Congenital Heart Disease Skills – Mastery of Common and Uncommon Challenges
Saturday, April 29, 2017
8:00 AM – 3:30 PM
Fix the Valve: Delaminate and Rehabilitate the Dysplastic Pulmonary Valve
*Giovanni Stellin, University of Padova
Build a Valve: Monocusp Pulmonary Valve Construction
*John W. Brown, Indiana University
Build a Valve: A Freehand Valved Conduit Construction
Patrick McConnell, Nationwide Children’s Hospital
Forget the Valve: Conduit-free Repair for Truncus Arteriosus
David Barron, Birmingham Children’s Hospital
No Slides and Audio Available
Deploy a Valve: Hybrid Pulmonary Valve Implantation
*Joseph A. Dearani, Mayo Clinic
Optimize Recovery: Simplified Modified Ultrafiltration at Boston Children’s Hospital
+Greg Matte, Boston Children’s Hospital
Part A: Optimizing Fontan Design and Construction, Minimizing the Insult of Surgery
Set up the Fontan: Hemifontan as the Second Stage
*Thomas L. Spray, Children’s Hospital of Philadelphia
Improve the Fontan: The “Y” Graft
*Kirk R. Kanter, Emory University
Redo the Fontan: Fontan Conversion
*Carl L. Backer, Lurie Children’s Hospital of Chicago
Part B: Optimizing Perfusion
Reduce the Physiologic Insult: Circuit Miniaturization
+Ron Angona, University of Oklahoma Health Sciences Center
When Less is More: Nationwide Children’s Hospital Approach to Bloodless Congenital Cardiac Surgery
+Ashley Hodge, Nationwide Children’s Hospital
Part C: How it Should Look: Imaging to Plan an Optimal Repair
Timothy C. Slesnick, Emory University
Plan your Arch: Computational Modeling of the Optimal Arch
*Tain-Yen Hsia, Great Ormond Street Hospital
Gerald Marx, Boston Children’s Hospital
Part A: Aortic Arch Reconstruction- Integrating Perfusion and Surcical Strategies
Arch Reconstruction Strategy: The CHOP Approach
*Thomas L. Spray, Children’s Hospital of Philadelphia
+Tami Rosenthal, Children’s Hospital of Philadelphia
Arch Reconstruction Strategy: The Cincinnati Approach
*James S. Tweddell, Cincinnati Children’s Hospital Medical Center
+Jim Reagor, Cincinnati Children’s Hospital Medical Center
Arch Reconstruction Strategy: The Vanderbilt Approach
*David P. Bichell, Vanderbilt University/Children’s Hospital
+Tom M. Klein, Vanderbilt University/Children’s Hospital
The Redo Arch
*Harold M. Burkhart, University of Oklahoma
The Late-Presenting Arch
*Robert D. Jaquiss, Duke University
Connective Tissue Disorders: Special Considerations in Aortic Arch Reconstruction
*Duke E. Cameron, Johns Hopkins Hospital
Part B: Atrioventricular Valve Repair Methods
Ebstein’s Tricuspid Repair: The Cone Repair Technique
*Joseph A. Dearani, Mayo Clinic
Techniques for Atrioventricular Valve Repair for the Single Ventricle Patient
*Richard G. Ohye, University of Michigan
Beyond Repair: Cylinder Replacement of the Mitral Valve in Infants
Patrick McConnell, Nationwide Children’s Hospital
Reoperative Valve Repair for Atrioventricular Septal Defect
*Pedro J. del Nido, Children’s Hospital
* AATS Member
+ AATS New Member
Congenital Hands-On Session
Chairs: David Bichell & Bohdan Maruszewski
3D Model Workshop – Congenital Heart Defects (DORV)
Proctors: Thomas L. Spray, Erle H. Austin, III
Supported by CryoLife, Scanlan, Ethicon
AATS/STS Congenital Heart Disease Symposium: Innovations and Controversies in the Surgical Management of Congenital Heart Disease
Sunday, April 30, 2017
8:00 AM – 5:00 PM
Course Chair: *Michael E. Mitchell, Children’s Hospital of Wisconsin
- *Giovanni Stellin, University of Padova
- +Ron Angona, University of Oklahoma Health Sciences Center
Device Innovations and Options for Biventrical Mechanical Circulatory Support
+Mark Shepard, St. Louis Children’s Hospital
Support for the Single Ventricle/Failing Fontan
*J. William Gaynor, Children’s Hospital of Philadelphia
Support of the Neonate and Infant
Katsuhide Maeda, Stanford University
UNOS Status Update- New Donor Allocation Scheme
Ryan R. Davies, A.I. duPont Hospital for Children
Mistakes in Timing and Listing for Transplant
*James S. Tweddell, Cincinnati Children’s Hospital Medical Center
The Difficult Recipient: Complex Transplantation in Congenital Heart Disease
+Tami R. Rosenthal, Children’s Hospital of Philadelphia
Arch Reconstruction at Stage II
Hakan Akintürk, Justus Liebig University
The “Tweener” Arch – Front vs. Side
*Charles D. Fraser, Texas Children’s Hospital
Fetal Intervention – Ready for Prime Time?
Audrey C. Marshall, Boston Children’s Hospital
Congenital AS – The Case for Surgical Valvotomy
*Viktor Hraska, Children’s Hospital of Wisconsin
Aortic Valve Repair for Insufficiency
*Michael N. Ilbawi, Hope Children’s Hospital
Repair for Mitral Stenosis
Eva Maria Delmo Walter, Cardio Centrum Berlin
Repair Techniques for Mitral Valve Insufficiency in Children
*Giovanni Stellin, University of Padua
Mitral Valve Replacement Techniques in Children
*Pedro J. del Nido, Boston Children’s Hospital
*William I. Norwood
Result of the National Perfusion Survey
+Ashley Hodge, Nationwide Children’s Hospital
Management of Perfusion in HLHS – DHCA vs. Warm Antegrade and Distal Perfusion
*David P. Bichell, Vanderbilt University/Children’s Hospital
*Thomas L. Spray, Children’s Hospital of Philadelphia
Slides and Audio:
To MUF or Not to MUF: Are We Making America Great Again?
+Rich Walczak, Duke University
+Craig McRobb, Children’s Hospital Colorado
Slides and Audio:
Management of MAPCAs in Repair TOF – Unifocalize or Not
*Frank L. Hanley, Stanford University
*Christian Brizard, Royal Children’s Hospital
Slides and Audio:
*Tain-Yen Hsia, Great Ormond Street Hospital
+Alex Robertson, Great Ormond Street Hospital
*James S. Tweddell, Cincinnati Children’s Hospital Medical Center
+Jim Reagor, Cincinnati Children’s Hospital Medical Center
*Charles D. Fraser, Texas Children’s Hospital
+Kimberly Madigan, Texas Children’s Hospital
Nikaidoh vs. Rastelli
Vladimir Sojak, Leiden University
*V. Mohan Reddy, Stanford University
Surgical Innovations with the Fontan
*Brian E. Kogon, Emory University
Surgical Techniques for TAPVR
*Christopher A. Caldarone, Hospital for Sick Children
Approaches to TOF with MAPCAs
David Barron, Birmingham Children’s Hospital
Paul H. Schoof, University Medical Center Utrecht
* AATS Member
+ AATS New Member
Congenital Heart Disease Simultaneous Scientific Session
Monday, May 1, 2017
2:00 pm – 5:15 pm
15. Long term Outcomes of the Expanded Polytetrafluoroethylene Conduit with Bulging Sinuses and a Fan shaped Valve in the Right Ventricular Outflow Tract Reconstruction
Takako Miyazaki, Masaaki Yamagishi, Yoshinobu Maeda, Satoshi Taniguchi, Shuhei Fujita, Hisayuki Hongu, Haruka Fu, *Hitoshi Yaku
Kyoto Prefectural University of Medicine, Kyoto, Japan
Objective: Various types of conduits are available for the right ventricular outflow tract (RVOT) reconstruction. However, the clinical results of conventional conduits were not satisfactory. We have developed the expanded polytetrafluoroethylene (ePTFE) conduit with bulging sinuses and a fan-shaped bi- or tricuspid ePTFE valve. This study summarized the results of a multicenter study for the evaluation of the valved ePTFE conduit.
Methods: We retrospectively investigated the valve functions of 1024 patients (median age: 3.8 years, range: 0 days-57.2 years, median body weight: 12.4 kg, range: 2.1-91.3 kg) who received the RVOT reconstruction using the valved ePTFE conduits (10 different sizes, 6-24 mm in diameter) at 65 hospitals between 2001 and 2015. The valve functions were assessed by echocardiogram, cardiac catheterization and magnetic resonance angiography.
Results: There was no late death related to the ePTFE conduit in the hospitals. The peak RVOT gradient was 16.5 ± 13.1 mmHg and the pulmonary insufficiency graded better than mild was 95.9 %. The conduit re-intervention was performed in 55 patients (5.3 %). The causes of the re-intervention were somatic growth (12 patients, 1.2 %), peripheral pulmonary artery stenosis (12 patients, 1.2 %), valvular stenosis (10 patients, 1.0%), RVOT stenosis (3 patients, 0.3%) and graft infection (3 patients, 0.3 %). The freedom at 5 years and 10 years from a re-intervention caused by overall size conduits was 96.1% and 94.3%, respectively. The freedom at 5 years and 10 years from the intervention by small conduits (6-16 mm in diameter) were 89.8% and 68.6%, respectively, whilst the freedom from the intervention caused by large conduits (18-24 mm in diameter) was 98.8% and 93.8%, respectively.
Conclusions: The long-term outcomes by the ePTFE conduit with a fan-shaped valve and bulging sinuses may be clinically satisfactory. We believe that the longevity of small-sized conduits can yield sufficient time to exchange the larger-sized conduits without any loss of their valve functions. With regard to longevity and resistance to infections, this ePTFE valved conduit can be one of the optimal ways to reconstruct RVOT.
16. Valve-sparing Repair with Intraoperative Balloon Dilation in Tetralogy of Fallot: Mid-Term Results and Therapeutic Implications
Sophie C. Hofferberth, Meena Nathan, Lynn A Sleeper, Audrey C. Marshall, Christopher W. Baird, *Pedro J. del Nido, *Sitaram M. Emani
Boston Children’s Hospital, Harvard Medical School, Boston, MA
Objective(s): The significant morbidity of long-term pulmonary insufficiency has driven recent efforts towards preservation of pulmonary valve (PV) function at the time of primary repair of tetralogy of Fallot (ToF). Several approaches to PV preservation have been reported in the past decade, including valve-sparing repair with intraoperative balloon dilation (VS-IBD). The purpose of this study was to evaluate late PV function in patients who underwent complete primary repair of ToF with VS-IBD.
Methods: This single center retrospective analysis included all patients <1 year of age who underwent complete primary repair of ToF with VS-IBD between 2007 and 2015. Adequacy of repair was assessed based on a pulmonary valve-specific technical performance score (TPS) at discharge, defined as Optimal (peak gradient <20mmHg, none/trivial regurgitation), Adequate (peak gradient 20-40mmHg, mild regurgitation) or Inadequate (peak gradient >40mmHg, ≥ moderate regurgitation). Risk factors for PV reintervention, freedom from significant pulmonary regurgitation (PR) and longitudinal PV annulus growth were evaluated.
Results: Among 162 consecutive patients who underwent VS-IBD repair of ToF, median age at surgery was 98 days (IQR: 73, 98). Median follow-up was 29.7 months (IQR: 7.0, 59.2). Median pre-operative PV annulus Z score was -2.2 (IQR: -2.5, -1.8). Twenty-five (15%) patients required PV reintervention for residual valvar stenosis post discharge. Multivariable regression analysis demonstrated baseline PV annulus Z score of <-2.45 (HR: 3.33, CI 1.44 – 7.68, p = 0.005), younger age at surgery (months, HR 0.58, CI 0.43 – 0.78, p = <0.001) and sub-optimal TPS class (Adequate: HR 2.35, CI 0.64 – 8.60; Inadequate: HR 9.23, CI 2.29 – 37.23, p = 0.002) were independently associated with higher hazard of PV reintervention. Freedom from significant PR (defined as ≥ moderate) was approximately 50% at 5-years and 20% at 8 years post surgery (Figure). The cohort exhibited significant PV annular growth over time; median z-score was -1.97 at discharge, then increased to -1.57 and -1.01 at intermediate and latest follow-up, p = <0.001.
Conclusions: Valve-sparing repair with intraoperative balloon dilation in ToF is associated with the development of progressive PV insufficiency. In younger patients and those with significant PV hypoplasia, alternative approaches to PV preservation should be explored. While ToF patients who undergo VS-IBD exhibit significant longitudinal PV annular growth, the observed lack of valve leaflet remodeling warrants further investigation. Compared to traditional trans-annular patch repair, the VS-IBD technique offers relief from significant PR for a period of time and therefore may delay the onset of right ventricular dilation. Nonetheless, the results of this study suggest that VS-IBD repair is not a suitable long-term solution to preserve PV function in ToF patients.
Deep Dive Session: Paper 16. Valve-sparing Repair with Intraoperative Balloon Dilation in Tetralogy of Fallot: Mid-Term Results and Therapeutic Implications
Panelists: **Sitaram Emani, Sophie C. Hofferberth, *Giovanni Stellin
17. Polytetrafluoroethylene (PTFE) Conduits vs Homografts for Right Ventricular Outflow Tract Reconstruction in Infants and Young Children: An Institutional Experience
Christopher W. Mercer,Shawn C. West, Mahesh S. Sharma, Masahiro Yoshida, Victor O. Morell
Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, PA
Objective: Our institution has developed a handmade bicuspid valved PTFE conduit as an alternative to homograft conduits. The objective of this study is to investigate the performance of these PTFE conduits vs homografts used for RVOT reconstruction in children less than 2 years old, and to evaluate risk factors for earlier conduit explant.
Methods: IRB approved retrospective chart review of all patients less than 2 years of age who underwent surgical RVOT reconstruction with either a PTFE conduit or homograft (pulmonary or aortic) from July 2004 through December 2014. The endpoints of the study were defined as: conduit explant, conduit explant or re-intervention, moderate conduit stenosis, and moderate conduit insufficiency.
Results: Fifty-five patients underwent 65 RVOT reconstructions with either a PTFE conduit (n = 39) or a homograft (n = 26, 23 pulmonary, 3 aortic). The majority of diagnoses were truncus arteriosus (n= 28) and tetralogy of Fallot with pulmonary atresia (n = 19). Median age of surgery was 134 [8-323] days and 128 [7-384] days and mean conduit size was 11.8 +/- 2.2 mm and 11.2 +/- 2.9 mm for PTFE and homografts respectively. There was no difference in age, gender, weight, height, BSA, diagnosis, conduit size, conduit z-score, bypass time, or cross clamp time between the 2 groups. There were 2 early deaths (both homografts) and 2 late deaths (1 homograft, 1 PTFE). Adjusting for year of surgery, hospital length of stay (LOS) was significantly different between the 2 groups. Median LOS was 14 [6-30] days for the PTFE group and 24.5 [17-46] days for the homograft group (p = 0.002). Multivariable analysis showed that younger age at surgery was a risk factor for conduit explant (HR 1.104 per 30 days younger, p < 0.001). The cumulative incidence of conduit explant at 1, 3, and 5 years was 17%, 52%, and 55% for PTFE conduits and 23%, 43%, and 56% for homografts. Time to event analysis demonstrated no difference in time to explant (p = 0.474) or time to explant or re-intervention (p = 0.213) between the 2 conduit types. There was no significant influence of conduit type on the development of moderate conduit stenosis (p = 0.931), severe conduit stenosis (p = 0.523), moderate conduit insufficiency (p = 0.830) or severe conduit insufficiency (p = 0.880). Larger conduit z-score was associated with a lower hazard rate for developing moderate conduit stenosis (HR 0.46, p = 0.001) and severe conduit stenosis (HR 0.42, p = 0.035).
Conclusion: Larger conduit z-score is associated with improved conduit function. PTFE conduits are associated with a decrease in hospital LOS. In our experience, the performance of bicuspid valved PTFE conduits at least matches that of homograft conduits in patients under 2 years of age for RVOT reconstruction, and should be considered a valid alternative. Their low cost and lack of potential sensitization makes them an even more appealing alternative to homograft conduits at our institution.
18. Transcatheter Pulmonary Valve Replacement for Treatment of Dysfunctional Surgical Bioprostheses: A Multicenter Study
Allison K. Cabalka1, Jeremy D. Asnes2, David T. Balzer3, John P. Cheatham4, Matthew J. Gillespie5, Thomas K. Jones6, Henri Justino7, Dennis W. Kim8, Te-Hsin Lung9, Daniel R. Turner10, Doff B. McElhinney11
1Mayo Clinic, Rochester, MN; 2Yale University, New Haven, CT; 3Washington University, St. Louis, MO; 4Nationwide Children’s Hospital, Columbus, OH; 5Children’s Hospital of Pennsylvania, Philadelphia, PA; 6Seattle Children’s Hospital, Seattle, WA; 7Texas Children’s Hospital, Houston, TX; 8Children’s Healthcare of Atlanta/Emory University, Atlanta, GA;9Medtronic, Santa Rosa, CA; 10Children’s Hospital of Michigan, Detroit, MI; 11Lucille Packard Children’s Hospital, Stanford University, Palo Alto, CA
Objective: Stented bioprosthetic valves (BPV) are commonly used for surgical pulmonary valve replacement in postoperative congenital heart disease. As in other implant locations, pulmonary BPVs develop structural failure in a time-related fashion. In 2010, a balloon-expandable transcatheter pulmonary valve (TPV) device consisting of a bovine jugular venous valve was approved in the US for treatment of dysfunctional right ventricular outflow tract (RVOT) conduits. TPV replacement (TPVR) within obstructed or regurgitant BPVs has been utilized widely, but there is limited published data regarding outcomes of this procedure, Therefore, we sought to evaluate TPV function in a cohort of patients who underwent TPVR into a failing pulmonary BPV.
Methods: Patients who underwent TPVR within a BPV for ≥ moderate stenosis and/or pulmonary regurgitation (PR) at 10 centers were enrolled retrospectively. Baseline, procedural, and follow-up data were collected on standardized case report forms, with all cases audited for accuracy. The primary outcomes were freedom from reintervention and TPV dysfunction (≥ moderate PR or mean Doppler gradient >40mmHg).
Results: A total of 100 patients who underwent TPVR for treatment of PR and/or RVOT obstruction at 10 centers between 1/2010 and 6/2015 were enrolled. The median age and weight were 22 [5-79] yrs and 62 [15-161] kg, respectively, and 48 patients were children. The underlying diagnosis included tetralogy of Fallot in 73 patients, and 84% of patients had moderate or severe PR. The TPV was implanted into multiple different types of surgical BPVs. The median BPV size was 23mm; 24 valves were ≤21mm, 25 were 23mm, 27 were 25mm, 11 were 27mm, and 10 were >27mm. In most patients (n=78), the TPV was implanted on a 22mm delivery system. Acute hemodynamic outcomes included reduction of PR to ≤mild in all but 1 patient and of the mean Doppler RVOT gradient from a median of 29 to 16 mmHg (P<0.001). During a median follow-up of 12.4 months (0-4.4 yrs), no patients underwent surgical explant or transcatheter reintervention on the TPV. Endocarditis (IE) was diagnosed in 1 patient, who was managed medically without intervention on the TPV, although there was severe PR after treatment. The most recent mean Doppler gradients were similar (median 14mmHg, P=NS) compared to early post-implant. No patient had a gradient >35mmHg, 86% of patients had no or trivial PR, with only 1 greater than mild (following the episode of IE as mentioned); no other patient met criteria for TPV dysfunction.
Conclusions: TPVR within dysfunctional surgical BPVs in the pulmonary position can be used to restore competence and relieve obstruction, with excellent medium term results. It will be important for surgeons and cardiologists to collaborate in an effort to determine the best lifetime management, combining surgical and transcatheter pulmonary valve replacement, in this population.
19. Modified Repair of Type I and II Truncus Arteriosus Limits Early Right Ventricular Outflow Tract Re-operation
Clauden Louis, Michael F. Swartz, Jill M. Cholette, Francisco Gensini, *George M. Alfieris
University of Rochester, Rochester, NY
Objective: As a result of branch pulmonary artery manipulation and changes in somatic growth, infants repaired with Type I and II truncus arteriosus often require early right ventricular outflow tract (RVOT) reoperation. Using a modified repair of truncus arteriosus, the branch pulmonary arteries are left in-situ, minimizing branch pulmonary artery manipulation which may limit early RVOT reoperation. We hypothesized that using a modified approach for the correction of Type I and II truncus arteriosus would minimize early RVOT re-operation.
Methods: Infants, requiring repair for type I or type II truncus arteriosus were reviewed from 1990-2014, and divided into two groups based upon the type of repair. For infants repaired using the traditional technique, the branch pulmonary arteries were excised from the truncal root, and the corresponding aortic defect closed primarily or patched. Following closure of the ventricular septal defect (VSD), either a valved bioprosthetic conduit, or valved homograft was then used for reconstruction of the RVOT. For infants repaired using a modified approach, a hockey stick incision was made on the truncal root, and extended into the left pulmonary artery. The truncal root was then septated using a Gore-Tex patch, the VSD closed, and a short (< 2 cm) aortic homograft used to re-establish right ventricular to pulmonary artery continuity.
Results: Fifty-five infants were repaired, 35 using a modified approach and 20 using the traditional technique. Although there were no significant differences in the pre-operative age, gender, or weight, there was a greater percentage of infants with interrupted aortic arch who were repaired using a modified approach (Table 1). There was no difference in conduit size between either group (11.3 ± 1.4 mm vs. 11.8 ± 2.2 mm; p=0.4). There was 100% follow-up, at 9.0 ± 5.5 years and 10.3 ± 8.8 years for the modified and traditional technique respectively. There were no cases of the septation patch causing branch pulmonary artery or aortic obstruction. Freedom from RVOT re-operation was significantly greater at 5 (Modified-79.4 % vs Traditional-36.6 %; p=0.004) and 10 years (Modified-49.3 % vs Traditional-29.3 %; p=0.05) using the modified approach. In addition, the percentage of patients who required a second re-operation for RVOT reconstruction was significantly lower using the modified approach (Modified-0 vs Traditional-15% (3/20); p= 0.04).
Conclusion: Maintenance of branch pulmonary artery architecture using a modified approach in infants with type I and type II truncus arteriosus results in improved survival and greater freedom from right ventricular outflow tract reconstruction.
20. Impact of Truncal Valve Insufficiency on the Outcomes of Truncus Arteriosus Repair20. Impact of Truncal Valve Insufficiency on the Outcomes of Truncus Arteriosus Repair
Phillip Naimo, Tyson Fricke, *Yves d’Udekem, Robert Weintraub, Johann Brink, *Christian Brizard, *Igor Konstantinov
Royal Children’s Hospital, Melbourne, Australia
Objectives: The impact of truncal valve (TV) insufficiency on the outcomes of truncus arteriosus (TA) repair is not well defined. We therefore sought to determine the impact of TV insufficiency on a large cohort of children, who underwent TA repair at a single institution.
Methods: We retrospectively reviewed 180 consecutive patients with median age 52 days (mean 144 days; range 1 day to 8.7 years) who underwent TA repair between 1979 and 2016. Preoperative echocardiography determined TV insufficiency in 80 patients (mild: 33.9%, 61/180; moderate: 9.4%, 17/180; and severe: 1.1%, 2/180). The TV was bicuspid in 13.3 % (24/180), tricuspid in 65% (117/180), and quadricuspid in 26.7% (48/180). Concomitant TV surgery at the time of TA repair was performed in 21 patients.
Results: There were 21 early deaths (11.7%, 21/180) and 20 late deaths in the entire cohort. Overall survival was 73.5 ± 3.9% (95% CI: 65.0, 80.3) at 25 years. Of the 21 patients who underwent concomitant TA-TV repair, early mortality was 19% (4/21), and survival was 70.8 ± 10.1% (95% CI: 46.2, 85.7). Neither concomitant TA-TV repair (p=0.5) nor degree of preoperative TV insufficiency (p=0.94) were associated with mortality.
TV reoperation was common in patients with concomitant TV surgery, with freedom from TV reoperation was 9.5 ± 8.6% (95% CI: 0.6, 33.2) at 25 years (Figure 1). Of the remaining 159 patients who did not undergo concomitant TA-TV repair, 14 patients required late TV surgery due to persisting or worsening TV insufficiency. Median time to TV operation was 7.9 years (range 1 day to 25 years). Freedom from TV operation was 84.0 ± 4.6% (95% CI: 72.5, 91.0) at 25 years. Only 7 patients with preoperative moderate TV insufficiency did not undergo concomitant TA-TV repair, of whom 3 have required late TV surgery to address TV insufficiency.
Follow-up was 98.6% (137/139) complete. TV insufficiency was none or trivial in 79.6% (109/137), and mild or less in 98.5% (135/137) of patients at median follow-up of 18 years
(mean 16 years; range 1 to 34 years). In the 28 surviving patients who have had TV surgery, 5 patients had mild TV insufficiency, while 1 patient had moderate TV insufficiency.
Conclusion: Although TV insufficiency is not a risk factor for death, moderate or greater preoperative TV insufficiency is associated high reoperation rate on the TV. The majority of patients with no or mild preoperative TV insufficiency are free from TV surgery up to 25 years
Congenital Emerging Technologies and Techniques / Case Video Forum
Tuesday, May 2, 2017
7:00 am – 8:25 am
Shenzhen Children’s Hospital, Shenzhen, China
Objective: This case video aims to demonstrate primary repair of infracardiac total anomalous pulmonary venous connection (TAPVC) with sutureless strategy and to explore the safeguards and pitfalls of this technique.
Case Video Summary: The patient was a 6-day-old boy with diagnosis of infracardiac total anomalous pulmonary venous connection with pulmonary venous obstruction, patent ductus arteriosus (PDA) and patent foreman ovale (PFO). Because of low blood pressure and extremely low arterial saturation, the patient underwent emergent TAPVC repair. A standard median sternotomy was performed followed by initiating cardiopulmonary bypass with aortic cannulation and single right atrial cannula. After the patent ductus arteriosus was ligated, the patient’s core temperature was cooled down to 18°C. After aortic cross-clamp and antegrade infusion of cardioplegia, the circulation was arrested. The patent foreman ovale was closed with primary closure via a right atrial incision. After opening the right thoracic cavity, the heart was rotated and put into the right thoracic cavity to expose the pulmonary veins and the vertical vein. After opening the pericardium and the vertical vein, the incision of the vertical vein was extended into each individual pulmonary vein and the vertical vein beyond stenotic segments to fully relieve any preoperative pulmonary venous obstruction. Another incision was made on the posterior wall of the left atrium longitudinally, the right end of which reached the interatrial septum. The left atrial incision and the pericardial incision were anastomosed together with running sutures. Most of the sutures did not touch the pulmonary venous wall to avoid injury to the venous intima, which could have triggered fibrous proliferative response and further led to new-onset obstruction. However, at the apex of the triangle zone between two individual pulmonary incisions, one stitch should suspend the pulmonary venous flap up to the pericardium and the left atrium to prevent the flaps from dropping down into the lumen. After finishing the anastomosis and closing the right atrial incision, the cardiopulmonary bypass resumed, and the heart restarted perfusion. The patient was weaned off the cardiopulmonary bypass and the operation was successfully terminated. The patient was discharged and followed up for 9 months. The echocardiography showed no pulmonary venous obstruction.
Conclusions: Sutureless strategy is an effective and reproducible technique for primary repair of infracardiac TAPVC. This strategy includes three important components: relieving any preoperative pulmonary venous obstruction, atrio-pericardial anastomosis and “no touch” technique.
T10. Anatomical Correction Including Aortic Root Translocation and Hemi-Senning / Bidirectional Glenn Atrial Switch Procedure in a Patient with Congenitally Corrected Transposition of the Great Arteries, Ventricular Septal Defect, Pulmonary Stenosis and Dextro
*Eun Seok Choi, *Chang-Ha Lee, *Sungkyu Cho
Sejong General Hosptial, Bucheon, Republic of Korea
Objective: We describe anatomical correction including aortic root translocation and hemi-Senning/bidirectional Glenn atrial switch procedure in a patient with congenitally corrected transposition of the great arteries (ccTGA), ventricular septal defect (VSD), pulmonary stenosis (PS) and dextrocardia.
Case Video Summary: A 8-year-old boy was referred to our hospital for cyanosis. He was diagnosed with ccTGA, VSD, PS and dextrocardia. Anatomical correction was considered in this patient.
Standard bicaval cardiopulmonary bypass was established with moderate hypothermia. Venting was performed via left atrial auricle. Antegrade cold cardioplegia was delivered and the aorta and pulmonary trunk were transected in sequence. The coronary buttons were fashioned and the aortic root was harvested. The pulmonary annulus and conal septum were completely divided in the middle to avoid injury to conduction pathway. The harvested aortic root was half-turned and translocated posteriorly. After the posterior side of aortic root was anastomosed to the pulmonary annulus, VSD was closed with autologous pericardial patch. Anterior side of aortic root was anastomosed to the VSD patch. Hypertrophied right ventricular muscle was resected. The coronary buttons were reimplanted in the aortic root. Branch pulmonary arteries were widened with autologous pericardial patch. The ascending aorta was re-anastomosed. A hemi-Senning procedure was performed using bovine pericardium and in-situ pericardium. The aortic cross-clamp was released and right ventricular outflow tract was reconstructed with a 18 mm tissue valved conduit on a fibrillating heart. Bidirectional Glenn was performed on a beating heart. Cardiopulmonary bypass and aortic cross-clamp time were 401 and 227 minutes, respectively.
The patient was weaned from cardiopulmonary bypass in sinus rhythm. The patient was extubated on postoperative day 4 and transferred to general ward on day 8. The patient was discharged 37 days after the operation because of prolonged pleural effusion. Postoperative echocardiography showed good both ventricular function, wide LVOT, good hemi-Senning pathway but mild AR.
Conclusions: Aortic root translocation and hemi-Senning/bidirectional Glenn atrial switch procedures are feasible treatment options for anatomical correction of ccTGA, VSD, PS and dextrocardia.
T11. Novel Surgical Strategy for Complicated Pulmonary Stenosis Using Hemodynamic Analysis Based on a Virtual Operation with Numerical Flow Analysis
*Kagami Miyaji1, Shohei Miyazaki1, Keiichi Itatani2, Koichi Sughimoto1, Tadashi Kitamura1, Tetsuya Horai1, Mamika Motokawa1
1Kitasato University, Sagamihara, Japan; 2Kyoto Prefectual Universty of Medicine, Kyoto, Japan
Objective: It is very difficult to successfully complete an optimal pulmonary artery plasty for complicated pulmonary stenosis (PS). A novel surgical strategy, using hemodynamic analyses based on virtual operation with computational simulations, has been induced for this patient group. We evaluated this strategy for complicated pulmonary artery plasty.
Methods: Six patients (Rastelli type operation: 4, TOF repair and PA plasty: 1, and Williams syndrome supra-valvular and branch PS: 1,) were enrolled. Before surgery, the optimal pulmonary arteries constructed based on computational fluid dynamics (CFD) using 3D-CT. Energy loss (EL, mW) and wall shear stress (WSS, Pa) were calculated. We compared with the shapes of preoperative and optimal pulmonary arteries, and decided surgical strategy, including incision line and patch shape (virtual surgery). EL and WSS were compared between virtual and real surgery using a flow analysis. These 6 patients were compared with 5 patients who underwent pulmonary plasty using a conventional approach without virtual surgery as a control group.
Results: The result of patient with Williams syndrome was shown in figure 1. Postoperative EL and max WSS was 7.0 mW and 67 Pa, respectively, compared to 10 mW and 70 Pa in virtual surgery. Postoperative right ventricular systolic pressure was 39.0 ± 11 mmHg in all 6 patients. Preoperative EL and max WSS were 22.8 ± 27.9 mW and 112 ± 130 Pa. Virtual and postoperative EL decreased to 3.9 ± 3.6 mW and 5.1 ± 3.2 mW (P=0.12, and P=0.16, respectively). Virtual and postoperative max WSS significantly decreased to 23 ± 20 Pa and 30 ± 23 Pa (P=0.028, and P=0.047, respectively). There was no significant difference between virtual and real surgery in both EL and max WSS (P=0.55, and P=0.67, respectively). In control group, postoperative right ventricular systolic pressure was 49 ± 16 mmHg. Postoperative EL was 8.0 ± 4.8 mW, and max WSS was 59 ± 46 Pa. There were tendencies that EL and WSS in control group were greater than those in study group, although there was no statistically significant differences (P = 0.27, and P = 0.21, respectively).
Conclusions: Pulmonary artery plasty, using hemodynamic analysis based on virtual surgery, is an efficient surgical strategy for complicated pulmonary stenosis. This novel strategy can easily and successfully provide an optimal pulmonary artery plasty, equivalent or superior to the conventional approach, which is based on the surgeons’ personal experiences and judgements.
T12. Repair Quadricuspid Truncal Valve with Utilization of Pulmonary Cusp to Reconstruct RVOT, Repair without Conduit
Shu-chien Huang, Ling-Yi Wei
National Taiwan University Hospital, Taipei, Taiwan
Objective: Quadricuspid truncal valves are susceptible to regurgitation, and tricuspid configuration is considered more durable after repairing the truncal valve. We report a new method, with translocate the pulmonary cuspid and its aortic wall as an everted flap to reconstruct the right ventricular outflow tract (RVOT).
Case Video Summary: This is a 4-month-and-1-day-old male baby, who was prenatally diagnosed of truncus arteriosus. Post-natal echocardiography also showed small bilateral pulmonary arteries with severe truncal regurgitation. CT showed hairpin aorta compressing right pulmonary artery and airway. He received bilateral banding initially, and transferred to our hospital for total repair. The aorta was transected and pulmonary artery button was separated, then a trans-annular incision was carried into RVOT. We performed truncal valve repair by translocating pulmonary cusp, its annulus and wall to RVOT side and reconstruct the new-aortic valve to tricuspid. The redundant aortic posterior wall was resect to relieve the condition of hairpin aorta before re-anastomosis of ascending aorta. Ventricular septal defect was repair with Dacron patch via RVOT approach. The floor of RVOT was made by the pulmonary cusp including its wall and the resected posterior aortic wall. Then the anterior wall was reconstructed with bovine pericardial patch with a 19mm porcine vale. Normal sinus rhythm resumed and cardiopulmonary bypass was weaned smoothly. Post-operative echocardiography confirmed a tricuspid neo-aortic valve with trivial aortic regurgitation, no residual VSD, and a patent right ventricular outflow tract.
Conclusions: We report a surgical technique of restoring competence in truncal insufficiency with tricuspidization of truncal valve and reconstructing truncus arteriosus without using a RVOT conduit.
T13. Chimney Reconstruction of the Aortic Arch in the Norwood Procedure
Satoshi Asada, Masaaki Yamagishi, Takako Miyazaki, Yoshinobu Maeda, Shuhei Fujita, Hisayuki Hongu, Haruka Fu, Keiichi Itatani, *Hitoshi Yaku
Kyoto Prefectural University of Medicine, Kyoto, Japan
Objective: The use of patch supplementation during aortic arch reconstruction in the Norwood procedure for hypoplastic left heart syndrome (HLHS) has become the norm. However, use of patch materials involves certain critical issues, such as the lack of growth, degeneration and calcification, possibly causing re-coarctation. On the other hand, autologous aortic arch reconstruction theoretically increases the risk of postoperative compression of the left pulmonary artery (PA) followed by narrowing of the aorto-pulmonary space. Further, neo-aortic root dilatation and subsequent neo-aortic regurgitation is another late critical complication. In order to avoid these complications, we developed a new aortic arch reconstruction technique referred to as “Chimney reconstruction” that does not require patch supplementation.
Case Video Summary: A 2-month-old boy weighing 3.9 kg was diagnosed with HLHS with aortic and mitral atresia. After bilateral PA banding in the neonatal period as the first palliation, he underwent the Norwood operation. Cardiopulmonary bypass was established under mild hypothermia through a median re-sternotomy and the ductal tissue was completely resected. The right and left PA orifices, which were longitudinally arranged, were detached in a U-shaped fashion from the posterior wall of the pulmonary trunk, instead of transecting the pulmonary trunk just beneath the bifurcation. The posterior U-shaped defect was closed longitudinally without any patch supplementation and was formed into a chimney-like shape. This maneuver enabled longitudinal extension and horizontal plication of the arterial trunk, resulting in an anastomosis with less tension and a wide aorto-pulmonary space. Subsequently, this arterial trunk was anastomosed to the neo-aortic arch. A modified Blalock-Taussig shunt was constructed on the right PA. Postoperative computed tomography showed a widely secured aorto-pulmonary space without aortic arch obstruction or compression of the left PA. There was no pressure gradient in the neo-aortic arch. Computational fluid dynamics analysis of this neo-aortic arch showed less turbulent flow with little wall shear stress at the isthmus, resulting in low energy-loss performance. The patient subsequently underwent the Fontan procedure at 2 years of age without developing re-coarctation, neo-aortic root dilatation or compression of the left PA.
Conclusions: We developed the novel “Chimney reconstruction” technique for aortic arch reconstruction without patch supplementation in the Norwood procedure. The procedure, which could be employed in settings of low availability of homograft, results in a wide aorto-pulmonary space, producing laminar blood flow without excessive energy loss. The technique preserves growth potential and potentially prevents future neo-aortic root dilatation.
T14. Through Tricuspid Closure for Doubly Committed Subarterial Ventricular Septal Defect with Right Vertical Subaxillary Mini-incision: a Matched-pair Analysis
Rui Liu, Zhongdong Hua
Chinese Academy of Medical Sciences & Peking Union Medical College Fuwai Hospital, Beijing, China
Objective: To evaluate the feasibility and efficacy of the right subaxillary vertical mini-incision (RAVI) used for the closure of doubly committed subarterial ventricular septal defect (SAVSD) through tricuspid approach only.
Methods: From June 2015 to September 2016, 32 SAVSD patients (mean age 2.4±1.9 years, range 0.7-8 years) underwent surgical repair with either RAVI (incision length 3-5cm) through tricuspid (group A, n=16) or conventional median sternotomy incision through the main pulmonary artery approach (group B, n=16). A retrospective 1:1 matched-pair analysis was performed with the group B was matched for defect size, body weight, gender, patching and operation year.
Results: The demographics characteristics in both groups were similar. No patient died and only 1 patient in group B needed reoperation for sternal infection. The mean cardiopulmonary bypass (CPB) time and aortic cross-clamp time was 48.6 ± 12.6 min, 29.3± 8.5 min in the group A and 57.8 ± 14.1 min (p= 0.03), 34.3 ± 12.1 min ( p = 0.18) in the group B. There was no significant difference between the two groups in the ICU stay (17.8 ± 8.9 hours in group A, 18.7 ± 9.5 hours in group B, p= 0.79), mechanical ventilation support time (2.7 ± 1.7 hours in group A, 3.6 ± 1.5 hours in group B, p= 0.11), postoperative hospital stay(6.3 ± 1.5 days in group A, 7.4 ± 1.7 days in group B, p= 0.06) and chest tube drainage (6.4 ± 4.3 ml/kg in group A, 8.5 ± 3.8 ml/kg in group B, p= 0.16). No significant residual defects were found in both groups. The post-operation pressure gradient across the right ventricular outflow tract (RVOT) were significantly different between the two groups with 4.6± 1.8 mmHg in group A and 10.0± 6.8 mmHg in group B (p = 0.004) even if no significant difference was found between both groups before operation. No arrhythmia was found after operation. All the patients or the parents (100%) in the group A were satisfied with the cosmetic results while the number in B group was 7 (43.8%) in questionare.
Conclusions: The RAVI through tricuspid approach to repair doubly committed subarterial ventricular septal defect is a safe and feasible procedure with better hemodynamic performance of RVOT and less CPB time because of keeping pulmonary artery intact comparing to conventional approach. More importantly, the RAVI through tricuspid approach can be performed with favourable cosmetic results.
T15. A Successful Biventricular Repair for a Neonate with Critical Aortic Stenosis Complicated With Left Ventricular Aneurysm and Endocardial Fibroelastosis
Mt. Fuji Shizuoka Children’s Hospital, Shizuoka City, Japan
Objective: Endocardial Fibroelastosis(EFE) sometimes develops in patients with critical aortic stenosis(cAS) or hypo plastic left heart syndrome. However its severity and influence on LV function are still unpredictable. Moreover, congenital left ventricular aneurysm(LVAn) combined in this disease has not reported before. We present our precious experience to treat this rare disease subset.
Case Video Summary: This patient was diagnosed in fetus as cAS complicated with EFE and LVAn. The patient was delivered by Cesarean at the gestational age of 38 week with 3.3kg of birth weight. Echocardiography revealed a ductus dependent circulation (antegrade blood flow through aortic valve reached only to the first branch of the aortic arch) and a “swing like motion” of LV wall almost without centripetal contraction(16% of LVEF). His aortic valve was bicuspid and 5.5mm in diameter with 15mmHg of peak pressure gradient. His mitral valve was 6.3mm in diameter with minimum inflow. The LVAn was located on the apex and it was dyskinetic. Because he developed pulmonary over circulation on day 2, bilateral pulmonary artery banding was performed in anticipation of future univentricular palliation. After the procedure, his condition became stable under continuous prostagrandin infusion. On day 20, however, echocardiography showed different hemodynamics; LV contracted centripetally with 79% of EF and antegrade blood flow through aortic valve(peak pressure gradient was 71mmHg) reached to the descending aorta, although there were no significant changes in EFE and LVAn. On day 21, we performed biventricular repair by surgical aortic valvotomy, division of patent ductus arteriosus, bilateral PA debanding and LV reconstruction with Dor procedure without EFE resection. In case he couldn’t tolerate with post-repair, we would convert to palliative surgery (Damus-Kay-Stansel anastomosis and BT shunt placement). CPB and Cross clamp time was 136min and 68min, respectively. Although he was complicated with mediastinitis and required chest explorations, he was extubated finally on POD30. Postoperative catheter examination demonstrated an acceptable hemodynamics with 66/42mmHg of systemic pressure and 3.5L/min/m2 of cardiac index, although mild aortic stenosis(peak pressure gradient of 32mmHg) remained and LA pressure was relatively high(15mmHg). Then he was discharged home on POD59. He has been followed for more than 1 year without additional surgical nor catheter interventions. The latest echocardiography revealed a successful maintenance of biventricular circulation even though diastolic dysfunction still existed.
Conclusions: A cAS neonate with EFE and LVAn showed dramatical change in his LV contractility within a short time and could achieve biventricular circulation after aortic valvotomy and exclusion of LVAn, although his LV seemed impossible to support a systemic circulation at his birth.
Congenital Heart Disease Moderated Poster Competition
Tuesday, May 2, 2017
12:45 pm – 2:00 pm
P13. Surgical Strategy of Anatomical Repair for Congenitally Corrected Transposition of The Great Arteries
Kai Ma, Shoujun Li, Lei Qi, Zhongdong Hua, Keming Yang, Hao Zhang, Jun Yan, Sen Zhang, Qiuming Chen
Fuwai Hospital, Beijing, China
Objective: Anatomical repair for congenitally corrected transposition of the great arteries (CCTGA) remains controversial. This study was to review our surgical strategy in pediatric patients with CCTGA.
Methods: From Jan 2005 to Feb 2016, consecutive CCTGA patients who underwent anatomical repair at our institution were enrolled in this retrospective study. As showed in the Figure, various types of biventricular repair were customized individually.
Results: A total of 85 patients were included. The median age of the patients underwent anatomical repair was 4.8 years (range, 8 months to 12 years). Left ventricular outflow tract obstruction (LVOTO) was presented in 51 (60.0%) patients (44 pulmonary stenosis and 7 pulmonary atresia). Positional anomaly (25 dextrocardia and 10 mesocardia) and situs inversus were documented in 35 (41.2%) and 10 (11.8%) patients, respectively. Sixty-four patients had an unrestrictive VSD and 10 of them presented with pulmonary vascular obstructive disease. Moderate-to-severe tricuspid regurgitation was presented in 39 (45.9%) patients. Prior pulmonary artery banding was required in 21 patients (1 to reduce pulmonary blood and 20 to retrain the LV). As showed in the Figure, 33, 19 and 32 patients accepted Senning/arterial switch operation (ASO), Senning/Rastelli and Hemi-Mustard/BDG/Rastelli, respectively. There were no differences considering both cardiopulmonary bypass time (ANOVA: P = 0.332) and cross-clamp time (ANOVA: P = 0.472) between these approaches. Early after repair, there were 5 in-hospital deaths and 9 reoperations. In multivariate analysis, the only risk factor for early deaths was the year of surgery before 2012. During a 4.6-years (0.5 to 10.3 years) follow-up, 7 late deaths were documented without risk factors found. Estimated overall survival rate after anatomical repair was 91.7%, 82.9% and 82.9% at 1 year, 5 years and 10 years, respectively. Instead of Senning/ASO, most (3/4, 75.0%) early LV dysfunction were noted in patients underwent Senning/Rastelli procedure. However, all the late LV dysfunction were found in patients underwent Senning/ASO and previous retraining. At the latest follow-up, 94.5% (69/73) of the survivors were in NYHA functional class I-II. Compared with patients underwent Senning/Rastelli, a lower early mortality was documented in patients who had Hemi-Mustard/Rastelli/BDG, although more positional cardiac anomalies presented.
Conclusions: Favorable outcomes of anatomical repair for CCTGA can be achieved with appropriate surgical strategies. Postoperative LV dysfunction is significant to the outcomes after both Senning/ASO and Senning/Rastelli. Hemi-Mustard/Rastelli/BDG may be an option for patients with cardiac malposition, providing lower early mortality.
P14. MELD score and Ventilation Indices are Strong Determinants of Death, Intensive Care Morbidity and Massive Transfusion after Adult Congenital Heart Disease Surgery
Jane E. Heggie1, Emma Lei Lei2, Jesse Creamer1, Karim Ladha1, Tait Gordon1, Jo Carroll1, Erwin Oechslin1, Lucy Roche1, *Vivek Rao1, *Christopher Caldarone1, *Glen Van Arsdell1, *William G. Williams1, Edward Hickey1
1Toronto General, Toronto, ON, Canada; 2Westmead Hospital, Sydney, Australia
Objective: Predicting peri-operative death and morbidity after ACHD surgery is difficult, due to patients’ complex histories and paucity of published outcomes data. Existing perioperative risk score calculators fail to capture comorbidities unique to the Adult Congenital Heart Disease population. We sought to identify robust determinants of ACHD intensive care morbidity.
Methods: Following REB approval, data was acquired from 4 prospectively maintained databases on 772 consecutive adults undergoing surgery (excluding transplants) by our congenital team between 2004 and 2015. Thorough chart review was undertaken by congenital cardiac intensivists and anesthesiologists, focusing on pre-operative biomarkers and physiologic parameters. Endpoints included: stroke, renal failure requiring dialysis (AKI), blood product usage and death. Analysis was via parametric univariate (UV) and multivariable (MV) risk-adjusted regressions, guided by bootstrapping for reliability.
Results: Underlying baseline diagnosis included: tetralogy of Fallot (36%), septal defects (29%), bicuspid aortic valve disease (7%) and Fontan, Ebstein’s, endocarditis, ccTGA, coarctation (all 3%). Median age was 37 (17-77) and the majority had undergone multiple previous sternotomies (none=39%, 1=30%, 2=19%, 3-7=11%). Overall in-hospital mortality was 4.4% (2004-2007=, 4.6%, 2008-2011=5.1%, 2012-2015=3.7%).
AKI, duration on ventilator, blood product usage and death all have strong and reliable risk factors in common (table). FEV1, FVC, creatinine, albumin, MELD scores, multiple previous sternotomies and single ventricle physiology were highly significant and reliable predictors of all these endpoints. Of these, FEV1, albumin and MELD IX were most commonly incorporated in multivariable risk-adjusted models. For MELD categories 0-9, 10-19 and 20+ mortality was 2.7%, 11% and 33% respectively (P<.0001).
Blood product usage was massive: 10% were exposed to 33 or more units (max 423). Only 37% avoided any exposure (predictors included: ASD repair, later operative year, fewer sternotomies). Of those who received products, median exposure was: PRBC 3 (max 138), platelets 5 (max 175), FFP 4 (max 130).
No biomarkers or physiologic parameters were strong predictors of stroke. Instead, multivariable models of stroke included older age, bicuspid aortic valve disease and previous Mustard surgery (table).
Conclusions: MELD score, ventilation parameters and biomarkers such as albumin are very strong and reliable determinants of severe morbidity, massive transfusion and death after ACHD surgery. Stroke is not easily predicted and is determined more by operation type. These peri-operative prediction models will help better counsel ACHD patients. Predicting massive transfusion will also help strategize for future transplant eligibility.
P15. The CHSS Complete Atrioventricular Septal Defect Inception Cohort: Pre-Intervention Echocardiographic Characteristics
James M. Meza1, Luc Mertens1, Gina Baffa2, Meryl S. Cohen3, Michael D. Quartermain3, David Gremmels4, Cheryl Fackoury1, *Christopher A. Caldarone1, *William G. Williams1, *William M. DeCampli5, David M. Overman4
1Hospital for Sick Children, Toronto, ON, Canada; 2Nemours Cardiac Center, Wilmington, DE; 3Children’s Hospital of Philadelphia, Philadelphia, PA; 4Children’s Hospitals and Clinics of Minnesota, Minneapolis, MN; 5Arnold Palmer Hospital for Children, Orlando, FL, Canada
Objective: Previous studies have demonstrated that the modified Atrioventricular Valve Index (mAVVI), Right Ventricular-Left Ventricular (RV/LV) inflow angle, and Left Ventricular Inflow Index effectively differentiate balanced vs. unbalanced Complete Atrioventricular Septal Defect (CAVSD) and may be useful in informing surgical management. This core lab analysis of baseline, pre-intervention echocardiograms of infants diagnosed with CAVSD provides a description of the echocardiographic spectrum of disease and assesses the relationship of known measures of inflow physiology with known measures of ventricular geometry.
Methods: Patients diagnosed with CAVSD before one year of age with atrioventricular and ventriculoarterial concordance were prospectively enrolled into an inception cohort. Pre-intervention echocardiograms were submitted by participating institutions. A single sonographer analyzed the images according to a standardized protocol of 111 morphologic and functional characteristics. Data are summarized using descriptive statistics and Pearson correlation coefficients.
Results: Baseline echocardiograms were available for 59% (194/328) of patients. The median age at which echocardiograms were performed was 15 days old (IQR 1-83), at a median weight of 3.3 kg (IQR 2.8-4.2). Right-dominant AVSD (mAVVI ≤ 0.4) was present in 22% (42/194), left-dominant (mAVVI ≥ 0.6) in 5% (9/194), and balanced (0.4 < mAVVI > 0.6) in 73% (143/194). Septal malalignment was present in 15% (25/186), with left malalignment accounting for 92% (23/25). The median mAVVI was 0.5 (IQR 0.4-0.5, absolute range=0.2-0.7). The median RV/LV inflow angle was 89.0 (IQR 80.0-101.3) degrees. The median LVII was 0.5 (IQR 0.5-0.6). The median right atrioventricular valve (AVV) area was 1.6 (IQR 1.2-2.1) cm2. The median left AVV area was 1.2 (IQR 0.9-1.7) cm2. The median total AVV area was 3.0 (IQR 2.2-3.8) cm2. mAVVI was weakly correlated with left ventricular size and inversely correlated with right ventricular size. The RV/LV inflow angle was not correlated with RV or LV size and only moderately correlated with RAVV and LAVV annular sizes. LVII was weakly correlated with LV size (Table).
Conclusions: In this large data set of baseline echocardiographic characteristics of patients with CAVSD, measures of inflow physiology are, at best, only moderately correlated with ventricular geometry. The role and impact of these observations on surgical strategy and clinical outcomes will require the continued study of this cohort.
P16. Effect of Atrioventricular Valve Repair on Multi-stage Palliation Outcomes of Single Ventricle Anomalies
Raina Sinha, Firat Altin, Courtney McCracken, Andrew Well, Joshua Rosenblum, *Brian Kogon, Subhadra Shashidharan, *Bahaaldin AlSoufi
Emory University, Atlanta, GA
Objectives: Accomplishment of multi-stage palliation of single ventricle (SV) anomalies depends on set anatomic and hemodynamic criteria. The presence of significant atrioventricular valve (AVV) regurgitation results in unfavorable conditions that affect the success of this palliation strategy. We report our institution’s experience with AVV repair and examine factors affecting outcomes.
Methods: We followed 604 infants who underwent their initial palliative surgery between 2002-12. Our cohort comprised those who underwent AVV repair at various palliation points. We examined patients’ characteristics and anatomic details associated with outcomes. Additionally, comparative analysis using propensity-matched control was performed.
Results: Fifty-seven patients received AVV repair during the first-stage (n=8), Glenn (n=30) and Fontan (n=19) stages. Median age at time of initial palliation was 5 days (IQR 3-16) while median age at time of AVV repair was 6.6 months (IQR 4.2-24.1). Underlying SV anomaly was hypoplastic left heart syndrome (n=29), atrial isomerism (n=14), other (n=14). The AVV was tricuspid (n=30), mitral (n=8), common (n=19) while dominant ventricle morphology was right (n=41), left (n=13), both (n=3). On preoperative echocardiogram, 55 (97%) had AVV regurgitation ≥ moderate and 8 (14%) had depressed ventricular function ≥ moderate. Post-repair, AVV regurgitation was none or trivial (n=21, 37%), mild (n=19, 33%), moderate (n=17, 30%).
Competing risks analysis showed that at 10 years following AVV repair, 15 % had died, 15% had received heart transplantation, 14% had undergone AVV reoperation and 56% were alive without transplantation or AVV reoperation. Overall survival and transplant-free survival at 10 years following AVV repair was 78% and 68%, respectively. Factors associated with transplant-free survival were AVV repair at first-stage surgery (HR 1.8 (0.6-5.6), p=0.005) and post-repair depressed ventricular function ≥ moderate (HR 5.4 (1.5-19.8), p=0.036).
When comparing with a matched control group, transplant-free survival was lower in our patient cohort (68% vs. 87%, p=0.013).
Conclusions: The presence of significant AVV regurgitation affects SV palliation survival. This is especially evident in patients who require AVV repair at first-stage surgery and those with diminished ventricular function. Different approaches are warranted in these high-risk patients and those might implicate the mode of initial palliation, timing of AVV repair and listing for transplantation.
P17. Long-term Results of Tissue-engineered Vascular Grafts in Pediatric Patients with Congenital Heart Disease
Tadahisa Sugiura1, Goki Matsumura2, Shinka Miyamoto1, Hideki Miyachi1, Christopher K. Breuer1, *Toshiharu Shinoka1
1Nationwide Children’s Hospital, Columbus, OH; 2Tokyo Women’s Medical University, Tokyo, Japan
Objective: Tissue engineering holds great promise for the advancement of cardiovascular surgery as well as other medical fields. Tissue-engineered vascular grafts have the ability to grow and remodel and could therefore make substantial advances in pediatric cardiovascular surgery. In 2001, we began a human clinical trial evaluating these grafts in patients with univentricular physiology. Herein, we report the long-term results of patients who underwent implantation of tissue-engineered vascular grafts as extracardiac total cavopulmonary conduits.
Methods: Tissue-engineered vascular grafts seeded with autologous bone marrow mononuclear cells were implanted in 25 patients with univentricular physiology as extracardiac total cavopulmonary conduits. The graft is composed of a woven fabric of poly-l-lactide acid or polyglycolic acid and a 50:50 poly (l-lactic-co-ε-caprolactone) copolymer. Patients were followed up postoperatively in a multidisciplinary clinic. The length and cross-sectional area of the graft were measured using both postoperative and late-term angiography or CT angiography at three locations: the side near the inferior vena cava, the mid graft, and the side near the pulmonary artery.
Results: Median patient age at operation was 5.5 years and the mean follow-up period was 10.3 years. Eight patients died during the follow-up period. There was no graft-related mortality. There was no evidence of aneurysmal formation, graft rupture, graft infection, or calcification. Ten patients (40%) had asymptomatic graft stenosis. Six of 10 patients underwent successful balloon angioplasty. One patient had thrombus formation in the graft one year after surgery, which was successfully resolved by anticoagulation therapy. For patients who were less than 5 years old at implantation, angiographical assessment shows the growth of graft (graft length; 33.9±7.3 mm vs. 47.4±12.4 mm, p=0.001, cross-sectional area at the side near inferior vena cava; 101.1±77.3 mm2 vs. 160.7±96.5 mm2, p=0.01, cross-sectional area at the side near pulmonary artery; 100.7±74.8 mm2 vs. 198.6±111.6 mm2, p=0.001, postoperative and late-term, respectively) (Figure A, B).
Conclusions: Our results indicate that tissue-engineered vascular grafts have grown according to the patients growth. Stenosis is the primary mode of failure of the tissue-engineered vascular graft.Tissue-engineered vascular grafts have feasibility in pediatric cardiovascular surgery.
P18. Outcomes and Prognostic Factors for Acquired Pulmonary Vein Stenosis in the Current Era
David Kalfa1, *Emre Belli2, *Emile Bacha1, Virginie Lambert2, Duccio di Carlo3, *Martin Kostolny4, Matej Nosal5, Jurgen Horer6, Jukka Salminen7, Jean Rubay8, Illya Yemets9, Mark Hazekamp10, *Bohdan Maruszewski11, *George Sarris12, Hakan Berggren13, François Lacour-Gayet14
1Columbia University, New York, NY; 2Marie Lannelongue Hospital, Paris, France; 3Ospedale Pediatrico Bambino Gesù, Roma, Italy; 4Great Ormond Street Hospital, London, United Kingdom; 5Childrens Heart Center, Bratislava, Slovakia; 6German Heart Center, Munich, Germany; 7University of Helsinki, Helsinki, Finland; 8Saint-Luc Hospital, Brussels, Belgium; 9Ukrainian Childrens Cardiac Center, Kyiv, Ukraine; 10Leiden University, Leiden, Netherlands; 11Children’s Memorial Health Institute, Warsaw, Poland; 12Athens Heart Surgery Institute and Iaso Children’s Hospital, Athens, Greece; 13The Queen Silvia Children’s Hospital, Goteborg, Sweden; 14Royal Hospital, Muscat, Oman
Objective: The optimal management and prognostic factors of acquired pulmonary vein stenosis (APVS) remain controversial. We sought to determine current APVS outcomes and prognostic factors in a multicentric study in the current era.
Methods: Seventy-five patients with APVS who underwent 103 procedures in 14 European/North-American centers between 2000 and 2012 were included retrospectively. A specific PVS severity score was developed, based on the echographic pressure gradient and the focal/diffuse aspect of the stenosis for each PV. A risk analysis was performed. Mean follow-up was 43±39 months. Seventy-six% (57/75) of APVS occurred after repair of a total anomalous pulmonary venous return. Mean preoperative score was 8.1±3.8. The mean number of affected PV per patient was 2.9±1.1. Sutureless repair was used in 42/103 procedures (41%), patch veinoplasty in 28/103 (27%), endarterectomy in 16/103 (16%). Median age and weight at surgery were 5 months (range: 5d.-184m.) and 5.5kg (range:2.8-42) respectively.
Results: Overall PV restenosis, reoperation and mortality occurred in 56%(n=58/103), 49%(n=50/103) and 27%(n=20/75) respectively. Kaplan-Meier cumulative patient survival and reoperation-free survival at 10 years were 69±11% and 48±10% respectively. Preoperative PVS scores >8.5 and >9 had the best predictive accuracy for postoperative PV restenosis and reoperation respectively. Restenosis and reoperation rates were significantly lower after sutureless repair compared to non-sutureless repair : 40%(n=17/42) vs. 67%(n=41/61) (p=0.007) for restenosis and 31%(n=13/42) vs. 61%(n=37/61) (p=0.003) for reoperation. Mortality rate after sutureless repair (20%; 7/35) was not significantly different from non-sutureless repair (33%; 13/40) (p=0.22). Kaplan-Meier cumulative reoperation-free survival at 8 years was significantly lower in patients with preoperative PVS score>9 (35±14% vs. 59±11% for PVS score<=9 ; logrank p=0.002), non-sutureless repair (34±13% vs. 68±12% for sutureless repair ; logrank p=0.003), and postoperative residual pulmonary hypertension (36±17% vs. 69±12% without postoperative pulmonary hypertension; logrank p=0.02). Results of the univariate analysis are showed in the Table. Multivariate analyis showed that a high postoperative PVS score was an independent risk factor for PV restenosis (OR=7.76 (95%CI:1.16-51.77); p=0.034), PV reoperation (OR=2.18 (1.25-3.8); p=0.006) and PVS-related mortality (OR=1.57 (1.11-2.2); p=0.009).
Conclusion: Acquired PVS still has a guarded prognosis in the current era. The sutureless technique used for acquired PVS seems to be associated with a lower risk of PV restenosis and reoperation but does not seem to reduce significantly the risk of mortality. The severity of the residual disease evaluated by a new severity score is an independent risk factor for poor outcomes regardless of surgical technique.
P19. Autosomal Dominant Mannose-Binding Lecithin (MBL) Binding Deficiency is Associated with Worse Neurodevelopmental Outcomes After Cardiac Surgery in Infants
Daniel Seung Kim1, Yatong K Li2, Jerry H Kim1, Curtis Bergquist2, Marsha Gerdes3, Judy Bernbaum3, Nancy Burnham3, Donna M. McDonald-McGinn3, Elaine H Zackai3, Susan C. Nicolson3, *Thomas L. Spray3, Deborah A. Nickerson1, Hakon Hakonarson3, Gail P Jarvik1, J. William Gaynor3
1University of Washington, Seattle, WA; 2University of Michigan, Ann Arbor, MI; 3Children’s Hospital of Philadelphia, Philadelphia, PA
Objective: Mannose-binding lectin (MBL) is an acute-phase reactant. Low MBL levels have been associated with adverse outcomes, including neurodevelopment, in preterm infants. The MBL2 gene is the major genetic determinant of MBL plasma protein levels. The MBL2 missense variant rs1800450 (p.Gly54Asp, minor allele frequency of 10.26%) causes autosomal dominant MBL deficiency. We tested the hypothesis that this variant, which causes low MBL levels, is associated with worse neurodevelopmental outcomes after cardiac surgery in neonates.
Methods: This is an analysis of a previously described cohort of non-syndromic congenital heart disease (CHD) patients who underwent cardiac surgery with cardiopulmonary bypass before 6 months of age (n=295). Four-year neurodevelopment was assessed in three domains: Full-Scale Intellectual Quotient (FSIQ), the Visual Motor Integration (VMI) development test, and the Child Behavior Checklist (CBCL) to assess behavioral problems. The CBCL measured total behavioral problems, pervasive developmental problems (PDPs), and internalizing/externalizing problems. To assess the effect of autosomal dominant MBL deficiency, patients with at least one minor allele at MBL2 missense variant rs1800450 were grouped together. Multivariable linear regression models, adjusting for confounders (see Figure 1A for full list of covariates), were fit. A Bonferroni-adjusted threshold for significance was set at α=0.0083 to adjust for the 6 total neurodevelopmental outcomes tested (0.05/6).
Results: Autosomal dominant MBL deficiency was associated with a significantly increased covariate-adjusted PDP score (β=3.98, P=0.0025, see Figure 1A). Examination of the interaction between age at first surgery and MBL genotype revealed significant effect modification for the patients with autosomal dominant MBL deficiency (Pinteraction=0.039, Figure 1B), with the poorest neurodevelopment scores occurring in children who had surgery earlier in life. There was a trend toward significance for MBL deficiency and CBCL total problems (β=3.23, P=0.097) and internalizing problems (β=3.33, P=0.093). While there was no significant association between autosomal dominant MBL deficiency and FSIQ, VMI, or CBCL externalizing problems score, the beta coefficients for each outcome (see Figure 1A) correspond to a deleterious effect of low MBL levels on neurodevelopment.
Conclusions: After cardiac surgery, children with autosomal dominant MBL deficiency have increased PDP scores at 4-year neurodevelopmental follow-up, independent of other covariates. There is a significant interaction between MBL levels and age at first surgery suggesting that the poorest neurodevelopmental outcome of low MBL levels occurs at early age. These data provide additional evidence that genetic variants are important modifiers of morbidity and disability after surgery for CHD.
P20. Bovine Arch Anatomy Influences Re-Coarctation Rates in the Era of the Extended End-to-End Anastomosis
*Joseph W. Turek, Brian D. Conway, Nicholas B. Cavanaugh, Alex M. Meyer, Osamah Aldoss, Ben E. Reinking, Ahmed El-Hattab, *Nicholas P. Rossi
University of Iowa Children’s Hospital, Iowa City, IA
Objective: Bovine arch anatomy has never been shown to influence re-coarctation after extended end-to-end anastomosis via a left thoracotomy, yet in all these studies the bovine arch is grossly underreported (prevalence in these studies no more than 5%, while imaging and autopsy studies show 15-37% prevalence of the bovine arch). This study aims to: 1) assess chart review reliability in bovine arch identification, 2) determine re-coarctation risk with a bovine arch, and 3) explore an anatomic explanation for recurrent arch obstruction based on arch anatomy.
Methods: 49 patients underwent surgical repair for aortic coarctation via an extended end-to-end anastomosis at a single institution over a 6 year period (2007-2012). Echocardiographic images from these patients were specifically reviewed for arch anatomy and compared to a chart review of the echocardiographic reports looking for the same. Recurrent arch obstruction was defined as an echocardiographic gradient across the repair ≥20 mmHg and compared across arch anatomies. For cases with angiographic images (18/49; 6 bovine arches; 12 normal arches), a scaled clamping distance (indexed to the diameter of the sinotubular junction) between the left subclavian artery and the maximal proximal clamp location on orthogonal projections was then calculated for normal anatomy versus bovine arch anatomy.
Results: Only 3/49 (6.1%) patients were indicated on chart review to have a bovine arch, compared to 14/49 (28.6%) on targeted retrospective review. For patients with a bovine arch, 4/14 (28.6%) had a follow-up gradient ≥20 mm Hg. Conversely, for patients with normal aortic arch anatomy, 2/35 (5.7%) had a follow-up gradient ≥20 mm Hg. Figure 1 shows the Kaplan-Meier curve for freedom from re-coarctation between the two arch anatomies (p <0.03). The mean clamping index for patients with normal arch anatomy was 1.31 ±0.55, while the mean for patients with bovine arch anatomy was only 0.80 ±0.39 (p <0.05). Age and weight at time of operation were not significantly different between study groups.
Conclusions: Bovine arch anatomy often goes undocumented on preoperative imaging assessment, yet children undergoing extended end-to-end repair with bovine arch anatomy are at a significantly increased risk of recurrent arch obstruction. This may be due to a reduced clampable distance to facilitate repair. These results should trigger a profound paradigm shift in preoperative assessment, parental counseling and surgical approach for children with discrete aortic coarctation.
P21. Atrioventricular Valve Regurgitation in Patients Undergoing Total Cavopulmonary Connection: Impact of Valve Morphology on Survival and Reintervention
Masamichi Ono, Julie Cleuziou, Jelena Pabst von Ohain, Elisabeth Beran, Melchior Strbad, Alfred Hager, Christian Schreiber, *Rüdiger Lange
German Heart Center Munich, Munich, Germany
Objective: To evaluate the morphology of atrioventricular valve (AVV), the mechanisms of AVV regurgitation and outcomes of AVV surgery in single ventricle patients, and to determine their influence on clinical outcomes after total cavopulmonary connection (TCPC).
Methods: A total of 460 patients underwent a TCPC between 1994 and 2015, including 101 (22%) patients who had at least one AVV surgery before or at time of TCPC. AVV morphology was classified into single mitral valve (MV), single tricuspid valve (TV), two separated valves (2AVVs), and common AVV (CAVV). Morphological feature of AVV regurgitation in patients who needed AVV surgery were analyzed. Outcomes following TCPC were compared to the remaining 359 patients who did not require AVV surgery. Factors influencing mortality, AVV reoperation and systemic ventricular function, were analyzed.
Results: In 101 patients who had AVV surgery, AVV morphology showed 2AVVs in 33 patients, MV in 11, TV in 41, and a CAVV in 16. Patients with a TV and a CAVV underwent AVV surgery more frequently, 27 and 36 %, respectively, than patients with a MV, 10% (p<0.001). AVV regurgitation was due to one or more of the following mechanism: dysplastic leaflet (n=62), prolapse (53), annular dilation (27), cleft (22), and chordal anomaly (14). Morphological anomalies were observed in 89 patients (88%). The primary procedure was 81 AVV repairs, 16 AVV closures, and 4 AVV replacements. Among 81 initial repairs, repeat repair was required in 20 patients, AVV replacement in 7, and AVV closure in 3. Overall survival following TCPC was lower in patients who had AVV surgery than those who did not. (88 % vs. 95 % at 15 years, p=0.01). In patients who had AVV surgery, freedom from reoperation on AVV was 75 % at 15 years and significant AVV regurgitation (moderate or more) was observed in 12 patients at medial follow-up of 6.5 years. Patients undergoing AVV replacement showed lower survival compared to those who had repair or closure of AVV (p=0.02). Risk factor analysis revealed papillary muscle anomaly as a risk for mortality following TCPC (p=0.001), leaflet prolapse for AVV reoperation (p=0.009), and chordal anomaly for AVV replacement (p<0.001). Systemic ventricular function did not differ in patients with and without AVV surgery at last follow-up. However, annular dilation (p=0.006 at TCPC and p=0.061 at last follow-up) and the number of AVV surgeries (p=0.024 at last follow-up) were identified as risk factors for reduced ventricular function.
Conclusions: AVV regurgitation in functional single ventricle is more frequently associated with TV or CAVV morphology. Morphological anomalies are the primary cause of AVV regurgitation. AVV regurgitation requiring surgical intervention influences survival following TCPC. Surgical management based on mechanisms of regurgitation is mandatory and early intervention is suggested to improve ventricular function and clinical outcomes.
P22. Cardiac Progenitor Cell Infusion to Treat the Patients with Single Ventricle Univentricular Heart Disease Strategy using Cardiac Progenitor Cell Infusion in Children with Single Ventricle Regenerative Therapy Using Cardiac Progenitor Cell for Congenital Heart Disease
*Shunji Sano, Shuta Ishigami, Shinichi Ohtsuki, Toshikazu Sano, Daiki Ousaka, Shingo Kasahara, Hidemasa Oh
Okayama University, Okayama, Japan
Objective: Cardiac regenerative strategy using cardiac progenitor cell infusion in children with single ventricle physiology including hypoplastic left heart syndrome has conducted since 2011.The aim of this study is to investigate the prognostic factors that may affect the cardiac function improvements after cell therapy.
Methods: Between January, 2011, and March, 2015, 41 children (2.8±1.4 yr) with single ventricle physiology were assigned to receive intracoronary infusion of cardiac progenitor cells after staged palliation. Cardiac tissues were harvested during surgery and progenitor cells were isolated and cultured. A total of 3.0×105 cells per kilogram of the body weight was selectively infused into each coronary artery about 1-2 months after surgical procedure. The primary endpoint was to assess the feasibility and safety; the secondary endpoint was to evaluate cardiac function and heart failure status up to 12 months after cell infusion. Multiple linear regression analysis was performed to investigate the prognostic factors impact of progenitor cell infusion on cardiac function
Results: Intracoronary injection of cardiac progenitor cells was successfully carried out in all 41 children. There were no major adverse events up to 1 year. Based on cMRI and catheter examination, children received progenitor cell infusion showed a significant improvement of ventricular ejection fraction, ventricular mechanical efficiency (Ea/Ees; systemic ventricle and ventriculoarterial coupling) and ventricular stiffness. In addition, plasma BNP levels, heart failure status (Ross scale, NYUPHF index), stressful aspects of parent-child interaction (Parenting Stress Index), and quality of life assessed by ITQOL-SF47 were all markedly improved during follow-up observation. Multiple linear regression analysis showed that ejection fraction and WAZ (weight for age z score) at cell infusion seemed to be a pivotal predictor for cardiac function improvement (P=0.001).
Conclusions: We report here the 12-month of follow-up results of intracoronary infusion of cardiac progenitor cells in 41 children with single ventricle physiology. The trial revealed improvement in ventricular ejection fraction, reduction of heart failure status and parenting stress index, and greatly improvement quality of life after cell therapy. Baseline cardiac function and WAZ may be a predictor to determine the therapeutic efficacy of progenitor cell infusion
P23. Selective Versus Standard Cerebro-Myocardiall Perfusion in Neonates Undergoing Aortic Arch Repair: a Multi-Center European Study. Versus Standard Cerebro-Myocardial Perfusion in Neonates Undergoing Aortic Arch Repair: a Multi-Center European Study
**Giovanni Battista Luciani1, Stiljan Hoxha1, Emanuela Angeli2, Francesco Petridis2, Lucio Careddu2, Alessio Rungatscher1, *Massimo Caputo3, Gaetano Gargiulo2
1University of Verona, Verona, Italy; 2University of Bologna, Bologna, Italy; 3University of Bristol, Bristol, United Kingdom
Objective: Myocardial protection during neonatal aortic arch surgery using selective ante-grade cerebral perfusion may be provided by concomitant myocardial perfusion. A novel technique for cerebro-myocardial protection was developed, where regional low-flow perfusion is combined with controlled and independent coronary perfusion, using separate pump rotors. This study aimed to compare results of selective and independent cerebro-myocardial perfusion (CMP) with standard CMP, using an arterial line Y-connector, in neonatal aortic arch surgery.
Methods: Between May 2008 and May 2016, 69 consecutive neonates underwent aortic arch repair using cerebro-myocardial perfusion for indications other than HLHS at three European Centers. Selective and independent CMP (Group 1) was used in 34 patients and standard (Group 2) in 35. Baseline demography was comparable in Group 1 vs. Group 2 relative to age (19±18 vs. 10±6, p=0.7), gender (22/34 vs. 21/35 male, p=0.9), weight (3.1±1.1 vs. 2.8±0.9 kg, p=0.2), BSA (0.20±0.05 vs. 0.19±0.03, p=0.1). Prevalence of single stage biventricular repair (20/34 vs. 23/35, p=0.9), staged repair (8/34 vs. 10/35, p=0.7) and single ventricle palliation (6/34 vs. 2/35, p=0.1) were also similar. Duration of splanchnic arrest at 25°C was similar (27±8 vs. 28±7 min., p=0.9), although CMP flows were higher in Group 1 (179±86 vs. 109±53 mL/min, p=0.007). Cardioplegic arrest to complete intra-cardiac repair was more common in Group 2 (13/34 vs. 23/35, p=0.02), although duration of myocardial ischemia was comparable (44±22 vs. 28±26 min, p=0.6). Arch repair was achieved by end-to-side anastomosis (25/34 vs. 35/35, p=0.04) or by patch augmentation (9/34 vs. none).
Results: There were 2 (2.9%) hospital deaths, one in each group, due to sepsis and multiple organ failure, respectively. There was no permanent neurological injury in either group. Perioperative cardiac dysfunction (high inotropes; ECMO; ischemia at EKG or laboratory; EF<30%) (1/34 vs. 7/35, p=0.02) and renal dysfunction (9/34 vs. 24/35, p=0.007) were more common in Group 2, although need for peritoneal dialysis was similar (4/34 vs. 1/35, p=0.06). During a mean follow-up of 3.2±2.4 years (0.3-7.3), there were 2 (cardiac, multiple organ failure) late deaths in Group 1 and 3 (cardiac, sepsis, multiple organ failure) in Group 2, with comparable 5-year survival (75±17% vs. 88±6%, p=0.7). Further catheter or surgical procedure on the arch was necessary in 4/33 vs. 5/34 hospital survivors, with comparable 5-year freedom from reintervention (86±6% vs. 84±7%, p=0.6).
Conclusions: Overall CMP is a safe and effective perfusion strategy in patients requiring neonatal arch repair. Selective and independent CMP is associated with reduced cardiac morbidity and may thus be better suited in neonates needing complex (TGA, DORV, truncus) arch repair.
P24. The Most Prevalent Tetralogy of Fallot Surgical Repair Strategy is Associated with Unfavourable Right Bundle Branch Block
Sara Hussain1, Ahmad Makhdoum2, Charis Tan3, Prisca Pondorfer4, Quazi Ibrahim1, *Yves D’Udekem3, Richard Whitlock1, *Glen Van Arsdell4
1Population Health Research Institute, Hamilton, ON, Canada; 2University of Toronto, Toronto, ON, Canada; 3The Royal Children’s Hospital Melbourne, Melbourne, Australia; 4The Hospital for Sick Children, Toronto, ON, Canada
Objectives: There are multiple strategies that can be employed for effective repair of Tetralogy of Fallot (TOF). Published STS and EACTS data shows that more than 50% of repairs involve transventricular VSD closure and transannular patch (TAP). We sought to determine if the type of TOF repair impacts the prevalence of late RBBB given that emerging literature demonstrates an association between right bundle branch block (RBBB), ventricular asynchrony, and decreasing LV function.
Methods: Cases performed between 1996-2004 at 2 large pediatric centers were reviewed for operative details and ECG findings. The primary outcome of interest was RBBB on ECG or Holter monitoring reports. Logistic regression analyses were performed to study the association between RBBB and TOF surgical repair strategies, adjusted for confounding effects.
Results: A total of 402 TOF repair cases were performed with a mean age of 1.03 years (1.2). The main repair strategies performed were annulus preservation (AP=141, 35%), annulus preservation and infundibular incision (AP+Infund=44, 11%), minimal TAP (miniTAP= 202, 50%) and transannular patching (TAP=15, 4%). The VSD was closed through a transatrial approach in 378 (94%) of cases. RBBB was evident in 212 patients (53%) with a mean follow-up of 17.0 years. Univariate analysis demonstrated that RVOT repair strategy and approach to VSD closure were significant predictors of RBBB. Significant association between RVOT repair strategy and approach to VSD closure were observed (p<0.001). The odds of developing RBBB was 3.4 higher when a trans-ventricular incision is used to close the VSD compared to a transatrial approach (95% CI of OR: 1.1, 10.3, p=0.03). Use of a large transannular patch increases the odds for developing RBBB by 4.6 times in comparison to an annulus preservation strategy (95% CI of OR: 0.99, 21.3, p=0.05).
Conclusions: A trans-ventricular approach to VSD closure and/or large TAP repair were found to significantly increase the odds of developing RBBB in repaired TOF patients. This data suggests that the approach to VSD closure and relief of outflow obstruction have an impact on RBBB – a finding which could prove to be important to ventricular function.
Odds of developing right bundle branch block:
|Model 1 (VSD closure)||Transventricular VSD closure||3.4||0.032||1.1, 10.3|
|Model 2 (RVOT repair strategy)||miniTAP||0.75||0.197||0.481, 1.162|
Congenital Heart Disease Simultaneous Scientific Session
Tuesday, May 2, 2017
2:00 pm – 5:35 pm
Insights from the Congenital Heart Surgeons’ Society Anomalous Coronary Artery Database
*Marhsall L. Jacobs, Johns Hopskins Hospital, Baltimore, MD
66. Patients with Anomalous Aortic Origin of the Coronary Artery Remain at Risk Even After Surgical Repair
Shannon N. Nees, Jonathan N. Flyer, Anjali Chelliah, Jeffrey D. Dayton, David Kalfa, *Paul J. Chai, *Emile A. Bacha, Brett R. Anderson
Columbia University, New York, NY
Objective: Anomalous aortic origin of the coronary artery (AAOCA) is a rare anomaly associated with sudden cardiac death (SCD). Single center studies describe surgical repair as safe, though medium and long-term effects on symptoms and SCD risk remain unknown. We sought to describe medium-term outcomes of surgical repair of AAOCA.
Methods: We reviewed institutional records for patients who underwent surgery for AAOCA, 2001- 2016. Follow-up data were obtained via phone calls and written questionnaires to patients and cardiologists. Patients with associated heart disease were excluded.
Results: In total, 45 patients underwent surgery for AAOCA (24 left, 21 right). Median age was 15.4 years (IQR 11.8-19.1 yrs; range 4 mos – 68 yrs). Most common symptoms were chest pain (n=25, 56%) and shortness of breath (SOB) (n=13, 29%). Cardiac arrest was the presenting symptom in 5 (11%) patients. Coronary unroofing was performed in 42 (93%), coronary translocation in 2 (4%) and removal of a fibrous sheath in 1 (2%). Early post-operative complications occurred in 10 (22%) patients, including post-pericardotomy syndrome (n=6, 13%), supravalvar aortic stenosis (n=1, 2%), transient arrhythmia (n=1, 2%), pleural effusion (n=1, 2%) and sternal wound infection (n=1, 2%). One patient who presented post-arrest went to and returned from the operating room on ECMO. Median length of stay was 4 days (IQR 4-5; range 3 – 42 days). Follow-up data were available for 38 (84%) patients over a median of 1.1 years (IQR 0.3-3.0 yrs; range 10days – 9 yrs). Of 32 patients with symptoms at presentation, 23 (72%) had resolution post-operatively. Of 6 patients who were initially asymptomatic, 4 (67%) developed post-operative symptoms. Of those with follow-up testing, 1/32 (3%) had evidence of ischemia on ECG and 4/25 (16%) had evidence of ischemia on stress test. Of 4 patients with abnormal stress tests, 1 (25%) had SOB with exertion and is currently exercise restricted and 3 (75%) were asymptomatic and cleared for exercise. Two (4%) patients required reoperation. The first experienced a cardiac arrest 6 years post-surgery despite a negative stress test. Noninvasive imaging was concerning for stenosis. Intra-operative examination revealed fibrosis around the left coronary orifice. The second patient had recurrent chest pain 3 months post-surgery. Significant stenosis was found at the anomalous left coronary orifice on catheterization and coronary bypass was performed. Both patients were asymptomatic at 0.5 and 3 years after the second operation, respectively.
Conclusions: Surgical repair of AAOCA is generally safe and significant early post-operative complications are rare. Restenosis of the anomalous coronary orifice can occur and patients may be at risk of SCD even after operative repair. Patients should be monitored longitudinally for signs and symptoms of ischemia, though current testing might not always predict clinical events.
67. Selection of Prosthetic Aortic Valve and Root Replacement in Young Patients Less Than Thirty Years of Age
Rita K. Milewski, Andreas Habertheuer, *Joseph E. Bavaria, Stephanie Fuller, *Wilson Szeto, *Nimesh Desai, Varun Korutla, Prashanth Vallabhajosyula
University of Pennsylvania, Philadelphia, PA
Objective: Valve repair or replacement with pulmonary autograft is an established option for young patients with aortic valve disease with or without ascending aortopathy. However, long term outcomes in young patients undergoing prosthetic aortic valve/ root replacement are not well studied. We assessed our single institution experience with prosthetic aortic valve/ root replacement in patients age less than 30 years.
Methods: From 1998 to 2016, 91 patients (n=54/91, 59.3% with bicuspid aortic valve) between the ages of 16 and 29 (mean age 25.0±2.9 years, 70.3% male) underwent aortic valve (AVR, n=51/91), aortic valve and supracoronary aorta (AVSAAR, n=4/91), or aortic root (ARR, n=36/91) replacement. Freedom from reoperation was evaluated both by inclusion and exclusion of endocarditis events. Total follow-up was 396 patient years. Prospectively maintained aortic valve database was retrospectively reviewed.
Results: Indications included primary aortic stenosis/insufficiency in 61.5% (n=56/91), Marfan syndrome in 11.0% (n=10/91), and endocarditis in 27.5% (n=25/91). Indications for reoperation included patient-prosthesis mismatch in 2.2% (n=2/91), prosthetic valve degeneration in 7.7% (n=7/91), and prosthetic valve endocarditis in 9.9% (n=9/91). The 30-day/ in-hospital mortality was 3.3 % (n=3/91), all endocarditis cases. Stroke rate was 1.1% (n=1/91, mechanical) and renal failure was 1.1% (n=1/91, biovalve). Overall valve replacement included 38.5% (n=35/91) bioprosthetic/biologic and 61.5 % (n=56/91) mechanical valves. There was a trend towards improved in-hospital survival in patients receiving bioprosthetic valve (p=0.07). Median follow-up was 52.24 months. Overall freedom from aortic valve reoperation at 1, 5, and 10 years was: 92%, 81%, 67% including endocarditis cases, and 99%, 93%, 77% excluding endocarditis cases. There was no significant difference in reoperation between bioprosthetic/biologic and mechanical valves either including (log rank p=0.34) (Figure 1A) or excluding (log rank p=0.29) endocarditis cases (Figure 1B). Overall survival at 1, 5, and 10 years was 94%, 94%, 89%, with increased mortality in prosthetic valve endocarditis cases (1 and 5 year survival of 56% and 56%) (p<0.0001). Mid and long term survival (exclusive of endocarditis) was similar in patients with mechanical versus bioprosthetic/ biologic valves (mechanical= 94% and 86% and bioprosthetic/biologic= 94% and 94% respectively at 5 and 10 years, log rank p=0.8).
Conclusions: Aortic valve and root replacement is associated with good long term freedom from aortic valve reoperation and survival in patients under 30 years. The choice of mechanical versus biological valve does not affect freedom from reoperation rates in this young cohort at 10 years. It will be important to understand >20% reoperative rate at 10 years in this young patient cohort.
68. Outcomes of the Arterial Switch Operation in ≤2.5kg Neonates: A 10 Year Study
Michael Salna, *Paul J. Chai, David M. Kalfa, Yuki Nakamura, Ganga Krishnamurthy, Marc Najjar, Amee Shah, Stephanie Levasseur, Brett R Anderson, *Emile A. Bacha
Columbia University, New York, NY
Objectives: While low birth weight is a known risk factor for mortality in congenital heart lesions and may consequently delay surgical repair, outcomes in low weight neonates undergoing the arterial switch operation (ASO) have not been well described. Our primary objective was to determine whether there were any differences in outcomes between infants weighing less than or equal to 2.5kg and those weighing more than 2.5kg at the time of surgery.
Methods: We retrospectively analyzed outcomes in low weight neonates (weighting less than or equal to 2.5 kg) undergoing the arterial switch operation from 2005 to 2014 at our institution. In-hospital and long-term mortality, post-operative complications, the need for early reintervention, and post-operative lengths of stay were assessed as outcomes. The mean follow-up time was 33.0 ± 41.7 months.
Results: A total of 220 neonates underwent the arterial switch operation from 2005 to 2014 with 31 (14%) patients weighing ≤ 2.5kg at the date of surgery. Neonates weighing ≤2.5 kg at surgery had significantly lower birth weights and were more likely to be premature but there was no difference in age at the time of surgery between the two groups. In-hospital mortality was 3.2% (n=1) in the ≤2.5kg group compared with 2.1% (n=4) in the > 2.5kg group (p =0.536) with no surgeon-specific differences. Compared with neonates > 2.5kg, the low weight group had significantly longer post-operative ICU lengths of stay (median: 19 days (interquartile range [IQR]: 13-26 days) vs. 14 days (IQR: 11-20 days)). Rates of early reintervention and post-operative complications were also comparable between the two groups (Table 1). Amongst a cohort of eight neonates weighing < 2.0 kg, in-hospital mortality occurred in one patient but there was a significantly higher incidence of cardiovascular complications compared with patients weighing > 2kg (n = 4 (50%) vs. n =31 (15%), p = 0.024). There was no mortality in any of the patients at the date of most recent follow-up.
Conclusion: The arterial switch operation can be performed safely in neonates weighing less than 2.5kg and yields comparable results to infants weighing more than 2.5kg. Imposed delays for corrective surgery may not be necessary for low weight infants with transposition of the great arteries.
Table 1. Characteristics and outcomes of ASO patients stratified by Operative Weight
(n = 220)
(n = 189)
(n = 31)
|Age at operation (days)||6 (4-8)||6 (4-8)||6 (4-11)||0.229|
|Male Gender (%)||135 (61.4%)||117 (61.9%)||18 (58.1%)||0.695|
|Birth weight (kg)||3.26 (2.80-3.52)||3.31 (3.02-3.59)||2.38 (1.88-2.49)||< 0.001|
|Weight at operation (kg)||3.22 (2.80-3.60)||3.34 (3.00-3.64)||2.30 (1.95-2.40)||< 0.001|
|Premature birth (%)||29 (13.2%)||14 (7.4%)||15 (48.4%)||< 0.001|
|TGA Subtype (%)||0.259|
|TGA, intact ventricular septum||123 (55.9%)||107 (56.6%)||16 (51.6%)|
|TGA, ventricular septal defect||71 (32.3%)||57 (30.2%)||14 (42.8%)|
|TGA, VSD LVOTO||1 (0.5%)||1 (0.5%)||0 (0%)|
|Taussig-Bing||25 (11.3%)||24 (12.7%)||1 (3.2%)|
|Cardiovascular||35 (15.9%)||30 (15.9%)||5 (16.1%)||1.000|
|Pulmonary||11 (5.0%)||9 (4.8%)||2 (6.5%)||1.000|
|Neurologic||6 (2.7%)||4 (2.1%)||2 (6.5%)||0.201|
|Vocal Cord Paralysis||13 (5.9%)||11 (5.8%)||2 (6.5%)||1.000|
|Sepsis||3 (1.4%)||3 (1.6%)||0 (0%)||1.000|
|Early Reintervention (%)||3 (1.4%)||2 (1.1%)||1 (3.2%)||0.367|
|In-Hospital Mortality (%)||5 (2.3%)||4 (2.1%)||1 (3.2%)||0.536|
|Post-op ICU Length of Stay (days)||9 (6-14)||8 (6-13)||14 (7-18)||0.010|
|Total Length of Stay (days)||14 (11-20)||14 (11-20)||19 (13-26)||0.002|
|Post-Discharge Mortality (%)||0 (0%)||0 (0%)||0 (0%)||1.000|
ASO- Arterial Switch Operation; VSD – Ventricular Septal Defect; LVOTO – Left Ventricular Outflow Tract Obstruction. Data are presented as median (interquartile range) unless otherwise specified.
69. Reoperation for Coronary Artery Stenosis after Arterial Switch Operation
Joon Chul Jung, Eung Re Kim, Ji Hyun Bang, Jae Gun Kwak, Woong-han Kim
Seoul National University Hospital, Seoul, Republic of Korea
Objective: Coronary artery stenosis can be a cause of delayed reoperation after arterial switch operation (ASO). We investigated freedom from reoperation, risk factors for reoperation and results of reoperation.
Methods: Between 2003 and 2016, 77 consecutive patients who underwent arterial switch operation and survived early postoperative period were included. We reviewed their diagnosis, coronary artery pattern, coronary artery anomaly, coronary artery transfer technique for risk factor analysis. We analyzed reoperation techniques and operative results.
Results: Diagnosis included d-TGA with IVS (32/77, 41.6%), d-TGA with VSD (32/77, 41.6%), d-TGA with CoA (4/77, 5.2%), d-TGA with IAA (3/77, 3.9%), Taussig-bing anomaly (2/77 2.6%), Taussig-bing anomaly with CoA (3/77, 3.9%), Taussig-bing anomaly with IAA (1/77 1.3%). Coronary artery pattern included usual (1AD,Cx;2R, 61/77, 79.2%), LCx from RCA (1AD;2Cx,R, 5/77, 6.5%), inverted (1R;2AD,Cx, 4/77, 5.2%), intramural left main coronary artery (LMCA) (2AD,Cx;2R, 3/77, 3.9%), single LCA (1AD,Cx,R, 2/77, 2.6%), single RCA (2AD,Cx,R, 2/77, 2.6%). Coronary artery anomaly included intramural LMCA (3/77, 3.9%) and high take-off LMCA (2/77, 2.6%). Coronary artery transfer technique included Trap door (68/77, 88.3%), punch out (5/77, 6.5%), modified Aubert (3/77, 3.9%), tube reconstruction (1/77, 1.3%). There was no late death during follow up period. Total 6 patients underwent reoperation for coronary artery stenosis. Freedom from reoperation at 5-year and 10-year were 93.9% and 89.4%. In multivariate analysis, intramural LMCA and high take-off LMCA were significant risk factors for reoperation (relative hazard ratio 14.3 (95% CI 2.1-96.4, p=0.006) and 36.4 (95% CI 2.7-482.2, p=0.006)). Reoperation techniques included coronary artery os un-roofing (3/6, 57.1%), cut-back angioplasty (1/6, 14.3%), ostioplasty (2/6, 28.6%). All patients who underwent un-roofing for intramural LMCA and cut-back angioplasty had no acute complication, death, or restenosis during 23.0-62.6 months of follow-up period. However, 2 patients who underwent ostioplasty needed additional reoperation for coronary artery restenosis within 2 months.
Conclusion: In arterial switch operation, intramural LMCA and high take-off LMCA were significant risk factors for reoperation. In patient with intramural LMCA, coronary artery os un-roofing can be considered during initial arterial switch operation. The results of reoperation were good with un-roofing and cut-back angioplsaty techniques.
70. Laryngeal Ultrasound Detects A High Prevalence of Vocal Cord Paresis After Aortic Arch Repair in Neonates and Young Children
Melissa G. Y. Lee, Johnny Millar, Elizabeth Rose, Aleesha Jones, Dora Wood, Taryn L. Luitingh, Johann Brink, *Igor E. Konstantinov, *Christian P. Brizard, *Yves d’Udekem
Royal Children’s Hospital, Parkville, Australia
Objective: To determine (1) the prevalence of vocal cord paresis after aortic arch repair or Norwood-type procedure via sternotomy, and (2) the effectiveness of non-invasive laryngeal ultrasound in detecting vocal cord paresis compared with gold standard invasive laryngoscopy.
Methods: Twenty-seven patients who had an aortic arch repair (22/27, 81%) or Norwood-type procedure (5/27, 19%) via sternotomy between April 2015 – October 2016 underwent a laryngeal ultrasound 48-72 hours after endotracheal extubation. Fourteen patients (14/27, 52%) also consented to a laryngoscopy. Primary arch diagnoses were coarctation of the aorta in 52% (14/27), isolated hypoplastic aortic arch in 22% (6/27), hypoplastic left heart syndrome in 15% (4/27), and interrupted aortic arch in 11% (3/27). Median age at surgery was 5 days (interquartile range (IQR): 3-10). Median intubation time was 3 days (IQR 2-8). Two patients (2/27, 7%) required an arch reoperation for arch reobstruction prior to extubation.
Results: Left vocal cord paresis was present in 58% (14/24) and 57% (8/14) on laryngeal ultrasound and laryngoscopy, respectively, and an additional 3 patients had an inconclusive result on laryngeal ultrasound (Figure 1). The degree of vocal cord paresis on laryngeal ultrasound was partial in 29% (4/14) and full in 71% (10/14). There was agreement between the results of laryngoscopy and the results of laryngeal ultrasound in all cases. Patients who required pre-operative endotracheal intubation had a higher prevalence of vocal cord paresis on laryngeal ultrasound compared to patients who did not require pre-operative intubation (100% [7/7] vs. 41% [7/17], p=0.02). After excluding the 7 patients who required pre-operative intubation, patients who underwent an arch repair had a higher prevalence of vocal cord paresis on laryngeal ultrasound compared to patients who underwent a Norwood-type procedure (54% [7/13] vs. 0% [0/4], p=0.1).
Conclusions: There is a high prevalence of vocal cord paresis after aortic arch repair via sternotomy. Strategies to preserve left recurrent laryngeal nerve function should be explored. Laryngeal ultrasound seems to be an effective and non-invasive method to detect vocal cord paresis in neonates and young children.
71. Management of Aortic Aneurysms Following Repair of Congenital Heart Disease
*Christian Pizarro, Gina M. Baffa, Majeed A. Bhat, Ryan Robert Davies, Kristi Fitzgerald
Alfred I duPont Hospital for Children, Wilmington, DE
Objective: Progressive aortic root dilatation has been documented during follow-up of children after repair of congenital heart disease (CHD). A significant knowledge gap exists regarding the natural history and management of this issue. We report our early experience with surgical intervention for aortic root dilatation following surgery for CHD.
Methods: Contemporary review of indications, timing of surgery and outcomes among patients with aneurysmal dilatation of the aortic or neo-aortic root following repair of CHD who underwent surgical intervention at a single institution (2005-2015). Patients with known connective tissue disorders were excluded.
Results: Nineteen patients underwent aortic intervention following surgery for CHD at a median age of 14 years (5-21) and median weight of 46.8 (20-103). The most common congenital diagnosis was HLHS in 7, transposition of the great arteries in 6, and aortic coarctation/IAA in 4 patients. Previous intervention included Fontan completion in 7, aortic valvuloplasty in 5, arterial switch procedure in 4 and repair of aortic arch/coarctation in 4 patients. Ten patients had an abnormal aortic valve (bicuspid 8, unicuspid 2). Median aneurysm size was 48 mm (40-70); aortic root and ascending aorta z-scores were 6.2 (4.4-13) and 6.4 (5.4-9.5) respectively. Indications for intervention included progressive aortic dilatation in all, plus significant aortic or neo-aortic valve regurgitation in 10, aneurysmal growth in 8, pulmonary artery stenosis in 6, recurrent chest pain in 2 and tracheal compression in 1. A valve-sparing aortic root replacement and a Bentall were the procedures most commonly performed (7 cases respectively). A tailoring procedure was performed in 4 patients. Associated procedures were common, including aortic arch replacement in 9, pulmonary arterioplasty in 5, a tricuspid valve repair, cryo Maze and Konno in 1. Histological findings included moderate to severe cystic medial degeneration and micro-dissection. A genetic abnormality associated with aneurysmal disease was newly identified in 5 cases. Median cardiopulmonary bypass and aortic cross clamp were 194 (103-264) and 105 minutes (62-172) respectively. Median ICU and hospital stay were 2 (1-4) and 7 (5-12) days. There was no operative mortality. At median follow up of 58 months, there were three late deaths, all survivors remain in functional class I and free of aortic regurgitation.
Conclusions: Despite high complexity, management of aortic aneurysmal disease following repair of CHD can afford excellent outcomes. Use of valve sparing root techniques can effectively restore aortic and neo aortic valve competency. Genetic screening should be performed routinely in order to inform timing of intervention and subsequent screening for aneurysmal disease.
72. Outcomes of Patients Undergoing Surgical Repair of Multiple Ventricular Septal Defects: a 22-year Study of 157 Patients
Michael Daley1, *Christian P. Brizard1, *Igor E Konstantinov1, Johann Brink1, Andrew Kelly2, Bryn O. Jones1, Diana Zannino3, *Yves d’Udekem1
1Royal Children’s Hospital, Melbourne, Australia; 2Women’s and Children’s Hospital, Adelaide, Australia; 3Murdoch Children’s Research Institute, Melbourne, Australia
Objective: Surgical treatment of multiple ventricular septal defects (VSDs) remains technically challenging. Residual defects, complete heart block and ventricular dysfunction have been reported as common complications but late outcomes of these procedures have not yet been defined.
Methods: From 1988 to 2015, 157 consecutive patients underwent surgical repair of multiple VSDs at a median age of 2.2 months (2 days – 16.2 years). Sixty-nine patients (44%) had exclusively multiple VSDs, 62 patients (39%) had multiple VSDs with concomitant intra-cardiac anomalies and 26 patients (17%) had multiple VSDs with aortic arch anomalies. Initial operations involved 150 VSD closures in 102 patients: direct closure (46), patch closure (88), sandwich technique (13), perventricular device closure (3). Pulmonary artery banding was performed in 83 patients. Fifty-four patients had banding only and 45 had an absorbable polydioxanone band. Eighteen patients (11%) required a ventriculotomy: right (15), left (2) or both (1).
Results: Operative mortality was 1% (2/157). Mean follow-up was 8 ± 6 years (1 day – 22 years). Survival was 94% (95% CI 88% – 97%) at 18 years. Ten patients required reoperation during hospital stay and 52 after hospital discharge consisting in 40 operations related to banding, and 50 additional procedures on the VSDs: direct closure (4), patch closure (38) and sandwich technique (8) with 13 patients requiring a ventriculotomy: right (10), left (1), both (2). Freedom from late reoperation related to residual VSDs was 51% (95% CI 41% – 60%) at 15 years (Figure 1). Thirty-one of the 45 patients treated with an absorbable PA band (69%) underwent only one procedure. At last follow-up, six patients (4%) were on anti-failure therapy for significant VSD while complete closure of the VSDs was observed in 89 patients (58%). Pacemaker implantation was ultimately required in 14 patients (9%). Follow-up left ventricular end-systolic and diastolic diameter Z-scores was available in 75 patients and was reported to be a mean 0.6 ± 1.6 and 0.9 ± 1.8 respectively. No deleterious impact of a ventriculotomy could be detected.
Conclusions: Surgical treatment of multiple VSDs can be performed with excellent short and long-term survival and normal late functional outcome. Half of the patients may require more than one procedure and the rate of pacemaker implantation is ultimately higher than reported with single VSDs. The use of absorbable pulmonary artery bands, limited ventriculotomies and sandwich techniques are useful adjuncts to the array of techniques necessary to treat patients with multiple VSDs.
Congenital Heart Disease Simultaneous Scientific Session
Wednesday, May 3, 2017
7:30 am – 9:35 am
90. Staged Ventricular Recruitment via Atrial Septation Alone in Patients with Borderline Ventricles and Large Ventricular Septal Defects
Olubunmi Oladunjoye, Puja Banka, Gerald Marx, Roger Breitbart, *Pedro del Nido, *Sitaram Emani
Boston Children’s Hospital, Boston, MA
Objective: Patients with borderline ventricular size and ventricular septal defect (VSD) including double outlet right ventricles and unbalanced atrioventricular canal defects who have previously undergone single ventricle palliation may be candidates for staged ventricular recruitment to maximize ventricular growth with the ultimate goal of eventual biventricular conversion. The aim of this study was to determine the impact of atrial septation alone i.e. diverting flow into the hypoplastic ventricle, upon ventricular growth in patients with borderline right or left ventricles and VSD who had previously undergone single ventricle palliation.
Methods: Patients with borderline ventricles and VSD who underwent recruitment procedure with fenestrated atrial septation alone without VSD closure between 2009 and 2016 were retrospectively reviewed. Patient demographics, diagnosis, surgical procedures and outcomes were abstracted from medical record and left and right heart volumes from pre- and post-recruitment cardiac magnetic resonance (CMR) examinations. Pre- and post-recruitment values were then compared using Wilcoxon Signed Ranks test.
Results: A total of 16 patients underwent staged ventricular recruitment via atrial septation as the sole procedure at median age of 19.4 (IQR 8.1-44.4) months. Among the group, 11 (68.8%) were females and 12 (75%) had borderline left ventricles. Time between pre- and post- recruitment CMR examination was 10.3 (IQR 8.4-12.5) months. The median indexed ventricular diastolic volume increased from 30.7 (IQR 24.4-35.4) ml/m2 to 44.4 (IQR 38.4-58.0) ml/m2 after the recruitment procedures (p<0.01) while the median indexed systolic volume increased from 12.3 (IQR 9.5-17.0) ml/m2 to 19.5 (IQR 16.0-28.2) ml/m2 after recruitment (p<0.01). There was also an increase in the median indexed stroke volume from 18.2 (IQR 13.8-20.1) ml/m2 to 29.1 (IQR 21.3-31.8) ml/m2 (p<0.01). Indexed ventricular mass of the ventricle went from 22.7 (IQR 18.0-30.4) g to 27.3 (IQR 18.2-31.7) g but it was not statistically significant (p0.333). Biventricular conversion was achieved in 10 patients (62.5%), 4 (25.0%) are yet to undergo conversion, one patient underwent Fontan completion and atrial septectomy and only one was listed for transplant as the patient was not fit for single ventricle palliation or biventricular conversion. There were no complications related to the procedure.
Conclusions: Staged ventricular recruitment by fenestrated atrial septation to divert flow into the hypoplastic ventricle, without VSD closure can result in growth of the hypoplastic ventricle enabling subsequent biventricular conversion in a subset of patients. Further studies are needed to identify candidate patients who may be best suited to this approach.
91. Planned Growth of Hypoplastic Cardiac Structures to Achieve Improved Long-Term Outcomes
Daniel Labuz1, Lee Pyles2, James Berry3, *John Foker3
1Oregon Health Sciences University, Portland, OR; 2West Virginia University, Morgantown, WV; 3University of Minnesota, Minneapolis, MN
Objective: Congenital heart defects (CHD) may include hypoplastic valves, ventricles and/or vessels which complicate repairs and compromise long term outcomes. Our hypotheses were that (1) hypoplastic structures are developmental rather than primarily genetic in origin and (2) the correct biomechanical signal would induce catch up growth. Our corollary hypothesis was that the signal is flow, not pressure. The first stage operations, therefore, were designed to increase flow and induce growth. Once sufficient growth had occurred, a secondary operation completed the repair. We report the results for flow induced growth of hypoplastic structures in three types of CHD repairs that led to improved outcomes such as two-ventricle repairs (2VRs).
Methods: Three groups of CHD patients with associated hypoplastic structures were reviewed: Unbalanced atrioventricular canal defects (UAVC); Pulmonary atresia with intact ventricular septum (PAIVS); and Coarctation of the aorta (CoA). Evaluation was by pre- and post-operative echocardiographic analysis, which was converted to Z-scores (Table). Operations included creating restrictive septal defects to increase flow through hypoplastic AV valves and ventricles (UAVC, PAIVS) and the relief of obstruction (principally CoA patients) which increased aortic valve and arch flow. No operation required circulatory arrest. All procedures increased flow through the hypoplastic structures.
Results: Follow up evaluation found virtually all (85/90) hypoplastic structures reached normal size and the remaining 5 had showed significant growth and were within one SD of normal. Subsequent growth had proceeded normally and few required cardiac medications (Table). In no case was a single ventricle repair track needed.
Conclusions: 1) Operations designed to increase flow through hypoplastic structures reliably induced catch up growth in UAVC, PAIVS and CoA lesions. 2) The growth response supported that underdevelopment was the cause of hypoplasia and flow was the growth signal which could reverse it. 3) Growth induction reliably allowed 2VRs in UAVC and PAIVS patients and avoided extended arch repairs in CoA. 4) Following catch up growth of the hypoplastic lesions, the outlook improved to that of patients with balanced AVCs and normal sized RVs in pulmonary stenosis. 5) Once normal size was reached, growth continued normally, producing a durable result. 6) Normal sized but previously hypoplastic structures still allowed adequate function and reduced the need for cardiac medications.
Staged Ventricular Recruitment- Strategies to Rehabilitate Borderline Ventricles
*Sitaram Emani, Boston Children’s Hospital, Boston, MA
92. Surgical Algorithm and Results for Repair of Pulmonary Atresia/Ventricular Septal Defect/Major Aortopulmonary Collaterals
*Frank L. Hanley, *Richard Mainwaring, William L. Patrick, Steve Roth, Komal Kamra, Lisa Wise-Faberowski
Stanford University, Stanford, CA
Objective: Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex and heterogeneous form of congenital heart disease. There is an ongoing controversy regarding the optimal treatment of PA/VSD/MAPCAs. The purpose of this study was to summarize our algorithm and surgical results for PA/VSD/MAPCAs.
Methods: This was a retrospective review of 301 patients (2001-2016) undergoing primary surgical treatment of PA/VSD/MAPCAs. Excluded from this analysis were patients who had undergone prior surgical treatment at another institution and patients with single ventricle and MAPCAs. There were three surgical pathways, including: 1) Mid-line unifocalization (n=233), 2) Creation of an aortopulmonary window (n=45), and 3) Other (n=23).
Results: For the 233 patients who underwent mid-line unifocalization, 199 (85.4%) had a single stage complete repair (including complete unifocalization with closure of the VSD and right conduit). The average right ventricle to aortic pressure ratio following complete repair for these 199 patients was 0.36 ± 0.09.
There were 34 patients who underwent a mid-line unifocalization and did not undergo single stage complete repair. These patients underwent an initial unifocalization and shunt and 22 have subsequently had a complete repair.
Forty five patients underwent an aortopulmonary window, of whom 33 have subsequently undergone complete repair. There were 23 patients who had complex anatomy and underwent procedures other than described above. Seventeen of these patients have subsequently undergone complete repair. Thus, for the patients currently eligible, 271 (92.4%) have achieved complete repair.
For the 72 patients who underwent a staged complete repair, the average right ventricle to aortic pressure ratio was 0.39 ± 0.07. This was slightly higher compared to those patients who underwent single stage complete repair (p < 0.05). There were a total of 6 (2.0%) operative mortalities at the initial surgical procedure, and 23 (7.6%) late mortalities (as summarized in the figure). The combined early and late mortality was 3.2 X lower in patients who underwent single stage complete repair compared with all other initial procedures (p < 0.01).
Conclusions: The data demonstrate that more than 90% of patients with PA/VSD/MAPCAs underwent complete repair, with an average right ventricular pressures less than 40% systemic. The overall mortality was significantly lower in the sub-group of patients who were able to undergo single stage complete repair. These results demonstrate the favorable early and mid-term outcomes that can be achieved in this heterogeneous group of patients with PA/VSD/MAPCA
93. Descending Aortopexy and Posterior Tracheopexy for Severe Tracheomalacia and Left Mainstem Bronchomalacia
Hester F. Shieh, C. Jason Smithers, Thomas E. Hamilton, David Zurakowski, Gary A. Visner, Michael A. Manfredi, Russell W. Jennings, Christopher W. Baird
Boston Children’s Hospital, Boston, MA
Objective: In severe tracheomalacia, posterior tracheopexy has been shown to improve airway patency by addressing posterior membranous tracheal intrusion. Its effectiveness can be limited by left mainstem bronchomalacia from compression between the descending aorta and pulmonary artery. Posterior descending aortopexy can be used to relieve this left mainstem compression. We review a series of patients who underwent descending aortopexy and posterior tracheopexy for severe symptomatic tracheobronchomalacia with posterior intrusion and left mainstem compression to determine if there were resolution of clinical symptoms and bronchoscopic evidence of improvement in airway collapse.
Methods: All patients who underwent both descending aortopexy and posterior tracheopexy at our institution from October 2012 to October 2016 were retrospectively reviewed. Clinical symptoms, tracheomalacia scores based on standardized dynamic airway evaluation, and persistent airway intrusion requiring reoperation were collected. To determine tracheomalacia scores, the tracheobronchial tree was evaluated on bronchoscopy by anatomical region and open airway with a standardized scoring system. The percentage of open airway was scored out of 100 for each anatomical region (upper, middle, and lower trachea; right and left mainstem bronchi). Data were analyzed by the Wald and Wilcoxon signed-ranks tests.
Results: 32 patients (63% male) underwent descending aortopexy and posterior tracheopexy at median (IQR) age 18 months (6-40). 66% were associated with esophageal atresia (EA) and 69% with cardiac disease. 63% had a prior EA repair and 19% had a prior anterior aortopexy. Median (IQR) follow up was 3 months (1-7). There were statistically significant improvements in clinical symptoms postoperatively, including cough, noisy breathing, prolonged and recurrent respiratory infections, ventilator dependence, blue spells, and apparent life-threatening events (ALTEs) (all p<0.001), as well as exercise intolerance (p=0.033), transient respiratory distress requiring positive pressure (p=0.003), and oxygen dependence (p=0.007) (figure). Total tracheomalacia scores (median (IQR)) on bronchoscopy improved significantly from 215 (145-268) to 450 (360-475) (p<0.001), with significant segmental improvements in the middle (p=0.003) and lower (p<0.001) trachea, and the right (p=0.011) and left (p<0.001) mainstem bronchi. 2 patients (6%) had persistent airway intrusion requiring reoperation with anterior aortopexy or tracheopexy. There was no mortality.
Conclusions: Descending aortopexy and posterior tracheopexy are effective in treating severe tracheobronchomalacia and left mainstem compression with significant improvements in clinical symptoms and degree of airway collapse on bronchoscopy.
94. Early and Mid-Term Results of Autograft-Sparing/Ross Reversal: A One-Valve Disease need not become a Two-Valve Disease
Syed T. Hussain, David Majdalany, Robert D. Stewart1 Antoine Addoumieh, *Eugene H. Blackstone, Gosta B. Pettersson
Cleveland Clinic, Cleveland, OH
Objective: Risk of reoperation and loss of a second native valve are major drawbacks of the Ross operation. Allograft or composite root replacement for a failed autograft leaves the patient with an allograft in the pulmonic position that is subject to further deterioration and need for a subsequent technically-demanding reoperation. Rather than sacrifice the failed autograft, it is reused, placing it back into the native pulmonary position: “Ross reversal”. We reviewed our early and mid-term results of this operation.
Methods: From 9/2006-10/2016, 39 patients underwent reoperation for autograft dysfunction. The autograft was successfully salvaged in 35 patients, by Ross reversal in 30, David procedure in 4, and autograft repair in 1. The autograft could not be salvaged in 4 patients, 1 each due to previous subcoronary autograft implantation, a failed autograft repair with aortic valve replacement, attempted David procedure, and poor autograft quality. Medical records were reviewed for patient characteristics, prior operations, indications for reoperation, hospital outcomes, and echocardiographic findings for the 30 patients undergoing successful Ross reversal.
Results: Mean age was 45±13 years (range 18-67 years) and 26 were male. Median interval between original Ross procedure and Ross reversal was 12.5 years (range 5-19 years). All 30 patients had autograft dysfunction with regurgitation and/or root dilatation. 21 patients also had an indication for reoperation on the pulmonary allograft. 22 were first-time reoperations, 5 second-time, 1 third-time and 2 fourth-time. 8 previous reoperations were performed in 6 patients for pulmonary allograft dysfunction, including 3 in one patient. The autograft was excised and replaced into the native pulmonary position followed by either aortic root replacement with a composite root or an allograft. 12 concomitant procedures were performed, including 5 hemiarch replacements; 6 patients required brief hypothermic circulatory arrest. There was no in-hospital or operative mortality. One patient required reoperation for bleeding. Another had abdominal aorta injury from use of an endo-clamp. There was no other major postoperative morbidity and median postoperative hospital stay was 8.0 days (range 4-41 days). None of the patients required reoperation in the follow-up period (median 1.7 years, range 1 month-10 years). On echocardiography, 21 patients had excellent pulmonary valve function, while 9 developed mild-to-moderate regurgitation or pressure gradient that is clinically well tolerated.
Conclusions: Ross reversal can be performed with low morbidity and good pulmonary valve function in the majority of patients. This may provide some reassurance to patients considering the Ross operation, and weakens the argument that the Ross procedure transforms a single-valve disease into a two-valve disease.
95. Surgical Unroofing of Hemodynamically Significant Myocardial Bridges in a Pediatric Population
Katsuhide Maeda, Daniel J. Murphy, Ingela Schnittger, Jennifer A. Tremmel, *Frank L. Hanley, Robert Scott Mitchell, Ian S. Rogers
Stanford University, Stanford, CA
Objective: Although myocardial bridges are generally regarded as incidental findings, it has been reported that adults patients with symptomatic myocardial bridges refractory to medical therapy benefit from surgical unroofing. However, there is very limited data in the pediatric literature regarding the indication for surgery, the optimal surgical procedure, and the postoperative outcomes. The aim of our study was to evaluate the indications for unroofing and the surgical outcomes in our pediatric patients.
Methods: We retrospectively reviewed all myocardial bridge patients in our institution who underwent surgical relief of myocardial bridges. Baseline clinical characteristics, relevant diagnostic data (stress echocardiography, computed tomography angiography, intravascular ultrasound, and invasive hemodynamic assessment with dobutamine for measurement of diastolic fractional flow reserve), intraoperative findings, and post-operative outcomes were evaluated.
Results: Between Sep 2012 and Sep 2016, 12 pediatric patients (7 male, 5 female; average age 15.8 years, range 11-20 years; body weight; 61.2±11.2 kg) underwent surgical unroofing of LAD myocardial bridges. Eleven of the 12 patients had chest pain refractory to medical therapy. Two of the 11 experienced prior syncope, 1 of which had inducible VF on EP study. One of the 12 patients was asymptomatic until experiencing aborted sudden cardiac arrest during an athletic event. One patient was previously treated for Kawasaki disease, though was without sequela. One patient had previously undergone surgical repair of subaortic membrane and aortic coarctation. Eleven of the 12 patients underwent exercise stress echo, all of which had mid septal dyssynergy on stress images suggestive of a myocardial bridge. Coronary CT confirmed the anatomic presence of myocardial bridges in all patients. IVUS confirmed the length of myocardial bridges: 29.3±16.5 mm, halo thickness: 0.6±0.3 mm, compression at resting heart rate: 30.8±10.8%, and number of jailed septal branches: 2.6±1.7. Invasive hemodynamic assessment with dobutamine confirmed the physiologic significance of the bridges with diastolic fractional flow reserve at peak heart rate: 0.6 ± 0.1. Myocardial bridge unroofing was performed under cardiopulmonary bypass in the initial 9 cases and without the use of bypass in the subsequent 3 cases. All patients were discharged without major complications. The 11 patients with chest pain reported resolution of symptoms on follow up. The patient with inducible VF was no longer inducible on repeat post-operative EP study.
Conclusions: Surgical unroofing of myocardial bridges can be safely performed in pediatric patients, with or without the use of cardiopulmonary bypass. In symptomatic patients in whom physiologic significance of a bridge is established, unroofing can provide relief of chest pain refractory to medical therapy.
96. Intraoperative Completion Angiogram may be Superior to Transesophageal Echocardiogram for Detection of Pulmonary Artery Residual Lesions in Congenital Heart Surgery
Luke Lamers, Erick Jimenez, Catherine Allen, Derreck Hoyme, Entela B. Lushaj, Petros V. Anagnostopoulos
University of Wisconsin, Madison, WI
Objective: Intraoperative residual pulmonary artery (PA) stenosis is difficult to diagnose with trans-esophageal echocardiography (TEE). We hypothesized that intraoperative completion angiogram is superior to TEE for detection of residual PA lesions.
Methods: All patients who had surgery involving the PAs in a hybrid suite over a 2-year period had postoperative TEE and completion angiograms that were reviewed retrospectively. Post-operative TEE imaging was interpreted by 2 physicians blinded to surgical and completion angiography results. TEE imaging results were categorized as adequate repair, inadequate requiring revision or unable to assess. TEE data was compared to results of completion angiography obtained immediately post-repair and to operative notes to determine the ability of each modality to reliably detect significant residual lesions.
Results: Nineteen patients, [median age 5 months (range 0-17) and weight 6 kg (2.7-10.8)], had TEE and completion angiography following PA surgery. Diagnosis included single ventricle variants (n=13), Tetralogy of Fallot variants (n=4), corrected transposition (n=1) and multiple ventricular septal defects (n=1). Surgeries included: Glenn operation (n=8), PA reconstructions (n=4), main PA bands (n=4) and bilateral PA bands (n=3). 50% of TEE imaging of the surgical repair was graded as inadequate. Based on TEE results surgical revision was indicated in 2/19 patients. Completion angiography documented residual PA stenosis prompting surgical revision in 5/19 patients (26%). Four patients required left and 2 right PA stenosis revision. An additional main PA band was tightened following hemodynamic and angiographic assessment due to increased PA pressures and excessive pulsatility into a concomitant Glenn anastomosis. No patient with PA abnormalities had hemodynamic instability or excessive desaturations coming off bypass. One Glenn patient with adequate image quality and repair by TEE, was found to have moderate left PA stenosis by angiography (Figure 1). All other discrepancies occurred in patients with inadequate TEE imaging of the surgical repair. Intraoperative angiography related complications included atrial tachycardia (n=2) and transient complete heart block (n=1).
Conclusions: Completion angiography may be more effective in detecting post-operative PA stenosis compared to TEE even in patients who do not manifest clinical symptoms. Documentation of PA stenosis with completion angiography is low risk and allows immediate surgical revision potentially limiting necessity for future PA interventions.
Congenital Masters of Surgery Video Session
Wednesday, May 3, 2017
9:45 am – 11:30 am
Moderators: *Jonathan M. Chen and *Robert D. Jaquiss
*Frank L. Hanley, Stanford University
*James S. Tweddell, Cincinnati Children’s Hospital
*Pedro J. del Nido, Boston Children’s Hospital
Valve Sparing Aortic Root Repair in Children
*Duke E. Cameron, Massachusetts General Hospital
*Jose Da Silva, Children’s Hospital of Pittsburgh
*Richard G. Ohye, University of Michigan