April 25-29th, 2015

Congenital Heart Disease Skills Dealing with Challenging Conditions – Pearls and Pitfalls

Saturday, April 25, 2015 | 8:00 AM – 3:30 PM

PA/VSD/MAPCAs – Technique for Early Complete Repair

*Frank L. Hanley, Stanford University

Neonatal Pulmonary Artery Rehab without Unifocalization

*Yves d’Udekem, Royal Children’s Hospital

Panel Discussion with Case Presentations

• Erle H. Austin, III, University of Louisville
• Yves d’Udekem, Royal Children’s Hospital
• Frank L. Hanley, Stanford University

 The Borderline Left Ventricle: What is it? Recruit it or Forget it?

*James S. Tweddell, Medical College of Wisconsin

Techniques for Recruiting the Borderline Left Ventricle

Sitaram Emani, Boston Children’s Hospital

 Where to Draw the Line

*Thomas L. Spray, Children’s Hospital of Philadelphia

Modified Truncus Arteriosus

George Alfieris, University of Rochester

Repairing/Replacing the Insufficient Truncal Valve

Mark Gerard Hazekamp, Leiden University

Repair of Truncus Arteriosus with Interrupted Aortic

*Ralph S. Mosca, NYU Langone Medical Center

Mechanical Support in the Pediatric Population

Holger Buchholz, University of Alberta

ECMO: Decision Making, Preferred Components, Techniques

Peter D. Wearden, Children’s Hospital of Pittsburgh

Implantation of the Berlin Heart

Michael Hübler, University of Zurich

Implantation of the Heartware Device

*Ivan M. Rebeyka, University of Alberta

3 Dimensional Imaging of the Complex Congenital Heart

Ajit Yoganathan, Georgia Institute of Technology

Impact of a 3D Model on Surgical Planning

*Glen Van Arsdell, Hospital for Sick Children

AATS/STS Congenital Heart Disease Symposium – Unsettled and Unanswered Questions in Congenital Heart Surgery

Sunday, April 26, 2015 | 8:00 AM – 5:00 PM

Course Chair: *Robert Jaquiss, Duke University Course Co-Chair:

*Victor Tsang, Great Ormond Street Hospital

Controversies & Debates

Moderators:

• Robert D. Jaquiss, Duke University
• Thomas L. Spray, Children’s Hospital of Philadelphia

Debate: Neonatal Aortic Stenosis Is a Surgical Disease

Pro: Viktor Hraska, German Pediatric Heart Centre

Con: Lee Benson, Hospital for Sick Children Toronto

Debate: Anomalous Aortic Origin of a Coronary Artery is Always a Surgical Disease

Pro: *Pascal R. Vouhe, L’Université Paris Descartes

Con: *Ralph S. Mosca, NYU Lagone Medical Center

Debate: Larger Centers Produce Better Outcomes In Pediatric Cardiac Surgery – Regionalization Is a Superior Model

Pro: *Charles D. Fraser, Texas Children’s Hospital

Con:Mark Danton, Royal Hospital for Sick Children Glasgow

Getting Out of Trouble – Rescue – Tough Case Management

Moderator: *Thomas L. Spray, Children’s Hospital of Philadelphia

Expert Panelists:

• *Scott M. Bradley, Medical University of South Carolina
• *Charles D. Fraser, Texas Children’s Hospital
• Viktor Hraska, German Pediatric Heart Centre

Introduction of Format & Panelists *Victor T. Tsang, Great Ormond Street Hospital

• Case One *James S. Tweddell, Medical College of Wisconsin
• Case Two *Kirk R. Kanter, Emory University
• Case Three *Christopher A. Caldarone, University of Toronto

Legends Luncheon

*Marc R. de Leval, International Congenital Cardiac Centre, London

The Single Ventricle & Its Problems

Moderators: *Fred A. Crawford, Jr., Medical University of South Carolina *Robert D. Jaquiss, Duke University

Lessons learned from the SVR Trial: Update at Three Years

*Richard G. Ohye, University of Michigan

The Relentless Effects of Fontan Paradox

Jack Rychik, Children’s Hospital of Philadelphia

Transplantation for Fontan: Better than People Think

*Kirk R. Kanter, Emory University

Is “Four-stage Management” the Future for Univentricular Hearts? – Destination Therapy in the Young

*Robert D. Jaquiss, Duke University

Outflow Valve Surgery in Children & Adults with Congenital Heart: Part I: Pulmonary & Aortic Valve Surgery

Moderators: *Robert D. Jaquiss, Duke University *Victor T. Tsang, Great Ormond Street Hospital

Preservation of the Pulmonary Valve (Not Just the Annulus) in Repair of Tetralogy of Fallot

*Giovanni Stellin, University of Padova

Mechanical Pulmonary Valve Replacement

*Joseph A. Dearani, Mayo Clinic

Are Stented Bioprostheses Appropriate for Aortic Valve Replacement in Young Patients

*Frank A. Pigula, Boston Children’s Hospital

Transcatheter/Hybrid Aortic Valves in the Young

Mirko Doss, Kerckhoff Heartcenter

Outflow Valve Surgery in Children & Adults with Congenital Heart: Part II: Aortic Valve Replacement

Moderators: *Pedro J. del Nido, Boston Children’s Hospital

*Victor T. Tsang, Great Ormond Street Hospital

Reinforced Ross Procedure

*Thierry-Pierre Carrel, University of Bern

When & How to Enlarge the Aortic Root

*Victor T. Tsang, Great Ormond Street Hospital

Mechanical AVR in the Young: Lower Anticoagulation – Pregnancy – Novel Anticoagulants

*Hartzell V. Schaff, Mayo Clinic

Aortic Valve Repair in Teenagers & Young Adults: When to Do It & When Not to Do It

*Pedro J. del Nido, Boston Children’s Hospital

Monday Afternoon, April 27, 2015

Congenital Heart Disease – Simultaneous Scientific Session

8 minute presentation, 12 minute discussion

Moderators: *Carl L. Backer and *Pirooz Eghtesady

13. Outcomes Following Simple and Complex (Damus-Kaye-Stansel Takedown) Ross Operations in 58 Consecutive Pediatric Patients

Alejandra Bueno, David Zurakowski, Suyog A. Mokashi, Vijayakumar Raju, Michele J. Borisuk, *Pedro J. del Nido, Gerald R. Marx, Christopher W. Baird Boston Children’s Hospital & Harvard Medical School, Boston, MA

Invited Discussant: *Jonathan M. Chen

INTRODUCTION: The Ross operation has acceptable outcomes in selected pediatric patients with a biventricular circulation. However, there are little data concerning outcomes of the Ross operation in patients with uni-ventricular circulation undergoing biventricular conversion. The purpose of this study was to review the outcomes before and after Ross operations, including patients undergoing biventricular conversion with Damus-Kaye-Stansel (DKS) takedown.

METHODS: A retrospective review was performed on 58 consecutive patients who underwent simple and complex Ross operations at a single institution from March 2000 to March 2014, including eight patients who underwent biventricular conversion with Ross operation and DKS takedown ± reversal of cavopulmonary connection (complex). Clinical data were collected from medical records and a single reviewer independently measured pre-operative, discharge and follow-up indexed echocardiographic data. Outcome variables included mortality, surgical or catheter-based re-interventions and echocardiographic measurements of pulmonary valve (PV) annulus, aortic root (AoR), ascending aorta (AA) and aortic regurgitation (AR) vena contracta. Statistical analysis included t-tests, repeated-measures ANOVA and Kaplan-Meier curves with 95% confidence intervals according to Greenwood’s formula.

RESULTS: The cohort of 58 patients (44 males) had a median age of 4.5 years (IQR: 1–12.7 years) and median weight of 16.4 kg (IQR: 7.6–42.7 kg). Median follow-up was 2.5 yrs (IQR: 8 months to 4.5 years). Simple and complex patients having DKS takedown and biventricular conversion had similar results for all outcome variables. In the simple group, there were two deaths (both within 12 months), two late neo-aortic valve-sparring root replacements and 10 right ventricular outflow tract (RVOT) re-interventions. In the complex group, there was one death; no neo-aortic valve or AoR re-interventions and two had RVOT re-interventions (see Figure). In both groups, there were no differences between pre- and late follow-up AoR and AA dimensions and no correlations were found between pre-operative PV annulus sizes (p = 0.19), late AR (p = 0.25), late AoR (p = 0.26) or AA z-scores (p = 0.91). Additionally, there were no differences between simple and complex groups in the distribution of late AR categories (none, mild, moderate, severe) (p = 0.54, chi-square test). 83% of all patients had either no AR (20%) or mild AR (63%) and 17% (8 simple, 1 complex) had moderate or severe AR.

CONCLUSIONS: The Ross procedure has good similar short-term results in simple and complex patients and should be considered in those undergoing Ross operation with biventricular conversion and DKS takedown. Moreover, native PV size should not be a contraindication for Ross procedure.

14. Ultra Long Term Outcomes in Adult Survivors of Tetralogy of Fallot and the Effect of Pulmonary Valve Replacement

Richard Dobson(1) , Mark Danton(1) , Nicola Walker(1) , Nikolaos Tzemos(2) , Hamish Walker(1)

1 Scottish Adult Congenital Cardiac Service, Glasgow, United Kingdom; 2 University of Glasgow, Glasgow, United Kingdom

Invited Discussant: *Bahaaldin Alsoufi

OBJECTIVE: To define the long-term outcomes of adult survivors of surgical repair of tetralogy of Fallot with respect to, 1.) survival, functional capacity and adverse events, and, 2.) the effect of pulmonary valve replacement (PVR) on clinical and functional outcome.

METHODS: Retrospective cohort analysis of 376 adult survivors (16 years or greater) of repaired tetralogy of Fallot (age at repair 5.3 ± 7.3 years) enrolled through a national adult congenital cardiac database. Male:female 59:41, and mean age at last follow up was 34.3 ± 12.4 years. 6,838 patient-years were available for analysis.

RESULTS: At 40-year follow-up from date of repair, freedom from death was 83%, atrial arrhythmia 60%, ventricular arrhythmia 73%, pacemaker/ICD insertion 70%, and surgical/percutaneous reintervention 27%. In a multivariate model the risk of death was increased by older age at repair (HR, 1.11; p = 0.006), atrial arrhythmia by older age at repair (HR, 1.10; p ≤ 0.001), device insertion by the presence of a genetic syndrome (HR, 4.75; p = 0.001), and reintervention by any non-classical form of tetralogy of Fallot (HR, 2.66; p = 0.003) and transannular patch repair (HR, 1.71; p = 0.002). 330 (86.9%) patients were NYHA class 1. There was no difference in the distribution of social deprivation scores compared to the general population (p = 0.51). 29/154 female patients (19%) had one or more successful pregnancy. Mean QRS duration was 147 ± 25 ms. Each ms increase raised the risk of all-cause mortality in a univariate model (HR, 1.07; p = 0.002). There were 130 (34.7%) patients with moderate or greater pulmonary regurgitation (PR) at latest follow-up, with mean PR fraction 25 ± 17%. 181 patients (48%) underwent cardiac MRI with mean RV EDVi 126 ± 35 ml/ m2 , RV ESVi 68 ± 27 ml/m2 , and RV ejection fraction 47 ± 9%. 169 (45%) patients had a cardiopulmonary exercise test. Mean VO2 max was 23 ± 7.4 ml/kg/m2 . This correlated with RV ejection fraction (p = 0.002), but not RV EDVi (p = 0.197) or PR fraction (p = 0.244). 163 (43%) patients underwent repeat intervention. 150 PVRs were performed in 138 (37%) patients. Of these 18 were implanted percutaneously. Other procedures included 24 balloon pulmonary angioplasties, 15 redo repairs, 9 RVOT reconstructions, 8 tricuspid valve repair/replacements, 1 Bentall and 1 aortic valve replacement.

PVR was not shown to confer a survival benefit in patients with moderate or greater pulmonary regurgitation (p = 0.925) (see Figure). However, following PVR a significant reduction in RV volumes was observed (mean RV EDVi 156ml/m2 vs. 110 ml/m2 ; p = 0.001) and the VE/VCO2 slope improved (34.9 vs. 32.3; p = 0.046)

CONCLUSIONS: In adult patients with repaired tetralogy of Fallot long-term survival remains excellent. However morbidity, arrhythmias and repeat intervention are frequent. Pulmonary valve replacement did not improve survival but improved exercise efficiency and normalised RV volume parameters.

15. Long-Term Mortality After the Fontan Operation: Twenty Years of Experience at a Single Center

Tacy E. Downing(1) , Kiona Y. Allen(1) , Andrew C. Glatz(1) , Lindsay S. Rogers(2) , Chitra Ravishankar(1) , Jack Rychik(1) , Stephanie Fuller(1) , Lisa M. Montenegro(1) , James M. Steven(1) , *Thomas L. Spray(1) , Susan C. Nicolson(1) , *J. William Gaynor(1) , David J. Goldberg(1)

1 The Children’s Hospital of Philadelphia, Philadelphia, PA; 2 Cincinnati Children’s Hospital Medical Center, Cincinnati, OH

Invited Discussant: *James K. Kirklin

OBJECTIVE: To describe short and long-term mortality outcomes and identify risk factors in a modern cohort of patients with single ventricle physiology palliated with the Fontan operation.

METHODS: All patients who underwent a first Fontan operation at a single institution between 1992 and 2009 were retrospectively reviewed. Demographic and clinical variables were abstracted from the medical record. For patients lost to follow-up, vital status was ascertained using the National Center for Health Statistics’ National Death Index. Multivariate logistic regression was used to identify covariates independently associated with early mortality (<1 year post-Fontan), and Cox regression was used to analyze late mortality. Because pre-Fontan catheterization data were available in only 79% of patients, subset analyses were performed to include hemodynamic parameters.

RESULTS: A total of 774 patients met inclusion criteria, with a median age at Fontan of 2.3 years (range: 0.9–38.4). The most common anatomic diagnosis was hypoplastic left heart syndrome (381, 50%), and 89 (12%) had a common atrioventricular valve (AVV). Fontan type was divided nearly equally between lateral tunnel (409) and extracardiac (365), and 90% (693) were fenestrated.

Vital status on or after 1/1/2012 was ascertained for 768 or 99.2% of patients, representing 7,548 patient-years of follow-up and a median follow-up of 8.8 years. 76 patients died during the study period, 37 within one year of Fontan. At 20 years post-Fontan, the Kaplan-Meier survival estimate was 77% (95% CI [65–86%]) for the entire cohort (see Figure). For patients surviving to one year, the conditional survival estimate was 81% (95% CI [68–90%]) at 20 years.

Multivariate regression results are summarized in the figure that follows. Fontan prior to 1996, post-operative pleural drainage >14 days, and longer aortic cross-clamp time were all independently associated with early mortality. Distinct risk factors for late mortality included post-Fontan ICU stay >1 week, common AV valve, and presence of ≥ mild AV valve regurgitation prior to Fontan. In a subset analysis of the 609 patients with pre-Fontan hemodynamic data, pulmonary artery pressure >15 mm Hg was independently associated with both early (OR, 6.8; 95% CI [2.9–16]; p < 0.001), and late mortality (HR, 3.1; 95% CI [1.3–7.3]; p < 0.009). Hypoplastic left heart syndrome, morphology of systemic ventricle, Fontan type and presence of fenestration were not independently associated with either early or late mortality.

CONCLUSIONS: In our single center experience, the 20-year survival estimate following Fontan operation is 77%. Risk factors for early and late post-Fontan mortality differ. Prolonged post-operative pleural drainage was associated with early mortality, but did not predict late mortality if patients survived to one year. Elevated pre-Fontan pulmonary artery pressure was associated with early and late mortality.

16. Long-Term Outcomes After First-Onset Arrhythmia in Fontan Physiology

Thomas A. Carins(1) , Ajay J. Iyengar(1) , Ashley Nisbet(2) , Victoria Forsdick(3) , Tom Gentles(4) , Dorothy Radford(5) , Robert Justo(6) , David S. Celermajer(7) , Andrew Bullock(8) , David Winlaw(9) , Gavin Wheaton(10), Leeanne Grigg(2) , Yves d’Udekem(11)

1 The University of Melbourne, Parkville, VIC, Australia; 2 The Royal Melbourne Hospital, Parkville, VIC, Australia; 3 The University of Notre Dame, Darlinghurst, Sydney, Australia; 4 Starship Children’s Hospital, Auckland, New Zealand; 5 The Prince Charles Hospital, Chermside, Brisbane, QLD, Australia; 6 Mater Children’s Hospital, Brisbane, QLD, Australia; 7 Royal Prince Alfred Hospital, Sydney, Australia; 8 Princess Margaret Hospital for Children, Subiaco, WA, Australia; 9 Children’s Hospital at Westmead, Sydney, NSW, Australia; 10 Women’s & Children’s Hospital, Adelaide, SA, Australia; 11The Royal Children’s Hospital, Parkville, VIC, Australia

OBJECTIVES: To determine long-term outcomes of patients with a Fontan circulation after first onset of arrhythmias.

METHODS: Out of the 1336 patients of Australia and New Zealand who have undergone a Fontan operation, 213 were identified to have tachyarrhythmias (176) and bradyarrhythmias (80; 72 pacemakers): 111 atrio-pulmonary connections, 49 lateral tunnel and 53 extra-cardiac conduits.

RESULTS: Twenty-three years freedom from tachyarrhythmias and bradyarrhythmias in the entire population were respectively 62% (95% CI [54–69]) and 86% (95% CI [82–90]). Detailed description of the time-course of the tachyarrhthmias was available for 172 patients. A single episode was observed in 27 (16%) of patients with no recurrence after a mean of 11.4 ± 2.5 years. Cardioversion was used in 51% (90/177) and radiofrequency ablation in 33% (58/177).

After a mean of 6.9 ± 1.3 years after a first onset of tachy- or bradyarrhythmias, 43 patients died; 12 underwent a heart transplantation and 43 a Fontan conversion; 13 had protein losing enteropathy; 20 had a thromboembolism and 3 were in NYHA class III/IV. Twenty years survival after a first onset of tachy- and brady-arrhythmias were respectively 71% (95% CI [60–80]) and 81% (95% CI [68–89]). Their 5-, 10-, and 20-year freedom from all adverse events after tachy- and brady-arrhythmias were respectively 78% (95% CI [70–84]), 60% (95% CI [51–68]), 32% (95% CI [21–43]), 80% (95% CI [67–88]), 62% (95% CI [47–73]), and 45% (95% CI [30–59]). No differences in outcomes were noted between the 3 forms of Fontan. Five years after cardioversion and radiofrequency ablation, freedom from all adverse events were respectively 70% (95% CI [58–80]) and 74% (95% CI [56–86]). During follow-up, 86 patients were noted by echocardiography to undergo a progressive deterioration of their systolic function. Ten years freedom from decreased systolic function were 55% (95% CI [45–65]) after tachyarrhythmias and 60% (95% CI [46– 72]) after bradyarrhythmias (see Figure). Freedom from all adverse events and decreased ventricular function after 5, 10, and 20 years were respectively for tachy- and bradyarrhythmias 64% (95% CI [56–72]), 43% (95% CI [35–52]), 14% (95% CI [6–25]) and 59% (95% CI [46–71]), 41% (95% CI [28–54]), 18% (95% CI [8–32]).

CONCLUSION: After a first onset of arrhythmia, patients who have undergone a Fontan procedure see a progressive deterioration of their ventricular function. Current conservative treatment with medications, cardioversion and ablation therapies provides symptomatic relief to close to three quarters of the patients for five years.

17. The Impact of Non-Cardiac and Genetic Abnormalities on Outcomes Following Neonatal Congenital Heart Surgery

*Bahaaldin Alsoufi , Shriprasad Deshpande, William Mahle, Scott Gillespie, *Brian Kogon, Kevin Maher, *Kirk Kanter Emory University, Atlanta, GA

Invited Discussant: *Peter J. Gruber

OBJECTIVE: Significant non-cardiac and genetic abnormalities (NC/GA) are common in neonates with congenital heart defects. We sought to examine the current era effect of those abnormalities on early and late outcomes following cardiac surgery.

METHODS: From 2002–12, 1538 neonates ≤ 30 days old underwent repair (n = 859, 56%) or palliation (n = 677, 44%) of congenital heart defects. Regression models examined the effect of NC/GA on operative results, resource utilization and late outcomes.

RESULTS: Neonates with NC/GA (n = 312, 20.3%) had higher incidence of prematurity (21.4% vs. 12.5%; p < 0.001) and low weight ≤2.5 Kg (24.4% vs. 12.3%; p < 0.001) than neonates without NC/GA (n = 1,226, 79.7%). Although the incidence of single ventricle was comparable (33.7% vs. 31.0%; p = 0.37), neonates with NC/GA underwent more palliation (52.2% vs. 42.0%; p = 0.001) and subsequently had higher percentage of STAT risk category 4 & 5 procedures (78.2% vs. 66.2%; p < 0.001).

Adjusted logistic regression models that included the disparate patient and operative variables showed that the presence of NC/GA was associated with increased unplanned reoperation (OR, 1.7; 95% CI [1.1–2.7]); p = 0.03) and hospital mortality (OR, 2.2; 95% CI [1.3–3.6]; p = 0.002). NC/GA neonates had longer mean mechanical ventilation requirement (6.3 vs. 4.0 days; p < 0.001), intensive care unit stay (10.5 vs. 6.3 days; p < 0.001), and hospital stay (18.3 vs. 10.8 days; p < 0.001). Adjusted linear regression models continued to show significant association between NC/GA and increased post-operative mechanical ventilation duration, intensive care unit and hospital stays (p < 0.001 each).

Adjusted hazard analysis showed that the presence of NC/GA was associated with diminished late survival (HR, 2.4; 95% CI [1.9–3.1]; p < 0.001) and that was evident in all subgroups of patients (p < 0.001 each, as shown in the figure).

CONCLUSIONS: Neonates with NC/GA commonly have associated risk factors for morbidity and mortality such as prematurity and low weight. After adjusting for those factors, the presence of NC/GA continues to have significant association with increased unplanned reoperation, early mortality risk and resource utilization after palliative and corrective cardiac surgery. Importantly, the hazard of death in those patients continues beyond the peri-operative period for at least one year. Our findings show that the presence of NC/ GA should be emphasized during parent counseling and decision making; and underscore the need to explore strategies to improve outcomes for this high risk population that must address peri-operative care, outpatient surveillance and management.

18. Health-Related Quality of Life in Adult Survivors After the Fontan Operation

*James K. Kirklin University of Alabama at Birmingham, Birmingham, AL

Invited Discussant: *Charles B. Huddleston

OBJECTIVE: There is a growing population of survivors of single ventricle congenital anomalies after these children undergo staged reconstructive Fontan procedures, but limited information is available about the quality of life (QOL) among adult Fontan survivors. The objective of this cross sectional study was to assess the QOL outcomes on long term Fontan survivors and compare them with the general population and with heart transplant recipients.

METHODS: Quality of life was ascertained using the Short Form-36 (SF-36) survey on adult Fontan survivors (19 years or older), as well as pediatric heart transplant recipients who were adults at the time of the survey. The eight components of health related QOL were determined and compared to United States Norms. Follow-up was based on QOL survey completion date of 7/15/14.

RESULTS: Sixty surviving patients, currently 19 years or older, had undergone Fontan operations at UAB between January 1988 and December 2011. The study group consisted of 49 Fontan patients who responded to the QOL survey (82% response rate); 13 Heart transplant survivors met the eligibility criteria for comparison (QOL survey response rate 100%). Duration of follow-up for the Fontan study group averaged 18 years (range: 3–24 years) and for the heart transplant patients 13 years (range: 3–23 years). The overall QOL scores among responders in regard to physical functioning, general health, vitality, social functioning, and emotional/mental health of Fontan population were comparable with that of healthy United States population. The Fontan group had a trend towards diminished role-physical health (p = 0.02), while bodily pains among Fontan patients were significantly higher as compared to normative data (p < 0.01). There were no significant difference in any of the 8 subjective measures of health related QOL between Fontan and heart transplant recipients.

CONCLUSIONS: Surviving patients after a Fontan procedure for treatment of single ventricle enjoy, on average for nearly 2 decades, functional and health-related QOL (6 of 8 domains) outcomes that are similar to the general population and heart transplant recipients. This generally favorable extended life-satisfaction period (average of 18 years) should be considered when informing patients and families of expectations with the Fontan pathway versus certain higher risk procedures when both the Fontan and 2-ventricle options are considered.

19. Aortic Dilatation Is Not Associated with Aortic Valvulopathy in Patients with Bicuspid Aortic Valve

Byron K. Yip, Colleen Clennon, Adin-Cristian Andrei, S. Chris Malaisrie

Northwestern University, Chicago, IL

Invited Discussant: *Duke E. Cameron

OBJECTIVE: Bicuspid aortic valve (BAV) is associated with a markedly increased risk for aortic dilatation leading to aneurysm and dissection. The aim of this study is to determine the influence of aortic valvulopathy on ascending aortic dimensions measured using cardiac magnetic resonance imaging (cMRI) in a cohort of BAV patients at our institution.

METHODS: From October 2003 to November 2013, 732 BAV patients received care at our institution. In an IRB-approved retrospective chart review, 375 (mean age 47 ± 14 years, male gender 69%) were identified as having undergone cMRI with evaluation of the aorta performed at our institution. No patients in this group had a history of intervention involving the aortic valve or aorta at the time of earliest cMRI from which dimensions were obtained nor any concomitant connective tissue or other congenital heart disease. Distributions of ascending aortic diameter (aAoD) were compared between patients with aortic stenosis (AS) (n = 163) and without AS (n = 212); with aortic insufficiency (AI) (n = 263) and without AI (n = 112); and with AS or AI (AS/AI) (n = 316) and without AS/AI (n = 59). Aortic valvulopathy (AoV), or AS and AI, were defined as present if indicated as mild or greater in severity.

RESULTS: Mean aAoD in this BAV cohort was 3.9 ± 0.6 cm (median 4.0 cm; range: 2.4–6.3 cm) in those with AoV and 3.8 ± 0.6 cm (median 3.7 cm; range: 2.3–4.8 cm) in those without AoV. Since aAoD was not normally distributed in this BAV cohort, non-parametric group comparisons using Wilcoxon’s rank-sum test were performed. There were no significant differences between groups by AS, AI or AS/AI (Figure 1A). The box plots presented reveal no associative relationship between aAoD and AS or AI severity grading (Figure 1B).

CONCLUSIONS: No association was found between degree of aortic dilatation and degree of aortic stenosis or insufficiency in this BAV cohort. Thus, the development of BAV-associated valvulopathy may not affect the progression of aortopathy. Similarities in mean, median, and standard deviation values across the groups without AoV, however, may inform future studies aimed at developing novel quantitative tools to enhance and standardize early clinical decision making for BAV patients.

Congenital Heart Disease Moderated Poster Competition

Moderator: *Jennifer C. Hirsch-Romano

P13. Outcomes of Heart Transplantation in Children with Hypoplastic Left Heart Syndrome Previously Palliated with the Norwood Procedure

*Bahaaldin Alsoufi(1) , William Mahle(1) , Cedric Manlhiot(2) , Shriprasad Deshpande(1) , *Brian Kogon(1) , Brian McCrindle(2) , *Kirk Kanter(1)

1 Emory University, Atlanta, GA; 2 University of Toronto, Toronto, ON, Canada

OBJECTIVE: Following the Norwood operation, unfavorable hemodynamic or anatomic factors might disqualify children with hypoplastic left heart syndrome (HLHS) from progressing through subsequent palliative surgeries or might cause late deterioration following Fontan completion; all necessitating enlistment to receive heart transplantation (HT). Those patients might have immune, clinical or anatomic risk factors that could preclude proper donor heart allocation, increase operative risk and diminish late survival following HT. Prior studies have shown that previously palliated HLHS patients had the worst outcomes following pediatric HT. We studied outcomes of HT in this patient cohort using the Pediatric Heart Transplant Study (PHTS) database.

METHODS: Between 1993 and 2013, 253 children with previously palliated HLHS were listed for HT. Competing risks analysis modeled events after listing (HT, death without HT), and after HT (death, retransplantation) and examined risk factors affecting outcomes.

RESULTS: Patients were listed at median age of 1.5 years (IQR, 0.4–4.2) and were in the following palliation stages: after Norwood (n = 89 [35%]), after Glenn (n = 96 [38%]), after Fontan (n = 68 [27%]). 188/253 (74%) were listed UNOS status I. Renal failure was present in 12 (5%). T-cell and B-cell panel reactive antibody (PRA) was >10% in 40% and 44%, respectively. Competing risks analysis showed that 2 years after listing, 23% of patients had died without HT, 74% had received HT, and 3% were alive without HT. [Figure a] Risk factors for death prior to HT were UNOS status 1 (HR, 4.3; p < 0.001), listing before Glenn (HR, 2.6; p < 0.001), younger age (HR, 2.8; p = 0.004), renal failure (HR, 3.0; p = 0.02) and mechanical circulatory support (HR, 2.0; p = 0.03). In the 188 HT recipients, competing risks analysis showed that 15 years following HT, 42% had died, 7% had received retransplantation, and 51% were alive without retransplantation. [Figure B] Risk factors for death following HT were black race (HR, 2.7; p = 0.02) and donor head trauma (HR, 1.8; p = 0.02). Following retransplantation, 3/8 (38%) died yielding an overall 15-year survival following HT of 54%

CONCLUSIONS: HT is an acceptable strategy to salvage previously palliated HLHS patients who fail to progress at different palliation stages. Long term results are encouraging and comparable to published reports on HT in children with other forms of congenital heart disease. Nonetheless, waiting list mortality is high and is more elevated in younger children who are listed soon after Norwood. Efforts to decrease this waiting list mortality could include early mechanical support prior to organ dysfunction, and ABO incompatible HT to increase donor pool. In patients who received HT, survival was not affected by last palliation stage or by the presence of elevated PRA, refl ecting advances in the current management strategies of sensitized patients undergoing HT.

P14. Burden of Potentially Pathologic Copy Number Variants Is Higher in Children with Isolated Congenital Heart Disease and Significantly Impairs Covariate-Adjusted Long-Term Survival

Daniel Seung Kim(1) , Jerry H. Kim(1) , Amber A. Burt(1) , David R. Crosslin(1) , Nancy Burnham(2) , Donna M. McDonald-McGinn(2) , Elaine H. Zackai(5) , Susan C. Nicolson(2) , *Thomas L. Spray(2) , Ian B. Stanaway(1) , Deborah A. Nickerson(1) , Patrick J. Heagerty(1) , Hakon Hakonarson(2) , Gail P. Jarvik(1) , J. William Gaynor(2)

1 University of Washington, Seattle, WA; 2 Children’s Hospital of Philadelphia, Philadelphia, PA

OBJECTIVE: Copy number variants (CNVs) are a common genomic polymorphism. Larger CNVs that duplicate or delete genes are rare, and potentially pathogenic. Prior work has found that large, gene-overlapping CNVs are enriched in children with congenital heart disease (CHD). In this work we sought to determine the frequency of large CNVs in children with isolated CHD and delineate the effect of these potentially pathogenic CNVs on longterm survival.

METHODS: This is an analysis of a previously described cohort of non-syndromic CHD patients who underwent cardiac surgery with cardiopulmonary bypass before 6 months of age (n = 419). Healthy pediatric controls (n = 500) were obtained from the Children’s Hospital of Philadelphia (CHOP) site of the electronic Medical Records and Genetic Epidemiology (eMERGE) study. Illumina 550 K or greater GWAS chips were typed and analyzed at Center for Applied Genomics at CHOP for both cases and controls. CNVs were discovered through the GWASTools and PennCNV algorithms and subsequently screened for >95% overlap between the two calling methods, size (>300 kb), quality score (>100), overlap with a gene, and novelty (not present in a known, non-pathologic database of CNVs). Survival curves, adjusting for confounding covariates identified through a priori analyses, were calculated using Cox Proportional Hazards modeling to evaluate the joint effect of CNV burden and covariates on long-term survival.

RESULTS: Compared to eMERGE healthy pediatric controls, children with isolated CHD had a significantly higher burden of potentially pathogenic CNVs (51/419 [12.2%] vs. 25/500 [5.0%]; p = 0.00015). Presence of a large, potentially pathogenic CNV was associated with significantly decreased long-term survival (HR = 2.23; p = 0.049) after confounder adjustment (e.g., CHD severity, total bypass duration) in children with isolated CHD after surgical palliation (see Figure).

P15. Intramural Coronary Arteries Are Not a Risk for Mortality in the Arterial Switch Operation

Tyson A. Fricke, Anne Eva Bulstra, Phillip S. Naimo, Yves d’Udekem, Christian P. Brizard, *Igor E. Konstantinov Royal Children’s Hospital, Melbourne, Australia

OBJECTIVE: Intramural coronary arteries (IMCAs) may complicate coronary artery transfer during the arterial switch operation (ASO). IMCAs are often considered a risk factor for mortality following ASO. We sought to determine the long-term outcomes of 29 patients with IMCAs who underwent an ASO at a single institution.

METHODS: All patients who had IMCAs and underwent an ASO were identified from the hospital database and retrospectively reviewed.

RESULTS: From 1983 to 2009, 675 patients underwent an ASO at our institution for transposition of great arteries (d-TGA, n = 618) and Taussig-Bing anomaly (TBA, n = 57). Twenty nine (29/675 [4.3%]) had IMCAs. Most patients (28/29 [97%]) had d-TGA with intact ventricular septum (n = 21) or ventricular septal defect (n = 7). One (3%, 1/29) patient had TBA. Intramural course involved the left main coronary artery in 27 (27/29 [93%]) patients, the left anterior descending artery in 1 (1/29 [3.5%]) patient and the right coronary artery in 1 (1/29 [3.5%]) patient. Median age at operation was 9 days (range: 2 days to 447 days) and median weight at operation was 3.5 kilograms (range: 2.6 kilograms to 10 kilograms). There were no early deaths. Follow-up was 100% complete for 27 local patients. Mean follow-up was 14.6 years (median 15.4 years; range: 4.5 years to 25 years). There were no late deaths. No patient required reoperation on the coronary arteries or transcatheter coronary angioplasty. Freedom from reoperation was 93% at 10 years. There were 8 reoperations in 4 patients that occurred at a mean 9 years after surgery (range: 1 day to 14 years). One patient had 2 reoperations for supra-aortic stenosis and aortic-to-pulmonary artery fistula, one patient had mitral valve repair, one patient had 4 reoperations due to left pulmonary artery stenosis and one patient had a Bentall procedure and aortic valve replacement with mechanical prosthesis. No patient had more than mild aortic regurgitation at last follow-up. Seven patients had coronary angiograms at median 21 months (range: 14 months to 17 years) after ASO. All patients were asymptomatic at the time of angiogram. One patient had mild stenosis of the circumflex coronary artery demonstrated on a routine coronary angiogram 14 months postoperatively. This patient remained asymptomatic with a myocardial perfusion scan negative for inducible ischemia 16 years postoperatively. All 27 patients were in New York Heart Association Class 1 at last follow-up.

CONCLUSION: Patients with IMCAs were not at increased risk of death or coronary re-interventions and have good late outcomes after the ASO.

P16. The Surgical Treatment of Atrial Isomerism: Single Center Experience of 353 Cases

*Hajime Ichikawa, Koji Kagisaki, *Toshikatsu Yagihara, Takaya Hoashi National Cerebral and Cardiovascular Center, Suita, Japan

OBJECTIVE: The surgical treatment of univentricular heart has improved by the successful treatment strategy to leads to single ventricular repair. However, the survival rate of the patients with atrial isomerism is inferior to that without. We have reviewed the 354 patients’ charts in our center retrospectively and analyzed the long term outcome.

METHODS: From 1978 to 2013, patients with right atrial isomerism (RAI; n = 246) and left atrial isomerism (LAI; n = 108) are treated surgically. Univentricular repair was indicated in 263 patients, whereas only 91 patients were indicated for biventricular repair. Among them, 244 patients tolerated first surgical procedure and survived.

RESULTS: Operative death was seen 65 out of 246 in RAI and 10 out of 108 in LAI at first surgery. The outcome is analyzed by dividing the decades from 1978 to 1989, 1990 to 1999, and 2000 to present. The operative mortality decreased by time (17%, 7%, and 2%, respectively). The late mortalities to present date are 13%, 7%, and 2%, respectively. The mean ages at the initial surgical procedure are 1.59 years, 0.94 years, and 0.22 years, respectively. The choice of systemic to pulmonary shunt as an initial palliative surgery was decreased by decades (50, 33, and 23 %, respectively). Contrarily, the choice of bidirectional Glenn procedure increased (2, 10, and 17%). The ratio of TCPC completion was improved by decades (23%, 38%, and 51%, respectively). The average oxygen saturation after the first palliation was higher in the earliest decades and lowest in the recent decades. The significant risk factor for operative and late death is the TAPVC repair as the first palliative procedure and a severe common AV valve regurgitation. With these two factors, there was no improvement in the survival among three decades. The survival rate 20 years after initial surgical procedure was 60 % and 36 % in left and right isomerism, respectively. The 10 year survival was improved by decades (40, 53, and 64% in 1980s, 1990s, and after 2000, respectively.

CONCLUSIONS: The outcome of the surgical treatment for atrial isomerism is still not satisfactory with high mortality. The low pulmonary blood flow strategy might improve the outcome possibly due to the prevention of high pulmonary vascular resistance and volume overload to the systemic ventricle to prevent AV valve regurgitation.

P17. The Impact of Age at Cavo-Pulmonary Shunt (Stage II) on Outcome After the Norwood Procedure. Importance of Elective Versus Non-Elective Intervention.

Intisar Ulhaq, Adrian Crucean, John Stickley, David Barron, Timothy Jones, Natasha Khan, William James Brawn Birmingham Children’s Hospital, Birmingham, United Kingdom

OBJECTIVE: The cavo-pulmonary shunt (CPS) is part of the staged strategy in univentricular heart palliation. Multiple studies have looked at the timing of this procedure highlighting the potential benefits of an early CPS especially in patients with hypoplastic left heart syndrome (HLHS). The experience with the CPS procedure in these particular patients is presented in this review.

METHODS: The study presents a retrospective analysis of 292 consecutive patients with dominant right ventricular morphology (HLHS and its variants) who have undergone CPS from 2002 to 2014. Variables analysed included age and weight at the time of CPS, atrio-ventricular(AV) valve function, ventricular function, interstage interventions, associated procedures, and the urgency of CPS (elective versus non-elective procedures based on clinical status and contemporary assessment of imaging data. End-points included length of hospital stay (LOS), freedom from death or transplantation, survival to the final stage of palliation (Fontan).

RESULTS: This is the largest series of its kind to date. The median patients’ age at the time of CPS was 155 days (IQR, 125–182) and weight was 6 kg (IQR, 5.3–6.7). There were 54 (18.5%) CPS performed under 4 months of age and 59 (20.2%) were non-elective. The follow-up was complete in all patients. The overall 30-day mortality post CPS was 2.4% and 154 patients have reached the final stage (III) of palliation to date. There were no significant differences in outcomes between patients undergoing CPS before or after the age of 4 months or whether patients underwent interstage procedures or not. There were significantly worse outcomes for the patients undergoing a non-elective procedure (30-day mortality 7.3% versus 1.3% in the elective group; p = 0.03; median LOS 15 versus 10 days in the elective group). At least moderate or worse AV valve regurgitation was an additional risk factor for mortality in the non-elective group (p = 0.01) but ventricular function was not.

CONCLUSIONS: Age at the time of stage II palliation did not affect outcome for patients with dominant right ventricular morphology. The CPS performed in non-elective settings yields worse outcomes irrespective of other variables that were traditionally found to be associated with poor prognosis. The function of the systemic AV valve only strengthens this association.

P18. More Than Repair the Valve – Effect of Cone Reconstruction on Right Ventricle Remodeling in Patients with Ebstein Anomaly: A CMR Study Qi An, Da Zhu, Xiao Li West China Hospital, Chengdu, China

OBJECTIVE: As a revolutionized procedure for Ebstein anomaly, cone reconstruction has yielded excellent results for valve repair. This study aims to determine the impact of cone reconstruction on right ventricle (RV) remodeling using cardiac magnetic resonance (CMR) technique.

METHODS: Patients with Ebstein anomaly were studied with CMR before and after cone reconstruction of tricuspid valve. Functional right ventricle (fRV) was traced manually on RV short axial planes, RV volume during end-systolic and end-diastolic phase as well as ejection fraction were than calculated. We also proposed the tricuspid annular movement synchronic index (TAMSI) to quantify the longitudinal movement pattern of tricuspid annuls during cardiac cycle. Three point at tricuspid annuls was chosen at four chamber and RV two chamber view of CMR indicating the anterior, posterior and septal leaflet, systolic excursion in reference to RV apex was then measured. TAMSI is defined as standard deviation of the systolic excursion divided by the average of the systolic excursion of at this three point.

RESULTS: During the study period, 17 patients were enrolled, with mean age 26 ± 12 years and mean follow-up time 6 ± 3 months. All patients survived without major complications except for one with a reoperation for valve repair. The atrialized right ventricle (aRV) was eliminated. The right ventricular enddiastolic volume was significantly reduced from 197.0 ± 64.3 ml/m2 to 154.7 ± 67.8 ml/m2 (p < 0.001; Figures A and B), with slight elevation of EF% after cone reconstruction (from 35.8% ± 8.9% to 38.2% ± 8.7%; p = 0.687). The TAMSI was reduced from 0.562 ± 0.152 to 0.233 ± 0.137 (p < 0.05; Figures C through F), which indicated more coordinated movement of tricuspid annuls and a positive effect of cone construction on RV remodeling.

CONCLUSIONS: Cone reconstruction significantly decreases the volume of right ventricle while improves the longitudinal movement synchronicity of right ventricle during cardiac cycle. This remodeling effect of cone reconstruction of right ventricle may benefit clinical outcome after cone reconstruction.

P19. Mild to Moderate Residual LVOTO May Stop or Reverse the Progression of Systemic Right Ventricle Remodeling Process in Adult CCTGA Patients After Physiological Repair

Fucheng Xiao, Jianping Xu, Hansong Sun Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China

OBJECTIVE: Left ventricular outflow tract obstruction (LVOTO) caused by pulmonary artery banding has been shown to retrain the sub-pulmonic left ventricle and to improve tricuspid regurgitation and morphological right ventricular dysfunction effectively in patients with congenitally corrected transposition of the great arteries (ccTGA). We aimed to evaluate the long-term effect of residual LVOTO in patients with ccTGA after physiological repair.

METHODS: Between January 2000 and August 2013, 47 consecutive adult ccTGA patients undergoing physiological repair were included. Residual LVOTO was defined as transpulmonary pressure gradient ≥15 mmHg. Patients were grouped into tricuspid valve replacement (TVR group) and non replacement group (NonTVR group). Based on the presence of residual LVOTO, those 2 groups were further divided into 4 subgroups, namely NonTVR+LVOTO subgroup (n = 13), NonTVR+No LVOTO subgroup (n = 5), TVR+LVOTO subgroup (n = 7) and TVR+No LVOTO subgroup (n = 22). Remodeling process of sub-aortic right ventricle after surgery, indicated by its ejection fraction (RVEF) and end diastolic diameter (RVEDD) were evaluated between postoperative and latest echocardiography in each subgroup. Late major adverse cardiac events (death, reintervention either surgical or percutaneous and recurrence of at least moderate tricuspid regurgitation or perivalvular leakage) were also analyzed.

RESULTS: There was 1 death and 7 surgically acquired complete heart blocks occurred during hospitalization. Mean echocardigrphic follow-up was 57.0 ± 39.4 months. In TVR+ LVOTO subgroup, RVEDD decreased significantly, from 52.8 ± 8.5 mm postoperatively to 50.5 ± 9.3 mm in latest echocardiography (P = 0.04), and RVEF increased dramatically from 51.3 ± 3.2% to 59.8 ± 7.1% (P = 0.01) whereas no significant improvement was observed in RVEDD and RVEF in TVR+No LVOTO subgroup (P = 0.62 and P = 0.09, respectively). In NonTVR group, RVEDD dilated progressively over time, from 45.2 ± 5.1 mm to 51.8 ± 7.0 mm in patients without LVOTO (P = 0.03) whereas it remained stable in patients with LVOTO (P = 0.82). However, RVEF did not differ significantly between postoperative and latest echocardiography in NonTVR group patients with or without LVOTO. The overall event free survival rate after physiological repair was 97.6%, 93.8%, 68.2%, and 45.5% at 12, 60, 120, and 143 months, respectively. In univariate analysis, residual LVOTO was associated with a trend toward being a significant protective factor against late adverse outcomes in NonTVR group but not in TVR group (OR = 0.15; P = 0.09 and OR = 3.82; P = 0.38, respectively).

CONCLUSIONS: Mild to moderate residual LVOTO may stop or even reverse the progression of right ventricular remodeling process and tend to decrease the long-term adverse outcomes in adult patients with ccTGA undergoing physiological repair.

P20. What Is the Procedure Impact on Ventricular Outflow Tract Hemodynamics and Reintervention in Patients with Complex Transposition? Mohammed K. Al-Jughiman, Maryam Al-Omair, Luc Mertens, *Christopher A. Caldarone, *Glen Van Arsdell Hospital for Sick Children, Toronto, ON, Canada

BACKGROUND: Over the past few decades, multiple surgical strategies have evolved in order to improve survival and ventricular outflow tract-related problems in patients with complex transposition and equivalents.

OBJECTIVE: We hypothesized that Nikaidoh procedure would produce better left ventricular outflow tract (LVOT) and right ventricular outflow tract (RVOT) hemodynamics and lower reintervention rates compared to the Rastelli and REV procedures.

METHOD: Over the past 32 years, 88 patients underwent repair with either Nikaidoh (n = 9 [10.2%]), Rastelli (n = 69 [78.4%]), or REV (n = 10 [11.4%]) ± atrial switch for either D-TGA (n = 54 [61.4%]), DORV (n = 12 [13.6%]), or CC-TGA (n = 22 [25%]). All had associated morphologic left ventricular outflow tract obstruction (LVOTO). Analysis was divided into eras; that is, first era 1982 to 1989 (n = 28 [31.8%]), second era 1990 to 1999 (n = 31 [35.2%]), and last era 2000 to 2013 (n = 29 [33%]).

RESULT: In the most recent era, freedom from LVOT reintervention at 10 years was 100%, 92.3%, and 100% after Nikaidoh, Rastelli, and REV respectively (P = 0.66). Freedom from RVOT reintervention at 10 years was 58.3%, 92.3%, and 66.7% after Nikaidoh, Rastelli, and REV respectively (P = 0.26). At 7 years, freedom from free PI was 41.7%, 53.2%, and 0.0% after Nikaidoh, Rastelli, and REV respectively (P = 0.014). In repeated measures analysis for the entire cohort (Figure 1A-B), Rastelli had a positive time relationship to increasing LVOT gradient with a slope of 0.47 (SE = 0.13; 95% CI [0.20–0.73]; P = 0.001), compared to –0.05 (SE = 0.07; 95% CI [–0.20–0.10]; P = 0.49) and 0.33 (SE = 0.24; 95% CI [–0.18–0.85]; P = 0.19) after Nikaidoh and REV respectively. There was no difference in LVOT mean gradient between groups; that is, 5.35, 11.50, and 7.17 in Nikaidoh, Rastelli, and REV, respectively (F = 2.26; p = 0.12). On RVOT, there was a positive time relationship to increasing gradient after Nikaidoh and Rastelli with a slope of 3.4 (SE = 1.09; 95% CI [0.96–5.83]; P = 0.01) and 0.89 (SE = 24; 95% CI [0.4–1.37]; P < 0.001) respectively, compared to 0.5 (SE = 0.69; 95% CI [–0.93–1.95]; P = 0.47) after REV. Mean RVOT gradient was also similar i.e. 28.79, 31.46, and 25.59 in Nikaidoh, Rastelli, and REV respectively (F = 1.05; P = 0.35). In the most recent era, freedom from mortality at 10 years was 88.9%, 93.8%, and 100% after Nikaidoh, Rastelli, and REV, respectively (P = 0.79).

CONCLUSION: The Nikaidoh yielded the most favorable LVOT hemodynamics. No advantage was demonstrated for survival, RVOT hemodynamics, or reintervention in the current era with midterm follow up. The REV produced the least RVOT obstruction/reintervention but at a cost of free pulmonary insufficiency.

P21. Increasing Complexity of Heart Transplantation in Patients with Congenital Heart Disease

William Y. Shi(1) , Pankaj Saxena(2) , Matthew S. Yong(1) , Silvana Marasco(2) , *David McGiffin(2) , Anne Shipp(1) , Robert G. Weintraub(1) , Yves d’Udekem(1) , Christian P. Brizard(1) , *Igor E. Konstantinov(1)

1 Royal Children’s Hospital, Parkville, Australia; 2 Alfred Hospital, Prahran, Australia

OBJECTIVES: Due to improved surgical results, there is a growing population of patients with repaired congenital heart disease (CHD) requiring heart transplantation.

METHODS: Retrospective review of outcomes of heart transplantation in patients with CHD (n = 77) was compared to age-matched patients without CHD (n = 442). Outcomes of early (1988 to 1999) and late (2000 to 2013) eras were compared.

RESULTS: From 1988 to 2013, 817 patients underwent orthotopic heart transplantation. Of these, 77 had CHD. There were 48 (62%) males and 29 (38%) females with a mean age of 18 ± 14 years (range: 16 days to 58 years). Most (70/77 [91%]) patients underwent a mean of 2.6 ± 1.3 (range: 1–6) cardiac operations prior to transplantation. Univentricular palliation was performed in 44 (57%) patients, 22 patients of whom had Fontan procedure. Seventeen (22%) patients required an additional procedure during transplantation (pulmonary artery reconstruction in 9, aorta arch replacement in 3, combined aortic and pulmonary reconstruction in 5). Hospital mortality was 13% (10/77) with primary graft failure being the cause of death in 7 patients. There were no difference in mean age (early: 15 ± 14 years vs. late: 21 ± 15 years; p = 0.06), number of prior cardiac procedures (early: 2.1 ± 1.4 vs. late: 2.7 ± 1.4; p = 0.09), waiting time to transplantation (early: 279 ± 449 days vs. late: 202 ± 163 days; p = 0.33), proportion univentricular palliations (early: 53%, 20/38 vs. late: 59%, 23/39; p = 0.65) or hospital mortality (early: 16%, 6/38 vs. late: 10%, 4/39; p = 0.52) in patients with CHD during 2 time periods. However, recipients with CHD in the later era had longer mean cardiopulmonary bypass (CPB) time (early: 190 ± 70 minutes vs. late: 271 ± 114 minutes; p < 0.001), donor ischaemic times (early: 222 ± 98 minutes vs. late: 276 ± 102 minutes; p = 0.02) and more often required postoperative ECMO (early: 8%, 3/38 vs. late: 72%, 28/39; p = 0.036). Patients (n = 442) without CHD (non-CHD) were used for comparison. The proportion of transplant recipients with CHD remained similar across the study period (early: 15% vs. late: 15%; p = 0.9). Univentricular physiology had no impact on survival. Recipients with CHD waited longer for transplantation (CHD: 240 ± 336 days vs. non-CHD: 152 ± 222; p = 0.028) and had longer mean CPB (CHD: 232 ± 103 minutes vs. non-CHD: 130 ± 52 minutes; p < 0.001) and cardiac ischaemia (CHD: 249 ± 103 minutes vs. non-CHD: 223 ± 92 minutes; p = 0.026) times, reflecting the need for complex intraoperative surgical preparation. CHD patients had greater 30-day mortality (CHD: 10%, 8/77 vs. non-CHD: 4%, 18/442; p = 0.04). However, long-term survival was similar in patients with and without CHD (see Figure). Survival adjusted for age and era was also similar.

CONCLUSIONS: Patients with CHD await heart transplantation longer, often require complex intraoperative surgical preparation and have higher early mortality. Long-term post-transplantation survival is similar in patients with and without CHD.

P22. Bicuspid Aortic Valve Morphology: Does the Pattern of Leaflet Fusion Determine the Success of the Ross Procedure?

Mark Ruzmetov, Randall S. Fortuna, Jitendra J. Shah, Dale M. Geiss, Karl F. Welke Children’s Hospital of Illinois, Peoria, IL

OBJECTIVES: In addition to being associated with aortopathy, bicuspid aortic valve (BAV) has been posed to be a risk factor for dilation of the pulmonary autograft in the aortic position. The aim of this study is to assess the association between the subtype of native aortic valve leaflet fusion (right and non-coronary leaflets [R/N] versus right and left leaflets [R/L]) and autograft dilatation and valve dysfunction after the Ross procedure.

METHODS: We performed a retrospective review of 43 patients with BAV who underwent a Ross procedure in our center from 1998 to 2012. Serial transthoracic echocardiography was used to measure changes in autograft and ascending aortic diameter over time. For each patient, the most recent echocardiogram or the last echocardiogram before intervention was reviewed. Moderate or greater aortic stenosis (AS) was defined as a valve gradient >3.5 m/s; aortic insufficiency (AI) was quantified using standard criteria. Aortic diameter was measured at 4 levels, and Z-values were computed. Aortic dilation was defined as a Z=value >3.

RESULTS: Mean age at the time of Ross procedure was 14.8 + 10.5 years (Table). R/L was the most prevalent native aortic valve subtype (R/L; n = 26 [61%] vs. R/N; n = 17 [49%]). Pre-Ross procedure, aortic dilation was more frequent in patients with R/N fusion (R/N, 72% vs. R/L, 32%; p = 0.02), whereas the initial aortic valve gradient and grade of AI did not differ between the subgroups. Patients had serial follow-up echocardiography performed for a mean of 9.6 + 4.3 years postoperatively. At follow-up, dilation of the autograft and ascending aorta was seen more often in patients with R/N leaflet fusion (R/N, 94% vs. R/L, 62%; p = 0.03). Conversely, the prevalence of more than moderate AI was significantly higher in patients with R/L leaflet fusion (R/L, 39% vs. R/N, 6%; p = 0.03). There was no significant difference between groups among numbers of late reintervention the aortic valve or root (R/L, 35% vs. R/N, 29%; p = 0.75); however the type of intervention varied by morphologic 294 subtype. Patients with R/L fusion underwent more aortic valve replacements while patients with R/N fusion underwent more valve sparing aortic root replacements.

CONCLUSIONS: Our study suggests that in young patients with BAV who undergo a Ross procedure, aortic valve morphology may be associated with autograft dilation and valve dysfunction. Patients with R/N leaflet fusion were more likely to have aortic root and ascending aortic dilation at follow-up, whereas patients with R/L fusion were more likely to have postoperative autograft insufficiency. This information may serve to guide patient and procedure selection for aortic valve replacement.

P23. Durable Ventricular Assist Device Support for End-Stage Heart Failure: An Extended Role in Pediatric and Congenital Population

Ed Peng, Neil Wrightson, Massimo Griselli, Richard Kirk, Tanveer Butt, John J. O’Sullivan, Guy A. MacGowan, David Crossland, Stephan Schueler, Asif Hasan Freeman Hospital, Newcastle Upon Tyne, United Kingdom

OBJECTIVE: Mechanical circulatory support in pediatric population is currently limited to pulsatile mechanical device (Berlin Heart Excor®). In recent years, the use of left ventricular assist device (LVAD) has increased substantially in adults with end-stage acquired heart disease with the availability of durable, newer generation, continuous flow device. We examined the extended role of this newer generation device in the pediatric and congenital population.

METHODS: Between 2010 and 2014, 16 patients who received HVAD (Heartware Inc, Framingham, MA) were studied and divided into two groups: (I) Pediatric group [N = 7; mean age: 11.3; range: 3.7–17.0 years]: 6-cardiomyopathy and 1-post-transplant rejection (II) Adult group (N = 9; mean age: 34.8; range: 24.5–41.0 years): all with complex congenital heart disease. Two patients were bridged from VA-ECMO and concomitant valve surgery was required in 3. RV function was optimised by pre-operative protocol using aggressive diuretics with/out inodilator and with/out hemofiltration. Temporary RVAD support if necessary was provided by a short-term device via neck or groin cannulation and pulmonary artery return via a vascular graft.

RESULTS: Overall, mortality on HVAD was 19% (3), 7 (44%) were transplanted, 2 (13%) were explanted and 4 (25%) were waiting for transplant. (I) In pediatric group: median length of support was 149 days (range: 17–638). The smallest patient to receive HVAD was 13.5 kg (age 3.7-year). Short term RVAD support was required in 3 patients but was successfully weaned off (range: 6–9 days) to LVAD support alone. The actuarial survival rate on HVAD was 100% at 30-day, 6-month, and 1-year on HVAD with no neurological event (0%). There was one death at day-638 from pneumonia/sepsis. 4 (57%) patients were transplanted and one pediatric HVAD was decommissioned following recovery from dilated cardiomyopathy (day-149). (II) In adult group, median length of support was 269 days (range: 64–685). The actuarial survival rates were 89% (30-day), 89% (6-month) and 76% (1-year). 3 patients were transplanted and 2 patients died (due to sepsis). One adult HVAD was explanted due to recurrent gastrointestinal bleeding, but also with evidence of improved heart function. 4 (44%) adult patients developed neurological complication (1-hemorrhagic, 3-embolic) but regained near complete or complete neurological recovery in all of them. CONCLUSION: Our early experience suggests that continuous flow, third generation device can provide durable support in the pediatric and congenital population with end-stage heart failure. Larger study is required to further assess the extended role of this device.

P24. Improving Outcomes with the Comprehensive Stage 2 Procedure After an initial Hybrid Stage 1 Palliation

*Mark E. Galantowicz, Andrew R. Yates Nationwide Children’s Hospital, Columbus, OH

OBJECTIVE: The Hybrid Approach has emerged as an alternative surgical strategy for the management of hypoplastic left heart syndrome (HLHS). A Hybrid Stage 1 is followed by a Comprehensive Stage 2 (removal of PDA stent and PA bands, aortic & pulmonary artery (PA) reconstruction, Damus-KayeStansel, atrial septectomy, Glenn). This is a challenging new procedure with very little published outcome data. We report our improving institutional experience.

METHODS: An IRB approved, retrospective review of prospectively collected data on all patients having a Comprehensive Stage 2 procedure between January 2002 to December 2013 including learning curve, high-risk, and standard risk patients were separated into Group 1 (January 2002 to March 2010) n = 64, and Group 2 (3/2010 to 12/2013) n = 36. The grouping eras fl ank the implementation of a peri-operative management protocol based on a review of the mode of death in Group 1.

RESULTS: Group 1 mortality was 12/64 (19%) where the most common mode of death involved pulmonary artery thrombosis in at least 6, with urgent vs elective indication for surgery, as well as age at procedure being factors. Care modifications instituted in 3/2010 included no procedures on an emergent basis or age <3 months, use of a BT shunt if the SVC and/or PA were too small, completion angiogram of Glenn with low threshold for intra-operative stenting, and post-op anticoagulation for 6 weeks. Group 2 mortality was 1/36 (3%) secondary to a hospital acquired viral pneumonia. There was no incidence of PA thrombosis in Group 2. ECMO utilization fell from 6/64 in Group 1 to 0/36 in Group 2.

CONCLUSION: Despite the technical challenges of the Comprehensive Stage 2 procedure excellent outcomes are attainable. Experience coupled with an internal quality review drove implementation of a successful peri-operative management protocol.

TUESDAY AFTERNOON, APRIL 28, 2015

Congenital Heart Disease – Simultaneous Scientific Session

8 minute presentation, 12 minute discussion

Moderators: *Jonathan M. Chen and *Charles B. Huddleston

60. Hybrid Therapy for the Hypoplastic Left Heart Syndrome – Myth, Alternative or Standard?

Can Yerebakan, Klaus Valeske, Hatem Elmontaser, Matthias Mueller, Juergen Bauer, Josef Thul, Dietmar Schranz, Hakan Akintuerk

Pediatric Heart Center Giessen, Giessen, Germany

Invited Discussant: *Mark E. Galantouitz

OBJECTIVE: The neonatal performance of the classical Norwood operation is still the standard treatment for patients with hypoplastic left heart syndrome (HLHS) worldwide. Alternative therapies such as the hybrid approach have only met acceptance as a niche therapy in most centers. We retrospectively evaluated our experience in hybrid treatment of the HLHS in a single center since June 1998.

METHODS: Patients with the diagnosis HLHS (n = 125) without exceptions were included into analysis who were treated with hybrid approach by one surgeon until October 2014. Hypoplastic left heart variants were excluded from this analysis. The different types of HLHS were distributed as follows: aortic and mitral atresia n = 38, 31%), aortic atresia and mitral stenosis (n = 43, 34%), aortic stenosis and mitral stenosis (n = 35, 28%), aortic stenosis and mitral atresia (n = 9, 7%). Median comprehensive aristotle score was 17.5 (14.5–27.5). Median weight was 3,200 g (1,210–7,050). All patients received an initial hybrid stage I procedure (surgical bilateral pulmonary artery banding and ductal stenting) with following comprehensive stage II operation, which included aortic arch reconstruction, bidirectional Glenn procedure, bilateral debanding of the pulmonary arteries and ductal stent removal at 4–6 months of age as indicated. Extracardiac total cavopulmonary anastomosis procedure completed the univentricular palliation.

RESULTS: Median follow-up time was 7.1 years (range: 1month to 15.6 years). Four early deaths occurred after hybrid stage I (3.2%). Out of the 121 survivors 3 patients received orthotopic heart transplantation (HTx). Seven patients did not survive until comprehensive stage II operation (5.1%). From these 4 patients received palliative care due to chromosomal defects after hybrid stage I. Comprehensive stage II operation was performed in 96 patients except 8 patients who are still awaiting stage II and 7 patients who received biventricular repair. Operative mortality after comprehensive stage II was observed in 5 cases (5.2%). Two patients underwent Htx. Interstage mortality after stage II comprised 5 patients (5.6%). Except 20 patients awaiting, Fontan/Kreutzer circulation was established in 62 patients without operative mortality and 329 *AATS Member TUESDAY, APRIL 28 two successful HTx thereafter. One patient died on the waiting list for HTx. Sixteen-year overall survival is 82% for the entire group. Survival beyond 10 years is 77.2% when patients who received biventricular repair and HTx are excluded from the cohort.

CONCLUSIONS: We adopted the hybrid approach as a standard treatment for all types of HLHS. Long-term results are highly satisfying in terms of mortality and morbidity in a complex group of unselected patients.

61. Selective Management Strategy for Neonates with Interrupted Aortic Arch Mitigates Future Left Ventricular Outflow Tract Obstruction Risk

*Bahaaldin Alsoufi , Brian Schlosser, Ritu Sachdeva, William Border, William Mahle, *Brian Kogon, *Kirk Kanter

Emory University, Atlanta, GA

Invited Discussant: *Ralph S. Mosca

OBJECTIVE: Following surgical repair of Interrupted aortic arch (IAA) and ventricular septal defect (VSD), left ventricular outflow tract obstruction (LVOTO) is a common complication that is associated with important morbidity and mortality, especially when early reoperation is required during infancy. Several anatomic factors that increase LVOTO risk have been identified and surgical strategies such as concomitant resection of the infundibular septum or LVOT bypass procedures (single stage Yasui or staged Norwood followed by Rastelli) have been proposed in those patients. We examined outcomes of our current selective management of neonates with IAA to address LVOTO risk.

METHODS: From 2002 to 2013, 77 neonates underwent IAA repair. Based on the presence of anatomic substrate for LVOTO, patients underwent standard IAA repair and VSD closure (n = 53 [69%]), concomitant infundibular resection (n = 7 [9%]), or LVOT bypass (n = 17 [22%], staged in 14). We analyzed anatomic and echocardiographic characteristics influencing procedure choice and explored reoperation risk and survival following IAA repair.

RESULTS: Median age at time of surgery was 7 days and median weight was 3.0 Kg; 41/77 (53%) had DiGeorge syndrome. Selected anatomic data is presented in the supplementary table. In comparison to neonates who had standard IAA repair and VSD closure, neonates who had infundibular resection or LVOT bypass had significantly smaller aortic valve and subaortic area and a trend for higher prevalence of type B IAA, aberrant right subclavian artery and bicuspid aortic valve. (See table) Overall freedom from LVOT reoperation was 94% and 76% at 1 and 8 years. Freedom from LVOT reoperation at 8 years was 80%, 43% and 88% for standard repair, infundibular resection and LVOT bypass, respectively (p = 0.004); 73% of LVOT reoperations were for discrete subaortic membrane resection. Overall 8-year survival was 86% and was not related to procedure type or LVOT reoperation. Eight-year freedom from arch and all-cause cardiac reoperation was 93% and 60%, respectively and was not related to procedure type. The majority of reoperations in the LVOT bypass group were for conduit change (67%).

CONCLUSIONS: This selective management strategy that is customized to the degree of aortic valve and subaortic area narrowing has mitigated and delayed LVOTO risk compared to published reports. With this tailored approach, most LVOT reoperations occur beyond infancy and are commonly related to the development of discrete subaortic membrane stenosis. Early and more complex LVOT reoperations, usually associated with higher morbidity and mortality, are required less often. The well established association between aortic valve and LVOT narrowing and increased LVOTO risk is neutralized with LVOT bypass procedures however reoperation for LVOTO continues to be the highest following infundibular resection.

62. Impact of Pacing on Left Ventricular Function in L-Transposition of the Great Arteries

Sophie C. Hofferberth, Mark E. Alexander, Douglas Y. Mah, Victor Baustista-Hernandez, *Pedro J. del Nido, Francis Fynn-Thompson

Boston Children’s Hospital, Harvard Medical School, Boston, MA

Invited Discussant: *Henry M. Spotnitz

OBJECTIVE: Late onset left ventricular (LV) dysfunction is a significant problem for patients with congenitally corrected transposition of the great arteries (ccTGA), particularly in those who are pacemaker dependent. The impact of univentricular versus biventricular (cardiac resynchronization therapy, CRT) pacing on LV function remains poorly understood in patients with ccTGA. In this study we sought to investigate the impact of ventricular pacing on LV function and assess whether the use of CRT as a primary or secondary (upgrade) mode of pacing precludes the development of late onset LV dysfunction in patients with ccTGA.

METHODS: We performed a retrospective review of all patients with a diagnosis of ccTGA who underwent pacemaker insertion. From 1993 to 2014, a total of 53 patients were identified from the cardiology database and surgical records at our institution. The primary endpoint was moderate or severe LV dysfunction as measured by echocardiography at latest follow up.

RESULTS: Overall mortality was 7.5 % (n = 4), with 1 patient requiring transplantation and 3 late deaths occurring secondary to end-stage heart failure. Median follow-up was 3.7 years (range: 4 days to 22.5 years). Seventeen (32%) patients underwent pacemaker insertion for spontaneous heart block (n = 8) or had a pacemaker placed at the time of surgical intervention preparing for anatomical repair (Pulmonary artery banding, n = 8; or BT shunt; n = 1). Eight (15%) underwent pacemaker insertion at the time of anatomical repair, while 28 (53%) patients became pacemaker dependent post anatomical repair. Twenty-eight (53%) underwent univentricular pacing only, of these, 10 (36%) patients developed moderate or severe LV dysfunction at latest follow-up. Twenty-five (47%) patients received CRT, 14 (26%) underwent secondary upgrade from a prior dual-chamber system, and 11 (21%) received CRT as the primary mode of pacing. Eleven of the 14 (85%) patients undergoing secondary CRT had significant LV dysfunction at the time of pacer upgrade, with only 4 (36%) demonstrating persistent LV dysfunction at latest follow-up. Therefore, of the 42 (79%) patients who initially underwent univentricular pacing, 21 (50%) went on to develop significant LV dysfunction. This is in contrast to the 11 patients who underwent primary CRT, none of whom developed LV dysfunction (see Figure, log rank test; p = 0.043).

CONCLUSIONS: Late-onset LV dysfunction is a clinically significant complication associated with the use of univentricular pacing in patients with ccTGA. All patients with ccTGA who develop heart block should undergo primary CRT as this prevents the development of left ventricular dysfunction. Furthermore, pre-emptive placement of biventricular pacing leads at the time of anatomical repair or other permanent palliative procedure will facilitate subsequent CRT should pacing be needed in ccTGA.

63. Comparison Between Two Surgical Techniques to Repair Total Anomalous Pulmonary Venous Connection at a Single Institute: 81 Cases with Sutureless Technique Versus 98 Cases with Traditional Technique

Yiqun Ding, Yanqiu Ou, Cheng Zhang, Jimei Chen, Jianzheng Cen, Shusheng Wen, Gang Xu, Jian Zhuang

Guangdong General Hospital, Guangzhou, China

Invited Discussant: *Christopher A. Caldarone

OBJECTIVE: Traditional surgical repair of total anomalous pulmonary venous connection (TAPVC) is associated with high mortality and morbidity, especially for patients with pulmonary venous obstruction. This study aims to compare the effect of two surgical techniques.

METHODS: From October 2007 to December 2013, 179 consecutive patients with TAPVC (Cardiac type was excluded) underwent biventricular surgical repair at our institute. Patients were allocated to 5 doctors, including 2 surgeons performing sutureless technique (Group 1, n = 81) and 3 surgeons performing traditional technique (Group 2, n = 98). All patients were divided into two categories: with/without preoperative pulmonary venous obstruction (PVO). Patient characteristics are summarized in the table that follows.

RESULTS: All patients were followed up from 7 days to 68.5 months.There were 23 deaths (4 in Group 1 [4/81, 4.9%], 19 in Group 2 [19/98, 19.4%]; P = 0.025). Postoperative PVO was present in 16 patients (2 in Group 1 [2/81, 2.5%], 14 in Group 2 [14/98, 14.3%]; P = 0.006). In the preoperative PVO category, the mortality of Group 1 and 2 were 12.5% (3/24) and 46.4% (13/28), respectively, P = 0.008. There was 1/24 (4.2%) patient in Group 1 present with mild PVO 18 days after the operation, the patient is symptomless, and being followed up. The survival rate and rate of free from PVO at 1 year were 87.5% (21/24) and 95.8% (23/24). There was 7/28 (25.0%) patients in Group 2 present with PVO at 1 to 7 months after the operations, with median as 3 months. 3 patients are symptomless and being followed up, 335 *AATS Member TUESDAY, APRIL 28 4 patients underwent reoperations, 75.0%(3/4) died. The survival rate and rate of free from PVO at 1 year were 53.6% (15/28) and 75.0% (21/28).In the non-preoperative PVO category, there were 1 and 6 deaths in Group 1 and 2, respectively, P = 0.199. There was 1/57 (1.8%) patient in Group 1 present with severe PVO 13 days after the operation, the patient underwent reoperation and survived. The survival rate and rate of free from PVO at 1 year were 98.2% (56/57) and 98.2% (56/57). There was 7/70 (10.0%) patients in Group 2 present with PVO 6 days to 8.74 months after the operations, with median as 1.5 months. 5 patients are being followed up, 2 patients underwent reoperations, 50.0%(1/2) died. The survival rate and rate of free from PVO at 1 year were 91.4% (64/70) and 90.0% (63/70).By using Cox’s proportional hazard model, traditional technique was the risk factor for death (OR, 4.59; 95% CI [1.53–13.89]) and postoperative PVO (OR, 8.20; 95% CI: [1.70–40.00]) for patients with preoperative PVO when adjusted for preoperative PVO, mixed type, neonates and weight at operation.

CONCLUSIONS: For patients with supracardiac, infracardiac and mixed type of TAPVC with preoperative PVO, sutureless technique in primary repair can decrease mortality and morbidity significantly. For patients without preoperative PVO, both sutureless and traditional techniques show satisfactory results.

64. Characterization and Outcomes of Severe Primary Multi-Vessel Pulmonary Vein Stenosis in Low-Birth Weight Infants

Ashley Dickens, Kimberley Gauvereau, Sanjay P. Prabhu, Christina Ireland, Michele J. Borisuk, Kathy Jenkins, Christopher W. Baird

Boston Children’s Hospital, Harvard Medical School, Boston, MA

Invited Discussant: *Emile A. Bacha

BACKGROUND: Primary pulmonary vein stenosis (PVS) is generally the most aggressive form of the disease; infants are often born premature and develop signs and symptoms within weeks to months after birth and frequently do not survive. The aim of this study was to characterize primary PVS in young and pre-mature infants and evaluate outcomes following surgical intervention.

METHODS: Patients undergoing surgical repair for primary PVS from February 2008 to July 2014 at Boston Children’s Hospital were identified. An aggressive modified “sutureless” surgical approach was used and additional consideration was given to modifying the relationship between the affected PVs and associated external structures. Outcome measures included mortality, re-intervention, degree of individual PV involvement and the relationship of externally related structures. Survival was determined by Kaplan-Meier analysis.

RESULTS: Twenty-five patients were identified with severe multi-vessel primary PVS. Median gestational age at birth was 34 weeks (25–40) and weight was 1.54 kg (0.39–4.22). Most patients underwent a pre-operative CT scan. Figure 1 shows direct relationships between the most affected PVs segments and external structures. The common findings included the following: right upper PV associated with the right pulmonary artery, left upper PV associated with the left bronchus and left lower PV associated with the descending aorta and left lung atelectasis. The right lower PV was generally unaffected. At operation, median age was 5.8 months (1–35) and weight was 4.9 kg (2.7–14). 92% had bilateral PV disease and 84% had involvement of three or more PV. The most common PV involved was RU (96%), LU (88%) and LL (80%) while the RL PV was involved least (44%). PV atresia was found in nearly half the patients (48%) and most commonly associated with the RU PV (28%) and LU PV (16%). All patients underwent post-operative catheterizations and 22 patients (88%) had interventions. The median number of catheterizations per patient was 3 (1–13). The median number of catheterizations with intervention per patient was 2.5 (1–12). No patients had PV re-operation. One patient died within 30 days following operation and 11 patients (44%) died in the follow-up period at a median follow-up of 0.8 years (13 days to 5.2 years). Eight patients were listed for lung transplantation: three patients were delisted, three patients died waiting and two patients were transplanted.

CONCLUSIONS: Severe multi-vessel primary PVS is uniformly fatal if not intervened upon. Aggressive multi-modal medical, surgical and interventional approach has improved survival in these patients, but mortality remains high. Continued characterization of primary PVS including the role of external anatomic structures will help to identify these patients earlier to prevent progression and allow earlier intervention and continued improved survival.

65. Pre-Operative Predictors of Survival After Repair of Pulmonary Vein Stenosis

Mauro Lo Rito, Tamadhir Gazzaz, Travis Wilder, *Glen S. Van Arsdell, Osami Honjo, Shi-Joon Yoo, *Christopher A. Caldarone

Hospital for Sick Children, Toronto, ON, Canada

Invited Discussant: *Francois G. Lacour-Gayet

OBJECTIVES: Pulmonary vein (PV) stenosis is associated with high mortality. CT/MRI imaging allows characterization of pulmonary vein pathology which may influence survival. We sought to develop a predictive model for survival after repair of PV stenosis.

METHODS: Patients who underwent repair of congenital or acquired PV stenosis (1990 to 2012) and preoperative CT/MRI were retrospectively reviewed. We measured short/long cross-sectional diameters of each PV at the left atrial junction (downstream), PV bifurcation (upstream), and at the narrowest point (maximum stenosis) and calculated total cross sectional area of PVs indexed for BSA (TCSAi). Survival after operation was evaluated with parametric multiphase risk-adjusted models. Univariate and multivariable analyses were used to identify relationships between PV dimensions and survival, and predictive nomograms were generated.

RESULTS: 145 patients underwent surgical repair of PV stenosis; 31 had preoperative imaging that met inclusion criteria. PV stenosis was congenital in 20 and acquired in 11. Surgical repair techniques included sutureless (n = 30) and pericardial patch reconstruction (n = 1). Follow-up duration is 4.28 ± 4.2 years. In-hospital mortality was 9.7% (3/31). Unadjusted 6-year freedom from death was 75 ± 7%, 69 ± 9% and 61 ± 10% at 1, 3 and 6 years respectively. Mean TCSAi downstream was 194 ± 109 mm2 /m2 , upstream was 302 ± 122 mm2 /m2 and site of maximum stenosis was 170 ± 90 mm2 /m2 . Although nearly 2/3 patients has the site of maximal narrowing which was localized to the left atrial junction, 1/3 of patients had sites of maximal narrowing which were between the left atrial junction and the first branching point of the pulmonary vein. A smaller upstream TCSAi was associated with an increased risk of early mortality (p = 0.06) and the TCSAi at the site of maximal stenosis was associated with late risk of death (p = 0.02) (see Figure).

CONCLUSION: Although intuitive to many surgeons, the anatomic characteristics of pulmonary veins which are amenable (or not amenable) to surgical repair are not objectively defined. The severity of maximal stenosis (which is often surgically accessible at the left atrial junction and associated with late mortality) has a complex predictive relationship with the extent of progression of PV pathology upstream into the lung parenchyma (which is not surgically accessible). The TCSAi is an objective measurement which can help to inform surgical decision making and stratify patients for enrollment in clinical trials of agents directed at upstream pulmonary vein pathology

66. Improvement of Cardiopulmonary Exercise Capacity After Pulmonary Valve Replacement and Its Predictors in Patients with Pulmonary Regurgitation After Repair of Tetralogy of Fallot

Yu Rim Shin, Jong Keun Kim, Hong Ju Shin, Young-Hwan Park, Han Ki Park

Yonsei University Severance Hospital, Seoul, Republic of Korea

Invited Discussant: *E. Dean McKenzie

OBJECTIVE: Chronic pulmonary regurgitation in patients after repair of tetralogy of Fallot results in progressive right ventricular dilatation and dysfunction. However, the association of exercise capacity and the right ventricular dilatation has not been clarified. The purpose of this study is to evaluate the change of exercise capacity and its association with right ventricular volume in the patients who underwent pulmonary valve replacement (PVR) after repair of tetralogy of Fallot.

METHODS: Among the patients who underwent PVR after repair of tetralogy of Fallot from January 2005 to August 2013, 40 patients underwent cardiopulmonary exercise test as well as magnetic resonance imaging (MRI) preoperatively and 1 year postoperatively. The medical records were reviewed retrospectively

RESULTS: The patients undergoing PVR had reduced preoperative cardiopulmonary exercise capacity (peak oxygen consumption, peak VO2 , 28.9 ± 5.8 ml/kg/min; percentage of predicted peak VO2 , 61.1 ± 10.5%). Preoperative peak VO2 had significant association with right ventricular end-diastolic volume index (RVEDVI) from preoperative MRI (r = –0.46; p < 0.01). After PVR, RVEDVI has decreased (173.1 ± 35.0 vs.110.7 ± 18.4 ml/m2 ; p < 0.01) and right ventricular ejection fraction improved (48.9 ± 9.1 vs. 52.2 ± 7.5%; p < 0.01) on the MRI. On the postoperative cardiopulmonary exercise test, percentage of predicted peak VO2 showed significant improvement (61.1 ± 10.5 vs.66.2 ± 10.7%; p = 0.03). The ratio of minute ventilation to carbon dioxide production (VE/VCO2 ) significantly improved (31.2 ± 6.8 vs.27.6 ± 3.3; p = 0.02), but the heart rate reserve showed no significant difference postoperatively. The postoperative peak oxygen consumption showed association with preoperative RVEDVI (r = –0.33; p = 0.04). On the risk factor analysis, moreover, preoperative RVEDVI larger than 160 ml/m2 was a negative predictive factor for the improvement of exercise capacity in the patients with reduced preoperative peak VO2 (OR, 0.10; 95% CI [0.014–0.838]; p = 0.03).

CONCLUSIONS: The overall exercise capacity showed significant improvement after PVR. The preoperative right ventricular volume had significant inverse correlation not only with the preoperative exercise capacity but also with the postoperative exercise performance. In the patients with larger RVEDVI more than 160 ml/m2 , the change of exercise capacity was less favorable after the PVR. The timely operation before severe dilatation of the right ventricle might be crucial in terms of exercise performance.

67. Intermediate-Term Neuro-Developmental Outcomes After Neonatal Cardiac Surgery: Role of Cortical Iso-Electric Activity

Michael Swartz, Laurie Seltzer, Jennifer Kwon, James Burchfiel, Jill M. Cholette, Dawn Sweeney, Cecilia Meagher, Ron Angona, Ronnie Guillet, George M. Alfieris

University of Rochester, Rochester, NY

Invited Discussant: *J. William Gaynor

OBJECTIVE: Neonates with congenital heart disease are at risk for adverse neuro-developmental outcomes following surgery. This multifactorial risk is likely related to fluctuations in blood pressure, flow, and temperature during cardiopulmonary bypass (CPB) and circulatory arrest. Electro-encephalograms (EEG’s) reflect cortical brain activity, and may be useful as an intraoperative monitoring tool. We hypothesized that intra-operative EEG activity may provide insight into future neuro-developmental outcomes.

METHODS: Neonates requiring surgery had baseline and continuous intraoperative EEG and hemodynamic monitoring. EEG activity was classified as either, 1) appropriate for age, 2) moderate burst suppression, 3) severe burst suppression, or 4) iso-electric (absent brain activity for >3 minutes). Follow-up neuro-developmental outcomes were assessed using the Vineland Adaptive Behavior Scale (VABS), providing a composite score and scores in specific activities (i.e., daily living, socialization, and communication).

RESULTS: Twenty-one neonates requiring cardiac surgery had baseline EEG activity which was appropriate for age. All neonates developed moderate burst suppression with the induction of general anesthesia. This pattern was unchanged throughout the case in the absence of hypothermia <25°C. Eleven neonates (52%) developed severe burst suppression that progressed into iso-electric activity during the severe hypothermic period required for circulatory arrest. The duration of iso-electric activity was significantly greater than circulatory arrest times (103.1 ± 50 vs. 24.6 ± 16 minutes; p < 0.001), and the onset of iso-electricity occurred with severe hypothermia 23.1 ± 4°C. However, iso-electric activity was not influenced by mean arterial pressure, or CPB fl ow. At a mean follow-up at 5.6 ± 1.0 years, neonates who required severe hypothermia had lower VABS scores in all areas and significantly lower communication scores (43.4 ± 9 vs. 50.1 ± 5; p = 0.05) when compared to neonates not requiring severe hypothermia. Comparison of composite VABS scores demonstrated an inverse correlation with the duration of iso-electric activity (R2 = 0.4; p = 0.03) but not circulatory arrest times (R2 = 0.001; p = 0.9) (see Figure). Further, in the neonates that developed iso-electric activity, those with iso-electric times >90 minutes, had the lowest VABS scores (459 ± 34 vs. 301.2 ± 62; p < 0.01).

CONCLUSIONS: The duration of cortical iso-electric activity associated with circulatory arrest is directly related to poor intermediate-term neuro-developmental outcomes, particularly if >90 minutes. Strategies which utilize moderate hypothermia, and continuous intra-operative EEG monitoring, may be useful during complex congenital heart surgery to predict risk.

Deep Dive Session Featuring

2. Late Survival and Right Ventricular Performance in 332 Matched Children: Classic Norwood-BT Shunt Versus Norwood-Sano Modification

Moderated by *Jonathan M. Chen

Wednesday Morning, April 29, 2015

Congenital Heart Disease – Simultaneous Scientific Session

5 minute presentation, 6 minute discussion

Moderators: *Duke E. Cameron and *Andrew J. Lodge

In Case You Missed It!

Listen to a summary of the 95th Annual Meeting scientific programming from the congenital surgeon perspective.

*Marshall L. Jacobs, Johns Hopkins University

94. Late Incidence of Endocarditis and Conduit Failure in Patients Undergoing Placement of Right Ventricular to Pulmonary Arterial Conduits with Bovine Jugular Grafts

Carlos M. Mery, Francisco Guzman-Pruneda, Matthew D. Terwelp, Luis E. DeLeon, Iki Adachi, *Jeffrey S. Heinle, *E. Dean McKenzie, *Charles D. Fraser, Jr.

Texas Children’s Hospital, Houston, TX

OBJECTIVE: Bovine jugular grafts (BJG) have been frequently used as right ventricular to pulmonary arterial (RV-PA) conduits. We have anecdotally observed several patients developing late endocarditis in the setting of a BJG after this procedure. The goal of this study was to analyze the long-term results of BJG’s as RV-PA conduits in terms of endocarditis and failure risk.

METHODS: All BJG RV-PA conduits placed at our institution from 2001 to 2014 were included. Freedom from endocarditis, freedom from conduit replacement (surgical or interventional), and freedom from reintervention (surgical or interventional) were calculated for each conduit. Univariate analyses were performed using Kaplan-Meier curves and log-rank tests. Multivariate analyses were performed using Cox proportional hazard models including statistically significant and/or clinically relevant variables.

RESULTS: 245 conduits were placed in 223 patients (103 [46%] females, 61 [27%] with genetic syndromes). BJG’s were inserted in 65 (27%) neonates/ infants, 98 (40%) patients 1–5 years, and 82 (33%) patients >5 years. Median conduit size was 16 mm (12–22 mm) with 125 (51%) conduits ≤16 mm in size. Diagnoses included pulmonary atresia or tetralogy of Fallot (88 [36%]), tetralogy of Fallot s/p simple repair (26 [11%]), truncus arteriosus (64 [26%]), and other (67 [27%]). 117 (48%) conduits were replacements of prior RV-PA conduits (89 [76%] homograft, 21 [18%] bovine jugular, 7 [6%] others). Median follow-up for non-replaced conduits was 4 years (3 days to 14 years). During the study period, endocarditis complicated 14 (6%) of the conduits at a median time of 7 years after implantation (34 days to 10 years); only 2 during the first year after implantation. 12 conduits were replaced and 2 were managed medically. Actuarial 5- and 10-year freedom from endocarditis was 94% and 76%, respectively (see Figure). Actuarial freedom from replacement 385 *AATS Member WEDNESDAY, APRIL 29 (5-year: 80%, 10-year: 46%) and freedom from reintervention (5-year: 76%, 10-year: 33%) can be seen in the figure. On multivariate analysis, no variables were found to be independent risk factors for development of endocarditis, conduit replacement, or conduit reintervention.

CONCLUSIONS: Despite reasonable short-term performance, bovine jugular RV-PA conduits are associated with considerable risk of late endocarditis and need for conduit replacement and reintervention. Due to the risk of endocarditis increased surveillance is warranted for these patients. Alternative graft conduits should be considered for patients in need for RV-PA reconstruction.

95. Pulmonary Artery Flow Studies Are Useful and Valid in Intraoperative Decision-Making for Patients Undergoing Repair of Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

Jiaquan Zhu, Atsuko Kato, Arezou Saedi, Devin Chetan, Rachel Parker, *John G. Coles, *Christopher A. Caldarone, *Glen S. Van Arsdell, Osami Honjo

Hospital for Sick Children, Toronto, ON, Canada

OBJECTIVES: We evaluated the predictive value of the intraoperative pulmonary artery flow (PAF) study on VSD closure and mortality in patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) who underwent complete unifocalization.

METHODS: Fifty patients with PA/VSD/MAPCAs (2000 to 2013) underwent repair and 40 consecutive patients since 2003 had a PAF study. The mean pulmonary artery pressure (PAP) was measured when calibrated pulmonary artery inflow reached 2.5 L/min/m2 . The cut off mean PAP to close the VSD in the study period was 30 mmHg. The ability to predict VSD closure was analyzed using a receiver operating characteristic curve.

RESULTS: Forty-seven patients (94%) had complete unifocalization of all MAPCAs. The median age of complete MAPCA unifocalization was 0.88 years (range: 0.05–16.2 years). Among 40 patients who had a PAF study, six (15%) patients had a mean PAP ≥ 30 mmHg and VSD was intentionally left open. VSD was closed in 34 patients who had a mean PAP ≤ 30 mmHg, of which four (11.8%) patients required salvage fenestration of the VSD patch due to suprasystemic right ventricular pressure (RVP) after weaning from cardiopulmonary bypass. The mean PAPs of these 4 patients were 18, 22, 24, and 25 mmHg (Figure 1A). The mean PAP accurately predicted VSD closure (area under the PAP curve 0.835; P < 0.001; Figure 1B). The sensitivity and specificity at the cut off value of 30 mmHg are 30.0% and 100.0%, and 60.0% and 83.3% if the cut off value of 25 mmHg. Within the whole cohort, there were 2 in-hospital deaths, 7 late deaths and 7 re-operations. Overall survival was 83.9%, 77.6% and 73.7% at 1, 3, and 5 years, respectively. Among the patients who had VSD closed, there is a trend toward higher mortality in patients with a higher PAP (25–30 mmHg) (37.5% vs. 4.5%; P = 0.08). Pulmonary artery catheter reinterventions were required in 27 (54%) patients. Patients who were not able to tolerate VSD closure had higher risks of death (P = 0.053), re-operation (P = 0.001), and pulmonary artery catheter re-interventions (P = 0.010) compared to the ones with VSD closed. Among patients who tolerated VSD closure, the follow-up systolic RVP did not drop in patients who needed pulmonary artery re-intervention (48.0 ± 11.0 vs. 49.3 ± 13.4 mmHg; P = 0.747), but significantly decreased in those who didn’t require re-intervention (45.9 ± 14.2 vs. 32.9 ± 9.3 mmHg; P = 0.011).

CONCLUSIONS: The intraoperative PAF study accurately predicted VSD closure in most patients with PA/VSD/MAPCAs and potentially predicts death. Careful interpretation is required if the mean PAP is greater than 25 mmHg

96. The Modification of Right Ventricle to Pulmonary Artery Conduit for Norwood Procedure Reduces the Number of Unintended Shunt-Related Events

Tomasz M. Mroczek, Rafal Zurek, Aleksandra Morka, Jerzy Jarosz, Katarzyna Szymanska, Janusz H. Skalski

Jagiellonian University, Krakow, Poland

Invited Discussant: *Minoo N. Kavarana

OBJECTIVE: The introduction of right ventricle to pulmonary artery conduit (RV-PAc) during Norwood procedure (NP) for hypoplastic left heart syndrome (HLHS) instead of traditional modified Blalock-Taussig shunt resulted in a higher survival rate in many centers, but increased number of unintended pulmonary and shunt interventions. We describe the impact of several modification applied to RV-PAc for NP on interstage course, surgical or catheter based interventions and pulmonary arteries development in HLHS cohort of patients.

METHODS: We retrospectively analyzed the three groups of non-selected, consecutive neonates who underwent NP between 2011 and 2014, with different RVPAc surgical techniques: Group I, left RV-PAc with distal homograft cuff (N = 32); Group II, right RVPAc with distal homograft cuff (N = 28); Group III, “dunk” right reinforced RV-PAc with direct distal anastomosis (N = 41). The technique of reconstruction of aorta during the NP was consistent during the entire study.

RESULTS: There was no difference in terms of age, weight, prevalence of aortic atresia, diameter of ascending aorta, deep hypothermic circulatory arrest time and hospital mortality rate (9.3 vs. 14.2 vs. 7.3%, respectively ) between groups. There was significant reduction in numbers of catheter-based interventions during the interstage period in third group (34 vs. 25 vs 0%, respectively; p = 0.01) and /or concomitant surgical interventions (17.2 vs. 4.1 vs. 2.6%, respectively). The diameter of pulmonary arteries was most homogenous in third group.

CONCLUSIONS: Modified strategy of using “dunk,” right reinforced RVPAc with direct distal anastomosis during Norwood procedures for HLHS significantly reduces the number of catheter-based and surgical-unintended shunt- related interventions during the interstage period. This strategy allows for more homogenous development of pulmonary arteries before the second, surgical stage.

97. Right Ventricular Outflow Tract Reconstruction with a Polytetrafluoroethylene Monocusp Valve: A Twenty-Year Experience

*John Brown, Mohineesh Kumar, *Mark Rodefeld, *Mark Turrentine

Indiana University, Indianapolis, IN

Invited Discussant: *James A. Quintessenza

OBJECTIVE: In patients with tetralogy of Fallot, pulmonary atresia, and other congenital right ventricular outfl ow tract (RVOT) obstruction/lesions, polytetrafluoroethylene (PTFE) monocusp valves provide two means: addressing RVOT competence and unloading the RV volume. They have been widely used at our institution over the past two decades. The purpose of this study was to determine whether a PTFE monocusp valve was an acceptable short and midterm remedy for patients with RVOT or pulmonary valve disease.

METHODS: From 1994 to 2014, 259 patients (mean age 2.8 ± 4.1 years; median 1.2 years; IQR, 0.7, 2.7 years) underwent reconstruction of the RVOT with a PTFE monsocusp valve. Patients were divided into 3 preoperative diagnostic subgroups: patients undergoing initial repair of tetralogy of Fallot or pulmonary atresia/ventricular septal defect (group 1: 176 pts, 68%), patients undergoing redo RVOT procedures (group 2: 37 pts, 14%), and patients undergoing complex initial repairs (group 3: 46 pts, 18%). Patients were studied intra-operatively, and serially postoperatively to determine pulmonary valve dysfunction defined as a peak gradient >40 mmHg or valve regurgitation > moderate. Valve failure was defined as the need for pulmonary valve balloon dilation or replacement. The mean follow-up duration was 11.2 ± 5.5 years (range: 1 month to 20 years).

RESULTS: There were 7 early and 8 late deaths (total 15/256; 5.8%). There was a significant difference between the preoperative and postoperative peak RVOT gradients (72.3 vs. 26.5 mm Hg). Of the 259 patients, 53 (20%) were lost to follow-up, and 77 (30%) required replacement of the monocusp valve 10.1 ± 4.4 years (range: 3 months to 18.9 years) after the original monocusp insertion. The remaining 129 patients have their original monocusp valve. Kaplan-Meier analysis showed no significant difference in all-cause mortality but there was a significant decrease in valve durability in group 2 as compared to groups 1 and 3 (see Figure).

CONCLUSIONS: The PTFE monocusp valve is an excellent choice for reducing pulmonary insufficiency in patients with congenital RVOT disease, especially in tetralogy of Fallot and pulmonary atresia (group 1). It is easy to construct, inexpensive and remains free from significant stenosis. In the long-term, patients will likely require a pulmonary valve replacement for valve regurgitation and RV dilation.

On Building a Heart: Lessons from Man and Nature

*Duke E. Cameron, Johns Hopkins Hospital

Moderators: *Bahaaldin Alsoufi and *J. William Gaynor

98. Repair of Parachute and Hammock Valves in Infants and Children and Its Long-Term Outcome

Eva Maria Delmo Walter, Henryk Siniawski, Takeshi Komoda, *Roland Hetzer

Deutsches Herzzentrum Berlin, Berlin, Germany

Invited Discussant: *Emre Belli

OBJECTIVE: Surgical management of parachute and hammock valves in children remains the most challenging congenital malformations to correct. We aim to report our institutional experience with valve-preserving repair techniques and the long-term outcome in parachute and hammock valves in infants and children.

METHODS: From 1992 to 2013, 20 infants and children with parachute (n = 12, mean age 5.16 ± 5.0, median 2. 2 months to 13 years) and hammock (n = 8, mean age 4.3 ± 0.7 years, median 7 months, range: 1 month to 14.9 years) valves underwent mitral valve (MV) repair . Ten (50%) belong to age group <1 year. Seven had class IV mitral insufficiency (MI) and 13 had class III MI. All had moderate mitral stenosis (MS).

RESULTS: Intraoperative findings included fused and shortened chordae with single papillary muscles in children with parachute valves. MV repair was performed using annuloplasty, commissurotomy, leaflet incision extended towards the body of the papillary muscles and split towards its base assuring sufficient thickness of both “new papillary muscle heads.” The children with hammock valves have dysplastic and shortened chordae, absence of papillary muscles with fused and thickened commissures in children with hammock valves (HV) with two patients having annular dilatation. Mitral valve repair consisted of carving off a suitably thick part of the left ventricular wall carrying the rudimentary chordae, ensuring that both the remaining left ventricular wall and the “new papillary muscles” maintain sufficient muscle thickness to maintain their function. The degree and extent of incision, commissurotomy and fenestration is determined by the minimal age-related acceptable mitral valve diameter to avoid mitral stenosis. Postoperative echocardiography showed absence or minimal MI, except for a one-month old infant whose MI was progressive and underwent MV replacement using a 14 mm biological prosthesis but died a week postoperatively. Another 4 month-old infant underwent repeat MV reconstruction a month after the initial repair, but severe MI persisted, hence underwent replacement with mechanical valve 2 weeks later and survived. During the 19-year follow-up, 5 patients with hammock valves and one with parachute valve underwent repeat MV reconstruction. A 7-month-old infant died of unknown cause 5 years after the initial repair. Freedom from reoperation was 60% and survival rate was 83.4%. Age less than 1 year proved to be a high-risk factor for reoperation and mortality (p = 0.00).

CONCLUSIONS: In children with parachute and hammock valves, surgical repair offered a satisfactory functional outcome during the long-term followup. Repeat MV repair may be necessary during the course of follow-up. Infants have a greater risk for reoperation and mortality.

99. Preliminary Experience in the Use of an Extracellular Matrix As a Tube Graft: Word of Caution

Narutoshi Hibino, Patrick McConnell, *Toshiharu Shinoka, *Mark Galantowicz

Nationwide Children’s Hospital, Columbus, OH

Invited Discussant: *David L. Morales

OBJECTIVE: A number of materials have been used for the repair of congenital heart disease. However an ideal material has yet to be discovered. Decellularized extracellular matrix from porcine small intestinal submucosa (CorMatrix) has been developed and commercialized as a biologic tissue substitute. This has been used for valvuloplasty, septal defect repair, or angioplasty as a patch. In this study, we demonstrate our preliminary experience using CorMatrix as a tube graft.

METHODS: From May 2012 to May 2013, a retrospective review of 13 patients who underwent cardiac surgery using CorMatrix as an interposition graft was performed (10 patients for central pulmonary artery (PA) reconstruction in comprehensive stage II surgery of hybrid procedure for hypoplastic left heart syndrome variant, 3 patients for aortic arch reconstruction in interrupted aortic arch). Chart review consisted of assessment of diagnosis, operative procedures, implant location, echocardiograms, reinterventions and pathology studies related to any explanted CorMatrix.

RESULTS: The average diameter of the original CorMatrix tube was 8.3 ± 0.7 mm in PA graft and 11.3 ± 0.6 mm in aortic graft. At a mean follow-up of 9.7 months, 9 of 10 patients who underwent central pulmonary artery reconstruction using CorMatrix tube showed progressive significant stenosis. The stenosed graft was dilated from 1.96 ± 1.47 mm to 4.66 ± 1.12 mm using catheter intervention at 4.2 ± 0.8 months after surgery. One of nine patients underwent replacement of CorMatrix tube with homograft due to severe stenosis due to neointmal hyperplasia after the placement of stent. All 3 patients who had aortic arch reconstruction with CorMatrix tube demonstrated no stenosis, no dilatation, and no aneurysm formation.

CONCLUSIONS: While angioplasty using CorMatrix as an interposition tube vascular graft is feasible and safe in aorta, high ratio of intimal hyperplasia formation with significant stenosis was found in venous circulation. Longer-term follow-up is required to assess the potential for growth of arterial conduit. CorMatrix may not be the ideal material as conduit in venous circulation to provide long-term durable outcomes.

100. Neo-Aortic Valve Regurgitation After Arterial Switch: Outcomes from a Single Center

Kai Ma, Shoujun Li, *Shengshou Hu, Zhongdong Hua, Keming Yang, Jun Yan, Hao Zhang, Qiuming Chen, Sen Zhang

Fuwai Hospital, Beijing, China

OBJECTIVE: 1) To identify the risk factors of neo-aortic valve regurgitation (NAR) after arterial switch for patients with complete transposition of the great arteries (TGA) and corrected transposition of the great arteries (CTGA).

METHODS: From 2003 to 2013, a total of 583 patients who underwent arterial switch operation for TGA and 31 patients who underwent double switch (atrial + arterial switch) operation for CTGA at our institution were included in this retrospective study. The NAR was evaluated by color Doppler imaging and graded as “none-trivial,” “mild,” “moderate,” or “severe” (0, 1 to 3), and was considered significant when documented as moderate or severe. Since 2011, concomitant neo-aortic sinotublar junction reconstruction was added if the aorta and pulmonary artery discrepancy (diameter ratio < 1:2) presented in patients with TGA.

RESULTS: The long-term survival rate was 92.5% (544/583) in TGA patients and 74.2% (23/31) in CTGA patients. No early postoperative significant NAR were noted in the total cohort. During the follow-up (Mean time: 46.0 ± 38.2 months) period, more NAR developed in patients with CTGA than patients with TGA (39.1% (9/23) versus 10.4% (56/539); p < 0.001), with a mean regurgitation level of 1.9 ± 0.8 and 1.8 ± 0.6, respectively. Moreover, the significant NAR (7.1% [38/539] vs. 26.1% [6/23]; p = 0.010) and the aortic valve replacement (0.6% [3/539] vs. 8.7% [2/23]; p = 0.003) were less in patients with TGA when compared with patients with CTGA. For TGA patients, estimated freedom from significant NAR was 99.8% at 1 year, 98.0% at 3 years and 94.4% at 5 years, respectively. In multivariate analysis for patients with TGA, previous left ventricular retraining and two great arteries diameter discrepancy were identified as risk factors for significant NAR. With the application of neo-aortic sinotublar junction reconstruction, no significant NAR was recorded in patients with two great vessels discrepancy. Respective freedom from significant NAR for CTGA patients was 100%, 75.0% and 68.2% at 1 year, 3 years and 5 years. There was no risk factor identified for significant NAR in patients with CTGA. However, all the 2 neo-aortic valve replacement in this subcategory of patient had previous morphological left ventricular retraining.

CONCLUSIONS: After ASO, favorable incidence of NAR and rare neo-aortic valve replacement are documented. The failure of neo-aortic regurgitation may be related to CTGA diagnosis and previous left ventricular retraining. Patients with aorta and pulmonary artery discrepancy may benefit from neo-aortic sinotublar junction reconstruction.

101. Anatomic Variability of the Thoracic Duct in Pediatric Patients with Complex Congenital Heart Disease

Ji Hyun Bang, Chun Soo Park, Jeong-Jun Park, Tae-Jin Yun

Asan Medical Center, Seoul, Republic of Korea

OBJECTIVE: Thoracic duct (TD) mass ligation through right thoracotomy, regardless of the sidedness of pleural effusion, is the standard procedure for the treatment of chylothorax. However, this procedure may not be successful, necessitating additional left peri-aortic mass ligation. We hypothesized that failure of right side approach may be attributable to the anatomical variation of the course of TD.

METHODS: Among the 8,880 children who underwent open heart surgery for congenital heart disease between 1992 and 2014, 70 patients (70/8,880, 0.8%) developed massive chylothorax and underwent TD mass ligation. Median age at cardiac surgery was 164 days (0 day to 70 months). Laboratory diagnosis of chylothorax was confirmed at 8.5 days (range: 2–118 days) after initial operation, and the first TD ligation was performed after a median duration of 31 days (range: 7–120 days) of medical treatment with a maximal drainage of 52 mL/kg/day (range: 14 to 259 mL/kg/day). Chylothorax resolved after right-side approach in most of patients (Group 1, n = 51) while additional left thoracotomy was required in 16 patients. In 3 patients with left chylothorax, left thoracotomy was initially performed. Thus, a total of 19 patients had left thoracotomy (Group 2). Demographic data, cardiac morphology, atrial situs, characteristics of chylous drainage, and postoperative outcomes were compared between the two groups.

RESULTS: In cardiac morphology, patients in group 2 were more likely to have dextrocardia (21.1% vs. 3.9%; p = 0.04), left isomerism (15.8% vs. 5.9%; p = 0.02), situs inversus (15.8% vs. 3.9%; p = 0.02) and abnormal great artery relationships (L-TGA, 15.8% vs. 5.9%; p = 0.02: side by side, 15.8% vs. 3.9%; p = 0.02). Incidence of right arch, right descending aorta, left superior vena cava, congenital esophageal anomaly were comparable between the two groups. There was no 30-days mortality, but 18 patients (20%) died during follow-up. Excluding 11 patients who died before chest tube removal, there was a gradual decrease in drainage after TD ligation, and chest tubes were eventually removed in 20 days (range: 4 to 79 days). After adjustment, both groups of patients showed a similar risk of death at 1 year (HR, 1.15; 95% CI [0.4–3.2]; p = 0.78). On multiple logistic regression analysis, peripheral leak syndrome (RR, 38.6; 95% CI [10.2–148.2]; p < 0.001) and postoperative low cardiac output (RR, 111.1; 95% CI [18.8–660.4]; p < 0.0.001) were identified as risk factos for death after TD ligation.

CONCLUSIONS: The course of the thoracic duct may be variable in pediatric patients with complex congenital heart disease. Left peri-aortic mass ligation should be considered in chylothorax refractory to right side TD mass ligation, especially in patients with abnormal atrial situs, dextrocardia, and great artery malposition.

102. Double Outlet Right Ventricle with Non-Committed Ventricular Septal Defect

Olivier Villemain(1) , Damien Bonnet(1) , Mathieu Vergnat(2) , Magalie Ladouceur(1) , Virginie Lambert(1) , Zakaria Jalal(1) , Pascal Vouhé(1) , *Emré Belli(2)

1 Université Paris Descartes, Sorbonne Paris Cité, Paris, France; 2 Université Paris Sud, Le Plessis Robinson, France

Invited Discussant: *Jennifer C. Hirsch-Romano

OBJECTIVE: The management of Double Outlet Right Ventricle (DORV) associated with anatomically non-committed Ventricular Septal Defect (NCVSD) constitutes a surgical challenge. The limits for, and the specific outcomes after anatomical versus univentricular repair still remain to be established.

METHODS: Between 1993 and 2011, 35 consecutive patients presenting with DORV/NC-VSD and 2 adequately sized ventricles were included into the study at two centers forming the National Referral Center. The selection criteria included the absence of outflow tract VSD: 21 inlet (4 complete atrio-ventricular septal defect [AVSD]), 9 muscular and 5 perimembraneous. RVOTO was present in 18/35 (51%). Twenty patients had undergone 25 initial palliative procedures.

RESULTS: Anatomical repair by means of intraventricular baffle construction was performed in 23 (Group l) at a median age of 10.5 months. VSD was surgically enlarged in 11 (48%). An associated RVOT reconstruction was required in 11 and Arterial Switch Operation (ASO) was done in 5. The remaining 12 patients underwent univentricular palliative repair (Group ll). There were 4 hospital deaths (11.4%): 3 in Group l and one in Group ll (p = .06). 8/20 survivors of group l patients underwent 13 reoperations after a median delay of 24 months, with subaortic stenosis being the main cause for reoperation (6/8). There was one late death in group 2. At last visit, all survivors were in NYHA class l-ll. Ten years actuarial survival rate and freedom from reoperation were respectively 74.7 ± 5% and 58 ± 5 % in Group l, and, 80 ± 7% and 71 ± 7% in Group ll. Univariate analysis showed that AVSD and/or isolated mitral cleft were associated with death (p = .04) and need for reoperation (p = .038).

CONCLUSIONS: Despite the need for complex procedure and the high incidence of reoperation for subaortic obstruction, our results suggested the long-term advantages of anatomical repair in DORV with NCVSD. The presence of associated AVSD and/or isolated mitral cleft was the only risk factors for mortality and reoperation.

Congenital Masters of Surgery Video Session

Moderators: *Jonathan M. Chen and *Pirooz Eghtesady

Starnes Procedure

*Vaughn A. Starnes, Keck School of Medicine

Mitral Valve Replacement in Infants with Stent Mounted Valve

Sitaram Emani, Boston Children’s Hospital

Pulmonary-Aorto Fistula (Pott’s Shunt) for Pulmonary Hypertension

*Emre Belli, Institut Jacques Cartier

Circumflex Aorta

*Carl L. Backer, Lurie Children’s Hospital