Congenital Rapid Fire Orals

Activity: 104th Annual Meeting
*Carl Backer Moderator
Cincinnati Children's Hospital Medical center
Winnetka, IL 
United States
 - Contact Me

Mayo Medical School, Northwestern CV Surgery and Congenital Heart Surgery.

Chief of Pediatric Cardiac Surgery  at Childrens Memorial in Chicago from 2008 - 2018

Current Chief of Pediatric Cardiac Surgery Kentucky Children's Hospital.

Co - author of the text Pediatric Cardiac Sugery.

Past President CHSS, Current director ABTS.

*Emre Belli Moderator
Marie Lannelongue Hospital
Bourg La Reine, NAP 
France
 - Contact Me

Chair, Department of Pediatric and Congenital Heart Disease, Marie Lannelongue Hospital, Univeristy Paris-Saclay, Le Plessis-Robinson, France

Domains of interest: Neoanatal Surgery, Anatomical Repair for Complex Anomalies, Valve Repair, Adult Congenital, Myocardial Protection

Sunday, April 28, 2024: 9:30 AM - 12:00 PM
Metro Toronto Convention Center 
Posted Room Name: Room 716 
Credits: 2.5 

Track

Congenital
104th Annual Meeting

Presentations

LB7. Outcomes of the "Univentricular Arterial Switch Operation" in Patients with Single Ventricle Morphology & Transposition of the Great Arteries

Total Time: 7 Minutes 
OBJECTIVE
To evaluate the intermediate survival in patients with univentricular hearts, transposition of great arteries & arch hypoplasia who underwent the univentricular arterial switch operation (uASO), in lieu of the conventional pulmonary artery band palliation.

METHODS
Patients with functionally univentricular hearts or single ventricle morphology undergoing uASO were shortlisted from the institutional congenital heart surgery database.

RESULTS
Seventeen patients underwent the uASO at a median age of 46 days (IQR: 30-49 days). There were 10 male children (59%). Primary diagnoses included Double Inlet LV in 13 patients (76%), hypoplastic right ventricular apparatus in 3 patients (18%) and straddling tricuspid valve in 1 patient (6%).

Concomitant procedures at the initial operation included aortic arch reconstruction under antegrade perfusion in 10 patients (59%), atrial septectomy in 8 patients (47%), tricuspid valve repair & exclusion in 1 patient (6%), each.

As part of the ASO, pulmonary blood flow was regulated either with pulmonary artery banding in 4 patients (24%), or graft interposition in 13 patients. The LeCompte manoeuvre in the initial part of our experience was avoided thereafter to retain the normal alignment of the pulmonary arteries. The median size of the interposition graft was 6 mm.

Median durations of CPB & aortic cross clamp were 240 & 146 minutes, respectively. There was 1 immediate operative death (6%), and 2 early deaths in this series. Median intensive care & hospital stays were 17 and 26 days, respectively. Six of the 14 survivors (43%) have advanced to Stage-2 palliation, while the other 8 are awaiting it.

Survival remained steady at 82% in the overall cohort, with the interstage period witnessing no attrition in survival or reinterventions. All 14 survivors were alive at last follow-up (median, 20 months) with mild neoaortic insufficiency, no residual systemic outflow tract obstruction, normal arch configuration, good left ventricular function & adequate pulmonary blood flow.

CONCLUSION
In a single sitting, the uASO addresses the hypoplastic arch, offers anatomical stabilisation of the ventriculoarterial junction & regulates pulmonary over-circulation. On the edifice of these principles, the outcomes from our experience demonstrate the physiological superiority & safety of this operation as a novel strategy for the morphology of univentricular hearts with transposition & arch hypoplasia.

Authors
Smruti Ranjan Mohanty (1), Aditya Doddamane (1), Bipin Radhakrishnan (1), Prashant Bobhate (1), Tanuja Karande (1), Kamlesh Tailor (1), Shankar Kadam (1), Keyoor Bhavsar (1), Nilesh Bohra (1), Suresh Gururaja Rao (1)
Institutions
(1) Children's Heart Center, Kokilaben Dhirubhai Ambani Hospital & Medical Research Institute, Mumbai, India 

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Abstract Presenter

Smruti Ranjan Mohanty, KOKILABEN DHIRUBHAI AMBANI HOSPITAL  - Contact Me Mumbai, Maharashtra 
India

131. Longitudinal Assessment of Health-Related Quality of Life in Adults with Congenital Heart Disease Patients Undergoing Cardiac Surgery: A Novel Approach Using Automated EMR Follow-Up

Total Time: 7 Minutes 
Objective:
Patients with adult congenital heart disease (ACHD) require life-long medical treatment. Longitudinal follow-up of this cohort is challenged by fragmented care paradigms and impaired transition from pediatric to adult care. This complex healthcare landscape inevitably influences the equilibrium of patients' perceived health-related quality of life (HR-QoL). Our center developed a novel electronic medical record (EMR)--based follow-up platform to assess HR-QoL among ACHD patients undergoing cardiac surgery. This study reports the results of this initiative among a complex cohort at our center.
Method:
Between January 2022- September 2023, we enrolled 104 patients (mean age 52±14) who underwent congenital heart surgery at our center and responded to HR-QOL questionnaires. Our comprehensive 23-element questionnaire included generic PROMIS 10 physical and mental scores and cardiac-specific MacNew social scores. Automated EMR assessments were delivered: 7 days preoperatively and postoperatively at 1,3,6, 12 months. A PROMIS 10 score exceeding 50 indicated superior self-reported physical and mental health compared to a matched age and sex U.S. population. MacNew social score above 5 signified enhanced social health compared to peers with cardiac disease. Multivariable linear regression models assessed relationships between patient characteristics, clinical variables, and HR-QOL outcomes.
Results:
Our cohort included 52 (50%) females, 69 married individuals (67%), and 40 patients who had undergone aortic valve interventions (41.6%). Male gender was a predictor of higher PROMIS 10 physical (P=0.04) and mental scores (P=0.0197) (Figure 1A, B) Over time, all PROMIS 10 mental and physical scores and MacNew social scores increased, from 1 month postoperatively to 12 months postoperatively (43± 10, 42±7, 4.4 ±1.1 versus 48±10,49± 12, 5.5 ±1.4; respectively). Notably, MacNew social score demonstrated significant improvements at 3 months (P =0.01), 6 months (P=0.003), and 12 months (P=0.001) ( (Figure 1C).
Conclusion:
Our innovative time series tool for assessing HR-QoL in ACHD patients demonstrates improved patient-perceived outcomes across mental, physical, and social domains, which continues over the first postoperative year. Social and mental domain gains outpaced perceived physical domain gains. Patient factors, including gender disparities contributing to HR-QoL differences, require further study to develop optimized treatment pathways

Authors
Brittany Potz (1), Miza Salim Hammoud (2), Justin Robinson (3), Matthew Thompson (4), Carla Duvall (1), Tara Karamlou (1)
Institutions
(1) Cleveland Clinic, Cleveland, OH, (2) Department of Pediatric Cardiac Surgery, Cleveland Clinic, Cleveland, OH, (3) N/A, N/A, (4) Cleveland Clinic, Lakewood, OH 

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Abstract Presenter

Brittany Potz, Cleveland Clinic Children's Hospital  - Contact Me Cleveland Heights, OH 
United States

133. When to do the Arterial Switch Operation to Optimize Outcomes and Resource Utilization: A National Database Analysis

Total Time: 7 Minutes 
Objective:The optimal timing of Arterial Switch Operation(ASO) has been a matter of debate in patients with d-TGA(d-Transposition of Great Arteries). We sought to examine characteristics, outcomes, & costs of early versus late ASO for d-TGA + IVS(Intact Ventricular Septum) from a large national database.
Methods:The Pediatric Health Informational System was queried from 2004-2022 to identify patients with d-TGA undergoing ASO. Patients were excluded if they had a VSD, arch reconstruction, concomitant defects or if ASO was performed >30 day of life. Patients were divided according to age at ASO:very early(0-3 d), early(4-7 d), late(8-14 d) & very late(15-30 d) groups. Baseline characteristics, demographics, use of balloon atrial septostomy(BAS), prostaglandin(PGE) & Nitric oxide(iNO) were analyzed. In-hospital outcomes including post-op mortality, need for ECMO, dialysis, delayed sternum closure, ICU length of stay, total costs of hospitalization were compared.
Results:A total of 3,556 patients/ASO cases were identified of which 21.7%(n=773) were very early, 51.0%(n=1,815) early, 21.1%(n=752) late & 6.1%(n=216) were very late. The median age at ASO was 6 days(IQR 4-8). Patients were similar in gender & race/ethnicity & presence of ASD. Patients in the late & very late groups were more likely to be preterm, have lower birthweight & have government insurance vs very early & early ASO groups. They were also more likely to have pre-op stroke, iNO use, BAS, & pre-op ECMO use(all P<0.05). Overall, in-hospital mortality was 1.4% & was similar between groups (p=0.15). Late & very late groups had higher rates of non-home discharge, post-op ECMO use, delayed sternum closure & longer ICU LOS. There was an incremental increase in the composite outcome of in-hospital death/non-home discharge/post-op ECMO/delayed sternum closure by timing of ASO( in order, v early to v late,): 8% vs 9.6% vs 16.2% vs 25.0%. On multivariate analysis, patients in [late & v late groups] vs [v early & early groups] had almost double the odds of developing composite outcome(aOR 1.97, 95%CI: 1.57-2.43, p<0.001).Median total adjusted costs of hospitalization were significantly higher in late & very late ASO groups (Very Early: $115,060; Early: $129,297; Late: $162,746; Very Late: $213,707)(Table)
Conclusions:This is the largest study examining timing of ASO in a cohort exclusively of d-TGA + IVS. ASO performed late (>7 days) resulted in worse outcomes & higher healthcare resource utilization.

Authors
Muhammad Faateh (1), Hosam Ahmed (1), Muhammad Aanish Raees (1), David Lehenbauer (1), James F. Cnota (1), David Morales, Sr. (1), Awais Ashfaq (1)
Institutions
(1) The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 

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Abstract Presenter

Awais Ashfaq, Cincinnati Childrens  - Contact Me Cincinnati, OH 
United States

134. The Landscape of Congenital Heart Disease Treated with the Ross Procedure

Total Time: 7 Minutes 
Objective: The Ross procedure has excellent results in the pediatric population. Some series report age and anatomy dependent outcomes, but no comprehensive analysis stratified by these variables exists. We sought to identify the spectrum of congenital heart disease (CHD) treated with the Ross procedure and associated outcomes at our center.
Methods: Over 30 years, 317 pediatric patients underwent the Ross procedure. Patients were stratified into 4 age groups: neonates (<31 days, n=21), infants (31 days-1 year, n=40), children (1-12 years, n=165), and adolescents (13-18 years, n=91). Groups were compared across preoperative variables and outcomes. Multivariable logistic regression identified risk factors for mortality and morbidity. Kaplan-meier method compared survival and freedom from reintervention.
Results: The primary diagnosis was isolated aortic stenosis or regurgitation. Shone's complex 25/317 (8%), interrupted aortic arch (IAA) 20/317 (6%), hypoplastic arch (HA) 20/317 (6%), transposition of the great arteries (TGA) 7/317 (2%), and double outlet right ventricle (DORV) 3/317 (1%) were less common. Operative mortality and major morbidity were 8/21 (38%), 4/40 (10%), 2/165 (1%), 0/91 (0%) and 13/21 (62%), 10/40 (25%), 19/165 (12%), and 5/91 (6%) for ascending age groups (p<0.001, p <0.001). Neonates and infants with isolated aortic valve (AV) disease had an operative mortality of 3% (1/29). In multivariable logistic regression analysis Shone's complex predicted mortality in patients <1 year of age (OR 1.38, p=0.002). Mitral valve disease (OR 1.16, p=0.048) and subvalvular stenosis (OR 1.23, p<0.001) predicted morbidity for children aged 1-12. History of IAA (OR 1.55 p=0.009) predicted morbidity in adolescents. Survival was significantly different between age groups (p<0.001, Figure 1A) and lowest in patients with complex CHD (DORV, TGA, IAA, HA) (Figure 1B, p<0.001). Freedom from left ventricular outflow tract (LVOT) reintervention did not differ between age groups (Figure 1C, p=0.63). Complex CHD demonstrated higher rates of LVOT reintervention (Figure 1D, p=0.004). Multivariable cox regression confirmed this finding (HR 2.47, p=0.03).
Conclusions: Pediatric patients >1 year old and those <1 year old with isolated AV disease have outstanding outcomes after the Ross procedure. Neonates and infants with complex CHD have the highest rates of perioperative morbidity and mortality. Complex CHD is associated with increased risk of LVOT reintervention.

Authors
Markian Bojko (1), Luke Wiggins (1), John Cleveland (1), Ramsey Elsayed (2), David Cleveland (1), Vaughn Starnes (1)
Institutions
(1) Heart Institute, Children's Hospital of Los Angeles, Los Angeles, CA, (2) Southwest Healthcare MEC, Temecula, CA 

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Abstract Presenter

Markian Bojko, Division of Cardiac Surgery, Keck School of Medicine of USC  - Contact Me Manhattan Beach, CA 
United States

135. All Roads Lead to Flow: Open, Hybrid, and Endovascular Repair of Aortic Coarctation in Adolescents and Adults

Total Time: 7 Minutes 
Objective: Optimal management of coarctation of the aorta (CoA) in adolescents and adults remains controversial. We report early and late outcomes of open surgical, hybrid, and endovascular repair, evolution of approaches over time, and the longitudinal effect of treatment on anti-hypertensive medication regimens.
Methods: Patients, age >10 years, who underwent primary CoA repair between 1999–2023 were reviewed from a prospectively maintained institutional database. Operative repair trends were evaluated. Longitudinal gradient boosting models were used to predict the probability of postoperative anti-hypertensive use over time. Reoperation and survival were evaluated by Kaplan–Meier methods; median follow-up was 6.8 years with 790 patient-years available.
Results: One-hundred ten patients (age 38+/-16 years; 62% male) underwent CoA repair. Bicuspid aortic valve was present in 48% (N=53), and 38% (N=42) had additional congenital cardiac anomalies. Most patients (90%, N=90/100) had hypertension. Operative techniques were open repair (N=42, 38% [N=25, 60% extra-anatomic bypass]), hybrid repair (N=7, 6% [N=6, 86% frozen elephant trunk]), and endovascular repair (N=61, 56%). Endovascular repair increased in frequency over the study period. Devices used included balloon-expandable stents (bare metal [N=33, 51%] and covered [N=10, 15%]), and self-expanding stent grafts (N=22, 34%). The left subclavian artery was left uncovered in 74% (N=45/60) and revascularized in 21% (N=13/60). Operative mortality was 0.9% (N=1/110), major morbidity including stroke (2.1%, N=2/96) and acute renal failure requiring dialysis (1.0%, N=1/96) were uncommon. Probability of using anti-hypertensive medications fell and stabilized, by two years postoperatively following open repair (Figure 1A), versus an increasing probability of more anti-hypertensive medications beyond two-years following endovascular repair (Figure 1B). Freedom from reintervention at 1, 5, and 10 years following open repair was 98%, 95%, and 95% and following endovascular repair was 91%, 75%, and 64% (Figure 1C). Overall survival at 1, 5, and 10 years was 98%, 98%, and 94%.
Conclusions: A tailored approach to coarctation repair in adolescents and adults with a shift toward endovascular repair when anatomically feasible has yielded excellent outcomes with respect to reintervention and survival. However, the effect on blood pressure control may indicate more durable CoA repair following open interventions.

Authors
Matthew Thompson (1), Jonathan Putnam (2), William Frankel (1), Holliann Willekes (1), Benjamin Kramer (3), Ashley Lowry (4), Patrick Vargo (5), Patcharapong Suntharos (6), Joanna Ghobrial (1), Lars Svensson (1), Eric Roselli (1)
Institutions
(1) Cleveland Clinic, Cleveland, OH, (2) Case Western Reserve University School of Medicine, Cleveland, OH, (3) Cleveland Clinic, United States, (4) Cleveland Clinic, Department of Quantitative Health Sciences, Cleveland, OH, (5) Cleveland Clinic, Cleveland, Ohio, (6) Cleveland Clinic Foundation, Cleveland, OH 

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Abstract Presenter

Matthew Thompson, Cleveland Clinic  - Contact Me Lakewood, OH 
United States

136. Concomitant Tricuspid Valve Repair of Any Kind at Pulmonary Valve Replacement is Advantageous for Late Tricuspid Valve Function in Adults with Repaired Tetralogy of Fallot

Total Time: 9 Minutes 
Objective: To investigate late effect of concomitant tricuspid valve surgery (TVS) at time of pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot (rTOF).
Methods: 562 patients undergoing pulmonary valve replacement (PVR) were screened. Exclusion criteria were non-rTOF diagnosis, incomplete surgical note, echocardiographic, or cardiac magnetic resonance imaging (CMR). Outpatient echocardiography was obtained at mean follow-up 11.0±5.5 years. CMR was performed at mean follow-up 2.1±2.0 years and 6.8±6.0 years after surgery.
Results: A total of 196 rTOF patients were categorized into PVR (n=161) vs PVR-TVS (n=35). TVS techniques were reinforced annuloplasty (band or ring) (51.4%;n=18) and DeVega annuloplasty (48.6%;n=17). TV morphology was normal in 98.1% PVR (n=157) and 77.1% PVR-TVS (n=35) (HR 0.2±0.1[CI 0.1-0.6];p<0.01). Mean age at surgery was 43.2±11.7 years for PVR compared with 47.7±12.8 years for PVR-TVS (p=0.02). Preoperative moderate or greater tricuspid regurgitation (mTR) was present in 20.5% PVR (n=33), compared to expectedly higher incidence, 91.4% in PVR-TVS (n=32;p<0.01). Preoperative mean right ventricular end-diastolic volume index (RVEDVi) was higher in PVR-TVS than PVR (225±49 vs 186±35 mL/m2;p<0.01). There was no difference in preoperative RV ejection fraction (RVEF) between both groups. Concomitant arrhythmia surgery occurred in 9.3% PVR (n=15) and 34.3% PVR-TVS (n=12;p<0.01). There were no differences in RV outflow tract resection (19.3% PVR [n=31] vs 11.4% PVR-TVS [n=4]), residual atrial septal defect (21.1% PVR [n=34] vs 25.7% PVR-TVS [n=9]) or ventricular septal defect closure (5.6% PVR [n=9] vs 11.4% PVR-TVS [n=4]). Postoperatively, RVEDVi decreased in both groups (186±35 to 121±28 mL/m2 in PVR vs 225±49 to 143±38 mL/m2 in PVR-TVS;p<0.01). RVEF was unchanged. At mean follow-up 9.9±5.8 years, recurrent mTR was 9.9% PVR (n=16) vs 34.3% PVR-TVS (n=12;p<0.01). Despite much higher initial prevalence of mTR, benefit of PVR-TVS was sustained at 10 years with >65% mTR abolished whereas PVR showed progressive mTR over time. Freedom from recurrent mTR appeared similar irrespective of TVS technique (Figure).
Conclusions: Patients undergoing PVR-TVS were older with greater RV dimension, more thickened TV, and higher prevalence of mTR at presentation. Although both PVR and PVR-TVS reduced RV dimension and TR severity, concomitant TV repair of any kind produced sustained benefit against late recurrent mTR.

Authors
Myunghyun Michael Lee (1), Leyre Alvarez Rodriguez (2), Ayako Ishikita (2), Rachel Wald (2), Osami Honjo (1), David Barron (1)
Institutions
(1) Hospital for Sick Children, Toronto, Ontario, (2) Toronto General Hospital, Toronto, Ontario 

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Abstract Presenter

Myunghyun Michael Lee, Hospital for Sick Children  - Contact Me Toronto, ON 
Canada

137. Surgical Debulking of Large Ventricular Fibromas in Children

Total Time: 7 Minutes 
Objective:
The optimal surgical resection strategy for ventricular fibromas is uncertain. We describe the outcomes of tumor debulking, ventricular cavity reconstruction, and atrioventricular valve repair if needed.

Methods:
Single-center, retrospective cohort study of patients undergoing ventricular fibroma resection between 2000 and 2023. Over the course of the study period, the approach has evolved from complete resection to a strategy of tumor debulking.

Results
Among 52 patients, median age at surgery was 2.0 years (IQR 9.2 months-4.6 years) and median weight was 11.5 (IQR 8.5-18.9) kilogram. Median tumor volume index was 69 (IQR 49-169) milliliters/m2. Tumor distorted the AV valve/subvalvar apparatus in 30 (58%) patients, caused outflow tract obstruction in 3 (6%) patients, and 5 (10%) patients had ≥ moderate AV valve regurgitation. Surgery was indicated for arrythmia (n=45, 86%), symptoms (n=14, 27%), and/or hemodynamic compromise (n=11, 21%). Tumor was primarily debulked in 34 (65%) patients, including the last 21 patients. The ventricular cavity was entered in 15 (29%) patients. Concomitant AV valvuloplasty was performed in 18 patients (15 mitral and 3 tricuspid), while 3 patients received intraoperative cryoablation. Residual rim of tumor was left around coronary arteries in patients who had signs of extrinsic coronary compression (n=7, 13%), myocardial bridging (n=6, 12%), and/or intratumor coronary artery course (n=4, 8%). Two patients required early reoperation for residual AV valve insufficiency and one patient late reoperation for LVOT obstruction. Median duration of follow-up was 2.5 (IQR 0.8 -6.5) years. There was no mortality or heart transplantation. During the follow-up period, sustained VT occurred in 1 (2%) patient. No other patients had evidence of ventricular arrythmia on Holter monitoring or EKG. Among 38 patients with post-op v-stim studies, 3 (8%) experienced inducible VT / VF requiring cardioversion / defibrillation. Two of these patients received an ICD. Pre and post-debulking LV EF did not significantly differ (P=.069). No patients had signs of outflow tract obstruction or > moderate AV valve regurgitation on latest imaging.

Conclusions:
Large ventricular fibromas can be resected safely with appropriate surgical planning and an emphasis on debulking rather than complete resection. Most children are cured of life-threatening arrythmias and maintain LV function.

Authors
Ajami Gikandi (1), Peter Chiu (2), Meena Nathan (3), Edward O'Leary (3), Edward Walsh (1), Rebecca Beroukhim (3), Pedro del Nido (3)
Institutions
(1) N/A, N/A, (2) N/A, United States, (3) Boston Children's Hospital, Boston, MA 

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Abstract Presenter

Jordan Secor, Massachusetts General Hospital  - Contact Me Boston, MA 
United States

138. Early versus Late Arterial Switch Operation for d-Transposition of Great Arteries with Ventricular Septal Defect without Outflow Tract Obstruction

Total Time: 7 Minutes 
Introduction: The ideal timing for Arterial Switch Operation (ASO) in patients with d-Transposition of Great Arteries (d-TGA) and Ventricular Septal Defect (VSD) without outflow tract obstruction remains inconclusive. We assessed the impact of timing of surgical correction on outcomes.
Methods: Neonates with d-TGA + VSD who underwent ASO + VSD repair at age ≤ 30 days were identified from the Pediatric Health Information System database from 2004-2022. Patients with outflow tract obstruction were excluded. Patients were categorized into four groups based on age (in days) when ASO was performed: Group 1 (0-7 days), Group 2 (8-14 days), Group 3 (15-21 days), and Group 4 (>21 days). Baseline demographics, presence of Patent Ductus Arteriosus (PDA), Balloon Atrial Septostomy (BAS) were compared. Outcomes including mortality, length of stay, post-op ECMO use were analyzed by timing of ASO.
Results: A total of 1,005 patients were identified of which 652(64.9%) underwent ASO in group 1, 247(24.6%) in group 2, 72(7.2%) in group 3 and 34(3.4%) in group 4. Overall, groups were similar in terms of gender, prematurity, pre-op ECMO use, and presence of coronary anomalies (all P>0.05). Patients in groups 3 and 4 were more likely to be of lower birth weight. Unadjusted outcomes including in-hospital mortality, post-op ECMO, delayed sternum closure, non-home discharge, and post-op length of stay were similar between the groups. The multivariate odds of composite outcome of death/post-op ECMO/delayed sternum closure/non-home discharge were higher, comparing patients in group 1(0-7 days) vs group 2-4(>7 days) (aOR 1.54, 95% CI: 1.09-2.19, p=0.016). (Table)
Conclusion: Our analysis of >1,000 neonates with d-Transposition of Great Arteries + ventricular septal defect without outflow tract obstruction reveals that outcomes of Arterial Switch Operation + VSD repair are better if performed within the first week of life.

Authors
Muhammad Faateh (1), Spencer Hogue (2), Amir Mehdizadeh-Shrifi (1), Kevin Kulshrestha (1), David Lehenbauer (1), David Morales, Sr. (1), Awais Ashfaq (1)
Institutions
(1) The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, (2) Department of Cardiothoracic Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 

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Abstract Presenter

Spencer Hogue, Department of Cardiothoracic Surgery, Cincinnati Children’s Hospital Medical Center  - Contact Me Cincinnati, OH 
United States

139. Left Ventricular Outflow Tract Obstruction as Modifiable Risk Factor for Recurrence of Endocardial Fibroelastosis

Total Time: 7 Minutes 
Objectives: Congenital heart defects with left ventricular outflow tract obstruction are often accompanied by subendocardial accumulation of collagen and elastic fibers referred to as endocardial fibroelastosis (EFE). Resection of EFE is performed to improve left ventricular functional recovery, but EFE recurrence is often observed, occasionally hampering LV recruitment endeavors. We previously identified longstanding left ventricular outflow tract obstruction (LVOTO) as risk factor for faster EFE recurrence after primary resection. The goal of this study was to determine whether early surgical elimination of LVOTO at the time of primary EFE resection affects the risk of EFE recurrence.
Methods: A retrospective chart review included all patients with congenital aortic valve (AoV) stenosis/LVOTO (n=93) who underwent primary resection of EFE at two centers between 01/2010 and 12/2021. Patients with LV long axis z-score >-2, mitral valve area z-score >-2, less than severe mitral stenosis and at least moderate LV function were included (58/93). Children with surgical treatment of LVOTO by a Ross (+/-Konno) procedure (22/58) were compared to patients with resembling anatomy who underwent other kinds of LVOT surgeries, mostly aortic valvuloplasty (36/58). EFE recurrence was defined as increased thickness or progressive appearance in previously resected or new areas determined by echocardiography and/or MRI during follow up. The primary outcome measure was time to EFE recurrence.
Results: Freedom from EFE recurrence was significantly higher in Ross (+/- Konno) patients ( P=0.003), with a median time to recurrence in the non-Ross patients of 2.67 years (Figure 1). Freedom from reintervention on the LVOT/AoV was higher in the Ross (+/-Konno) group, 1/22 (4.5%) vs 15/36 (41.7%) (log-rank test = 8.46, P = 0.004). 18/22 (81.5%) of patients after Ross were alive with a biventricular circulation at most recent follow up.
Conclusions: Our data indicate that LVOTO is a modifiable risk factor for EFE recurrence, and early surgical relief by enlargement of the LVOT has the potential to reduce the risk of LV EFE recurrence. This patient cohort also showed a reduced rate of reoperations on the LVOT and AoV. The correct patient selection and timing for surgical LVOTO treatment in the larger context of LV recruitment efforts has yet to be identified through prospective trials.

Authors
Gregor Gierlinger (1), Daniel Diaz-Gil (1), Andreas Tulzer (2), Roland Mair (3), Eva Sames-Dolzer (3), Steven Staffa (4), David Zurakowski (4), Michaela Kreuzer (3), Sitaram Emani (1), Pedro del Nido (1), Rudolf Mair (3), Ingeborg Friehs (1)
Institutions
(1) Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA, (2) Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, JKU, Linz, Austria, (3) Division of Pediatric and Congenital Heart Surgery, Kepler University Hospital, JKU, Linz, Austria, (4) Department of Surgery, Boston Children's Hospital, Boston, MA, USA 

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Abstract Presenter

Gregor Gierlinger, Boston Children's Hospital  - Contact Me Linz
Austria

141. Combined pressure and volume loading for left ventricular training in patients with congenitally corrected transposition of the great arteries

Total Time: 7 Minutes 
Objective: Patients with congenitally corrected transposition of the great arteries (ccTGA) and unconditioned left ventricles (LV) may become candidates for double switch operation (DSO) when undergoing LV training with pulmonary artery banding (PAB) ± atrial septectomy. The aim of the study was to assess the success of this concept.
Methods: The medical records of all patients with ccTGA who underwent LV training between 2012-2022 were retrospectively reviewed.
Results: Fifteen patients underwent LV training at a median age of 1.5 years (IQR 0.7-5.6). Baseline MRI was done in 11 and cardiac catheterization in 12 patients. The median LV mass index was 20.4 g/m2 (IQR 18.9-36.6), LV end-diastolic volume (LVEDV) index was 67.6 ml/m2 (IQR 40.6-97.1) and systolic LV/RV pressure ratio was 0.35 (IQR 0.31-0.5). In addition to PAB, atrial septectomy was performed in twelve patients (80%). Two patients (13%) already had a relevant shunt due to partial anomalous pulmonary venous drainage. The maximum PAB gradient was 41 mmHg (IQR 40-52). Median ICU and hospital stay were 1 day (IQR 1-3) and 6 days (IQR 5-8), respectively. One patient required ventricular assist device implantation 8 months after PAB and underwent heart transplantation. The patient died 3.6 years after PAB.
Of the 14 survivors, follow-up MRIs and cardiac catheterization were done in 12 patients, respectively. Two patients were evaluated by echocardiography. After a median of 1.9 years (IQR 0.8-4.4) median LV mass index increased to 38.5 g/m2 (IQR 25-49, P=0.004), LVEDV index remained at 71.4 ml/m2 (IQR 50.5-91.8, P=0.49) and systolic LV/RV pressure ratio increased to 0.94 (IQR 0.84-1.1, P=0.004) (Figure 1). Maximum PAB gradient increased to 59 mmHg (IQR 52-97, P=0.003).
So far, six patients (6/14, 43%) have met eligibility criteria for DSO (5 performed, 1 scheduled). Their age at time of PAB was 1.7 years (IQR 0.5-3.7) and time between PAB and DSO was 3.1 years (IQR 1.5-5.2). One patient required DSO takedown due to LV failure. Two patients (11 and 14 years at time of PAB) were considered non-responders and remained in NYHA class II with preserved cardiac function 5.5 and 4.6 years after PAB. Six patients (6/14, 43%) continue LV training after a median follow-up time of 1.1 years (IQR 0.7-1.9).
Conclusion: Combined pressure and volume loading resulted in significant increase in LV mass index and LV/RV pressure ratio. Among older patients there were non-responders who remained not suitable for DSO

Authors
Antonia Schulz (1), Viktoria Weixler (1), Marcus Kelm (2), Peter Kramer (2), Mi-Young Cho (3), Stanislav Ovroutski (2), Felix Berger (2), Joachim Photiadis (4)
Institutions
(1) Deutsches Herzzentrum der Charité, Berlin, Germany, (2) Deutsches Herzzentrum der Charité, Berlin, NA, (3) German Heart Center Berlin, Berlin, Berlin, (4) N/A, N/A 

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Abstract Presenter

Antonia Schulz  - Contact Me Berlin
Germany

142. Comparison of pulmonary artery growth between ductus stent and systemic to pulmonary shunt as single-ventricle palliation

Total Time: 7 Minutes 
Objective: We aimed to compare the pulmonary artery (PA) growth between infants with univentricular hearts who underwent a ductus stenting and those who received a surgical systemic to pulmonary shunt.
Methods: All infants with univentricular heart and ductal-dependent pulmonary blood flow who underwent initial palliation with either a ductus stenting (DS) or a surgical systemic to pulmonary shunt (SPS) between 2009 and 2022 were reviewed. Patients who underwent SPS and concomitantly repair of total anomalous pulmonary venous connection and/or aortic arch repair were excluded. PA development was compared between the groups and the using the PA index after Nakata, ratio of left to right PA index, and the symmetry index after Gratz.
Results: A total of 130 patients were evaluated, and patients were divided into 2 groups: 39 patients who underwent ductus stenting (DS group) and 91 patients who underwent systemic to pulmonary shunts (SPS group). SPS group included 10 patients who initially performed DS and switched to SPS due to the dislocation or dysfunction of DS. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19, and double inlet left ventricle in 18 patients. At the time of the initial procedure, PA index (p=0.43), right PA index (p=0.81), and left PA index (P=26) were similar between the groups. The rate of reaching stage II palliation was similar between the groups (87.2 vs. 82.4%, p=0.50). At stage II palliation, the PA index (p=0.73), right PA index (p=0.32), left PA index (p=0.633), and symmetry index (p=0.83) were similar between the groups (Figure). However, the ratio of left to right PA index was lower in DS group compared to SPS group (p=0.02). At stage III Fontan completion, PA index (p=0.50), right PA index (p=0.61), left PA index (p=0.12), symmetry index (p=0.32) and the ratio of left to right PA index (p=0.06) were similar between DS and SPS group. The incidence of the development of aortopulmonary collaterals (28.6 vs. 48.3%, p=0.08) and venovenous collaterals (21.4 vs. 10.3%, p=0.17) were also similar between DS and SPS group.
Conclusions:
After successful ductus stenting in infants with univentricular heart, the development of pulmonary arteries is comparable to those after surgical systemic to pulmonary shunt. The impaired left PA development in the long-term might be a concern after ductus stent.

Authors
Dimitrij Grozdanov (1), Takuya Osawa (1), Thibault Schaeffer (1), Jonas Palm (2), Paul Philipp Heinisch (1), Alfred Hager (2), Peter Ewert (2), Jürgen Hörer (1), Masamichi Ono (1)
Institutions
(1) German Heart Center Munich, Munich, Germany, (2) Department of Pediatric Cardiology and Congenital Heart Disease, Munich, Germany 

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Abstract Presenter

Dimitrij Grozdanov, German Heart Center Munich  - Contact Me Munich
Germany

143. Burden of Reintervention after Tetralogy of Fallot Repair: A joint pediatric and adult congenital experience over 30 years.

Total Time: 7 Minutes 
Objective: The burden of reintervention (surgical and catheter-based) is high for patients with Tetralogy of Fallot (TOF). We report the impact of type of index surgery on late outcomes in our TOF cohort over a 30-year period.

Methods: Patients with index TOF repair with follow up at our adult center between 1990 and 2022 were included in our study (n=1,239). TOF with absent pulmonary valve and TOF with pulmonary atresia with or without major aortopulmonary collaterals were excluded. Descriptive statistics and survival analysis were performed, with comparison of reinterventions made between patients receiving a transannular patch (TAP, n= 551) or a pulmonary valve sparing repair (VSR, n=647) using cumulative incidence frequencies.

Results: Sixty percent of patient were male (736/1239) with 71% (878/1239) of the cohort under the age of 18. A staged repair was performed in 14% (178/1239), with 62% undergoing a TAP repair. Overall survival of the cohort was 97% at 15 years and 96% % at 25 years. Analysis of index surgical repair approach showed similar overall survival at 25 years between TAP and VSR (p=0.26), while need for major reinterventions at 25 years was overall higher in the TAP cohort (62% TAP vs 31% VSR, p<0.001), with 22.8% undergoing 2 reinterventions and 12% undergoing >2 reinterventions. Isolated placement of pulmonary artery stents was higher in TAP cohort at 15 years (10% TAP vs 4% VSR) and 25 years (19% TAP vs 10% VSR) post index repair (p<0.001). Children who had a VSR repair had higher incidence of RVOT reoperation within the first 10 years post index repair compared to the TAP repair (6.1% vs 3.3 %; p=0.047). At the time of discharge after index repair, 25% (159/647) of children with VSR repair had ≥ moderate pulmonary regurgitation. In the overall cohort, 15.8% had replacement of their pulmonary valve (PVR) at 15 years, while 43% had a PVR by 25 years follow up. PVR was higher after TAP repair at 25 years following the index operation (53% TAP vs 26% VSR, p< 0.001, Figure 1).

Conclusion: The burden of reintervention remains high in TOF population. The index surgical repair approach did not impact overall survival but resulted in higher need for reintervention for those receiving a TAP repair. Echocardiography and MRI imaging data will be integrated into propensity matching studies and risk factor analysis to understand patient characteristics contributing to reintervention burden over the lifespan.

Authors
Shouka Parvin Nejad (1), Crystal Tran (1), Adriana Goraieb (1), Gazelle Halajha (1), Sangkavi Kuhan (1), Chun-Po Steve Fan (2), Sudipta Saha (2), David Barron (1), Erwin Oechslin Oechslin (3), Lee Benson (1), Rachel Vanderlaan (1)
Institutions
(1) Hospital for Sick Children, Toronto, ON, Canada, (2) Peter Munk Cardiac Centre, University Health Network, Toronto, ON, Canada, (3) Toronto General Hospital, Toronto, ON, Canada 

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Abstract Presenter

Shouka Parvin Nejad, Hospital for Sick Children  - Contact Me ON 
Canada

144. Role of Modified Right-Ventricular Overhaul Procedure in Pulmonary Atresia with Intact Ventricular Septum: Promising Effect on Growth of Right Ventricle Based on Magnetic Resonance Imaging Study

Total Time: 7 Minutes 
Objective: Pulmonary atresia with intact ventricular septum(PA-IVS) has a broad spectrum of treatment strategies ranging from single ventricle palliation to biventricular repair. We aimed to evaluate the clinical outcomes of patients who underwent our modified RV overhaul(mRVoh) procedure.
Methods: We conducted a retrospective review of 25 cases in 23 patients with PA-IVS without RV-dependent coronary circulation who underwent the mRVoh procedure between 2008 and 2023. The operative strategy of the mRVoh procedure for PA-IVS included wide resection of hypertrophied infundibular and trabecular muscle with peeling off fibrotic endocardial tissue in the RV cavity, surgical pulmonary valvotomy and tricuspid valve(TV) repair. In the neonatal and young infant patients, we performed B-T shunt or temporary PDA banding simultaneously and considered PDA stenting depending on the antegrade pulmonary flow after the mRVoh procedure. The z-scores of TV and PV annulus, as well as valve function, were assessed by echocardiography. RV function and volume index were measured by magnetic resonance imaging(MRI).
Results: The median age and body weight at the mRVoh procedure were 7.0months and 7.6kg, respectively. Seven were neonates and three were young infants(< 3months). Twenty underwent intervention or surgery for PA-IVS before the mRVoh procedure. Four finally underwent 1.5-V repair; of these, three underwent 1.5-V repair before the mRVoh procedure. There were no early- and late mortalities. Three patients required reoperation(re-do mRVoh procedure in two and TV repair in one). During the median follow-up of 2.25years, the median TV annular z-score tended to increase from -2.53 to -1.01, and tricuspid regurgitation grade improved significantly(1.5 vs.1.0,p=0.041) before and after the mRVoh procedure. In the subgroup study of ten who underwent two MRIs at median intervals of 2.8years after the mRVoh procedure, MRI studies showed that the median RVEDVI (49.2[IQR,33.3-62.8]vs.87.1[IQR,69.4-111.2],p=0.005), RVESVI (19.6[IQR,14.6-35.0]vs.42.2[IQR,33.0-50.7], p=0.005), and RV cardiac output index(2.8[IQR,2.1-3.4]vs.4.1[IQR,3.13-4.65],p=0.007) had significantly increased, respectively.
Conclusions: We observed the effective growth of RV, including TV annulus, with acceptable PV function after mRVoh which ultimately could lead to 2-V repair. Our results showed that mRVoh procedure might be considered as feasible option in terms of RV growth in selected patients with PA-IV

Authors
Jae Hong Lee (1), Woong-Han Kim (2), Hye Won Kwon (2), Sungkyu Cho (3), Jae Gun Kwak (4)
Institutions
(1) Pusan National University Yangsan Hospital, Yangsan-si, (2) Seoul National University Children's Hospital, Seoul, (3) Seoul National University Children's Hospital, SEOUL, (4) Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul, 

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Abstract Presenter

Jae Hong Lee, Seoul National University Children's Hospital  - Contact Me Seoul, Jongno-gu 
South Korea

145. Ventricular Septation for Double Inlet Ventricle – Avoiding Conduction Injury

Total Time: 7 Minutes 
Objective: Ventricular septation is an alternative to Fontan in patients with double inlet left or right ventricle (DIV) but carries risk of complete heart block (CHB). This study describes our experience with ventricular septation and intraoperative conduction mapping.

Methods: Patients with DIV undergoing ventricular septation from 2017-2023 were identified. Conduction mapping was performed on the open, decompressed, beating heart to identify the His bundle (HB). Outcomes analyzed were CHB frequency, transplant-free survival, and freedom from reintervention.

Results: Ventricular septation was performed in 31 patients (25 DILV, 6 DIRV). Staged ventricular septation was performed in 27 patients. Seven progressed to complete septation and 1 underwent Fontan. Single-stage complete septation was performed in 4 patients. Twenty-five (83%) underwent conduction mapping during septation. Median mapping time was 7 minutes (range 1-18). Among 19 mapped DILV patients, the HB was localized to the region between the atrioventricular (AV) valves in 7 patients (4 superior, 2 inferior, 1 mid-inlet) and along the bulboventricular foramen (BVF) in 12 patients (7 anterior-superior margin, 5 anterior-inferior margin). All 5 mapped DIRV patients had conduction localized between the AV valves (3 inferior, 2 mid-inlet). Four patients required a permanent pacemaker (PPM) for CHB. One of these patients had transient CHB during preoperative cardiac catheterization and at surgery during heart retraction before mapping. Another patient had eventual recovery of AV conduction after PPM implantation. At a median follow-up of 14.5 months (IQR 1.4 months – 2.9 years) no deaths or transplants occurred. Late reoperations included resynchronization PPM implantation due to ventricular dyssynchrony (n=1), mitral valvuloplasty (n=3), and resection of subpulmonary outflow tract obstruction (n=2). Latest median oxygen saturation was 95% (IQR 93-98%). In DILV patients, L-looping (OR 7.7, 95% CI 1.6-51.2, P = 0.035) and L-malposed great vessels (OR 7.5, 95% CI 1.4-40.2, P = 0.019) were associated with conduction near the BVF on univariate regression analysis.

Conclusions: Ventricular septation can be performed safely in a subset of DIV patients. Conduction location can vary depending upon underlying anatomy. Intraoperative conduction mapping is a valuable adjunct that can guide surgical technique, including VSD patch positioning and suture placement, in order to avoid conduction injury.

Authors
Eric Feins (1), Ajami Gikandi (1), Jocelyn Davee (1), Edward O'Leary (1), Elizabeth DeWitt (1), Sunil Ghelani (1), Rebecca Beroukhim (1), Pedro del Nido (1), Sitaram Emani (1)
Institutions
(1) Boston Children's Hospital, Boston, MA 

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Abstract Presenter

Eric Feins, Boston Children's Hospital  - Contact Me Boston, MA 
United States

146. Delineating the Midterm Outcomes and Impacts of the Turbo Glenn

Total Time: 7 Minutes 
Objective: Studies have evaluated factors that enhance or derail progression through the single ventricle palliation pathway. The impacts of placing Blalock-Taussig-Thomas (BTT) shunts following superior cavopulmonary connection (SCPC), in patients who require additional pulmonary blood flow, remain to be delineated. This study evaluates the midterm outcomes of BTT shunts following SCPC, informally referred to as the turbo Glenn, on progression to Fontan circulation and transplant-free survival.

Methods: This is a retrospective analysis that selected single ventricle patients from 2004-2022 who had undergone a Norwood procedure and progressed to stage 2 palliation with SPCP. We compared isolated SCPC to SCPC followed by BTT shunt (turbo Glenn) in the early postoperative period. Primary outcomes were achievement of Fontan circulation and transplant-free survival. Secondary outcomes were the incidence of postoperative complications, timing of turbo Glenn, and length of hospital stay. Patients who underwent initial hybrid operations with comprehensive stage 2 palliation were excluded.

Results: 154 patients were included with 14 (9.1%) turbo Glenns and 140 (90.9%) standard SCPC. Cardiopulmonary bypass times were longer in patients (66.9 ± 32.6 vs 91.9 ± 45.9 minutes, p=0.04) who ultimately required a turbo Glenn and 100% of the turbo Glenns were unplanned reoperations. The median time to turbo Glenn following SCPC was 2 days [IQT 0.5, 3.8]. There were similar interstage durations, preoperative oxygen saturations (SpO2), inhaled oxygen requirements, pulmonary artery z-scores, and pulmonary to systemic ratios between the groups (all, p>0.05). However, postoperative SpO2 was substantially lower in the turbo Glenn group with higher inhaled nitric oxide requirements (all, p<0.05). There were higher rates of SCPC take-down in the turbo Glenn group (21.4% vs 5.0%, p=0.04). Yet, the incidence of all complications were similar (all, p>0.05). The rates of mortality, heart transplantation, and progression to Fontan circulation were also similar (all, p>0.05). However, hospital LOS was longer in the turbo Glenn group (10 [IQR 6, 19] vs 19.5 [14.5, 37] days, p=0.009.

Conclusion: When compared to patients who undergo isolated SCPC, a turbo Glenn, in patients who require additional pulmonary blood flow, is a viable option for early postoperative rescue with similar rates of progression to Fontan circulation, Glenn takedown, and transplant-free survival.

Authors
Laura Seese (1), Carlos Diaz Castrillon (2), Melita Viegas (3), mario castro-medina (3), Luciana Da Fonseca da Silva (4), Jose Da Silva (4), Victor Morell (2)
Institutions
(1) University of Pittsburgh Medical Center, Pittsburgh, PA, (2) Children's Hospital of Pittsburgh, Pittsburgh, PA, (3) N/A, Pittsburgh, PA, (4) UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA 

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Abstract Presenter

Laura Seese, University of Pittsburgh  - Contact Me Pittsburgh, PA 
United States

147. Pulmonary Artery Banding for Cardiomyopathy in Young Children: Beyond the Current Experience

Total Time: 7 Minutes 
Objective: This abstract highlights a series of successful single-center cases of PAB procedure, aiming to provide insights and guidance regarding the surgical indications for PAB in PHF.
Methods: We performed PAB for a total of 7 PHF patients. The preoperative conditions of the 7 patients varied, with common features of severe LV dilation (left ventricular end diastolic diameter Z-score greater than 5.0) and preserved RV function.
Results: During follow-up duration, none of the 7 patients encountered all-cause death, heart transplantation, or the need for ventricular assist devices. Three patients successfully regained normal LV function, one came remarkably close to normal, and three patients exhibited substantial improvement.
Conclusions: In cases of DCM combined with CHD and DCM with LBBB, PAB may be a pivotal component of PHF treatment, although they were once considered contraindications. In children with isolated DCM, PAB has also demonstrated notably positive treatment outcomes. The surgical indications for PAB remain a subject of debate and necessitate more cases support.

Authors
Zheng Dou (1), Kai Ma (2), Qiyu He (1), Yuze Liu (1), Shoujun Li (3)
Institutions
(1) N/A, N/A, (2) Fuwai Hospital, Beijing, Beijing, (3) Fuwai Hospital, CAMS&PUMC, Beijing, Beijing 

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Abstract Presenter

Zheng Dou, Fuwai Hospital of Chinese Academy of Medical Sciences  - Contact Me Beijing, Beijing 
China

148. The long-term impact of the tricuspid valve intervention after Fontan completion in patients with single right ventricle

Total Time: 7 Minutes 
Objective: Reveal the long-term impact of tricuspid valve intervention on survival after Fontan completion.
Methods: A single-center retrospective cohort study was conducted with patients who underwent Fontan completion from 1985 through 2017. Data was obtained by chart review, and transplant and survival outcomes were linked to the scientific registry of transplant recipients and the national death index. There were a total of 675 morphological single right ventricle patients. Among them, 128 patients underwent TV intervention at any stage. The primary outcome was transplant- and Fontan takedown-free survival, which was compared between the non-TV intervention (NTV) and TV intervention (TVi) groups. Sub-analysis was performed for patients requiring repeat TV interventions.
Results: Median follow-up was 8.8 (±7.6) years. Of the overall cohort of 675 patients, 487 had HLHS (72%) and 66 had heterotaxy (10%). Among the 128 TV intervention patients, 28 patients underwent repeat TV intervention (22%). Fifty-five (43%) patients underwent TV intervention before Fontan (40 - at stage 2, 15 - during interstage period), 60 (47%) patients at the time of Fontan completion, and 13 (10%) patients after Fontan. The NTV and TVi had no significant differences in background characteristics except for TV insufficiency before Fontan completion (NTV 8.4% vs. TVi 53.9%, P<0.001). There were no significant differences in early mortality after Fontan (NTV 6% vs. TVi 8.6%, P=0.32) nor prolonged chest tube drainage after Fontan (P=0.81). Overall transplant- and Fontan takedown-free survival was 62.5% at 20 years (Fig.1). There was no significant survival difference between NTV and TVi (NTV 76% and TVi 72% at 15 years, P=0.06). However, repeat TV interventions showed an inferior survival outcome compared to the NTV group (NTV 76%, vs. TVi 73%, vs. repeat TVi 56% at 15 years, P=0.03, HR 1.41). Follow-up echo data showed 34 (35%) patients in TVi and 14 (50%) patients in repeat TVi had more than moderate TV regurgitation.
Conclusions: Although repeat TV intervention was associated with inferior survival outcomes, successful TV intervention can provide comparable long-term survival outcomes to non-TV intervention patients undergoing staged single ventricle palliation.

Authors
Yuriy Kulyabin (1), Ilya Soynov (1), Nataliya Nichay (1), Timothy Lancaster (2), Vikram Sood (2), Jennifer Romano (2), Richard Ohye (2), Jiyong Moon (2)
Institutions
(1) E.Meshalkin National Medical Research Center, Novosibirsk, Russia, (2) C. S. Mott Children's Hospital, Ann Arbor, MI 

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Abstract Presenter

Jiyong Moon, Michigan Medicine - University of Michigan  - Contact Me Ann Arbor, MI 
United States

149. Alarming rate of liver cirrhosis after the small conduit Extracardiac Fontan. A comparative analysis with the Lateral Tunnel.

Total Time: 7 Minutes 
Objective: Liver cirrhosis is a well-recognized complication following the Fontan procedure. However, the association between the prevalence of cirrhosis and the types of Fontan operations remains unclear.

Methods: We conducted a retrospective chart review of 332 patients who underwent the Fontan procedure at our institution between November 1989 and July 2021. Four patients who underwent the atriopulmonary connection Fontan were excluded from the analysis. Patients who had intracardiac-extracardiac conduit (126) were pooled with those having extracardiac conduit (ECC) (134) as the conduit went from the IVC to the pulmonary arteries. The 260 patients who underwent the ECC and the 68 who had the lateral tunnel (LT) Fontan constitute the core of the study. Diagnosis of liver cirrhosis was based on histopathology from liver biopsy or elastography.

Results: Patients who underwent the LT and the ECC Fontan shared the same characteristics. Median age at the Fontan procedure was 23.7 months (interquartile range [IQR]: 20.8-32.6) in the LT group, compared to 28.8 months (IQR: 24.6-39.5) in the ECC group (p<0.01). F
enestration rates were 96% (65) in the LT group and 84.6% (220) in the ECC group. The conduit size was reported in 258 patients: 10 mm (1); 12 mm (2); 14 mm (25);16 mm (187); 18 mm (9); 19 mm (24) and 20 mm (10). The median follow-up was 14.8 years (IQR: 12.5-16.5) in the lateral tunnel group and 7 years (IQR: 2.8-10.4) in the extracardiac conduit group. During the follow-up period, two patients (2.9%) with LT and 15 (5.8%) in the with ECC (9 patients with 16 mm or less conduit size) were diagnosed with cirrhosis. The prevalence of cirrhosis at 1, 5, 10, and 15 years was 0%, 0%, 0%, and 2.4% in the lateral tunnel group, respectively, and 0%, 0.9%, 7.6% and 26.8% in the extracardiac conduit group (p<0.01 Figure). Rates of mortality, Fontan revision, Fontan takedown, and transplant were comparable between the two groups.

Conclusions: The Extracardiac Conduit Fontan seem to be associated with faster development of cirrhosis, especially in patients implanted with small conduits.

Authors
Eiri Kisamori (1), Alyssia Venna (1), Rahul Rajeev (1), Manan Desai (1), Aybala Tongut (1), Rittal Mehta (1), Sarah Clauss (1), Can Yerebakan (1), Yves d’Udekem (1)
Institutions
(1) Children's National Hospital, Washington, DC 

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Abstract Presenter

Eiri Kisamori, Children's National Hospital  - Contact Me Washington, DC 
United States

150. Unveiling the Complete Medical Burden of Hypoplastic Left Heart Syndrome: 18 Years of Insight from Childhood to Adolescence

Total Time: 7 Minutes 
Objective: While numerous studies view Hypoplastic Left Heart Syndrome (HLHS) through the lens of financial burden, an overall sense of total medical burden which includes total number of visitations, imaging, and encounters, are not part of the contemporary burden debate. We sought to describe a more complete analysis of burden from childhood to adolescence for a patient with HLHS to capture a more comprehensive view of the journey.
Methods: All children (≤ 18 years) with births from 2004-2022 were identified in the Pediatric Health Information System (PHIS). ICD-9/-10 codes were used for diagnosis of HLHS. Follow-Up was set at one, five, ten, fifteen and eighteen years of age.
Results: 18134 children were identified. 11239 were followed to 1 year of age (yo), 6194 to 5 yo, 2846 to 10 yo, 866 to 15 yo, and 151 to 18 yo. A 1 yo child spends a mean of 149 ± 221 days in hospital, has 27 ± 38 hospital encounters, and 147 ± 126 radiological exams (X-Rays: 74 ± 75, Ultrasound: 62 ± 54); total hospital cost ($) was 778214 ± 867389 (Inpatient: 713555 ± 843138) and imaging cost of 40981 ± 39766 (X-Ray: 7598 ± 7273). A 10 yo child spends a mean of 191 ± 313 days in hospital, has 46 ± 53 hospital encounters and 158 ± 123 radiologic exams (X-Rays: 77 ± 70, Ultrasound: 68 ± 50); total hospital costs were 756532 ± 731301 (Inpatient: 656230 ± 677488) and imaging costs of 43539 ± 43990 (X-Ray: 7676 ± 6575). An 18 yo child spends a mean of 223 ± 268 days in hospital, has 70 ± 81 hospital encounters and 187 ± 132 radiological exams (X-Rays: 93 ± 86, Ultrasound: 76 ± 60); total hospital cost ($) was 979964 ± 892171 (Inpatient: 838568 ± 800221) and imaging cost of 46323 ± 36405 (X-Rays: 8651 ± 7043).
Conclusion: HLHS represents a major burden for both children & family and rises throughout the patient's life. The present report offers a more comprehensive insight into the full medical burden at different ages and can give providers more information to help describe to patients and parents the journey in front of them.

Authors
Amir Mehdizadeh-Shrifi (1), Muhammad Faateh (1), Kevin Kulshrestha (1), Haleh Heydarian (1), Russel Hirsch (1), Monir Hossain (2), Spencer Hogue (1), Awais Ashfaq (1), David Morales, Sr. (1)
Institutions
(1) The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, (2) Division of Biostatistics and Epidemiology, Cincinnati Children’s Hospital Medical Center, Cincinnat, Cincinnati, OH 

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Abstract Presenter

Amir Mehdizadeh-Shrifi, Cincinnati Children's Hospital Medical center  - Contact Me Cincinnati, OH 
United States