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Background: Total anomalous pulmonary venous connection (TAPVC) with a functional single ventricle is a risk factor for mortality during staged palliation. This study aimed to assess TAPVC's impact on staged palliation outcomes., Methods: In a total of 602 patients with a functional single ventricle who underwent stage 1 palliation (S1P) at our center between 2001 and 2020, 39 (6.5%) patients were associated with TAPVC. Median age at S1P was 12.0 (interquartile range, 7-21) days with a body weight of 3.1 (interquartile range, 2.8-3.6) kg. Outcomes during staged palliation were compared with the remaining 563 patients without TAPVC. Risk factors for mortality were identified using a Cox proportional hazards regression model., Results: Primary diagnosis in functional single-ventricle patients with TAPVC included hypoplastic left heart syndromes (n = 13), unbalanced atrioventricular septal defects (n = 12) tricuspid atresias (n = 2), double inlet left ventricle (n = 1), and others (n = 11). Types of TAPVC were supracardiac (n = 21), cardiac (n = 10), infracardiac (n = 6), and mixed (n = 2). Pulmonary venous obstruction (PVO) was associated in 19 (49%) patients. S1Ps included Norwood (n = 13), aortopulmonary shunt (n = 21), and pulmonary artery banding (n = 5). Thirty-day mortality after S1P was significantly increased in patients with TAPVC vs without TAPVC (43.6% vs 16.3%; P < .001). After bidirectional cavopulmonary shunt and total cavopulmonary connection procedures, mortality was low in both groups, and no statistically significant differences were found. Correction of TAPVC at the time of S1P was not found to be a significant risk factor in univariable Cox regression analysis. In univariate and multivariate analysis, PVO was identified as an independent risk factor for mortality in patients with TAPVC (P < .001)., Conclusions: Overall survival is lower in TAPVC single-ventricle patients than in non-TAPVC patients. Most deaths after S1P were associated with TAPVC, but not after S2P. PVO is a mortality risk factor in TAPVC patients., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: This study was intended to determine the impact of extracardiac anomalies on outcomes in patients with functional single ventricle who underwent staged palliation., Methods: We reviewed medical records of patients who underwent first-stage palliation at our center between 2001 and 2020. The prevalence and type of extracardiac anomalies were evaluated, and their impact on outcomes during staged palliation was analyzed., Results: Among 602 patients who underwent first-stage palliation, 81 (14%) patients had associated with extracardiac anomalies. They were more frequently associated with prematurity (P = .03) and low birth weight below 2.5 kg (P < .01). Mortality between first-stage palliation and stage II was similar in patients with and without extracardiac anomalies (24.7% vs 17.1%, P = .10). However, mortality between stage II and stage III was significantly higher in patients with extracardiac anomalies compared with those without (22.2% vs 12.5%, P = .02). Mortality after stage III was also higher in patients with extracardiac anomalies compared with those without (4.9% vs 1.5%, P = .04). In the subgroup analysis of 81 patients with extracardiac anomalies, renal anomalies were identified as a significant risk factor for mortality (P = .03, hazard ratio 2.44)., Conclusions: The incidence of extracardiac anomalies in this study was 14%, and patients with extracardiac anomalies were highly associated with prematurity and low birth weight. Presence of extracardiac anomalies was associated with higher mortality between stage II and stage III palliation and after stage III phase, but not before stage II. Among extracardiac anomalies, renal anomalies were identified as a risk factor for mortality., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: This study sought to determine long-term outcomes of a primary Damus-Kaye-Stansel (DKS) procedure in patients with a functional single ventricle and to compare the results with those of our historical control subjects who underwent pulmonary artery banding before the DKS procedure., Methods: The study reviewed the medical records of all patients who underwent the DKS procedure at the German Heart Center of Munich, Germany between December 1994 and December 2019., Results: The DKS procedure was performed as initial palliation in 52 patients (primary DKS group) and as staged palliation after pulmonary artery banding in 24 patients (staged DKS group). The median follow-up period after the DKS procedure was 8.9 years in the primary DKS group and 8.0 years in the staged DKS group. The survival rates at 10 years after the DKS procedure were 89% in the primary DKS group and 68% in the staged DKS group (log-rank P = 0.04). Before total cavopulmonary connection, the pressure gradient through the systemic ventricular outflow tract was significantly lower in the primary DKS group than in the staged DKS group (P < .001). At last follow-up echocardiography, reduced ventricular function was observed in 1 patient in the primary DKS group and in 7 patients in the staged DKS group (P < .001). The degree of neoaortic regurgitation was significantly higher in the staged DKS group than in the primary DKS group (P < .001)., Conclusions: A primary DKS procedure in patients with a functional single ventricle and potential systemic ventricular outflow tract obstruction is recommended to obtain favorable long-term survival with preserved ventricular function and competent semilunar valve function., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Our aim was to evaluate the results of stage 2 palliation by means of bidirectional cavopulmonary shunt (BCPS) and to identify risk factors for failed Fontan completion., Methods: Between 1998 and 2018, BCPS was performed on 525 patients with functional single ventricle. Patient demographics, surgical data, and echocardiographic and cardiac catheterization measurements were analyzed, and outcomes after BCPS were evaluated., Results: The median age at BCPS was 4.7 months (interquartile range, 3 to 7.4). Hypoplastic left heart syndrome was the most frequent diagnosis. The median follow-up after BCPS was 3.4 years (interquartile range, 1.5 to 8.7); 407 patients underwent the Fontan procedure, 50 were waiting for Fontan completion, 47 died, 6 were considered not suitable for Fontan completion, and 15 were lost to follow-up. Of the 407 patients who underwent Fontan completion, there were 5 early deaths. Freedom from failed Fontan completion (mortality before, unsuitability for, and early mortality after the Fontan procedure) at 1, 2, and 3 years was 91.9%, 87.3%, and 86.1%, respectively. Risk factor analysis for failed Fontan completion revealed hypoplastic left heart syndrome (hazard ratio [HR] 4.1, P = .001), unbalanced atrioventricular septal defect (HR 10.1, P < .001), higher pulmonary artery pressure (HR 1.1, P = .040), and reduced ventricular function (HR 4.2, P = .001) as risks., Conclusions: Stage 2 palliation can be performed with minimal risk and provides excellent subsequent Fontan completion. Hypoplastic left heart syndrome, unbalanced atrioventricular septal defect, high pulmonary artery pressure, and reduced ventricular function at the time of BCPS were identified as risk factors for failure to successfully complete the Fontan procedure., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Mitral valve (MV) repair in children is challenging because of the broad spectrum of lesions and anticipated patient growth. The purpose of the study was to evaluate the outcome of MV repair in children below 10 years of age., Methods: We reviewed all MV repair procedures performed in children below 10 years of age. Endpoints of the study were survival after MV repair and cumulative incidence of reoperation., Results: MV repair was performed in 40 patients with congenital MV disease (MVD) and in 10 patients with acquired MVD. Median age at time of repair for congenital MVD was 1.2 years (range, 14 days to 9.8 years) and for acquired MVD 1.9 years (range, 10 days to 9.9 years). Indication for MV repair was mitral regurgitation in 31 congenital MVD patients (77.5%) and in all acquired MVD patients. In patients with congenital MVD operative mortality was 5% and late mortality was 10%. No deaths occurred in patients with acquired MVD. Patients with congenital mitral regurgitation showed a better, yet not significant, 6-year survival than patients with congenital mitral stenosis (85.3% ± 8.2% vs 60% ± 18.2%, P = .1). In patients with congenital MVD cumulative incidence of reoperation at 6 years was 38.6% ± 8.3%., Conclusions: In children below 10 years of age, MV repair is an effective treatment option for MVD. However it often just delays the time to valve replacement., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The development of recoarctation after the Norwood I procedure is a known complication in patients with hypoplastic left heart syndrome (HLHS)., Methods: We reviewed data on 194 consecutive patients with HLHS who underwent the Norwood I procedure between 2000 and 2015. All patients who survived until the second stage of the procedure were included. Recoarctation was defined by the need for intervention, either catheter based or surgical. The aim of our study was to determine if the patch material used for the initial arch reconstruction had an influence on the development of recoarctation., Results: The study population consisted of 145 patients. The patch material used for aortic arch reconstruction was either a homograft (n = 87), autologous pericardium (n = 23), equine pericardium (n = 28), or other material (n = 7). Recoarctation was documented in 27 patients (18%) at a median time of 4.3 months (range, 1 to 28 months) after completion of Norwood I. Freedom from recoarctation was 85% ± 4%, 86% ± 7%, and 30% ± 22% at 2 years for homograft, autologous pericardium, and equine pericardium, respectively. Thirty-six percent of patients with equine pericardium exhibited recoarctation, significantly more than homograft patch (p < 0.01) or autologous pericardium (p = 0.002). Treatment for recoarctation was percutaneous balloon angioplasty in 19 patients, stent implantation in 3, and operative enlargement of the aortic arch in 5. On univariate analysis, arch reconstruction with equine pericardium was the only risk factor for recoarctation (hazard ratio, 6.0; 95% confidence interval, 2.6-14; p < 0.01)., Conclusions: The patch material used for reconstruction of the aortic arch in HLHS influences the rate of recoarctation following the Norwood I procedure. Equine pericardium cannot be recommended., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: After the Fontan-Björk modification for tricuspid atresia, some patients show pulsatile systolic pulmonary flow. We compared the hemodynamic findings and the clinical presentation of patients with and without pulsatile systolic flow after atrioventricular connection., Methods: According to the pulmonary flow pattern by pulsed-wave Doppler assessment of transthoracic echocardiography, 41 patients after atrioventricular connection were divided into two groups: patients who showed dominant pulsatile systolic pulmonary flow (group P, n = 11), and patients who did not (group N, n = 30)., Results: Mean follow-up time was 27.8 ± 4.7 years in group P and 25.3 ± 3.8 years in group N (p = 0.1). Patients in group P had significantly less frequently catheter ablation procedures for tachyarrhythmia (9% versus 50%, p = 0.03). No patient in group P had had cardiac decompensation, whereas 7 patients (23%) in group N had had an episode of cardiac decompensation (p = 0.08). Cardiopulmonary exercise testing revealed that patients in group P showed higher oxygen uptake compared with patients in group N (25.0 ± 7.3 versus 19.6 ± 6.0 mL · kg(-1) · min(-1), p = 0.03). Patients in group P showed higher systolic pulmonary artery pressure (21.3 ± 8.4 versus 16.8 ± 4.5 mm Hg, p = 0.05), higher right ventricular end-diastolic volume index (88.6 ± 30.2 versus 50.3 ± 28.5 mL · L(-1) · m(-2), p = 0.03), and higher right ventricle to left ventricle ratio of end-diastolic volume index (1.4 ± 0.6 to 0.7 ± 0.3, p = 0.01)., Conclusions: Patients with pulsatile systolic flow in the pulmonary artery had better hemodynamic and better exercise performance compared with patients without pulsatile systolic flow after atrioventricular connection. A sufficient volume and function of the right ventricle is a prerequisite to create pulsatile systolic flow., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: With interrupted aortic arch (IAA), a direct anastomosis may produce an angular-shaped aortic arch instead of the normal arc-shaped aorta, when the discontinuity is considerably long. That may lead to aortic stenosis and to compression of the pulmonary artery or the main bronchus. If a tube graft is used, reoperation for graft exchange is inevitable. We demonstrate the results of using the subclavian artery for creating an arc-shaped aorta in IAA repair., Methods: Between February 2006 and October 2012, 23 patients underwent IAA repair using the subclavian artery. The subclavian artery was closed distally, divided, and longitudinally incised from the transected end to the aorta. This flap was used to bridge the gap of the IAA, by forming the posterior wall of a new aortic segment. The arch was completed using glutaraldehyde-fixed autologous pericardium (52%) or homograft (48%)., Results: Median follow-up time was 4.8 years (range, 1.3 to 6.9). There were no early deaths and 1 late death. On postoperative angiographic imaging, the aorta takes an arc-shaped course in all cases. Aortic arch stenosis developed in 7 patients (30%). Four patients were treated interventionally, and 3 surgically. During follow-up, there was no compression of the pulmonary artery or the main bronchus., Conclusions: By using the subclavian artery, an arc-shaped aorta can be accomplished without the use of tube grafts. With this technique, compression of the pulmonary artery or the main bronchus can be avoided. This technique is recommended if a direct anastomosis might be not applicable to bridge a long interruption., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)