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Background: Aortic atresia (AA) is considered a risk for the Norwood procedure. This study aimed to compare the longitudinal ventricular function (VF) and atrioventricular valve (AVV) regurgitation in patients with AA and aortic stenosis (AS).
Methods: Using serial echocardiographic images in patients undergoing the neonatal Norwood procedure between 2001 and 2020, systemic VF assessed by ejection fraction and the degree of AVV regurgitation were compared between the patients with AA and AS.
Results: A total of 335 consecutive patients were included, consisting of 273 with hypoplastic left heart syndrome and 62 with its variants. AA was observed in 146 patients (43.6%) and AS in 189 (56.4%). Longitudinal VF and AVV regurgitation were evaluated using a total of 4687 echocardiograms. Preoperatively, VF was better in AA patients than in AS patients (P = .01). After the Norwood procedure, VF was initially (1-30 days) worse in patients with AA than in those with AS (P < .01). However, after that (31 days to stage 2 palliation), it improved in the AA group but remained worse than in the AS group (P < .01). After stage 2 palliation, VF was reduced in the AA group compared to the AS group, especially between 31 to 90 days postoperatively (P < .01). The degree of AVV regurgitation after the Norwood procedure (P < .01) as well as after stage 2 palliation (P < .01), was worse in AA patients, compared with AS patients.
Conclusions: After the Norwood procedure, patients with AA demonstrated inferior systemic VF as well as worse AVV function throughout the palliation period before Fontan completion.
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http://dx.doi.org/10.1016/j.athoracsur.2025.01.014 | DOI Listing |
World J Pediatr Congenit Heart Surg
September 2025
Heart Center, Children's Healthcare of Atlanta; Division of Cardiothoracic Surgery, Department of Surgery, Emory University School of Medicine, Atlanta, GA, USA.
Delayed sternal closure (DSC) is frequently utilized to facilitate the recovery of myocardial function and edema following the Norwood procedure. At our institution, most patients undergo primary sternal closure (PSC), unless specified high-risk characteristics are present. We sought to analyze the outcomes of our approach.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
September 2025
Texas Center for Pediatric and Congenital Heart Disease, The University of Texas at Austin Dell Medical School, Austin, TX, USA.
Pericardial effusion (PCE) represents a significant postoperative complication following congenital heart surgery (CHS), contributing to more complex postoperative care and heightened morbidity. In this study, we aim to elucidate the risk factors contributing to PCE development post-CHS through analysis of data from a nationwide, multi-institutional database. Review of the Pediatric Health Information System Database from January 1, 2004, to December 30, 2023.
View Article and Find Full Text PDFPediatr Transplant
November 2025
D'Or Institute for Research and Education (IDOR), Rio de Janeiro, RJ, Brazil.
Background: Fontan-associated liver disease can progress to advanced fibrosis, raising the potential need for combined heart-liver transplantation (CHLT) in selected patients. However, the benefits of CHLT over isolated orthotopic heart transplantation (HT), particularly in terms of mortality, remain uncertain. In this systematic review, we compared mortality outcomes following CHLT versus HT in patients with Fontan circulation, with the aim of supporting clinical decision-making.
View Article and Find Full Text PDFAngiogenesis
August 2025
First Department of Cardiology, AHEPA University Hospital, Aristotle University of Thessaloniki, St. Kiriakidi 1, 54636, Thessaloniki, Greece.
The Fontan procedure is a definitive surgical approach for complex cardiac malformations, redirecting systemic venous blood into the pulmonary circulation through a staged repair that separates systemic and pulmonary venous returns in the absence of a subpulmonary ventricle. The ensuing unique hemodynamic conditions compromise the endothelial function both in the pulmonary and the systemic circulation. The underlying pathophysiological mechanisms, although distinct within each vascular bed, are interrelated and may collectively contribute to progressive end-organ dysfunction, ultimately accounting for the significant morbidity burden in Fontan patients.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
August 2025
Kirklin Solutions Inc. Birmingham, AL, USA.
The World Database for Pediatric and Congenital Heart Surgery (WDPCHS) was utilized to define the incidence of one-year mortality and identify associated risk factors following surgery for congenital heart disease. The WDPCHS was queried from January 2017 to September 2022 for all tier 1 surgeries submitted to the database. Out-of-hospital death was defined as a death between 30 days postoperatively and up to one year.
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