The purpose of this retrospective study was to determine the outcome of patients who underwent a Fontan procedure at National Heart Institute "Ignacio Chávez", Mexico, from January 1989 to December 2003. We had 81 patients with a mean age of 7 years old: 53 with tricuspid atresia, 14 with pulmonary atresia and intact septum, 11 with univentricular atrioventricular connection and 3 with Ebstein's anomaly. An intra-atrial tunnel was performed on 61 patients and an extracardiac conduit on the rest. Of all, 53 underwent a fenestration. The surgical mortality was 13% for tricuspid atresia and 25% for the other diseases. We had an overall mortality of 28.4%, being the main causes cardiogenic shock and arrhythmia. The global survival was 71% in 55 months of following. Takedown was indicated on 5 patients. The patients without fenestration showed to have 2.8 times more risk of death. The mean pulmonary artery pressure > or = 20 mmHg plus the left atrium pressure > or = 10 mmHg increased the death risk 3.6 times. Of the 52% who required readmission, the main causes were hemodynamical failure and infections. The more relevant complications were: arrhythmia (38%), protein-losing enteropathy (8%) and thrombotic cerebral events (1.4%).
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Ann Thorac Surg Short Rep
June 2024
Great Ormond Street Hospital Biomedical Research Centre and Institute of Cardiovascular Science, University College London, London, United Kingdom.
Background: Given their importance as a metric for health care evaluation, this study's aim was to evaluate the rates of surgical and catheter reinterventions for children with functionally single-ventricle (f-SV) congenital heart disease (CHD) undergoing staged palliation.
Methods: We undertook a retrospective cohort study of children born with f-SV CHD between 2000 and 2018 in England and Wales, using the national registry, with survival ascertained in 2020. Competing risk analysis was used to describe the incidence of additional procedures that occurred first, during follow-up, accounting for competing events of death or transplantation.
Cardiol Young
January 2025
Department of Cardiovascular Surgery, Hacettepe University, Ankara, Turkey.
Background: Ebstein's anomaly represents 40% of congenital tricuspid valve abnormalities. Studies about paediatric Ebstein's anomaly patients are limited.
Aim: To evaluate clinical characteristics, treatment (medical/arrhythmia ablation/surgical) results, and outcome of Ebstein's anomaly patients, and to determine factors affecting arrhythmia presence and mortality.
Cardiol Young
January 2025
Children's Cardiac Centre, Department of Cardiology, Perth Children's Hospital, Perth, WA, Australia.
Introduction: Pulmonary atresia with intact ventricular septum is a rare congenital cardiac lesion with significant anatomical heterogeneity. Surgical planning of borderline cases remains challenging and is primarily based on echocardiography. The aim was to identify echocardiographic parameters that correlate with surgical outcome and to develop a discriminatory calculator.
View Article and Find Full Text PDFDiagnostics (Basel)
December 2024
Zeynep Kamil Women and Children's Diseases Training and Research Hospital, Department of Perinatology, Health Science University, Istanbul 34668, Turkey.
To assess the prenatal course and early postnatal outcomes of fetuses diagnosed with tricuspid atresia and to identify predictors of survival. This was a retrospective study of 25 fetuses diagnosed with tricuspid atresia in a single tertiary referral center, evaluating prenatal echocardiographic features and postnatal outcomes. A total of 4 of 29 initially diagnosed fetuses were excluded due to changes in diagnosis or loss to follow-up, leaving 25 fetuses for analysis.
View Article and Find Full Text PDFEur Heart J Case Rep
December 2024
Department of Cardiology and Catheterization Laboratories, Shonan Kamakura General Hospital, Okamoto 1370-1, Kamakura City, Kanagawa 247-8533, Japan.
Background: In patients with adult congenital heart disease (ACHD), significant atrioventricular valve regurgitation is an important risk factor for poor outcomes, such as heart failure. However, in many cases, transcatheter intervention may reduce the risk profile to avoid a high surgical risk.
Case Summary: A 44-year-old man with complex ACHD in the form of a double-inlet left ventricle, congenitally corrected transposition of the great arteries, pulmonary atresia, atrial septal defect, and patent ductus arteriosus was referred for the treatment of severe tricuspid regurgitation.
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