Background: The Fontan procedure is the accepted standard for single-ventricle palliation. The goal of this study was to determine short- and midterm outcomes of patients undergoing a Fontan operation at a single institution and to identify contemporary risk factors for acute and chronic failure.
Methods: All patients undergoing a Fontan operation between 1995 and 2016 were included. Failure was defined as death, transplantation, Fontan takedown or revision, fenestration creation or enlargement, plastic bronchitis, protein-losing enteropathy, or major perioperative reintervention. Multivariable logistic and Cox regression models were used to identify risk factors for acute (perioperative) and chronic failure (after hospital discharge or 30 days postoperatively, or both).
Results: The cohort included 610 patients. Median age at surgery was 4 years. Median follow-up was 6.8 years. Trends showed increasing use of extracardiac conduits, nonfenestrated Fontan, and extubation in the operating room. Perioperative mortality was 0.5% (n = 3). Transplant-free survival at 5, 10, and 15 years was 97%, 94%, and 92%, respectively; freedom from failure was 91%, 89%, and 87%, respectively. Extubation in the operating room was associated with lower risk of acute failure (odds ratio, 0.30; 95% confidence interval [CI], 0.11 to 0.87). Independent risk factors for chronic failure included genetic syndrome (hazard ratio [HR], 2.54; 95% CI, 1.11 to 5.83), ventricular dysfunction (HR, 3.86; 95% CI, 1.81 to 8.24), cardiopulmonary bypass time in 30-minute intervals (HR, 1.242; 95% CI, 1.100 to 1.402), and persistent pleural effusions (HR, 4.26; 95% CI, 2.25 to 8.07). Moderate or severe atrioventricular valve regurgitation (HR, 2.61; 95% CI, 1.13 to 6.02) and cardiopulmonary bypass time (HR, 1.22; 95% CI, 1.03 to 1.45) were associated with reduced long-term transplant-free survival.
Conclusions: Contemporary midterm outcomes for Fontan patients are reassuring. Lifelong follow-up is mandatory to determine long-term outcomes and need for additional surgery as patients reach adulthood.
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http://dx.doi.org/10.1016/j.athoracsur.2019.05.039 | DOI Listing |
J Clin Med
January 2025
Department of Preventive Cardiology and Lipidology, Medical University of Lodz, 90-419 Lodz, Poland.
The Fontan operation has become the primary palliative treatment for patients with a functionally univentricular heart. The population of patients with Fontan circulation is constantly growing and aging. As the number of Fontan patients surviving into adulthood increases, there is a clear need for research on how best to follow these patients and manage their complications.
View Article and Find Full Text PDFJ Cardiol Cases
January 2025
Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan.
Unlabelled: The incidence of invasive group A Streptococcus (iGAS) infection has been increasing across all age groups, including pediatric patients, and is associated with high mortality rates. Although iGAS infection leads to streptococcal toxic shock syndrome and necrotizing soft tissue infections, iGAS-associated infective endocarditis (IE) is rare. Here, we report a case of iGAS-associated IE, streptococcal toxic shock syndrome, and pyomyositis that occurred after the Fontan procedure in a 7-year-old patient.
View Article and Find Full Text PDFPediatr Cardiol
January 2025
Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, USA.
Prolonged pleural drainage and chylothorax are common in postoperative Fontan patients and are associated with increased morbidity and mortality. Multiple medical and interventional treatment strategies exist and vary between centers. This is a retrospective multicenter observational cohort study of pediatric patients who underwent Fontan operation at 8 pediatric cardiac surgical institutions from 1/1/2019 to 12/31/2021.
View Article and Find Full Text PDFPediatr Cardiol
January 2025
Fetal Heart Program, Division of Cardiology. Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
The IMmediate Postpartum Access to Cardiac Therapy (IMPACT) procedure is a multidisciplinary, collaborative, highly coordinated clinical service in which a planned delivery and intensive neonatal care are offered for conditions where there is a high likelihood of postnatal instability. This process includes prenatal consultation with the parent(s), involving each service engaged with the delivery, postnatal resuscitation, and procedural care. A Cesarean section delivery is planned in an operating room with immediate access to a multifunctional procedural suite where the neonate can undergo rapid cardiac evaluation and initiation of interventional treatments which can have a positive, life-saving impact.
View Article and Find Full Text PDFOpen Heart
January 2025
Department of Cardiology, Rabin Medical Center, Petah Tikva, Israel.
Introduction: Data on the characteristics and outcomes of pregnancy and among patients with Fontan physiology are limited. We aimed to evaluate the immediate and long-term outcomes among these patients who were followed at our centre.
Methods: We included adult patients who had undergone Fontan surgery for congenital heart disease and were pregnant between 1994 and 2021.
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