Contemporary Outcomes of the Fontan Operation: A Large Single-Institution Cohort
Carlos M Mery1, Luis E De León2, Daniel Trujillo-Diaz2
1Texas Center for Pediatric and Congenital Heart Disease, University of Texas Dell Medical School/Dell Children's Medical Center, Austin, Texas.
Insights
Outcomes for the Fontan procedure are reassuring, with low perioperative mortality and high long-term survival. Key risk factors for chronic failure include genetic syndromes and ventricular dysfunction, necessitating lifelong follow-up.
Area of Science:
- Cardiology
- Pediatric Surgery
- Congenital Heart Disease
Background:
- The Fontan procedure is a standard surgical treatment for single-ventricle congenital heart defects.
- Assessing short- and midterm outcomes and identifying risk factors for failure is crucial for patient management.
Purpose of the Study:
- To evaluate the outcomes of the Fontan operation in a single institution.
- To identify risk factors associated with acute and chronic Fontan procedure failure.
Main Methods:
- A retrospective review of 610 patients who underwent the Fontan procedure between 1995 and 2016.
- Failure was defined by a composite of adverse events including death, transplantation, reoperation, or specific complications.
- Multivariable regression models were employed to determine risk factors for acute and chronic failure.
Main Results:
- Perioperative mortality was low at 0.5%.
- Five-, 10-, and 15-year transplant-free survival rates were 97%, 94%, and 92%, respectively.
- Risk factors for chronic failure included genetic syndromes, ventricular dysfunction, prolonged cardiopulmonary bypass time, and persistent pleural effusions. Early extubation in the operating room was linked to reduced acute failure.
Conclusions:
- Midterm outcomes for the Fontan procedure are generally positive.
- Lifelong patient monitoring is essential to manage long-term outcomes and potential future surgical needs.
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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