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Systemic ventricular outflow obstruction progresses after the Fontan operation
K M Finta1, R H Beekman, F M Lupinetti
1Division of Pediatric Cardiology, C. S. Mott Children's Hospital, University of Michigan, Ann Arbor.
Insights
Systemic ventricular outflow obstruction is a common and progressive complication following the Fontan operation. Early identification and monitoring are crucial for managing this condition in pediatric cardiac surgery patients.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Cardiac Physiology
Background:
- The Fontan operation is a palliative procedure for complex single-ventricle congenital heart defects.
- Systemic ventricular outflow obstruction (SVOO) is a potential complication after cardiac surgeries.
- Long-term outcomes and progression of SVOO post-Fontan require further evaluation.
Purpose of the Study:
- To assess the incidence and natural history of systemic ventricular outflow obstruction after the Fontan procedure.
- To identify risk factors associated with the development of SVOO post-Fontan.
- To understand the progression of SVOO in survivors of the Fontan operation.
Main Methods:
- Retrospective review of medical records for 57 hospital survivors of the Fontan operation.
- Categorization of patients into those with (Group 1) and without (Group 2) ventricular outflow obstruction.
- Comparison of demographic, surgical, and echocardiographic data between groups.
Main Results:
- Systemic ventricular outflow obstruction was identified in 12% of patients (7 of 57).
- In Group 1, outflow gradients significantly increased from pre-Fontan (6.3 mmHg) to 28 months postoperatively (80.1 mmHg).
- Patients with SVOO were more likely to have an aorta arising from a hypoplastic ventricle and prior pulmonary artery banding.
Conclusions:
- Systemic ventricular outflow obstruction is a common (12% incidence) and progressive complication after the Fontan procedure.
- Prior pulmonary artery banding and aortic origin from a hypoplastic ventricle are associated with SVOO post-Fontan.
- The progressive nature of SVOO necessitates vigilant monitoring in Fontan survivors.
Abstract:
To evaluate the course of systemic ventricular outflow obstruction after the Fontan operation, the records of 57 hospital survivors of that procedure were reviewed. Ventricular outflow obstruction was identified in 7 patients (group 1) and was absent in 50 patients (group 2). Overall, the ventricular outflow gradient in group 1 was 6.3 +/- 2.9 mm Hg (mean +/- standard error) before the Fontan operation and 7.6 +/- 3.9 mm Hg at hospital discharge. Ventricular outflow obstruction subsequently progressed to 80.1 +/- 17.3 mm Hg (range, 33 to 165 mm Hg; p < 0.02) a mean of 28 months postoperatively. One patient died of severe progressive ventricular outflow obstruction. Group 1 did not differ from group 2 in age, ventricular morphology, presence of a subaortic outflow chamber, prior shunt, or length of follow-up. Compared with group 2, however, patients in group 1 more commonly had an aorta arising from a hypoplastic ventricle (p < 0.001) and had undergone prior pulmonary artery banding (p = 0.005). We conclude that systemic ventricular outflow obstruction occurs commonly after a Fontan procedure (incidence, 12%; 70% confidence interval, 9% to 18%) and is a progressive lesion.