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Lifelong management of patients with a single functional ventricle: a protocol
D J Fisher1, T Geva, T F Feltes
1Lillie Frank Abercrombie Section of Pediatric Cardiology, Department of Pediatrics, Texas Children's Hospital, Houston 77030, USA.
Insights
Patients with a single functional ventricle face significant health challenges. This study introduces a comprehensive lifelong management protocol to improve outcomes for these complex congenital heart defect patients.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Single functional ventricle is a complex congenital heart defect with high morbidity and mortality.
- Patients often develop dilated cardiomyopathy a decade after modified Fontan repair.
- Existing management strategies are often retrospective and lack comprehensive prospective approaches.
Purpose of the Study:
- To present a comprehensive lifelong management protocol for patients with a single functional ventricle.
- To improve the understanding and long-term outcomes for this patient population.
- To establish a framework for prospective management and data review.
Main Methods:
- Development of a comprehensive lifelong management protocol.
- Regular review and analysis of patient outcomes.
- Prospective approach to managing single functional ventricle patients.
Main Results:
- The study presents an initial protocol for lifelong management.
- The protocol aims to enhance understanding of patient challenges.
- The goal is to improve long-term outcomes through systematic care.
Conclusions:
- A comprehensive, prospective management protocol is crucial for single functional ventricle patients.
- Regular outcome review is essential for refining care strategies.
- This approach holds promise for improving the lifelong health of individuals with single functional ventricles.
Abstract:
Of the wide variety of congenital heart defects, the single functional ventricle continues to be one of the most enigmatic. Compared with patients who have complex congenital heart defects and 2 functional ventricles, patients with a single functional ventricle have greater surgical and long-term morbidity and mortality, and use more medical resources. Recent investigations have shown that patients with a single functional ventricle often have very satisfactory hemodynamics soon after a modified Fontan repair but then develop dilated cardiomyopathy during the subsequent decade. The cause and pathogenesis of the cardiomyopathy have not yet been determined. Many publications have examined individual aspects of the care of these patients based on retrospective reviews, but very few have provided comprehensive prospective methods for managing patients with a single functional ventricle. We hypothesized that a comprehensive management protocol with regular review of the results would provide a better understanding of the problems encountered in these patients and thus improve long-term outcome. Herein, we present our initial protocol for the lifelong management of patients with a single functional ventricle.