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Management of Fontan circulation in pregnancy: a multidisciplinary approach to care
Natasha K Wolfe1, Bethany A Sabol2, Jeannie C Kelly2
1Cardiovascular Division, Department of Internal Medicine, Washington University School of Medicine in St. Louis, St. Louis, MO.
Insights
Women with Fontan circulation face unique pregnancy risks. This review analyzes current literature and outlines a multidisciplinary approach for optimal preconception, pregnancy, and postpartum care for these patients.
Area of Science:
- Cardiology
- Reproductive Medicine
- Pediatric Surgery
Background:
- The Fontan operation, a palliative surgery for single ventricle congenital heart disease, impacts cardiovascular physiology.
- Over 70,000 individuals worldwide have Fontan circulation, with a growing population of women reaching childbearing age.
- Increased Fontan population survival necessitates understanding pregnancy-related risks and management.
Purpose of the Study:
- To review existing literature on pregnancy in women with Fontan circulation.
- To describe a multidisciplinary approach for managing these pregnancies.
- To guide clinical care from preconception through postpartum.
Main Methods:
- Expert review of current literature on Fontan circulation and pregnancy.
- Analysis of cardiovascular challenges in Fontan patients during gestation.
- Description of a center's integrated care model.
Main Results:
- Limited clinical data currently exists for guiding pregnancy management in this population.
- Fontan circulation presents unique maternal and fetal risks due to altered hemodynamics.
- A structured, multidisciplinary approach is essential for optimizing outcomes.
Conclusions:
- Pregnancy in women with Fontan circulation requires specialized, comprehensive care.
- Further research is needed to establish evidence-based guidelines.
- Optimizing preconception, antepartum, intrapartum, and postpartum care is critical for maternal and fetal well-being.
Abstract:
The Fontan operation was first performed in 1968 and is a palliative procedure for children born with single ventricle forms of congenital heart disease. Today, 70,000 patients worldwide have Fontan circulation today, half of them women, and with an expected 30-year survival of >80%, this population is expected to double in the next 20 years. The Fontan operation surgically redirects systemic venous blood return directly to the pulmonary circulation, bypassing the single ventricle. This abnormal anatomy results in significant challenges for the cardiovascular system and is marked by a sustained, abnormally elevated systemic venous pressure combined with decreased cardiac output. As more women with Fontan circulation reach childbearing age, understanding the unique risks of pregnancy to the mother and fetus and how to best provide clinical care for these women during pregnancy is imperative. However, there are limited clinical data to guide counseling and management in this population. This expert review offers an analysis of the literature about Fontan circulation during pregnancy and describes our center's current multidisciplinary approach to care for these women in the preconception, antepartum, intrapartum, and postpartum periods.
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