Management of Fontan circulation in pregnancy: a multidisciplinary approach to care

Natasha K. Wolfe, Bethany A. Sabol, Jeannie C. Kelly, Michael Dombrowski, Amber C. Benhardt, Jaquelyn Fleckenstein, Molly J. Stout, Kathryn J. Lindley

Research output: Contribution to journalReview articlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)100257
Number of pages1
JournalAmerican journal of obstetrics & gynecology MFM
Volume3
Issue number1
DOIs
StatePublished - Jan 1 2021
Externally publishedYes

Bibliographical note

Publisher Copyright:
Copyright © 2020 Elsevier Inc. All rights reserved.

Keywords

  • Fontan circulation
  • adult congenital heart disease
  • cardio-obstetrics
  • high-risk pregnancy
  • multidisciplinary
  • single ventricle physiology

PubMed: MeSH publication types

  • Journal Article
  • Research Support, Non-U.S. Gov't
  • Review

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