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

doi:10.1016/j.ajogmf.2020.100257

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 journal › Review article › peer-review

11 Scopus citations

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 language	English (US)
Article number	100257
Journal	American Journal of Obstetrics and Gynecology MFM
Volume	3
Issue number	1
DOIs	https://doi.org/10.1016/j.ajogmf.2020.100257
State	Published - Jan 2021
Externally published	Yes

Bibliographical note

Funding Information:
We thank Vicki Friedman and Mary Lynn Brophy for the medical illustrations. We also thank the Barnes-Jewish Hospital Women's Heart Health Fund for the financial support for medical illustration. The medical illustrations prepared for this article were funded by the Barnes-Jewish Hospital Women's Heart Health Fund.

Publisher Copyright:
© 2020 Elsevier Inc.

Keywords

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

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1016/j.ajogmf.2020.100257

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title = "Management of Fontan circulation in pregnancy: a multidisciplinary approach to care",

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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author = "Wolfe, {Natasha K.} and Sabol, {Bethany A.} and Kelly, {Jeannie C.} and Michael Dombrowski and Benhardt, {Amber C.} and Jaquelyn Fleckenstein and Stout, {Molly J.} and Lindley, {Kathryn J.}",

note = "Funding Information: We thank Vicki Friedman and Mary Lynn Brophy for the medical illustrations. We also thank the Barnes-Jewish Hospital Women's Heart Health Fund for the financial support for medical illustration. The medical illustrations prepared for this article were funded by the Barnes-Jewish Hospital Women's Heart Health Fund. Publisher Copyright: {\textcopyright} 2020 Elsevier Inc.",

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AU - Stout, Molly J.

AU - Lindley, Kathryn J.

N1 - Funding Information: We thank Vicki Friedman and Mary Lynn Brophy for the medical illustrations. We also thank the Barnes-Jewish Hospital Women's Heart Health Fund for the financial support for medical illustration. The medical illustrations prepared for this article were funded by the Barnes-Jewish Hospital Women's Heart Health Fund. Publisher Copyright: © 2020 Elsevier Inc.

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N2 - 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.

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

Abstract

Bibliographical note

Keywords

UN SDGs

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