TY - JOUR
T1 - Management of Fontan circulation in pregnancy
T2 - a multidisciplinary approach to care
AU - Wolfe, Natasha K.
AU - Sabol, Bethany A.
AU - Kelly, Jeannie C.
AU - Dombrowski, Michael
AU - Benhardt, Amber C.
AU - Fleckenstein, Jaquelyn
AU - Stout, Molly J.
AU - Lindley, Kathryn J.
N1 - Publisher Copyright:
© 2020 Elsevier Inc.
PY - 2021/1
Y1 - 2021/1
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.
KW - Fontan circulation
KW - adult congenital heart disease
KW - cardio-obstetrics
KW - high-risk pregnancy
KW - multidisciplinary
KW - single ventricle physiology
UR - http://www.scopus.com/inward/record.url?scp=85100099833&partnerID=8YFLogxK
U2 - 10.1016/j.ajogmf.2020.100257
DO - 10.1016/j.ajogmf.2020.100257
M3 - Review article
C2 - 33451613
AN - SCOPUS:85100099833
SN - 2589-9333
VL - 3
JO - American Journal of Obstetrics and Gynecology MFM
JF - American Journal of Obstetrics and Gynecology MFM
IS - 1
M1 - 100257
ER -