Pregnancy in Women With Fontan Circulation

Study Questions:

What is the impact of preconception counseling (PCC) on patients with Fontan circulation, and what are their pregnancy rates and outcomes?


A retrospective review was performed at a single combined cardiac pregnancy service for patients undergoing PCC between 1994 and 2014.


A total of 58 women were offered PCC, of which three refused. Of the remaining 55 women, 15 decided against pursuing pregnancy, 16 decided to delay pregnancy, 19 attempted pregnancy, and five were lost to follow-up. Baseline hypoxemia and cardiac disease in pregnancy (CARPREG) score were similar in those opting for and against pregnancy. Of those attempting pregnancy, 14 succeeded, becoming pregnant a total of 43 times (median 1, range 1-9). Six women miscarried all pregnancies. Eight women carried 14 pregnancies to viability. Women exclusively miscarrying or unable to conceive were more likely to have baseline hypoxemia (oxygen saturation <92%) and higher CARPREG score. There was no maternal mortality. Cardiac complications included arrhythmia requiring treatment (n = 4) and one thromboembolism. Premature and small for gestational age infants were common in women with Fontan circulation.


Most women accept PCC and decide to pursue pregnancy. Pregnancy outcome was related to baseline hypoxia and CARPREG scores.


This study reinforces what we know regarding pregnancy in women with Fontan circulation. It has been known that women after Fontan procedure are at risk for delivering premature, small for gestational age infants. Particularly telling in this study was a mean birth weight of 1682 (range 600-2719) grams and median gestation at delivery of 34 weeks. Postpartum hemorrhage was extremely common, occurring in 50% of pregnancies, in the setting of a center recommending thromboprophylaxis for all women with Fontan circulation. Miscarriage remains a significant issue, particularly for women with baseline hypoxemia. Although women with Fontan circulation may tolerate pregnancy, maternal and fetal complications remain significant.

Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, Interventions and Structural Heart Disease

Keywords: Anoxia, Abortion, Spontaneous, Arrhythmias, Cardiac, Fontan Procedure, Gestational Age, Heart Defects, Congenital, Maternal Mortality, Postpartum Hemorrhage, Premature Birth, Pregnancy, Pregnancy, Heterotopic, Pregnancy Outcome, Pregnancy Rate, Thromboembolism

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