Maternal and fetal outcomes of pregnancy with Fontan circulation: A multicentric observational study

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Abstract

Background

Despite serious long-term sequel, women with Fontan palliation have reached childbearing age. However there is paucity of data on the pregnancy outcomes and management of this condition. We aimed to determine the maternal and fetal outcomes of pregnancy in women with Fontan palliation.

Methods

This multicentric, retrospective study included women with Fontan circulation followed in 13 French specialized centers from January 2000 to June 2014. All pregnancies were reviewed, including miscarriages, abortions, premature and term births. We reviewed maternal and fetal outcomes.

Results

Thirty-seven patients had 59 pregnancies. Mean age was 27 ± 5 years at first pregnancy. There were 16 miscarriages (27%) and 36 live births with 1 twin pregnancy. Cardiac events occurred in 6 (10%) pregnancies, with no maternal death. The most common cardiac complication was atrial arrhythmia, which occurred in 3 patients. Hematological complications including thromboembolic/hemorrhagic events (n = 3/7) occurred in 5 women antepartum (n = 2/3), and 4 women postpartum (n = 1/4). Two of the 3 thromboembolic events occurred in patients without anticoagulation. There was a high incidence of prematurity (n = 25/36, 69%). Anticoagulation was associated with adverse neonatal outcome (OR = 10.0, 95% CI [1.5–91.4], p < 0.01). After a median follow-up of 24 months, there was no significant worsening of clinical status and thromboembolic disease noted.

Conclusions

Pre-selected women can successfully complete pregnancy with Fontan circulation. There is an increase in cardiac and neonatal morbidity during pregnancy. Because thromboembolism could have a severe consequence on Fontan circulation, anticoagulation should be indicated during pregnancy and postpartum period.

Introduction

Since 1971, Fontan procedure has been adopted worldwide for the management of patients with complex congenital heart disease considered unsuitable for a biventricular repair. Surgical evolution of this technique [1], [2], [3] has significantly improved survival for patients with functional univentricular heart. However, intrinsic to the construction of the Fontan circulation, secondary complications due to extensive surgical distortion of cardiac anatomy and physiology are inevitable. The wide spectrum of complications includes arrhythmia, ventricular dysfunction, thromboembolic events, hepatic dysfunction, and protein losing enteropathy. Nevertheless, long-term survival is good [4], [5] (90% at 10 years, 83% at 20 years, and 70% to 76% at 25 years) and an increasing number of women reaching childbearing age following surgery express desire to have children. Hemodynamic changes in pregnancy are pronounced for the abnormal heart (cardiac output and stroke volume double, myocardial oxygen consumption increases by 20%, heart rate increases by 15–20%). Paucity of data on the maternal and fetal risks, as well as short and long-term outcomes, leaves preconception counseling with many unanswered questions, most importantly on the type and duration of anticoagulation to prevent thromboembolic accidents [6]. Thromboembolic complications during pregnancy are expected to increase, as Fontan state and pregnancies are both pro-thrombotic milieu [7], [8]. However, no study has shown that anticoagulation reduces the risk in this context. Moreover, the late consequences of pregnancy on the Fontan heart need to be analyzed given the limited data on long-term survival [4], [5]. Published literature includes anecdotal case reports and small series [9], [10], [11], which are insufficient to evaluate and identify predictors of maternal risk during pregnancy, to provide recommendations, and subsequently guide providers for counseling.

The aim of this study was to determine the fetal and maternal outcomes of pregnancy in women with Fontan palliation and impact of pregnancy on heart disease at last follow-up in a large series of patients.

Section snippets

Population

All pregnant women with Fontan palliation registered in one of the 13 centers of the French M3C network (Centres de Référence et de Compétence des Malformations Cardiaques Congénitales Complexes, M3C) during the time period from January 2000 to June 2014 were included in the study. We did not include ongoing pregnancies by the end of the study period. For each pregnancy, the cardiac and obstetric records were reviewed.

Baseline data included initial heart anatomy, prior surgical procedures,

Baseline characteristics

During the study period, 37 women had a total 59 pregnancies. Mean age at first pregnancy was 27 ± 5 years (19 to 41 years). Thirteen women had more than one pregnancy (up to 5 pregnancies). Underlying congenital heart lesions and maternal baseline characteristics are summarized in Table 1, Table 2.

Half of the population had tricuspid atresia, and half of palliations were intracardiac total cavo-pulmonary connection (TCPC). Mean age at Fontan palliation was 13 ± 7 years. Seven Fontan or Kreutzer

Discussion

Fontan surgery and its derivatives (Kreutzer, intra-auricular cavopulmonary connection or extracardiac tube) have allowed the population with functionally single ventricle to reach adulthood in the vast majority of cases. For post-Fontan women who become pregnant, the main question is the ability of the univentricular heart to tolerate the hemodynamic transformations due to pregnancy (cardiac output increase, overload circulating volume, heart rate increase) and the risk of exacerbating the

Limitations

Data were retrospectively collected. Follow-up, outcome assessment, and treatment strategies were not standardized. However, information bias was likely minimized by the completeness of data reported in the study and absence of losses to follow-up. In addition, patients deemed at highest risk may have been counseled against pregnancy. For example, no patient had severe systemic ventricle dysfunction, a NYHA functional class of III or IV, or protein losing enteropathy.

Conclusion

Our results show that pregnancy in women with Fontan palliation is possible, without maternal mortality. However, pregnancies remain at high risk for the mother with arrhythmias, and for the fetus, with a high rate of miscarriages, prematurity and SGA. Although anticoagulation therapies during pregnancy are still debated, thromboembolisms were some of the major complications we described during pregnancy or the postpartum period in women with Fontan palliation, which can threaten Fontan

Conflict of interest

None.

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