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

Abstract

a b s t r a c t Keywords: Pregnancy Fontan circulation Congenital heart disease AnticoagulationBackground: 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, abor-tions, 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 pa-tients. 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 oc-curred 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 b 0.01). After a median followup 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.

Pregnancy outcomes in patients with a fontan circulation and proposal for a risk-scoring system: single centre experience

“…In contrast to published series, we observed a high rate of pre-term delivery (92%) and a higher rate of CS delivery [7,8,10]. The latter may be attributed to a higher rate of maternal and fetal morbidity seen in our cohort necessitating delivery rather than allowing spontaneous labour to take place.…”

“…Maternal cardiovascular morbidity, primarily due to atrial arrhythmias and deterioration of functional status has been observed. The rate of spontaneous miscarriages varied (27-65%) may have been underestimated in some of the series [6,10]. Moreover, a significant proportion of pregnancies (69-81%) resulted in premature births in the contemporary series [7,8,10].…”

“…Published data regarding pregnancy in Fontan patients consists largely of small case series, as described in Table 1 [6][7][8][9][10][11][12][13]. Maternal cardiovascular morbidity, primarily due to atrial arrhythmias and deterioration of functional status has been observed.…”

“…The rate of spontaneous miscarriages varied (27-65%) may have been underestimated in some of the series [6,10]. Moreover, a significant proportion of pregnancies (69-81%) resulted in premature births in the contemporary series [7,8,10]. Drenthen et al and Cannobio et al advised against pregnancy in these women, whereas more contemporary literature suggests that despite the comorbidities associated with the Fontan circulation, women can undergo pregnancy, albeit at the risk of encountering significant maternal morbidity [6][7][8][9].…”

Trends in Congenital Heart Disease

“…80,81 Not surprisingly, among adult women with CHD who become pregnant, adverse cardiovascular events occurring in pregnancy and childbirth are considerably more likely during admission for delivery than the general population, particularly among those with complex CHD or pulmonary hypertension. [82][83][84][85] As a result of these pathophysiologies, significant, lesion-specific effects on life expectancy can be forecast (Figure 7).…”