Pregnancy outcomes after the Fontan repair

Abstract

Pregnancy after the Fontan operation appears to have been well tolerated in 13 to 14 gravidas. There does appear to be an increased risk of miscarriage. The tendency to routinely discourage pregnancy may need to be reconsidered.

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

“…This high rate of miscarriages, described in other series [9,10] during the first trimester, is twice that in the cardiac congenital population [22] and threefold that in the general population [23]. In our population, no predictive factor was found, neither the type of palliation, nor the treatment (including anticoagulation or anti arrhythmic), nor the NYHA functional class.…”

“…Despite several anecdotal and few multicentric evidence in literature on outcomes of pregnancy in women with Fontan circulation, there is still paucity of data on the predictors of maternal and fetal outcomes especially in this subset [9,10,[14][15][16][17]. The aim of this observational retrospective multicentric study was to determine, with a large number of pregnancies, the magnitude and frequency of these complications, with special focus on hematological complications.…”

“…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.…”

Human Heterotaxy Syndrome

“…70,84, 85 High risks of pregnancy and delivery, including deterioration of arrhythmias, progression of heart failure, coagulopathy, and miscarriage, have also been reported in women with Fontan circulation. 90 Representative long-term complications of the Fontan operation are illustrated in Figure 5. PLE PLE, one of the most severe manifestation of the failing Fontan circulation, occurs in 5-10% of the total postoperative cases.…”

Guidelines for Indication and Management of Pregnancy and Delivery in Women With Heart Disease (JCS 2010) - Digest Version -

“…12,61,62 The risk during pregnancy is not high among patients with complete transposition of the great arteries who underwent atrial switch operation (eg, Mustard operation or Senning operation), have favorable systemic ventricular function and only mild residua. The incidences of spontaneous abortion and obstetric complications are high.…”