Background
Aortopulmonary window is a rare congenital heart defect that results in severe pulmonary arterial hypertension (PAH), Eisenmenger syndrome, and congestive heart failure in the first months of life. Pregnancy is absolutely contraindicated in the patients with this condition [7].
Case summary
This paper describes two clinical cases of pregnancy in patients (28 and 20 years old) with aortopulmonary window defect, severe pulmonary arterial hypertension, and Eisenmenger syndrome that ended in preterm delivery by cesarean section. One patient died in the postpartum period due to progression of right ventricular heart failure. The younger patient survived childbirth and the postpartum period; later she continued therapy at the pulmonary arterial hypertension center.
Discussion
We describe unusual cases of clinical features in pregnants with aortopulmonary window defect. Due to the rare occurrence and low survival rate of patients with uncorrected aortopulmonary window defect, descriptions of clinical cases of this defect in adults are very rare. It is very important to note the necessity of observation of these patients in specialized centers by a multidisciplinary team of healthcare professionals, due to the high risk of cardiovascular, obstetric complications, and death.