Severe Pulmonary Hypertension in Pregnancy Associated with Unespecific Congenital Heart Disease: Case Report

Abstract

Pulmonary hypertension during pregnancy, particularly when associated with congenital heart disease, carries a high risk of maternal–fetal morbidity and mortality. Its management requires early referral to specialized centers, which remains challenging in settings where sociocultural factors influence clinical decision-making. The case presented involved a 40-year-old primigravida with previously uninvestigated cyanotic congenital heart disease. During the second trimester, she declined pregnancy termination and referral to a tertiary care center due to religious convictions. At 34.1 weeks of gestation, she was admitted in labor with severe cardiorespiratory decompensation, requiring an emergency cesarean section. She subsequently progressed to refractory right-sided heart failure and died 36 hours postpartum. The preterm newborn had a favorable clinical course. This fatal case underscores the extreme risk of pulmonary hypertension in pregnancy in the context of non-protocolized congenital heart disease and highlights the critical need for preconception counseling, early detection, timely referral, and a shared decision-making process that, while respecting patient values, prioritizes maternal–fetal safety through expert multidisciplinary management.