Portopulmonary hypertension Archives - Revista Brasileira de Ginecologia e Obstetrícia

The severity of the association of pulmonary hypertension with pregnancy is well known. Pulmonary arterial hypertension constitutes one of the highest risk conditions for maternal mortality in late pregnancy and postpartum. Patients with portal hypertension of varying etiology may develop pulmonary arterial hypertension (portopulmonary hypertension) and most cases present cirrhosis as the underlying disease; however, a few cases of noncirrhotic etiology have been described. Clinical and pathological findings in two cases of portopulmonary hypertension and pregnancy are presented here. The two patients (30 and 24 years old) developed severe right heart failure and shock just after the delivery and the disease progressed rapidly to death. Autopsy demonstrated fibrosis in hepatic portal tracts, as has been described in cases of idiopathic portal hypertension. Also, pulmonary hypertension classified as plexogenic was reported.