Multiple congenital visceral abnormalities as a rare cause of pulmonary arterial hypertension

Parham Rabiee; Sedigheh Saedi

doi:10.1186/s43044-022-00273-x

Multiple congenital visceral abnormalities as a rare cause of pulmonary arterial hypertension

Egypt Heart J. 2022 Apr 28;74(1):34. doi: 10.1186/s43044-022-00273-x.

Authors

Parham Rabiee¹, Sedigheh Saedi²

Affiliations

¹ Radiology Department, Rajaei Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran.
² Cardiology and Adult Congenital Heart Disease Department, Rajaei Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Vali-asr Ave, Adjacent to Mellat Park, 1995614331, Tehran, Iran. Sedsaedi@gmail.com.

Abstract

Background: Pulmonary arterial hypertension (PAH) is a rare, progressive disorder. PAH is caused by a wide spectrum of pathologies but the cause remains undetermined on many occasions and patients are classified in the idiopathic group.

Case presentation: Here we report a young woman with rare congenital visceral abnormalities presenting with severe pulmonary hypertension.

Conclusions: Pulmonary hypertension is a complex disorder. Search for uncommon conditions that lead to pulmonary hypertension is necessary to determine the best management options.

Keywords: Abernethy malformation; Congenital heart disease; Mayer–Rokitansky–Küster–Hauser syndrome; Pulmonary hypertension.