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

Abstract

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

• Congenital heart disease
• Pulmonary hypertension
• Mayer–Rokitansky–Küster–Hauser syndrome
• Abernethy malformation