A rare etiology of pulmonary hypertension: The scimitar syndrome.

Abstract

Scimitar syndrome is a rare anomaly where there is an occurrence of partial or complete abnormality of venous drainage of the right pulmonary vein into the inferior vena cava. Here we present a 34-year-old female patient complaining of dyspnea on exertion and dry cough, after undergoing extensive imaging and hemodynamic testing, was found to have a syndrome identified with lung hypoplasia with moderate pulmonary arterial hypertension (PAH). This case emphasizes how crucial it is to rule out Scimitar syndrome in adults as one of the causes of PAH from imaging and sonography. The initial presentation will be breathlessness where every physician keeps an insight of performing cardiac evaluation that may finally end up with these rare diagnosis.