Scimitar syndrome with late presentation

Abstract

Introduction: Scimitar syndrome is a congenital malformation with an incidence of 1-3 per 100,000 live births, consisting of abnormal drainage of the right pulmonary veins. The age of diagnosis varies according to the severity of the pathology, which depends on the degree of cardiac and pulmonary malformation. The case of a patient with a late diagnosis of the syndrome due to a silent course is reported despite severe anatomical alterations. Clinical Case: A 76-year-old female was admitted due to a 6-month clinical picture of progressive dyspnea to minimal efforts. A transthoracic echocardiogram was performed showing severe pulmonary hypertension of 96 mmHg with abnormal communication between the right pulmonary veins and the inferior vena cava, confirmed by CT angiography. Discussion: The late clinical course in relation to severe anatomical malformations has not been described in the scientific literature due to its high mortality in childhood.