CRA68 SCIMITAR SYNDROME AND ITS CONTINUUM

Scimitar syndrome is a rare congenital cardiovascular anomaly. It consists of a partial anomalous pulmonary venous drainage of right lung, right lung hypoplasia, dextroposition of heart, and anomalous systemic arterial supply from aorta or one of its branches to the right lung. Its presentation ranging from asymptomatic state to haemoptysis, heart failure and severe pulmonary hypertension. 

This case is reported in view of the rareness of the syndrome.

Case Report: 

A 33-year-old male presented with a 3-day history of cough and breathlessness. He reported a history of bronchial asthma and right lung hypoplasia with diaphragmatic eventration diagnosed at childhood. He had no history of recurrent hospitalization for respiratory infection until last year, where he required multiple hospitalizations for similar complaints. Auscultation revealed reduced breath sound over right lower zone. CXR showed the shadow of the descending pulmonary vein along the right cardiac border, a hypoplastic right lung and dextroposition of heart. Computed tomography (CT) thorax showed hypoplasia of the right lung. The right pulmonary vein drains into the inferior vena cava in keeping with anomalous pulmonary venous return. The normal left pulmonary vein drains into the left atrium and dextroposition of heart. His echocardiography revealed ejection fraction 51%, moderately enlarged right atrium and right ventricle with RVSP 69mmHg. He responded with diuresis for Scimitar syndrome with cor pulmonale and pulmonary hypertension. After a multi-disciplinary discussion, he was referred to cardiology team for right heart catheterization and discussion of surgical options. 

Conclusion: 

Scimitar syndrome is a rare entity. However, early recognition, diagnosis, and prompt treatment can significantly lower morbidity and complications and improve quality of life.