Journal of Rare Cardiovascular Diseases

A 48-year-old woman was admitted to our institution with a year’s history of uncharacteristic chest pain episodes and decrease in exercise tolerance. A CT lung scan performed nine months earlier revealed an anomalous vein arising from the right lower pulmonary lobe and draining into the inferior vena cava. A chest radiogram performed 2 months prior to the admission showed a curved shape adjacent to the right heart border. Right heart catheterization revealed: normal systolic, diastolic and mean pulmonary artery pressures, normal pulmonary resistance and insignificant shunt volume. Pulmonary artery angiography demonstrated an anastomotic vessel between the aberrant pulmonary vein and the right upper pulmonary vein. The patient was diagnosed with Partial Anomalous Pulmonary Venous Connection in form of Scimitar syndrome. Given a mild character of symptoms, no signs of the right-sided heart volume overload, insignificant shunt fraction and normal pulmonary circulation parameters, the patient was qualified to a conservative treatment with a close medical observation. JRCD2013; 1 (2): 39–41

Key words: partial anomalous pulmonary venous connection, scimitar syndrome, left-to-right shunt, pulmonary hypertension

partial anomalous pulmonary venous connection; scimitar syndrome; left-to-right shunt; pulmonary hypertension

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