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To the Editor:
Scimitar syndrome, also known as pulmonary venolobar syndrome, is a rare congenital heart condition that encompasses an array of anatomical abnormalities, including: hypoplastic right lung and right pulmonary artery with aortopulmonary collaterals, an anomalous venous return from the right lung (either partial or total) to the systemic venous drainage system (commonly the right atrium or inferior vena cava, and dextroposition of the heart (1, 2). These may be further associated with pulmonary sequestration.
The syndrome is named as such because the shape taken by the anomalous and dilated veins as they drain into the inferior vena cava resembles the curved blade of a middle-eastern saber. The condition often comes to medical attention at an early age because of symptoms related to the associated pulmonary hypertension. Less commonly, scimitar syndrome is discovered in adults, many of whom have mild or no symptoms at the time of diagnosis (3).
Conventionally the syndrome is corrected surgically by redirecting central venous return toward the left atrium. If not corrected, detrimental outcomes may ensue (4). Here, we report a unique transcatheter treatment approach that we used to treat an adult scimitar syndrome complicated by severe pulmonary hypertension.
Case Report
A 30-year-old man presented with dyspnea and hemoptysis. He had a history of congenital heart disease, which he referred to as "holes in my heart," stating he had undergone reparative surgery at age 2 years. Apparently, there was no subsequent medical follow up. The patient reported breathlessness for about 6 months, which progressed to severe exercise limitation and intermittent dyspnea at rest. This was associated with intermittent production of clear sputum, at times with specks of blood, until the day of presentation, when he produced more hemoptysis than usual. He also reported exertional substernal chest pain and wheezing. He noted that he was otherwise well except for systemic hypertension.
On presentation, the man's arterial oxygen saturation was 80% measured while he breathed ambient air. Supplemental oxygen increased his oxygen saturation to 93%. The blood pressure was 155/97 mm Hg. He was obese. Breath sounds were slightly diminished over both bases, without adventitious sounds. Auscultation of the precordium revealed prominent right-sided heart sounds and a...