Scimitar syndrome presenting in infancy

Scimitar syndrome has a variable presentation based on the age at which the diagnosis is made. In general, infants presenting in heart failure have a greater number of associated anomalies and their prognosis is much worse.

We reviewed the records of all patients under our care at St. Louis Children's Hospital who presented with symptoms attributable to scimitar syndrome during their infancy. Twelve patients were identified. The average age at presentation was 6 weeks.

The most common symptom at presentation was tachypnea. The chest roentgenogram demonstrated dextroposition of the heart and hypoplastic right lung. Only 1 patient had the classic "scimitar sign." Cardiac catheterization demonstrated pulmonary hypertension (pulmonary artery systolic pressure, 73.9+/-21.8 mm Hg). The Qp:Qs was 3.1+/-1.5:1. Two patients with severe associated anomalies were treated medically and both died. Two patients underwent occlusion of the systemic collaterals; one died and the other ultimately underwent complete repair due to persistence of the symptoms of heart failure. Two patients had primary right pneumonectomy and both are alive and well. Seven patients underwent complete repair (one after coil occlusion of the systemic arterial collaterals) and one died; three subsequently developed occlusion of the baffle from the orifice of the anomalous pulmonary vein and required pneumonectomy. Two patients required lung transplantation due to persistent pulmonary hypertension in one and recurrent bilateral pulmonary venous stenosis in the other.

Infants presenting with scimitar syndrome generally have symptoms of tachypnea and chest roentgenograms showing dextrocardia and hypoplastic right lung. Although repair of the anomalous venous return and ligation of collaterals is generally recommended, right pneumonectomy (either as primary therapy or if repair failed) had similar early and late results.