Scimitar syndrome presenting in infancy

Background. Scimitar syndrome has a variable presentation based on the age at which the diagnosis is made. In general, infants presenting in heart fai lure have a greater number of associated anomalies and their prognosis is m uch worse. Methods. We reviewed the records of all patients under our care at St. Loui s 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. Results. The most common symptom at presentation was tachypnea. The chest r oentgenogram demonstrated dextroposition of the heart and hypoplastic right lung. Only 1 patient had the classic "scimitar sign." Cardiac catheterizat ion 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 seve re associated anomalies were treated medically and both died. Two patients underwent occlusion of the systemic collaterals; one died and the other ult imately underwent complete repair due to persistence of the symptoms of hea rt failure. Two patients had primary right pneumonectomy and both are alive and well. Seven patients underwent complete repair (one after coil occlusi on of the systemic arterial collaterals) and one died; three subsequently d eveloped occlusion of the baffle from the orifice of the anomalous pulmonar y vein and required pneumonectomy. Two patients required lung transplantati on due to persistent pulmonary hypertension in one and recurrent bilateral pulmonary venous stenosis in the other. Conclusions. Infants presenting with scimitar syndrome generally have sympt oms of tachypnea and chest roentgenograms showing dextrocardia and hypoplas tic right lung. Although repair of the anomalous venous return and ligation of collaterals is generally recommended, right pneumonectomy (either as pr imary therapy or if repair failed) had similar early and late results. (C) 1999 by The Society of Thoracic Surgeons.