[Scimitar syndrome in infants. Physiopathology and therapeutic implications in 12 cases].

Twelve children, aged 6 days to 6 months (mean: 42 +/- 23 days) were diagnosed as presenting with the scimitar syndrome. In this syndrome, anomalous pulmonary venous drainage, hypoplasia of the right pulmonary artery and parenchyma, variety of "sequestrum" of the right pulmonary artery and parenchyma, variety of "sequestrum" of the right pulmonary base usually fed by subdiaphragmatic branches of the aorta are associated. Respiratory disorders were constant, severe in 8 cases. X-rays showed "dextro-cardia" and "hypoplasia of the right lung". Endocardiac investigation assessed the diagnosis and showed pulmonary arterial hypertension in 11 cases, with (5) or without (6) associated heart defect. One case with good tolerance remains under simple supervision; 4 were operated on from the associated lesions only and died; 7 took benefit of a simple surgical removal of the branches feeding the sequestrum, with one death and 6 successes. The poor prognostic significance of the sequestrum is thus indirectly demonstrated and the value of its devascularization is emphasized.