Key result
Two patients with Down syndrome and intracardiac communications presented early in life with significant, progressive stenosis of pulmonary veins.
Case Report (n=2)
Pulmonary venous stenosis in Down syndrome patients with intracardiac communications may contribute to the early onset of pulmonary vascular occlusive disease.
Pulmonary vein stenosis may contribute to early pulmonary vascular disease in Down syndrome with shunts; hypothesis-generating from case reports.
Two patients with Down syndrome, intracardiac communications and elevated pulmonary arteriolar resistance presented early in life. Both patients had significant stenosis of pulmonary veins. The progressive nature of the stenosis is illustrated in one patient. Pulmonary venous stenosis in Down syndrome has been recorded only twice before in the literature, and may play a part in the early onset of pulmonary vascular occlusive disease in some patients.
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Stewart et al. (1992) conducted a case report in Down syndrome with pulmonary vein stenosis (n=2). Pulmonary vein stenosis was evaluated. Two patients with Down syndrome and intracardiac communications presented early in life with significant, progressive stenosis of pulmonary veins.
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