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[Peripheral stenoses of the pulmonary arteries: possible causes and syndromic relationships (author's transl)]
Abstract:
The catheterization results and additional malformations and diseases in 20 infants and children with pulmonary artery stenosis are reported. In 17 cases additional cardiovascular malformations were found. In 15 cases extracardiac malformations or developmental retardation were observed. Peripheral stenoses of the pulmonary arteries are frequently established in rubella embryopathy, in the syndrome of supravalvular aortic stenosis, in Down s syndrome, in different types of nanism, in thalidomide embryopathy and in the socalled arteriohepatic dysplasia (Watson and Miller) associated with hypoplasia of the bile ducts. In 3 cases an arteriophepatic dysplasia was confirmed by angiocardiography and biopsy. Coarctations of pulmonary arteries rarely need operative interventions.