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Polymalformative syndrome characterized by hypoplastic pulmonary arteries and aorta and urinary tract malformations.

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Hypoplastic Pulmonary Arteries and Aorta with Obstructive Uropathy; Pulmonary and Aortic Stenosis with Obstructive Uropathy.

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Two cases in siblings have been described. Autosomal recessive inheritance.

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Diffuse hypoplasia of the pulmonary arteries and the ascending aorta resulted in progressive right heart failure, systemic hypertension, chronic malabsorption, and failure to thrive. Urinary tract abnormalities included obstructive uropathy with reflux and hydronephrosis. The authors characterized the disease as possibly representing hypoplasia of the arterial vascular system.

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Evaluate current cardiac status by history, physical examination, and investigations (electrocardiogram, echocardiogram). Consultation with cardiologist to evaluate and optimize management, especially if cor pulmonale is present. Endocarditis prophylaxis may be required. Evaluate renal function (creatinine, blood urea nitrogen levels). Anesthetic techniques that minimize myocardial depression and pulmonary vascular resistance may be tolerated best. Inotropic support may be required if cor pulmonale is present.

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Avoid drugs with predominantly renal elimination in the presence of renal insufficiency.

Kashani IA, Strom CM, Utley JE, et al: Hypoplastic pulmonary arteries and aorta with obstructive uropathy in 2 siblings. Angiology 35:252, 1984.  [PubMed: 6711910]

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