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At a glance

Polymalformative syndrome characterized by hypoplastic pulmonary arteries and aorta and urinary tract malformations. Predisposing factors include cigarette smoking, recurrent or chronic respiratory infections, air pollution, occupational exposure to chemicals, and allergies. Smoking is by far the most important of these factors—it impairs ciliary action and macrophage function, inflames airways, increases mucus production, destroys alveolar septae, and causes peribronchiolar fibrosis.

Synonyms

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

Incidence

Two cases in siblings have been described.

Genetic inheritance

An autosomal recessive inheritance trait is suggested based on the siblings reported.

Clinical aspects

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.

Anesthetic considerations

Evaluate current cardiac status by history, physical examination, and investigations (electrocardiogram, echocardiogram). A consultation with a cardiologist is recommended to evaluate the cardiac function and optimize the clinical 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.

Pharmacological implications

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

References

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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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