Natural history of supravalvular aortic stenosis and pulmonary artery stenosis

Abstract

Data from 35 patients with supravalvular aortic stenosis or pulmonary artery stenosis, or both, undergoing cardiac catheterization between 1973 and 1989 were analyzed retrospectively. Twenty-seven patients had supravalvular aortic stenosis: 11 required surgery after the first investigation and 8 (80%) of 10 others undergoing serial investigation showed an increase in the left ventricle to aorta pressure gradient. Angiographic measurements showed that the increase in the aortic pressure gradient was related to failure of normal growth of the ascending aorta lumen. Nineteen patients had pulmonary artery stenosis, with a right ventricular pressure greater than 33 mm Hg. At restudy, right ventricular pressure had decreased in 9 (82%) of 11 patients. This decrease in right ventricular pressure was associated with an increase in the systolic distensibility of the proximal pulmonary arteries, although there was no increase in the diastolic diameters. One patient had a rapid early increase in right ventricular pressure and no pulmonary artery growth. In two patients, multiple peripheral pulmonary artery stenoses became evident with time and produced persistent right ventricular hypertension. Supravalvular aortic stenosis is usually a progressive lesion, with an increase in left ventricular outflow tract pressure gradient related to poor growth of the ascending aorta. Pulmonary artery stenosis usually improves and only rarely limits prognosis.

Balloon Dilation Angioplasty of Peripheral Pulmonary Stenosis Associated With Williams Syndrome

“…3,29 Although mortality improved in the second half of this series, the incidence of complications remains significant. This finding, coupled with the possibility of spontaneous improvement has caused us to recommend frequent noninvasive observation in the asymptomatic infant or young patient with subsystemic RV pressure who does not have significant left-sided involvement.…”

Cardiovascular Disease in Williams Syndrome

“…16 The natural history of PAS in WS is one of improvement, which has been described in multiple studies. 16,37,38 This natural history is likely related to the change in arterial medial tension that occurs in the postnatal period. Pulmonary arterial concentrations of elastin normally decrease in the first few months of life, at a time when pulmonary vascular resistance is normalizing.…”

Supravalvular Aortic Stenosis and Peripheral Pulmonary Stenosis Coexisting With a Straight Thoracic Spine.

“…8 The association between SVAS and PPS is well recognized 1 and experience with children and adolescents has shown that in untreated patients, the degree of left ventricular outflow obstruction increases with age, whereas the associated PPS may become less severe 2,3,10 because of an increase in thes systolic distensibility of the pulmonary artery rather than to true luminal growth. 3 The same natural course occurred in the case; that is, regression of the PPS with reduction of right ventricular pressure, together with apparent progression of SVAS. Because of the progressive nature of SVAS, surgical treatment was recommended, either a standard patch aortoplasty or extended aortoplasty.…”

“…1 Further, its association with peripheral pulmonary stenosis (PPS) is well known. 2,3 A straight thoracic spine, with compression of the heart and great vessels by the chest deformity, has been designated as 'straight back syndrome' and it simulates organic heart disease by causing systolic murmurs and alterations of heart sounds in patients with a normal heart. 4,5 We present a rare case of SVAS and PPS with a straight thoracic spine.…”