Balloon Dilation Angioplasty of Peripheral Pulmonary Stenosis Associated With Williams Syndrome

Abstract

Background-Experience of balloon dilation of peripheral pulmonary stenosis (PPS) in Williams syndrome (WS) is limited. Methods and Results-Catheterizations in all patients with WS undergoing therapy for PPS from 1984 to 1999 were reviewed. Criteria for successful dilation included an increase Ͼ50% in predilation diameter and a decrease Ͼ20% in ratio of right ventricular (RV) to aortic (Ao) systolic pressure. Median age and weight were 1.5 years and 9.5 kg. There were 134 dilations during 39 procedures in 25 patients. The success rate for initial dilations was 51%. In multivariate analysis, successful dilation was more likely (1) in distal than in central pulmonary arteries (Pϭ0.02), (2)

Congenital heart defects in Williams syndrome

“…Del Pasqua et al 26 proposed that the major surgical or interventional indication for pulmonary stenosis in WS patients was a mean pressure gradient of >45 mmHg. Clinical studies by different surgical teams, including Stamm et al 27 , Monge et al 28 and Geggel et al 29 that the treatment of choice for pulmonary stenosis depended on the site of the lesion and the late results were fine (Fig. 1).…”

“…De Rubens Figueroa et al 3 reported that, in 58% of cases, a surgical operation was required, depending on the severity of pulmonary artery stenosis. Geggel et al 29 reported that balloon dilation of peripheral pulmonary stenosis in WS were 134 dilation maneuvers during 39 procedures in 25 patients with a success rate for initial dilations of 51%. Bruno et al 17 proposed, in cases of pulmonary artery branch stenosis, there was the possibility of stent placement.…”

Cardiovascular Disease in Williams Syndrome

“…15,30,33,45 The stenoses seen in the pulmonary arterial bed most commonly occur in the branch and peripheral pulmonary arteries. Although discrete stenosis can be seen, diffuse stenoses involving large segments of the pulmonary arterial tree are frequently encountered 46 ( Figure 5). Supravalvar pulmonary stenosis is also seen but is less common, occurring in ≈12% of all patients with WS 15 and 20% of those presenting in the first year of life.…”

Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome

“…For patients with persistent systemic or suprasystemic right ventricular pressure, marked asymmetry in pulmonary blood flow, or symptoms, balloon dilation angioplasty has been used to improve arterial diameter, especially in distal vessels. After catheter-based therapy, right ventricular pressure often remains elevated due to residual proximal obstruction, and the incidence of aneurysms is higher in comparison with non-WBS subjects (101). Patients with biventricular outflow obstruction with an indication for surgical relief of SVAS may undergo balloon angioplasty of pulmonary arterial stenosis prior to surgery.…”