Surgery for bilateral outflow tract obstruction in elastin arteriopathy

Objective: A number of patients with Williams syndrome or other forms of el astin arteriopathy have stenoses of pulmonary arteries in addition to supra valvular aortic stenosis. We sought to investigate the effect of the degree of pulmonary arterial stenosis on the prognosis after an operation for sup ravalvular aortic stenosis to help define the optimal treatment strategy fo r patients with severe forms of elastin arteriopathy. Methods: Between 1960 and 1999, 33 patients underwent operations for suprav alvular aortic stenosis while having significant stenoses of the pulmonary arteries. We retrospectively reviewed patient charts, obtained current foll ow-up information, and determined risk factors for survival and reoperation . Results: Fifteen patients with moderate right-sided obstructions (confirmed by pulmonary artery Z-scores and right ventricular/descending aortic press ure ratio) underwent operations for supravalvular aortic stenosis only. Eig hteen patients had more severe right-sided obstructions and underwent surgi cal relief of pulmonary arterial stenoses or right ventricular outflow trac t obstruction in addition to operations for supravalvular aortic stenosis. Eight patients had undergone preoperative balloon dilations of stenotic pul monary arteries. There were 6 early deaths and 1 late death in our series. Survival at 10 and 20 years was 76% (70% confidence interval, 68%-84%) and freedom from reintervention was 59% (70% confidence interval, 46%-71%) at 1 0 years and 49% (70% confidence interval, 35%-62%) at 20 years. Multivariat e analysis revealed that patients with a right ventricular/descending aorti c pressure ratio of 1.0 or more were at higher risk for reintervention but not for death. Conclusions: Surgical treatment of pulmonary artery obstructions in elastin arteriopathy is palliative but, in conjunction with balloon dilation of pe ripheral pulmonary arteries, offers good long-term survival to patients wit h the severest form of elastin arteriopathy.