Outcomes of Pulmonary Artery Reconstruction in Williams Syndrome.

BACKGROUND: The study sought to evaluate the short-term and midterm outcomes of surgical pulmonary artery reconstruction in patients with Williams syndrome (WS).

METHODS: We performed a retrospective cohort study of all patients with WS who underwent surgical pulmonary artery reconstruction at Lucile Packard Children's Hospital between January 2001 and May 2018.

RESULTS: There were 25 WS patients (52% female) who underwent pulmonary artery reconstruction during the study period. Median age at surgery was 2.4 (interquartile range [IQR], 0.9 to 4.5) years. Median preoperative right ventricular (RV) pressure was 80 (IQR, 70 to 90) mm Hg and aortic pressure was 96 (IQR, 90 to 107) mm Hg, with an RV-to-aortic pressure ratio of 0.8 (IQR, 0.7 to 1.0). The median number of pulmonary arterioplasty patches was 16.5 (IQR, 6.5 to 24). Median postoperative RV pressure was 27 (IQR 20 to 31) mm Hg and aortic pressure was 90 (IQR, 87 to 105) mm Hg, with an RV-to-aortic pressure ratio of 0.27 (IQR, 0.22 to 0.35). The postoperative RV pressure and RV-to-aortic pressure ratio were significantly lower than preoperative RV pressure and RV-to-aortic pressure ratio (p < 0.0001 for both). There was 1 (4%) postoperative death. In a median follow-up of 2.6 (IQR, 0.94 to 3.4) years, 1 (4.2%) patient has undergone RV outflow tract aneurysm repair and 2 (8.3%) patients have undergone balloon dilation of the pulmonary arteries.

CONCLUSIONS: Multilevel, surgical pulmonary artery reconstruction addressing severe extrapericardial stenoses is highly effective in patients with WS. This technique results in immediate normalization of RV pressure and has a low rate of reintervention in midterm follow-up.