Coronary artery lesions are associated with adverse cardiac events in children undergoing supravalvular aortic stenosis repair.

OBJECTIVES: To identify the prevalence and anatomic characteristics of coronary artery lesions and their associated postoperative risk in patients undergoing supravalvular aortic stenosis repair.

METHODS: The association between structural risk factors, postoperative ST-segment changes, and major adverse cardiac events were explored using logistic regression and the Fisher's exact test.

RESULTS: In 51 consecutive supravalvular aortic stenosis patients between 2000 and 2017, a total of 48 coronary lesions were identified in 27 patients(53%). Prominent ostial ridge(Type I) was the most common coronary lesion, followed by small ostium with(IIIb) or without(IIIa) diffuse long segment coronary narrowing, and adhesion of the coronary cusp(TypeⅡ). There were 54 concomitant coronary procedures, including 43 primary corrections and 11 revisions. Thirty-three patients underwent supravalvular aortic stenosis repair with a bifurcated patch; 13(39.4%) had right coronary artery distortion/kinking requiring patch plication(n = 8) and reimplantation(n = 5). Postoperative MACE occurred in nine patients(17.6%), including three deaths, four needing mechanical circulatory support, and six ventricular arrhythmias. Twenty-two patients(43.1%) had postoperative ST-segment changes, including 13 early changes that resolved within 24 hours, and 9 persistent changes lasting more than 24 hours. Patients with Type III lesions were associated with postoperative persistent ST-segment change(p = 0.04) and independently predicted postoperative MACE(p = 0.02). Patients with pre-existing coronary lesions were at elevated risk of right coronary artery distortion/kinking(p = 0.045).

CONCLUSIONS: The prevalence of ST-segment changes and MACE is high in patients undergoing supravalvular aortic stenosis repair. Preoperative presence of complex coronary lesions is the most important predictors for postoperative major adverse cardiac events.