ECGs Do Not Detect Myocardial Ischemia in Patients with Williams Syndrome and Non-Syndromic Elastin Arteriopathy with Coronary Artery Stenosis.

Abstract

Coronary artery stenosis (CAS) may affect up to 27% of patients with Williams syndrome (WS), which may lead to myocardial ischemia. WS patients face a 25 to 100-fold higher risk of sudden cardiac death, frequently linked to anesthesia. Assessing CAS requires either imaging while under general anesthesia or intraoperative assessment, with the latter considered the gold standard. Our study aimed to identify electrocardiogram (ECG) markers of myocardial ischemia in patients with WS or non-syndromic elastin arteriopathy and documented CAS. We retrospectively reviewed patients with WS/elastin arteriopathy who underwent supravalvar aortic stenosis (SVAS) surgery and CAS assessment from January 1, 2006, to April 30, 2021. A pediatric electrophysiologist, unaware of the patients' CAS status, reviewed their preoperative ECGs for markers of ischemia. We assessed associations of study parameters using Wilcoxon rank-sum and Fisher's exact tests. Out of 34 patients, 62% were male, with a median age of 20 months [IQR: 8, 34]. Coronary artery stenosis was present in 62% (21/34), 76% (16/21) of whom were male. There were no ECG indicators of myocardial ischemia in patients with CAS. In conclusion, CAS was present in over half of children with WS/elastin arteriopathy who underwent repair of SVAS. Coronary artery stenosis in WS/non-syndromic elastin arteriopathy does not appear to exhibit typical ECG-detectable myocardial ischemia. ECGs are not a useful screening tool for CAS in WS/elastin arteriopathy. Given the high anesthesia-related cardiac arrest risk, other noninvasive indicators of CAS are needed.

• Authors: Claudia Algaze, Henry Chubb, Anna M Deitch, R Thomas Collins 2nd
• Journal: The American journal of cardiology (Am J Cardiol) (Nov 12 2023) ISSN: 1879-1913 [Electronic] United States
• PMID: 37963512 (Publication Type: Journal Article)
• Copyright: Copyright © 2023. Published by Elsevier Inc.