Background: Sudden death is the leading cause of mortality in patients with transposition of the great arteries (TGA) and atrial switch surgery. Understanding underlying mechanisms could contribute to identifying high-risk patients and preventing such catastrophic deaths.
Methods: A total of 144 adults (≥18 years) with TGA and atrial switch surgery were followed at our adult congenital center since 1989. Four patients were excluded: two with double-outlet right ventricles and two with subsequent arterial switch surgery in childhood.
Results: Of the remaining 140 patients, age 37.6 ± 7.8 years, 37.1% female, 8 (6%) had a cardiac arrest of presumed arrhythmic etiology of whom 3 were resuscitated. The arrests occurred in 3 women and 5 men at age 30.5 ± 8.6 (range 22 to 50) years. None had established coronary artery disease, sustained ventricular arrhythmias, or syncope. Four (50%) had atrial arrhythmias and 6 (75%) had at least moderate systemic right ventricular dysfunction. For 5 patients in whom circumstances surrounding the arrests were documented, 3 occurred on exertion, 1 after consuming recreational methamphetamine, and 1 in the context of an atrial tachyarrhythmia. Autopsies were performed in 2 of 5 patients. Both revealed acute massive myocardial infarction of the hypertrophied systemic right ventricle with normal coronary arteries and chronic subendocardial ischemic lesions.
Conclusion: This is the first report to provide histopathological evidence in support of a myocardial ischemia hypothesis as a cause of sudden death in this patient population, despite the absence of coronary atherosclerosis.
Keywords: Atrial switch surgery; Mustard; Senning; Sudden cardiac death; Transposition of the great arteries.
Copyright © 2019 Elsevier B.V. All rights reserved.