Abstract 16590: Late Myocardial Infarction After Fontan in Pulmonary Atresia With Intact Ventricular Septum
Background: Right ventricle (RV)-to-coronary fistulae, coronary stenoses, and atresia are common in pulmonary atresia with intact ventricular septum (PA-IVS). Myocardial infarction (MI) and sudden death after RV decompression in the neonate have been associated with coronary anomalies that create a RV-dependent coronary circulation. There is a paucity of data, however, on long-term coronary sequelae after Fontan completion.
Methods: A retrospective review of patients followed at our institutions who underwent Fontan for PA-IVS between 1985 and 2013 was performed. Patients with antegrade pulmonary blood flow were excluded. Charts were reviewed for sudden death as well as for clinical, radiologic, and angiographic evidence of myocardial infarction/ischemia.
Results: Forty-six patients met inclusion criteria with a median age at Fontan of 2.6 (range 1.2 - 18.9) years. Median follow-up from Fontan was 3.5 (range 0 - 27.9) years. Twenty-three patients (50%) had adequate angiograms for review and of those, 18 (75%) had RV-to-coronary fistulae; 13 (54%) had fistulae with coronary stenoses; and 2 (8.3%) had aortocoronary atresia. There were 2 early deaths (4.3%) but neither had angiograms nor non-invasive tests of myocardial perfusion for review. Five patients (11%) had evidence of ischemia. Of those, 2 (4.3%) had non-fatal MI at 16.9 and 18.8 years post-Fontan. Both patients had fistulae and one had severe mid-right coronary stenosis. Coronary anomalies correlated with ischemic territory on non-invasive testing in both. Three additional patients had evidence of ischemia on stress test and/or cardiac MRI without clinical evidence of MI. Two of those had angiograms, both with fistulae and coronary stenoses that correlated with ischemic territory on non-invasive testing. No patient had traditional risk factors for atherosclerotic coronary disease. No patient underwent coronary revascularization. There was no sustained ventricular tachycardia or sudden death. There were no late deaths.
Conclusion: Patients with PA-IVS and single-ventricle palliation remain at risk for long-term myocardial ischemia. Further study is necessary to define risk factors for ischemic outcomes.
Author Disclosures: A.J. Small: None. Y.Y. Kim: None. A.C. Glatz: Research Grant; Modest; Children’s Heart Foundation. Consultant/Advisory Board; Modest; Bristol-Myers Squibb, Inc.. K.K. Whitehead: None. K.Y. Allen: None. T.E. Downing: None. R. Donner: None. S. Fuller: None. T.M. Giglia: None.
- © 2014 by American Heart Association, Inc.