Abstract
Children dying after operation for pulmonary atresia and intact ventricular septum (PA-IVS) may have evidence of myocardial ischemia. The etiology of ischemia in these patients is unknown. We assessed the relationship between myocardial ischemia and the presence of right ventricular to coronary artery fistulae in necropsy specimens from 17 patients (pts) with PA-IVS. Age at death was from 1 day to 16 years (mean 17 months). Six of 17 pts (35%) had right ventricular to coronary artery fistulae, 5 of whom had coronary dysplasia. Three pts had segmental or complete absence of a coronary artery. Histologic evidence of M.I. was present in 4/6 pts, 2 of whom had RV outflow reconstruction. Six of 11 pts without right ventricular to coronary artery fistulae had myocardial ischemia. Of these, 4 had right ventricular outflow reconstruction and 2 had shunt operations. Death occurred from 1 to 8 days (mean 3 days) postoperatively.
Pts with PA-IVS may have myocardial ischemia with or without either right ventricular to coronary artery fistulae or coronary dysplasia. Myocardial ischemia may follow right ventricular outflow reconstruction or shunt operations. Thus, myocardial ischemia occurs commonly with PA-IVS and does not always relate to coronary abnormalities or right ventricular outflow reconstruction. Myocardial ischemia may be due to poor subendocardial perfusion of a small chambered, yet hypertrophic and hypertensive ventricle.
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Fyfe, D., Edwards, W. & Driscoll, D. MYOCARDIAL ISCHEMIA IN PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM. Pediatr Res 18 (Suppl 4), 122 (1984). https://doi.org/10.1203/00006450-198404001-00173
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DOI: https://doi.org/10.1203/00006450-198404001-00173