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Myocardial ischemia in patients with pulmonary atresia and intact ventricular septum
Insights
Myocardial ischemia is common in children with pulmonary atresia and intact ventricular septum, often occurring after surgery. This condition can be present with or without coronary artery abnormalities.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Pathology
- Congenital Heart Disease
Background:
- Pulmonary atresia with intact ventricular septum is a complex congenital heart defect.
- Myocardial ischemia can be a serious complication in affected children.
- Understanding the causes of ischemia is crucial for patient management.
Purpose of the Study:
- To investigate the relationship between myocardial ischemia and coronary artery abnormalities in children with pulmonary atresia and intact ventricular septum.
- To assess the role of surgical interventions in the development of myocardial ischemia.
Main Methods:
- Histological examination of 17 autopsy specimens from children deceased after surgery for pulmonary atresia and intact ventricular septum.
- Assessment for evidence of myocardial ischemic injury.
- Evaluation of the presence of right ventricle to coronary artery fistulas, coronary artery dysplasia, and surgical history.
Main Results:
- Myocardial ischemia was observed in hearts with and without right ventricle to coronary artery fistulas or coronary artery dysplasia.
- Ischemia occurred in children who underwent right ventricular outflow reconstruction or shunt operations.
- Coronary artery abnormalities were present in a significant proportion of cases, but ischemia was not exclusively linked to them.
Conclusions:
- Myocardial ischemia is a frequent finding in pediatric patients with pulmonary atresia and intact ventricular septum.
- Ischemia can occur irrespective of coronary artery anomalies, such as fistulas or dysplasia.
- Surgical procedures, including right ventricular outflow reconstruction and shunt operations, may be associated with myocardial ischemia.
Abstract:
Children who die after operation for pulmonary atresia and intact ventricular septum may have myocardial ischemia. The relation between histologic evidence of myocardial ischemic injury and the presence of a right ventricle to coronary artery fistula, coronary artery dysplasia and operation in 17 autopsy specimens was assessed. Age at death ranged from 1 day to 16 years (median, 11 days). Of the 17 hearts, 6 (35%) had right ventricle to coronary artery fistulas, 5 of which had coronary artery dysplasia. In three cases, there was segmental or complete absence of a coronary artery. Ischemia was present in four of these six hearts, two of which had right ventricular outflow reconstruction. Six of the 11 hearts without right ventricle to coronary artery fistulas also had myocardial ischemia. Of these six cases, four had right ventricular outflow reconstruction and two had shunt operations. Death occurred from 1 to 8 days (mean 3) after operation. Hearts with pulmonary atresia and intact ventricular septum may have myocardial ischemia with or without either right ventricle to coronary artery fistulas or coronary artery dysplasia. Myocardial ischemia may occur after right ventricular outflow reconstruction or shunt operations. Thus, myocardial ischemia occurs commonly in patients with pulmonary atresia and intact ventricular septum and is not always related to coronary abnormalities or operation.