Isolated anomalous origin of right coronary artery from the main pulmonary artery
Chih-Ta Yao1, Jieh-Neng Wang, Cheng-Nan Yeh
1Department of Pediatrics, Sin Lau Hospital, Tainan, Taiwan.
Abstract:
The anomalous origin of the right coronary artery (ARCA) from the main pulmonary artery (MPA) is a rare congenital cardiac malformation and usually associated with other cardiac anomalies. Most patients with isolated ARCA from MPA remain asymptomatic, but they may develop myocardial ischemia and even sudden death. We reported an asymptomatic 7-year-old boy referred for evaluation of a heart murmur. Isolation of ARCA from MPA was diagnosed by echocardiography and then confirmed by cardiac catheterization and angiography. The right coronary artery was re-implanted into the ascending aorta. A preoperative thallium-201 myocardial perfusion showed a myocardial ischemia pattern in the anterolateral septal area after a dipyridamole stress test; the ischemia was completely resolved after surgery.
Insights
Anomalous origin of the right coronary artery (ARCA) from the main pulmonary artery (MPA) is rare. Surgical repair of isolated ARCA from MPA in an asymptomatic child resolved myocardial ischemia.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Pediatric Cardiac Surgery
Background:
- Anomalous origin of the right coronary artery from the main pulmonary artery (ARCA from MPA) is a rare congenital cardiac malformation.
- While often asymptomatic, ARCA from MPA can lead to myocardial ischemia and sudden death, especially when associated with other cardiac anomalies.
Observation:
- A 7-year-old asymptomatic boy presented with a heart murmur.
- Echocardiography, cardiac catheterization, and angiography confirmed isolated ARCA from MPA.
Findings:
- Preoperative thallium-201 myocardial perfusion imaging revealed myocardial ischemia in the anterolateral septal area following a dipyridamole stress test.
- Surgical re-implantation of the right coronary artery into the ascending aorta successfully resolved the ischemia.
Implications:
- This case highlights the importance of diagnosing and treating even asymptomatic ARCA from MPA to prevent potential ischemic events.
- Surgical correction of ARCA from MPA can effectively restore normal coronary perfusion and resolve myocardial ischemia in pediatric patients.
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