Anomalous origin of the right coronary artery evaluated with multidetector computed tomography and its clinical
Keiichi Hirono1, Yukiko Hata2, Naruaki Miyao1
1Department of Pediatrics, Graduate School of Medicine, University of Toyama, Toyama City, Toyama, Japan.
Insights
Anomalous origin of the right coronary artery (AORCA) is a rare congenital anomaly. Early diagnosis via MDCT and surgical intervention are crucial for preventing sudden cardiac death in affected individuals.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Imaging
Background:
- Anomalous origin of the right coronary artery (AORCA) is a rare congenital anomaly.
- AORCA can lead to myocardial ischemia and sudden cardiac death.
Purpose of the Study:
- To report on juvenile cases of AORCA with specific anatomical features.
- To highlight the diagnostic utility of MDCT in AORCA.
- To emphasize the importance of surgical intervention for AORCA.
Main Methods:
- Review of clinicopathological records of three juvenile AORCA cases.
- Comparison with two autopsy cases of sudden cardiac death due to AORCA.
- Diagnosis and surgical planning using multidetector computed tomography (MDCT).
Main Results:
- Three juvenile patients presented with exertional symptoms due to AORCA.
- MDCT accurately delineated the anomalous origin and intramural course of the RCA.
- All three juvenile patients underwent successful surgical unroofing of the RCA.
- Autopsy cases revealed slit-like orifices and long intramural RCA courses, similar to the juvenile cases.
Conclusions:
- Exertional symptoms may be the first sign of AORCA, necessitating vigilance.
- MDCT is highly effective in defining AORCA anatomy for surgical planning.
- Surgical unroofing of the RCA leads to favorable outcomes in AORCA patients.
Background:
Anomalous origin of the right coronary artery (AORCA) is a rare congenital anomaly that may cause myocardial ischemia and sudden death.
Methods:
We reviewed the clinicopathological records of three cases of AORCA, and compared these with two cases of sudden cardiac death with AORCA revealed by autopsy.
Results:
We report three juvenile cases with an AORCA originating above the commissural junction between the left and right aortic sinuses, with interarterial and intramural compression. They presented with exertional symptoms and were diagnosed with an AORCA by multidetector computed tomography (MDCT), which successfully delineated the spatial resolution of the anomalous origin and course of the right coronary artery (RCA), in the operating room. All three underwent successful surgical unroofing of the RCA. Two cases of sudden cardiac death with AORCA revealed by autopsy showed a slit-like orifice, acute-angled take-off, and long intramural course of the RCA, resembling the RCAs of three juvenile cases.
Conclusions:
It is crucial to be alert to the presentation of exertional symptoms, as sudden death may be the first manifestation of an anomalous coronary artery, such as those observed in these three cases. MDCT provided an excellent definition and spatial resolution of the unusual origin and intramural course of the RCA, facilitating the correct surgical remedy and resulting in a good outcome for the patients.
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