Anomalous origin of the left coronary artery from the proper sinus with acute angulation and an intramural segment
Tsukasa Torigoe1, Seiichi Sato, Chizuo Kikuchi
1Department of Pediatrics, Niigata City General Hospital, Niigata, Japan.
Insights
A rare coronary artery anomaly originating from the correct sinus caused ischemic events in a child. Surgical unroofing successfully resolved symptoms, highlighting risks of abnormal ostial location.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Coronary Artery Anomalies
Background:
- Anomalous origin of the coronary artery from the wrong sinus (AOCARS) is a known cause of myocardial ischemia.
- This case presents a rare variation where the coronary artery originates from the correct sinus but has an abnormal ostial course.
Observation:
- A child presented with ischemic events attributed to a left main coronary artery originating from the left sinus.
- Imaging revealed horizontal displacement of the ostium, proximity to valve commissures, an acute takeoff angle, and an intramural course.
Findings:
- The anomalous coronary artery ostium, despite originating from the proper sinus, possessed features (acute takeoff, intramural segment) predisposing to ischemia.
- Surgical unroofing was effective in correcting the anomaly and alleviating ischemic symptoms.
Implications:
- Abnormal coronary ostial location, even from the correct sinus, can mimic AOCARS and pose risks of ischemia and sudden cardiac death.
- Early diagnosis and surgical intervention are crucial for managing this rare but potentially life-threatening condition in pediatric patients.
Abstract:
This is the first report of a coronary artery with an anomalous origin from the proper sinus resulting in ischemic events in a child. Transthoracic echocardiogram, computed tomogram, and coronary angiogram revealed that, although the left main coronary trunk originated from the left sinus, its ostium was displaced horizontally and was located near the commissure between the left and noncoronary valve cusps. Moreover, it was associated with an acute take off angle and an intramural segment, which are known contributing features for ischemia in cases of anomalous origin of a coronary artery from the wrong sinus. Surgical intervention, involving the unroofing procedure, was employed successfully to eliminate the ischemic events. At the latest follow up, no chest pain was reported and the transthoracic echocardiogram showed no stenosis of the neo-ostium. Even in a coronary artery that originates from the proper sinus, an abnormal ostial location could be associated with an acute takeoff angle and an intramural segment. This finding is extremely rare but entails the risk of ischemia and sudden death.
Related Concept Videos
01:21Coronary Circulation
Coronary circulation begins at the base of the aorta, where two main arteries arise—the left and right coronary arteries. These arteries encircle the heart in the coronary sulcus and supply the...
01:22Mitral Stenosis I: Introduction
01:18Physiology of the Heart: The Cardiac Cycle
Diastole: The Relaxation Phase
During diastole, all four heart chambers relax. The atrioventricular (AV) valves open, and the semilunar valves close. This phase sees the lowest chamber pressures, promoting ventricular filling. Venous blood enters the heart through the...
01:24Cardiac Catheterization III: Left Heart Catheterization
01:30Acute Coronary Syndrome I: Introduction
01:10The Arch of Aorta
Encircling the heart, the coronary arteries form a ring-like structure before...