Anomalous Left Coronary Artery from Pulmonary Artery: An Important Cause of Ischemic Mitral Regurgitation in Children
Muhammad Kamran Younis Memon1, Muneer Amanullah2, Mehnaz Atiq3
1Pediatrics and Child Health, Liaquat National Hospital and Medical College, Karachi, USA.
Insights
Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect. Unexplained mitral regurgitation in survivors suggests ALCAPA, with surgical repair significantly improving heart function.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Pediatric Cardiology
Background:
- Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly.
- Infantile presentation typically involves congestive heart failure or inconsolable crying due to myocardial ischemia.
- Late survivors often present with mitral regurgitation from papillary muscle ischemia.
Purpose of the Study:
- To highlight unexplained mitral regurgitation as a diagnostic clue for ALCAPA.
- To evaluate the outcomes of surgical reimplantation in ALCAPA patients.
Main Methods:
- Retrospective review of six ALCAPA patients operated between June 2017 and May 2018.
- Analysis of clinical presentation, electrocardiography, echocardiography, and angiographic findings.
- Assessment of surgical outcomes, including ventricular function and mitral regurgitation.
Main Results:
- All six patients presented with mitral regurgitation; two had signs of cardiac failure.
- The mean left ventricular ejection fraction was 52 ± 12%, with all patients showing mitral regurgitation.
- Surgical reimplantation was successful, leading to improved left ventricular function and reduced mitral regurgitation post-operatively.
Conclusions:
- ALCAPA diagnosis can be challenging, especially in late presenters.
- Persistent, unexplained mitral regurgitation should prompt suspicion for ALCAPA.
- Surgical intervention for ALCAPA yields excellent outcomes, improving both cardiac function and mitral regurgitation.
Introduction:
Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. The usual presentation in infancy is inconsolable crying or congestive cardiac failure, both due to myocardial ischemia. Survivors after infancy have improved left ventricular function but continue to have mitral regurgitation due to papillary muscle ischemia. The present study emphasizes the importance of unexplained mitral regurgitation as a clue to the diagnosis.
Patients And Methods:
Patients with the diagnosis of ALCAPA operated between June 2017 and May 2018 were enrolled. Their ages at diagnosis, electrocardiography, and echocardiography findings were noted. A selective angiogram of the right coronary artery was done in all. Results of surgical reimplantation were analyzed. Postoperative data were collected, including ventricular function and mitral regurgitation.
Results:
Six patients were included. Clinical signs of cardiac failure were present in two patients, and a systolic murmur was heard in all. The mean left ventricular ejection fraction was 52 ± 12%. Mitral regurgitation was present in all of the patients. The right coronary artery was dilated (Z score > 2.5) in all except one. Selective right coronary angiogram and cardiac computerized tomography angiogram (CTA) were performed in all. Coronary reimplantation was successfully done. Follow-up echocardiography showed improved left ventricular ejection fraction and degree of mitral regurgitation in all patients.
Conclusion:
ALCAPA is an uncommon congenital anomaly, the diagnosis of which can be missed, particularly in late presenters. Unexplained mitral regurgitation should always raise the suspicion of this anomaly. Surgical intervention has excellent results with an improvement of left ventricular function and mitral regurgitation.
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