Unusual cause of left ventricular dysfunction in a child
Maria Emanuel Amaral1, Pedro Epifânio1, Natália Noronha1
1Serviço de Cardiologia Pediátrica - Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
Insights
An unusual congenital heart defect, Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA), was diagnosed in an 8-year-old. Successful surgical repair created a dual coronary artery system, highlighting diagnostic challenges.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiovascular Surgery
Background:
- Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) is a rare congenital heart defect.
- It often leads to myocardial ischemia in children and high mortality within the first year if undiagnosed.
- Early diagnosis and intervention are critical for survival.
Observation:
- An 8-year-old asymptomatic child presented with left ventricular dilatation and dysfunction.
- Echocardiogram revealed turbulent flows at the interventricular septum, suggesting ALCAPA.
- Previous diagnosis at 3 months was heart failure with dilated cardiomyopathy.
Findings:
- Computerized angiotomography and cardiac catheterization confirmed ALCAPA.
- The patient underwent successful surgical reimplantation of the left coronary artery to the aorta.
- This created a dual coronary perfusion system, restoring normal blood flow.
Implications:
- This case highlights an unusual long-term survival of undiagnosed ALCAPA.
- It underscores the importance of high clinical suspicion and multimodality imaging in diagnosing rare pediatric cardiac conditions.
- Successful surgical correction offers a favorable prognosis for ALCAPA patients.
Abstract:
Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery is a rare congenital heart disease and a cause of myocardial ischemia during childhood. Most undiagnosed cases die in the first year of life as an extensive collateral network is essential for survival. The diagnosis requires a high index of clinical suspicion. The authors present the case of an 8-year-old black asymptomatic child referred from Cape Verde Island in order to clarify left ventricular dilatation and dysfunction with systo-diastolic turbulent flows observed at the interventricular septum. At the age of 3 months, she was diagnosed with heart failure, in the context of showing dilated cardiomyopathy. She was managed and clinically improved with anticongestive therapy, which she was still taking at the time of admission to our Center. The echocardiogram findings suggested Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery and the diagnosis was confirmed by computerized angiotomography and cardiac catheterization. The patient was successfully submitted to direct implantation of the left coronary artery into the aorta, allowing the creation of a double coronary perfusion system. This case illustrates an unusual presentation of a rare pathology that survived without a diagnosis after the first year of life. It also reinforces the importance of multimodality image screening in these cases.
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