What is the clinical evidence from this study?

Study design: Case Report. Population: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) (n=1). Intervention: Left coronary artery reimplantation and mitral valvuloplasty.

What question did this study set out to answer?

This report aims to highlight the importance of recognizing ALCAPA in pediatric patients presenting with cardiac dysfunction.

May 11, 2026Open Access

Beyond the usual suspects: unmasking ALCAPA in pediatric cardiomyopathy – case report

Key Result

Surgical reimplantation of the left coronary artery and mitral valvuloplasty successfully restored ventricular function and improved mitral regurgitation in a 9-year-old boy with atypical ALCAPA.

Key Points

This report aims to highlight the importance of recognizing ALCAPA in pediatric patients presenting with cardiac dysfunction.
Case presentation of a 9-year-old boy with dilated cardiomyopathy and asthma exacerbation.
Diagnostic tools included transthoracic echocardiography, cardiac magnetic resonance imaging, and cardiac catheterization.
Surgical intervention involved left coronary artery reimplantation and mitral valvuloplasty.
Left ventricular ejection fraction was reduced, and significant mitral regurgitation and pulmonary hypertension were observed.
Echocardiographic signs typical of ALCAPA were absent, complicating the diagnosis.
Postoperative evolution was favorable following successful surgery.

Study Design

Type

Case Report (n=1)

Multicenter

Structured PICO

Population

1 9-year-old boy with a history of dilated cardiomyopathy and poorly controlled asthma, presenting with an asthma exacerbation, severe mitral regurgitation, and severe pulmonary hypertension.

Intervention

Left coronary artery reimplantation and mitral valvuloplasty

Outcome

Postoperative clinical and echocardiographic evolution

ALCAPA can present atypically in older children without classic echocardiographic signs, mimicking idiopathic dilated cardiomyopathy and asthma, and requires high clinical suspicion and targeted imaging for diagnosis.

Limitations

Echocardiography is highly operator-dependent and contingent upon adequate acoustic windows and appropriate clinical suspicion.
Standard cardiac MRI performed for cardiomyopathy assessment may not adequately visualize coronary origins without a dedicated protocol.
Electrocardiography in older children may lack classic ischemic features.

Abstract

INTRODUCTION: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially lethal congenital heart disease. Delayed recognition may result in irreversible myocardial injury. Although the classic presentation occurs in infancy, atypical forms may appear in older children, making this entity a diagnostic challenge. CASE PRESENTATION: We report the case of a 9-year-old boy with a history of dilated cardiomyopathy (DCM) and poorly controlled asthma who was admitted for an asthma exacerbation. Transthoracic echocardiography revealed Left ventricular ejection fraction (LVEF) reduced, left ventricular dysfunction, severe mitral regurgitation (MR), and pulmonary hypertension (PH). Notably, classic echocardiographic hallmarks of ALCAPA-such as coronary dilation, collateral circulation, and retrograde filling of the left coronary artery-were absent, rendering this case particularly unusual. Cardiac magnetic resonance imaging and cardiac catheterization ultimately confirmed the diagnosis. The patient underwent successful left coronary artery reimplantation and mitral valvuloplasty, with favorable postoperative evolution. DISCUSSION: This case illustrates how ALCAPA can mimic idiopathic dilated cardiomyopathy and asthma-related cardiopulmonary disease, underscoring the importance of systematically evaluating coronary arteries to avoid delayed recognition. Indirect echocardiographic signs, including segmental wall motion abnormalities and mitral regurgitation, should prompt careful coronary evaluation. Echocardiography and coronary CT/CMR are considered as the primary diagnostic tools, however catheterization or coronary CT angiography may be used in atypical cases although is not routine. CONCLUSION: ALCAPA should be considered in any child with unexplained ventricular dysfunction or severe mitral regurgitation. High clinical suspicion and targeted imaging are essential for timely diagnosis and optimal outcomes.

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Cite This Study

C et al. (Fri,) conducted a case report in Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) (n=1). Left coronary artery reimplantation and mitral valvuloplasty was evaluated. Surgical reimplantation of the left coronary artery and mitral valvuloplasty successfully restored ventricular function and improved mitral regurgitation in a 9-year-old boy with atypical ALCAPA.

synapsesocial.com/papers/6a0171ce3a9f334c28271cda https://doi.org/https://doi.org/10.1186/s12887-026-06652-6

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