ALCAPA syndrome: A rare etiology of cardiac arrest in a teenager

Abstract

ALCAPA syndrome is a rare congenital heart disease and a cause of myocardial ischemia in pediatric population. The authors present the case of a 10-year-old girl admitted to the emergency room after experiencing cardiac arrest at school. In the echocardiogram, the inability to identify the origin of the left coronary artery raised the hypothesis of abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA). A CT-scan and a cardiac catheterization were performed confirming the diagnosis. Infants with the syndrome may have myocardial infarction and congestive heart failure^. She successfully underwent cardiac surgery. Most undiagnosed patients die within the first year of life, and it is necessary to develop an extensive network of collaterals to ensure survival. ALCAPA syndrome rarely manifests in late childhood, teenagers, and adults and may be an important cause of sudden cardiac arrest.

1. Images in pediatrics

The authors present the case of a 10-year-old girl admitted to the emergency department after experiencing a sudden cardiac arrest at school. The first recorded rhythm was ventricular fibrillation, which was reverted to sinus rhythm after four minutes of advanced life support and electrical defibrillation.

The post event electrocardiogram revealed a left ventricular hypertrophy, subendocardial ischemia, and a mildly increased, corrected QT interval. Transthoracic echocardiography raised the hypothesis of an abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA), as the origin of the left coronary could not be identified and the right coronary artery was dilated. A CT-scan (Fig. 1, Fig. 2) and cardiac catheterization were performed, confirming the diagnosis (Fig. 3).

Fig. 1.

CT scan: Axial plane at the level of the aortic root where a dilated right coronary artery is shown, together with an absence of the left coronary artery ostium.

RCA: Right coronary artery; AO: Aorta.

Fig. 2.

CT scan: Origin of the LCA from the lower aspect pulmonary trunk.

LCA: Left coronary artery; MPA: Main pulmonary artery.

Fig. 3.

Selective angiography reveals a dilated right coronary artery with extensive collateralization to the left coronary artery, which is connected to the pulmonary trunk.

LCA: Left coronary artery; RCA: Right coronary artery.

She underwent cardiac surgery 13 days after presentation, with direct reimplantation of the proximal left coronary artery in the aortic root, with good postsurgical evolution. Her ECG at discharge presented only nonspecific repolarization abnormalities in the precordial leads. Now, 12 months later, the patient is doing well without any cardiovascular symptoms.

ALCAPA syndrome is a rare congenital defect being a recognized cause of myocardial ischemia in young patients [1]. Most undiagnosed cases die during the first year of life, with survival after infancy being related to the number of collaterals established between coronary arterial territories. Clinical presentation may include myocardial infarction and congestive heart failure [1,2]. ALCAPA syndrome rarely manifests in late childhood, teenagers, or adults, and may be an important cause of sudden cardiac arrest.

A high clinical suspicion is the key to establish the diagnosis [3]. Because high heart rates, typically observed in pediatric patients, may create some technical difficulties during the CT scan, investigation may need to be complemented with coronary angiography. Surgical correction should aim to establish dual coronary perfusion, which was carried out successfully in this patient [2].

Ethical statement

All authors listed have contributed sufficiently to the project to be included as authors. To the best of our knowledge, no conflict of interest, financial or other, exists.

Declaration of competing interest

None.