Case Report

Diagnosis of Anomalous Aortic Origin of the Left Coronary Artery in a Pediatric Patient

World Journal for Pediatric and Congenital Heart Surgery 2015, Vol. 6(3) 470-473 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135114558689 pch.sagepub.com

Alisa Arunamata, MD1, Katie Jo Buccola Stauffer, BS, RDCS1, Rajesh Punn, MD1, Frandics P. Chan, MD, PhD2, Katsuhide Maeda, MD, PhD3, and Sowmya Balasubramanian, MD1

Abstract Anomalous aortic origin of the left coronary artery is rare and confers increased risk of sudden cardiac death. Accurate diagnosis is crucial and often requires many diagnostic modalities. This case report highlights the echocardiographic characteristics and pitfalls in diagnosing the anomaly in addition to the advantages of using computed tomography and magnetic resonance imaging in a pediatric patient. Keywords coronary artery anomaly, coronary artery imaging (all modalities), congenital heart disease (CHD), pediatric Submitted May 28, 2014; Accepted October 13, 2014.

Background Anomalous aortic origin of the left coronary artery is rare and confers increased risk of sudden cardiac death during or after exercise in otherwise asymptomatic children and adolescents. An estimated 17% of sudden deaths among young athletes in the Unites States are attributed to this lesion.1 If clinically suspected, transthoracic echocardiography is typically the

primary screening tool for this diagnosis, although its accuracy can be limited, given the rare occurrence and often misleading two-dimensional images.2 This case report highlights the echocardiographic characteristics and pitfalls in diagnosing the anomaly in addition to the advantages of using computed tomography (CT) and magnetic resonance imaging (MRI) in a pediatric patient.

Case Presentation A previously healthy 8-year-old Asian male presented to our institution after a near-syncopal event while playing soccer. After arrival at the hospital, the patient had frequent runs of nonsustained, hemodynamically unstable ventricular tachycardia despite multiple lidocaine boluses and an amiodarone infusion. Given the incessant ventricular tachycardia and 1 Division of Pediatric Cardiology, Lucile Packard Children’s Hospital, Stanford University Medical Center, Palo Alto, CA, USA 2 Department of Radiology, Stanford University Medical Center, Stanford, CA, USA 3 Department of Cardiovascular Surgery, Stanford University Medical Center, Stanford, CA, USA

Figure 1. Transthoracic two-dimensional imaging in the parasternal short-axis view of the anomalous left coronary artery (LCA) appearing to arise from the left sinus as it exits the aortic wall. þ indicates right coronary cusp; *, left coronary cusp.

Corresponding Author: Alisa Arunamata, MD, Division of Pediatric Cardiology, Lucile Packard Children’s Hospital, Stanford University Medical Center, 750 Welch Road, Suite #305, Palo Alto, CA 94304, USA. Email: [email protected]

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Figure 2. Transthoracic parasternal short-axis view with color (A) and spectral Doppler (B) revealing high-velocity diastolic flow coursing away from the right sinus of Valsalva. LCA indicates left coronary artery; þ, right coronary cusp; *, left coronary cusp.

non-reassuring clinical examination, he was emergently intubated and cannulated onto venoarterial extracorporeal membrane oxygenation (VA-ECMO) with a presumed diagnosis of acute myocarditis. Initial echocardiogram (Phillips iE33, Phillips Medical Systems, Bothell, Washington) while supported on VA-ECMO had limited acoustic windows and revealed only a mildly dilated left atrium and left ventricle with estimated left ventricular ejection fraction of

Diagnosis of Anomalous Aortic Origin of the Left Coronary Artery in a Pediatric Patient.

Anomalous aortic origin of the left coronary artery is rare and confers increased risk of sudden cardiac death. Accurate diagnosis is crucial and ofte...
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