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Lynchburg Journal of Medical Science

Lynchburg Journal of Medical Science

Specialty

Congenital Heart Surgery

Advisor

Thomas Colletti, DHSc, PA-C, DFAAPA

Abstract

Abstract

Background:

Sudden cardiac death (SCD) in the pediatric population is alarming, rare, and tragic. Sudden cardiac death is defined as death occurring within an hour of onset of symptoms, arising from an underlying cardiac disease. This clinical review article focuses on the presentation, diagnosis, work-up, and treatment of anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva.

Objectives:

To understand the anatomy of an anomalous aortic origin of a coronary artery, the clinical presentation and work-up for the diagnosis, risk stratification of sudden cardiac death in patients with an anomalous coronary artery, and surgical interventions that are available for treatment.

Methods:

We performed a systematic review of articles identifying the risk of sudden cardiac death in the pediatric population due to an AAOCA, the diagnosis and management of patients with AAOCA, and the current guidelines for surgical intervention in patients with AAOCA

Conclusions:

Patients with an anomalous aortic origin of a right or left coronary artery who are symptomatic or have an anomalous left coronary artery and high-risk anatomy defined as a coronary artery with origin from the inappropriate sinus of Valsalva with an interarterial course with or without an intramural course should undergo surgical intervention to decrease his or her risk of SCD. In patients who are asymptomatic or at a lower calculated risk of sudden cardiac death, surgical intervention remains controversial.

Abstract word count: 233

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