An Uncommon Cause for Chest Pain in an Adult Patient with Cyanotic Congenital Heart Disease

Document Type

Article

Abstract

Background: Patients with adult cyanotic congenital heart disease (ACHD) are at decreased risk to develop atherosclerotic coronary disease yet can still present with signs and symptoms of coronary pathology. Coronary evaluation should be pursued to identify less common etiologies, including coronary artery fistula in this unique population.

Case: A 34-year-old female with heterotaxy syndrome complex single ventricle post Fontan palliation and sinus node dysfunction with pacemaker placement presented to the hospital with chest pressure. Acute coronary syndrome was ruled out. Coronary computed tomography angiogram revealed a coronary artery fistula between an anomalous coronary vessel that arose from the right coronary artery and the Fontan circuit proximal to the connection with the left pulmonary artery (Panel C). At cardiac catheterization, the coronary fistula (Panel A) was coil embolized with significant reduction in blood flow through the vessel (Panel B). At follow up her symptoms had completely resolved.

Decision-making: Coronary evaluation with advanced imaging should be considered in patients with ACHD who have symptoms of chest pain to identify complex coronary pathology including coronary fistula.

Conclusion: Coronary artery fistula should be considered in patients with cyanotic ACHD who have signs or symptoms of coronary insufficiency. This unique population requires special attention to less common etiologies for common presenting complaints during their initial evaluation.

First Page

2755

DOI

10.1016/S0735-1097(24)04745-4

Publication Date

4-2-2024

Link to Full Text

Share

COinS