Anomalous Origin of Left Coronary Artery From Right Pulmonary Artery in Association With Scimitar Syndrome

Safwat A Aly; Ramiro W Lizano Santamaria; Shi-Joon Yoo

doi:10.1016/j.jaccas.2019.11.048

Anomalous Origin of Left Coronary Artery From Right Pulmonary Artery in Association With Scimitar Syndrome

JACC Case Rep. 2020 Feb 19;2(2):319-323. doi: 10.1016/j.jaccas.2019.11.048. eCollection 2020 Feb.

Authors

Safwat A Aly¹, Ramiro W Lizano Santamaria¹, Shi-Joon Yoo¹

Affiliation

¹ Department of Pediatrics, Division of Cardiology, the Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Abstract

This paper reports a unique case of anomalous origin of the left coronary artery from the right pulmonary artery associated with scimitar syndrome. The presence of pulmonary hypertension may have contributed to maintain coronary perfusion, which likely prevented early significant coronary steal. This study reports the clinical course, diagnosis challenges, and management strategy. (Level of Difficulty: Intermediate.).

Keywords: ALCAPA; ALCAPA, anomalous origin of the left coronary artery from pulmonary artery; AP collaterals; CPAP, continuous positive airway pressure; FRAS1 mutation; IVC, inferior vena cava; LAD, left anterior descending; LMCA, left main coronary artery; MPA, main pulmonary artery; PAH, pulmonary arterial hypertension; RA, right atrium; RPA, right pulmonary artery; RV, right ventricle; scimitar syndrome.

Publication types

Case Reports