Congenital Left Coronary Atresia: Diagnostic Pearls and Management Challenges in Two Children Presenting with Syncope

Rachel T Sullivan,Jennifer F Gerardin,Peter C Frommelt,Todd M Gudausky

doi:10.1016/j.case.2021.05.003

Abstract

Congenital atresia of the left main coronary artery (LMCA) is a rare anomaly in which the LMCA does not receive antegrade flow from any vessel and instead receives exclusively retrograde flow from right coronary artery (RCA) collaterals. Its estimated incidence is 0.04%.1 Clinical presentation is varied and can manifest as heart failure and sudden cardiac death in infancy and childhood or as angina in adulthood.2 We present two pediatric cases with different clinical presentation, subtle initial diagnostic findings, and surgical management challenges.

Highlights

Congenital atresia of the left main coronary artery (LMCA) is a rare anomaly in which the LMCA does not receive antegrade flow from any vessel and instead receives exclusively retrograde flow from right coronary artery (RCA) collaterals
Cardiac catheterization was performed with angiography, demonstrating atresia of the left coronary ostia and long-segment LMCA atresia with retrograde filling of the left anterior descending (LAD) and circumflex through collaterals from a dilated RCA (Figure 3, Videos 3 and 4)
Their work highlights that symptoms are common in this lesion at any age, pediatric patients are more likely to present with severe symptoms that may include heart failure, syncope, or sudden cardiac death.[2]

Summary

INTRODUCTION

Congenital atresia of the left main coronary artery (LMCA) is a rare anomaly in which the LMCA does not receive antegrade flow from any vessel and instead receives exclusively retrograde flow from right coronary artery (RCA) collaterals. Cardiac catheterization was performed with angiography, demonstrating atresia of the left coronary ostia and long-segment LMCA atresia with retrograde filling of the LAD and circumflex through collaterals from a dilated RCA (Figure 3, Videos 3 and 4). Angiography demonstrated left coronary ostial atresia with retrograde filling of the left coronary artery through collaterals from the RCA, identifying both the LAD and circumflex, which joined to form a well-developed LMCA that ended blindly in proximity to the aortic root (Figure 7, Video 9). He went to the operating room on ECMO support for coronary artery revascularization. He has had normal interval growth and development without arrhythmias or cardiovascular symptoms

DISCUSSION

CONCLUSION