Extrinsic compression of an anomalous left main coronary artery in a patient with pulmonary arterial hypertension presenting with myocardial injury

Abstract

Extrinsic compression of the left main coronary artery (LMCA) caused by severe pulmonary arterial dilatation in the setting of pulmonary arterial hypertension (PAH) is a recognised entity. This can present with angina, cardiogenic shock, malignant arrhythmias or sudden cardiac death. We report the case of a 17-year-old female with a history of primary PAH who presented with acute chest discomfort, elevated biomarkers and ECG changes. Invasive and non-invasive imaging confirmed her diagnosis and identified an anomalous origin of the left main coronary artery and significant LMCA extrinsic compression by an enlarged main pulmonary artery (MPA). The abnormal anatomical location of the LMCA resulting from its anomalous origin could have further contributedto the risk of compression.