C57. Left Main Coronary Artery Compression by A Dilatated Pulmonary Artery in Patients with Pulmonary Arterial Hypertension: What Should We Do?

Abstract

Abstract Background Pulmonary artery hypertension (PAH) is often associated with angina and extrinsic compression of left main coronary artery (LMCA) by a dilatated pulmonary artery is recognized as the cause. The optimal management for pulmonary arterial hypertension with extrinsic LMCA compression has not been clearly established. Case Summary We report on 31-year-old female with secundum atrial septal defect and 44 -year-old female with incomplete atrioventricular septal defect complain of progressive typical angina that aggravated with exertion. The electrocardiogram showed right heart strain and chest radiograph showed cardiomegaly involving the right cavities with enlargement of the pulmonary arch. Right heart catheterization showed pulmonary arterial hypertension with the mean pulmonary artery pressure of 61 mmHg and 65 mmHg respectively. Cardiac CT demonstrated compression of proximal LMCA by dilatated pulmonary artery. Although with optimal phosphodiesterase-5 (PDE5) inhibitor and prostacyclin analog therapy, patients still complain of angina. Discussion LMCA compression may be associated with additional complications of severe myocardial ischemia, including myocardial infarction, left ventricle dysfunction, arrhythmia, and eventually, sudden death. It may occur in patients with PAH with severely dilated PA and can be identified with either computed tomographic coronary angiography or selective coronary angiography. Furthermore, there are no guidelines recommending optimal management of this disease entity. Optimalization of pharmacologic treatment of PAH may reduce compression-related symptoms. A stent implantation is a promising alternative option for the treatment of LMCA compression and provides an optimal risk-to-benefit ratio, both for symptom relief and for long-term outcomes.