Chest pain in a patient with pulmonary arterial hypertension

Chest pain is a frequent symptom in patients with pulmonary arterial hypertension (PAH). Left main coronary artery (LMCA) extrinsic compression from a pulmonary artery (PA) is an increasingly recognized cause of angina or complications, such as acute myocardial infarction, left ventricular dysfunction, arrythmia, and sudden death. We report the case of a 45-year-old patient with pre-capillary pulmonary hypertension (PH), a patent ductus arteriosus corrected surgically during adolescence, and chronic constrictive bronchiolitis. In 2016, the patient began to report oppressive chest pain and worsening fatigue. Computed tomography coronary angiography (CTCA) showed extrinsic LMCA compression by a dilated PA, which was confirmed by invasive coronary angiography and intravascular ultrasound. After stent implantation, the patient reported symptom resolution, and has been asymptomatic ever since. Imaging studies, in particular CTCA, play an important role in the diagnosis of LMCA compression in patients with PAH. The reported case supports the efficacy and safety of stent implantation as a therapeutic option, as already demonstrated in the literature. It shows the complexity of decision making on the operability of systemic-to-pulmonary shunts and reinforces the importance of continuous diagnostic testing.