Pulmonary arterial hypertension (PAH) is still a fatal disease persisting with poor prognosis, despite all the advances in treatment (new agents, new combination strategies) in recent years. Patients present with different symptoms which are not specific to the disease (dyspnea, angina, palpitation, syncope). Angina may occur secondary to myocardial ischemia due to increased right ventricular (RV) afterload (oxygen supply and demand mismatch) or external compression on the left main coronary artery (LMCA). Left main coronary artery compression is associated with post-exercise sudden cardiac death (SCD) in PAH patients. It should be kept in mind in differential diagnosis of angina in patients with PAH and should be treated immediately. Here, we report a PAH patient associated with secundum type atrial septal defect (ASD) presented with ostial LMCA compression caused by enlarged pulmonary artery (PA) and treated with IVUS-guided percutaneous coronary intervention (PCI).
Keywords: atrial septal defect, pulmonary arterial hypertension, LMCA compression, intravascular ultrasound, adult congenital heart disease, percutaneous coronary intervention.Copyright © 2023 Archives of the Turkish Society of Cardiology