Abstract

Abstract Introduction Pulmonary artery aneurysm (PAA) is a common condition among pulmonary arterial hypertension (PAH) patients (38%). A pulmonary artery diameter ≥48 mm is an independent risk factor for sudden cardiac death in PAH patients. In the past years, the diagnosis of potentially lethal complications related to PAA is increasing. These complications include pulmonary artery dissection, bronchial compression and left main coronary artery compression (LMCAC). Our aim is to compare the survival and cause of death between PAA patients with a diagnosed and treated LMCAC and the rest of our PAA population. Methods Among our PAH population, we selected those patients who had a PAA diagnosis, defined as main pulmonary artery dilatation >40 mm. All the information was gathered from our institutional database. Patients with a suspected LMCAC based on computed tomography findings were evaluated with coronary angiography and treated surgically or percutaneously when LMCAC was confirmed. Survival from PAH diagnosis was estimated by the Kaplan–Meier method and compared by log-rank test between groups. Results 86 patients in our cohort were diagnosed with a PAA (24% male, 49.8±12.5 years). In 10 (12%) patients, a LMCAC was diagnosed, 9 of them were treated with a percutaneous coronary intervention and the remaining one underwent PAA resection surgery due to severe pulmonary regurgitation. Among patients with LMCAC, only 3 presented chest pain and 2 had signs of ischemia in cardiopulmonary stress testing. In the other 5 patients, LMCAC was suspected after performing a thoracic computed tomography for different reasons: chronic cough in 1 patient, congenital heart disease evaluation in 2 patients and asymptomatic PAA follow-up in 2 patients. None of the patients presented complications secondary to revascularization. There were no differences between patients with and without LMCAC neither in age (49.5±12.9 vs. 49.6±12.4 years; p=0.982) nor in PAH hemodynamic severity (mean pulmonary artery pressure: 63.1±27.6 vs. 56.6±15.0 mmHg; p=0.486). Patients with LMCAC had larger median pulmonary artery diameter (52.5 [47–60.3] vs 43 [46–50] mm; p=0.024). Among LMCAC patients, 1 (10%) died during follow-up due to neoplastic disease. Among patients without LMCAC, 18 (24%) died (5 [6%] sudden cardiac death; 8 [11%] heart failure; 5 [6%] neoplastic or infectious diseases). Despite LMCAC diagnosis, there were no differences between both groups in time from PAH diagnosis to death (log rank test p=0.38). Conclusion LMCAC is a common complication among PAH patients with a PAA. Its early identification and treatment might reduce the rate of sudden cardiac death among PAH patients. Since LMCAC is often asymptomatic regular screening with non-invasive tests should be incorporated to PAH follow-up protocols.

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