Frequency, Predictors, and Prognostic Impact of Pulmonary Artery Aneurysms in Patients With Pulmonary Arterial Hypertension

Abstract

Detection of pulmonary artery aneurysms (PAA) in pulmonary arterial hypertension (PAH) is increasing. We sought to determine the frequency of PAA in a PAH cohort, variables related to its development and its prognostic impact. We conducted a retrospective analysis of PAH patients who underwent a computed tomography or magnetic resonance. PAA was defined as a pulmonary artery >40 mm. Baseline, echocardiographic, and hemodynamic findings at PAH diagnosis were compared. Freedom from death or lung transplant was estimated by Kaplan-Meier method and compared by log-rank test. Predictors of PAA development were analyzed with multivariate models. Two-hundred patients underwent a computed tomography and/or magnetic resonance. In 77 (38%), a PAA (48.3 ± 7.2 mm) was detected. Time-course (months) of PAH was an independent risk factor for PAA (hazard ratio 1.01; 95% confidence interval 1.002 to 1.019; p = 0.016) whilst connective tissue disease was associated with a lower risk (hazard ratio 0.236; 95% confidence interval 0.060 to 0.920; p = 0.037). PAA patients showed lower rates of death and lung transplant from PAH diagnosis (p = 0.005), but no differences appeared when survival analysis was performed from first imaging test (p = 0.269). PAA patients presented a nonsignificant higher rate of sudden death (5% PAA vs 1% no-PAA; p = 0.073). In conclusion, the frequency of PAA was 38%. PAH time-course was an independent risk factor for PAA development whereas connective tissue disease -related PAH patients showed a lower risk. PAA patients showed lower rates of death or lung transplant from PAH diagnosis but no differences were found from imaging test. PAA patients had a nonsignificant higher rate of sudden death.