Influence of long-standing pulmonary arterial hypertension and its severity on pulmonary artery aneurysm development.

Pulmonary artery aneurysm (PAA) is a common finding in patients with long-term pulmonary arterial hypertension (PAH). The influence of PAH severity in the development of PAA remains unclear. We sought to determine whether PAA development is related to PAH severity and whether treatment optimization based on risk profile estimation is effective to stop pulmonary artery (PA) enlargement. This is a retrospective study of 125 PAH patients who underwent an imaging test (computed tomography or magnetic resonance) combined with a right heart catheterization within a six-month period. A multivariate analysis was performed to identify independent risk factors for PAA. Patients who underwent an additional imaging-test and RHC during follow-up were analyzed to evaluate changes on PA dimensions. PAA was diagnosed in 42 (34%) patients. PAA was more frequent in patients with congenital heart disease and toxic oil syndrome. PAH time-course showed to be an independent risk factor for PAA (HR 1.051, 95% CI 1.013-1.091, p = 0.008) whereas PAH severity did not. Twenty-six patients underwent a follow-up imaging-test and catheterization. After treatment optimization, a non-significant reduction of mean PA pressure was observed (58.5 mmHg [43.5-70.8] vs. 55.5 mmHg [47.5-66.3], p = 0.115) and a higher proportion of patients achieved a low-risk profile (19% vs. 35%, p = 0.157). However, the PA diameter significantly increased (40.4 ± 10.1 mm vs. 42.1 ± 9.6 mm; p = 0.003). PAA is a common condition in long-standing PAH but its development is not necessarily related to PAH severity. Despite stabilization after treatment optimization, a progressive PA dilatation was observed.

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

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.

Management of incidentally diagnosed pulmonary artery dissection in patients with pulmonary arterial hypertension.

Pulmonary artery dissection is a rare entity, usually discovered post-mortem. In recent years, reported cases of living patients with a pulmonary artery dissection are growing and represent a challenging situation for clinicians.