Takayasu arteritis-associated pulmonary hypertension.

OBJECTIVE To investigate the probable pathogenesis, clinical features, diagnosis, and therapy of patients with pulmonary hypertension (PH) in Takayasu arteritis (TA). METHODS A total of 48 patients with TA who had PH, 20 patients with TA who had pulmonary arterial involvement (PA) without PH, and 30 patients with idiopathic pulmonary arterial hypertension (IPAH) were enrolled in the study from 2009 to 2013. RESULTS Among the 48 patients with TA who had PH, 36 (75.0%) had PA, and left heart disease (LHD) was present in 12 (25.0%). Serum levels of big endothelin 1 (ET-1) were independently correlated with pulmonary arterial systolic pressure (r = 0.33, p = 0.04). Compared to patients with IPAH, patients with PH because of PA who underwent right heart catheterization had lower average cardiac indexes (2.0 ± 0.5 vs 3.0 ± 1.2 l/min/m(2), p = 0.05), and they all developed favorable responses to acute vasodilator testing (100%) in comparison to 10 of the patients with IPAH (33.3%). During a mean followup of 36.0 ± 13.2 months (12.0-65.0 mos), of the patients with PH associated with PA, 3 died of heart failure. Six patients who underwent pulmonary artery revascularization were found to have good prognoses after followup for a mean duration of 6.2 ± 1.9 months. Additionally, 12 patients with PH with LHD were followed for 38.4 ± 15.6 months (12.0-60.0 mos), and 1 patient died of heart failure during the followup period. CONCLUSION Patients with TA are at increased risk for PH. Early screening of patients with TA with unexplained symptoms related to PH should be applied. PH-specific therapies or revascularization may be effective treatments in the early stages of patients with PA, PH, and severe pulmonary artery stenosis.