Pulmonary hypertension (PH), as a complication of bronchiectasis, is associated with increased mortality. However, hemodynamic characteristics and the efficacy of pulmonary arterial hypertension (PAH) therapies in patients with bronchiectasis and PH remain unknown. Patients with bilateral bronchiectasis and concurrent PH were included in the study. Patient characteristics at baseline and during follow-up, as well as survival, were analyzed. This observational study was conducted in 36 patients with a mean age of 51.5 years (range, 17?74 years). The 6 min walking distance was 300.8±93.3 m. The mean pulmonary arterial pressure (PAP) was 41.5±11.7 mmHg, cardiac output was 5.2±1.4 L/min, and pulmonary vascular resistance was 561.5±281.5 dyn·s·cm⁻⁵. The mean PAP was>35 mmHg in 75% of the cases. Mean PAP was inversely correlated with arterial oxygen saturation values (r = −0.45, P = 0.02). In 24 patients who received oral PAH therapy, systolic PAP was reduced from 82.4±27.0 mmHg to 65.5±20.9 mmHg (P = 0.025) on echocardiography after a median of 6 months of follow-up. The overall probability of survival was 97.1% at 1 year, 83.4% at 3 years, and 64.5% at 5 years. Given the results, we conclude that PH with severe hemodynamic impairment can occur in patients with bilateral bronchiectasis, and PAH therapy might improve hemodynamics in such patients. Prospective clinical trials focusing on this patient population are warranted.