• Lan Wang, Qin-Hua Zhao, Bigyan Pudasaini, Rong Jiang, Su-Gang Gong, Jing He, Ping Yuan, and Jin-Ming Liu.
• Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, 200433, People's Republic of China.
• Clin Respir J. 2018 Mar 1; 12 (3): 915-921.

BackgroundPulmonary hypertension (PH) is an important complication of interstitial lung disease (ILD). The aim of the present study was to investigate characteristics and prognosis of ILD associated PH in a predominantly Chinese patient cohort.MethodsConsecutive patients with ILD associated PH, confirmed by right heart catheterization at shanghai pulmonary hospital were included in the study. Characteristics at baseline and during follow-up, as well as survival, were analyzed.ResultsThis observational study was conducted in 26 patients with a mean age of 60.0 ± 12.2 y old. The 6-min walking distance was 335.0 ± 190.9 m. The mean pulmonary arterial pressure (PAP) was 42.9 ± 10.9 mmHg, cardiac output was 4.4 ± 1.0 L/min, and pulmonary vascular resistance (PVR) was 8.6 ± 4.1 Wood Units. The mean PAP was ≥35 mmHg in 69.2% of cases. There were no difference in lung function parameters between ILD with PH and ILD without PH. The overall survival rate was 92% at 1 y, 82% at 3 y, and 62% at 5 y. PVR was the only variable significantly associated with death.ConclusionsSevere hemodynamic impairment along with PH can occur in patients with ILD. These patients have more severely impaired exercise capacity and cardiac function than ILD patients without PH. PVR was the only prognostic factor identified in this group of patients.© 2017 John Wiley & Sons Ltd.

42 keywords...

hide…