Wang Lan, Zhao Qin-Hua, Pudasaini Bigyan, Jiang Rong, Gong Su-Gang, He Jing, Yuan Ping, Liu Jin-Ming
Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, 200433, People's Republic of China.
Clin Respir J. 2018 Mar;12(3):915-921. doi: 10.1111/crj.12604. Epub 2017 Jan 6.
Pulmonary 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.
Consecutive 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.
This 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.
Severe 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.
肺动脉高压（PH）是间质性肺疾病（ILD）的重要并发症。本研究旨在调查以中国患者为主的队列中ILD相关PH的特征和预后。
纳入在上海肺科医院经右心导管检查确诊的连续的ILD相关PH患者。分析基线和随访期间的特征以及生存率。
本观察性研究纳入了26例患者，平均年龄为60.0±12.2岁。6分钟步行距离为335.0±190.9米。平均肺动脉压（PAP）为42.9±10.9 mmHg，心输出量为4.4±1.0 L/分钟，肺血管阻力（PVR）为8.6±4.1伍德单位。69.2%的病例平均PAP≥35 mmHg。有PH的ILD患者和无PH的ILD患者在肺功能参数方面没有差异。1年总生存率为92%，3年为82%，5年为62%。PVR是唯一与死亡显著相关的变量。
ILD患者可出现严重的血流动力学损害及PH。与无PH的ILD患者相比，这些患者的运动能力和心功能受损更严重。PVR是该组患者中唯一确定的预后因素。
