Gadre Shruti K, Minai Omar A, Wang Xiao-Feng, Zhang Qi, Budev Marie, Tonelli Adriano R
From the Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, Ohio, USA.
Exp Clin Transplant. 2017 Dec;15(6):676-684. doi: 10.6002/ect.2016.0209. Epub 2017 May 22.
Little is known about recipient characteristics and outcomes of patients with pulmonary arterial hypertension undergoing lung transplant, particularly those with systemic sclerosis-associated disease. Here, we describe the characteristics and outcomes of patients with pulmonary arterial hypertension undergoing lung transplant, focusing on systemic sclerosis-associated disease.
This retrospective study included pulmonary arterial hypertension patients undergoing lung or heart-lung transplant between July 1992 and August 2013 at a single center.
We evaluated 51 patients with pulmonary arterial hypertension (37.3% males; mean age of 45.3 ± 11.9 y). Of 51 patients, 9 (17.6%) had systemic sclerosis-associated pulmonary arterial hypertension. Pulmonary arterial hypertension patients without systemic sclerosis-associated disease had higher mean pulmonary arterial pressure (P = .002), higher pulmonary vascular resistance (P = .008), and were more likely to have severe right ventricular systolic dysfunction (P = .006) than individuals with the disease. Mean hospital stay posttransplant was similar in the 2 groups (42.2 ± 43.3 vs 43.1 ± 19.4 d; P = .20). Higher pretransplant creatinine clearance (P = .0005), forced vital capacity (P = .01), and absence of vasopressor/inotrope use (P = .03) were associated with shorter hospital stay. Mortality for pulmonary arterial hypertension patients with versus without systemic sclerosis-associated disease was 0% versus 13% at 1 year, 29% versus 24% at 2 years, and 86% versus 53% at 5 years. Female sex (hazard ratio, 0.23; 95% confidence interval, 0.08-0.68) and less severe tricuspid regurgitation (hazard ratio, 0.31; 95% confidence interval, 0.11-0.89) were independently associated with long-term survival.
Pulmonary arterial hypertension patients with versus without systemic sclerosis-associated disease have comparable short-term and 2-year outcomes after lung or heart-lung transplant. Female sex and less severe tricuspid regurgitation were independently associated with better long-term survival. These outcomes did not vary when adjusted for the year of transplant.
对于接受肺移植的肺动脉高压患者的受体特征和预后了解甚少，尤其是那些患有系统性硬化症相关疾病的患者。在此，我们描述接受肺移植的肺动脉高压患者的特征和预后，重点关注系统性硬化症相关疾病。
这项回顾性研究纳入了1992年7月至2013年8月在单一中心接受肺移植或心肺联合移植的肺动脉高压患者。
我们评估了51例肺动脉高压患者（男性占37.3%；平均年龄45.3±11.9岁）。在51例患者中，9例（17.6%）患有系统性硬化症相关肺动脉高压。与患有系统性硬化症相关疾病的患者相比，无系统性硬化症相关疾病的肺动脉高压患者平均肺动脉压更高（P = 0.002），肺血管阻力更高（P = 0.008），且更易出现严重右心室收缩功能障碍（P = 0.006）。两组患者移植后的平均住院时间相似（42.2±43.3天对43.1±19.4天；P = 0.20）。移植前肌酐清除率较高（P = 0.0005）、用力肺活量较高（P = 0.01）以及未使用血管升压药/正性肌力药（P = 0.03）与较短的住院时间相关。患有系统性硬化症相关疾病和未患该疾病的肺动脉高压患者1年死亡率分别为0%和13%，2年分别为29%和24%，5年分别为86%和53%。女性（风险比，0.23；95%置信区间，0.08 - 0.68）和较轻的三尖瓣反流（风险比，0.31；95%置信区间，0.11 - 0.89）与长期生存独立相关。
患有和未患有系统性硬化症相关疾病的肺动脉高压患者在接受肺移植或心肺联合移植后的短期和2年预后相当。女性和较轻的三尖瓣反流与更好的长期生存独立相关。调整移植年份后，这些结果并无差异。
