Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blood Institute, Bethesda, MD, USA.
Am J Respir Crit Care Med. 2013 Apr 15;187(8):840-7. doi: 10.1164/rccm.201207-1222OC.
Pulmonary hypertension (PH) in adults with sickle cell disease (SCD) is associated with early mortality, but no prior studies have evaluated quantitative relationships of mortality to physiological measures of pre- and postcapillary PH.
To identify risk factors associated with mortality and to estimate the expected survival in a cohort of patients with SCD with PH documented by right heart catheterization.
Nine-year follow-up data (median, 4.7 yr) from the National Institutes of Health SCD PH screening study are reported. A total of 529 adults with SCD were screened by echocardiography between 2001 and 2010 with no exclusion criteria. Hemodynamic data were collected from 84 patients. PH was defined as mean pulmonary artery pressure (PAP) ≥ 25 mm Hg. Survival rates were estimated by the Kaplan-Meier method, and mortality risk factors were analyzed by the Cox proportional hazards regression.
Specific hemodynamic variables were independently related to mortality: mean PAP (hazard ratio [HR], 1.61; 95% confidence interval [CI], 1.05-2.45 per 10 mm Hg increase; P = 0.027), diastolic PAP (HR, 1.83; 95% CI, 1.09-3.08 per 10 mm Hg increase; P = 0.022), diastolic PAP - pulmonary capillary wedge pressure (HR, 2.19; 95% CI, 1.23-3.89 per 10 mm Hg increase; P = 0.008), transpulmonary gradient (HR, 1.78; 95% CI, 1.14-2.79 per 10 mm Hg increase; P = 0.011), and pulmonary vascular resistance (HR, 1.44; 95% CI, 1.09-1.89 per Wood unit increase; P = 0.009) as risk factors for mortality.
Mortality in adults with SCD and PH is proportional to the physiological severity of precapillary PH, demonstrating its prognostic and clinical relevance despite anemia-induced high cardiac output and less severely elevated pulmonary vascular resistance.
成人镰状细胞病（SCD）合并肺动脉高压（PH）与早期死亡率相关，但之前的研究并未评估死亡率与毛细血管前和毛细血管后 PH 的生理测量值之间的定量关系。
确定与死亡率相关的危险因素，并估计通过右心导管检查确诊为 PH 的 SCD 患者队列的预期生存率。
报告了美国国立卫生研究院 SCD PH 筛查研究的 9 年随访数据（中位数，4.7 年）。2001 年至 2010 年期间，通过超声心动图对 529 例 SCD 成人进行了筛查，无排除标准。从 84 例患者中收集了血流动力学数据。肺动脉平均压（PAP）≥25mmHg 定义为 PH。通过 Kaplan-Meier 法估计生存率，通过 Cox 比例风险回归分析死亡率的危险因素。
特定的血流动力学变量与死亡率独立相关：平均 PAP（危险比 [HR]，每增加 10mmHg 为 1.61；95%置信区间 [CI]，1.05-2.45；P=0.027）、舒张期 PAP（HR，每增加 10mmHg 为 1.83；95%CI，1.09-3.08；P=0.022）、舒张期 PAP-肺毛细血管楔压（HR，每增加 10mmHg 为 2.19；95%CI，1.23-3.89；P=0.008）、肺跨压梯度（HR，每增加 10mmHg 为 1.78；95%CI，1.14-2.79；P=0.011）和肺血管阻力（HR，每增加 1 个 Wood 单位为 1.44；95%CI，1.09-1.89；P=0.009）是死亡的危险因素。
SCD 合并 PH 的成年人死亡率与毛细血管前 PH 的生理严重程度成正比，尽管贫血导致心输出量增加和肺血管阻力升高不那么严重，但仍显示出其预后和临床相关性。
