Pulmonary hypertension as a risk factor for death in patients with sickle cell disease
Pulmonary hypertension as a risk factor for death in patients with sickle cell disease
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DOI：
10.1056/nejmoa035477
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发表时间：
2004-02-26
影响因子：
158.5
通讯作者：
Ognibene, FP
中科院分区：
文献类型：
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作者：
Gladwin, MT;Sachdev, V;Ognibene, FP
背景：成人镰状细胞病患者肺动脉高压的患病率、发展机制及其未来预后意义尚不清楚。方法：我们对195例连续患者（男性82例，女性113例；平均[+/-SD]年龄，36+/-12岁）进行了肺动脉收缩压的多普勒超声心动图评估。肺动脉高压的前瞻性定义为三尖瓣反流射流速度至少为2.5 m / s。患者平均随访18个月，在患者死亡或失去随访时对数据进行审查。结果：32%的患者发生了多普勒定义的肺动脉高压。多重logistic回归分析，使用二分变量三尖瓣反流喷射速度小于2.5米/秒或大于2.5米/秒，确定了自我报告的心血管或肾脏并发症史，收缩压升高，高乳酸脱氢酶水平（溶血标志物），高碱性磷酸酶水平和低转铁蛋白水平是肺动脉高压的重要独立相关因素。胎儿血红蛋白水平、白细胞计数、血小板计数和羟基脲治疗的使用与肺动脉高压无关。与低于2.5米/秒的三尖瓣反流射流速度相比，至少2.5米/秒的三尖瓣反流射流速度与死亡风险增加密切相关(比率比，10.1；95%置信区间，2.2至47.0；P
BACKGROUND:The prevalence of pulmonary hypertension in adults with sickle cell disease, the mechanism of its development, and its prospective prognostic significance are unknown.METHODS:We performed Doppler echocardiographic assessments of pulmonary-artery systolic pressure in 195 consecutive patients (82 men and 113 women; mean [+/-SD] age, 36+/-12 years). Pulmonary hypertension was prospectively defined as a tricuspid regurgitant jet velocity of at least 2.5 m per second. Patients were followed for a mean of 18 months, and data were censored at the time of death or loss to follow-up.RESULTS:Doppler-defined pulmonary hypertension occurred in 32 percent of patients. Multiple logistic-regression analysis, with the use of the dichotomous variable of a tricuspid regurgitant jet velocity of less than 2.5 m per second or 2.5 m per second or more, identified a self-reported history of cardiovascular or renal complications, increased systolic blood pressure, high lactate dehydrogenase levels (a marker of hemolysis), high levels of alkaline phosphatase, and low transferrin levels as significant independent correlates of pulmonary hypertension. The fetal hemoglobin level, white-cell count, and platelet count and the use of hydroxyurea therapy were unrelated to pulmonary hypertension. A tricuspid regurgitant jet velocity of at least 2.5 m per second, as compared with a velocity of less than 2.5 m per second, was strongly associated with an increased risk of death (rate ratio, 10.1; 95 percent confidence interval, 2.2 to 47.0; P