A Hemodynamic Study of Pulmonary Hypertension in Sickle Cell Disease
A Hemodynamic Study of Pulmonary Hypertension in Sickle Cell Disease
复制标题
DOI：
10.1056/nejmoa1005565
复制
发表时间：
2011-07-07
影响因子：
158.5
通讯作者：
Simonneau, Gerald
中科院分区：
文献类型：
--
作者：
Parent, Florence;Bachir, Dora;Simonneau, Gerald
BackgroundThe患病率和特点的肺动脉高压的成年人与镰状细胞病还没有明确established.MethodsIn这项前瞻性研究中，我们评估了398例门诊病人与镰状细胞病（平均年龄，34岁）在转诊中心在法国。所有患者均行多普勒超声心动图检查，测量三尖瓣返流速度。对96例三尖瓣返流速度至少为2.5 m/s的疑似肺动脉高压患者进行右心导管检查。肺动脉高压被定义为平均肺动脉压至少25 mm Hg.ResultsThe患病率的三尖瓣返流射流速度至少2.5米每秒为27%。相比之下，经导管检查证实的肺动脉高压患病率为6%。超声心动图检测肺动脉高压的阳性预测值为25%。在24例确诊为肺动脉高压的患者中，11例患者的肺毛细血管楔压为15 mm Hg或更低（表明毛细血管前肺动脉高压）。与其他患者相比，确诊肺动脉高压的患者年龄较大，功能能力较差，N末端脑钠肽前体水平较高。相比之下，三尖瓣返流喷射速度至少为2.5米每秒，而没有肺动脉高压和三尖瓣返流喷射速度小于2.5米每秒的患者有类似的临床feature.ConclusionsIn这项研究的成人镰状细胞病，肺动脉高压的患病率确认右心导管检查为6%。单独超声心动图评估对肺动脉高压的阳性预测值较低。
BackgroundThe prevalence and characteristics of pulmonary hypertension in adults with sickle cell disease have not been clearly established.MethodsIn this prospective study, we evaluated 398 outpatients with sickle cell disease (mean age, 34 years) at referral centers in France. All patients underwent Doppler echocardiography, with measurement of tricuspid-valve regurgitant jet velocity. Right heart catheterization was performed in 96 patients in whom pulmonary hypertension was suspected on the basis of a tricuspid regurgitant jet velocity of at least 2.5 m per second. Pulmonary hypertension was defined as a mean pulmonary arterial pressure of at least 25 mm Hg.ResultsThe prevalence of a tricuspid regurgitant jet velocity of at least 2.5 m per second was 27%. In contrast, the prevalence of pulmonary hypertension as confirmed on catheterization was 6%. The positive predictive value of echocardiography for the detection of pulmonary hypertension was 25%. Among the 24 patients with confirmed pulmonary hypertension, the pulmonary-capillary wedge pressure was 15 mm Hg or less (indicating precapillary pulmonary hypertension) in 11 patients. Patients with confirmed pulmonary hypertension were older and had poorer functional capacity and higher levels of N-terminal pro-brain natriuretic peptide than other patients. In contrast, patients who had a tricuspid regurgitant jet velocity of at least 2.5 m per second without pulmonary hypertension and patients with a tricuspid regurgitant jet velocity of less than 2.5 m per second had similar clinical characteristics.ConclusionsIn this study of adults with sickle cell disease, the prevalence of pulmonary hypertension as confirmed on right heart catheterization was 6%. Echocardiographic evaluation alone had a low positive predictive value for pulmonary hypertension.