Early detection of pulmonary arterial hypertension in systemic sclerosis -: A French nationwide prospective multicenter study
Early detection of pulmonary arterial hypertension in systemic sclerosis -: A French nationwide prospective multicenter study
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DOI：
10.1002/art.21433
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发表时间：
2005-12-01
影响因子：
--
通讯作者：
Humbert, M
中科院分区：
文献类型：
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作者：
Hachulla, E;Gressin, V;Humbert, M
Objective.筛查允许早期管理肺动脉高压（PAH），这是系统性硬化症（SSc）的严重并发症。由于没有达成共识的方法和标准的最佳筛选，我们试图开发一种算法的基础上的症状，多普勒超声心动图，右心导管（RHC）的应用程序在法国的全国多中心SSc的人口。这项前瞻性研究由21家SSc中心的专家于2002年9月至2003年7月进行。在每家临床试验机构，无重度肺功能异常的SSc患者由经验丰富的心脏病专家进行多普勒超声心动图检查。对三尖瓣返流（VTR）峰值速度> 3 m/s或2.5 ~ 3 m/s伴不明原因呼吸困难的患者，根据国际指南进行RHC确诊PAH。在分析的599例患者中，根据多普勒超声心动图，29例已知PAH，33例疑似PAH，并接受了RHC。在这33例患者中，发现18例PAH，3例左心室功能不全，12例无PAH。新诊断的PAH病例的严重程度为轻度（平均+/- SD肺动脉压[mPAP] 30 +/- 9 mm Hg，平均+/- SD总肺阻力[TPR] 524 +/- 382达因x秒/cm（5））。已知PAH患者的血流动力学结果为mPAP 49 17 mm Hg和TPR 1，007 615 dynes x second/cm（5）。PAH患病率为7.85%（95%可信区间5.70-10.00）。该筛选算法基于呼吸困难、多普勒超声心动图对VTR和RHC的评价，能够在轻度阶段早期检测PAH。将在该队列正在进行的3年随访中评价报告病例中轻度PAH是否会演变为重度PAH，以及这种早期诊断是否会转化为轻度PAH患者的预后改善。
Objective. Screening allows for early management of pulmonary arterial hypertension (PAH), a severe complication of systemic sclerosis (SSc). Since no consensus has been reached on the method and criteria for optimal screening, we sought to develop an algorithm based on symptoms, Doppler echocardiography, and right heart catheterization (RHC) for application to a nationwide multicenter SSc population in France.Methods. This prospective study was conducted from September 2002 to July 2003 by experts at 21 SSc centers. At each center, SSc patients without severe pulmonary function abnormalities underwent Doppler echocardiography by an experienced cardiologist. Patients with a peak velocity of tricuspid regurgitation (VTR) of > 3 meters/second or 2.5-3 meters/second with unexplained dyspnea were asked to undergo RHC to confirm PAH according to international guidelines.Results. Of the 599 patients analyzed, 29 had known PAH and 33 had suspected PAH, based on Doppler echocardiography, and underwent RHC. Of these 33, 18 were found to have PAH, 3 had left ventricular dysfunction, and 12 had no PAH. Newly diagnosed cases of PAH were of mild severity (mean +/- SD pulmonary artery pressure [mPAP] 30 +/- 9 mm Hg, mean +/- SD total pulmonary resistance [TPR] 524 +/- 382 dynes x second/cm(5)). Hemodynamic findings in patients with known PAH were mPAP 49 17 mm Hg and TPR 1,007 615 dynes x second/cm(5). The estimate of PAH prevalence was 7.85% (95% confidence interval 5.70-10.00).Conclusion. This screening algorithm, based on dyspnea, Doppler echocardiographic evaluation of VTR, and RHC, enabled early detection of PAH at a mild stage. Whether mild PAH will evolve to severe PAH in reported cases and whether this early diagnosis translates into improved prognosis for patients with mild PAH will be evaluated in the ongoing 3-year followup of this cohort.