Hachulla Eric, Gressin Virginie, Guillevin Loïc, Carpentier Patrick, Diot Elisabeth, Sibilia Jean, Kahan André, Cabane Jean, Francès Camille, Launay David, Mouthon Luc, Allanore Yannick, Tiev Kiet Phong, Clerson Pierre, de Groote Pascal, Humbert Marc
Hôpital Claude-Huriez, Lille, France.
Arthritis Rheum. 2005 Dec;52(12):3792-800. doi: 10.1002/art.21433.
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.
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.
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).
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.
筛查有助于对肺动脉高压（PAH）进行早期管理，PAH是系统性硬化症（SSc）的一种严重并发症。由于在最佳筛查方法和标准上尚未达成共识，我们试图基于症状、多普勒超声心动图和右心导管检查（RHC）开发一种算法，以应用于法国全国范围内的多中心SSc人群。
这项前瞻性研究由21个SSc中心的专家于2002年9月至2003年7月进行。在每个中心，没有严重肺功能异常的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。三尖瓣反流峰值速度（VTR）>3米/秒或2.5 - 3米/秒且伴有无法解释的呼吸困难的患者，根据国际指南被要求进行RHC以确诊PAH。
在分析的599例患者中，基于多普勒超声心动图，29例已知患有PAH，33例疑似患有PAH并接受了RHC检查。在这33例患者中，18例被发现患有PAH，3例有左心室功能障碍，12例没有PAH。新诊断出的PAH病例病情较轻（平均±标准差肺动脉压[mPAP] 30±9毫米汞柱，平均±标准差总肺阻力[TPR] 524±382达因×秒/厘米⁵）。已知患有PAH的患者的血流动力学结果为mPAP 49±17毫米汞柱和TPR 1007±615达因×秒/厘米⁵。PAH患病率估计为7.85%（95%置信区间5.70 - 10.00）。
这种基于呼吸困难、VTR的多普勒超声心动图评估和RHC的筛查算法能够在轻度阶段早期检测出PAH。在该队列正在进行的3年随访中，将评估报告病例中轻度PAH是否会发展为重度PAH，以及这种早期诊断是否会改善轻度PAH患者的预后。
