Hsu Vivien M, Moreyra Abel E, Wilson Alan C, Shinnar Meir, Shindler Daniel M, Wilson Julianne E, Desai Ami, Seibold James R
University of Medicine and Dentistry of New Jersey Scleroderma Program, UMDNJ Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
J Rheumatol. 2008 Mar;35(3):458-65. Epub 2008 Jan 15.
Pulmonary hypertension (PH) is an ominous complication in patients with scleroderma (systemic sclerosis, SSc). We compared noninvasive assessment of PH with pulmonary artery (PA) pressures obtained by right-heart catheterization (RHC).
Forty-nine patients with SSc were evaluated for suspected PH based on clinical findings, progressive dyspnea, and pulmonary function tests (PFT). PH was defined as mean PA pressure > or = 25 mm Hg, or > or = 30 mm Hg after exercise, with normal pulmonary capillary wedge pressure (PCW). Doppler echocardiography (echo) and cardiac magnetic resonance imaging (MRI) were performed within 4 hours of RHC, and the predictive accuracy of the tests was compared.
RHC identified 24/49 (49%) patients with PH. The noninvasive cutpoints were: estimated right ventricular systolic pressure > 47 mm Hg by echo; diameter of the main PA > 28 mm by MRI; and the ratio of forced vital capacity to diffusion capacity (%FVC/%DLCO) > 2.0 by PFT. Echo classified 38 subjects correctly (14/24 with and 24/25 without PH; sensitivity 58%, specificity 96%). The area under receiver-operating characteristic curve (AUC) was 0.84 for echo. MRI measurement of PA diameter had a sensitivity of 68% and specificity 71% (AUC 0.78). PFT evaluation had a sensitivity of 71% and specificity of 72% (AUC 0.76).
In evaluation of SSc with suspected PH, echo appeared to be the most useful among the noninvasive tests, mainly due to the high specificity, high positive predictive value, and highest AUC. However, due to the low sensitivity of noninvasive testing, RHC should remain the gold standard.
肺动脉高压（PH）是硬皮病（系统性硬化症，SSc）患者的一种严重并发症。我们比较了PH的非侵入性评估与通过右心导管检查（RHC）获得的肺动脉（PA）压力。
根据临床表现、进行性呼吸困难和肺功能测试（PFT），对49例SSc患者进行疑似PH评估。PH定义为平均PA压力≥25 mmHg，或运动后≥30 mmHg，且肺毛细血管楔压（PCW）正常。在RHC后4小时内进行多普勒超声心动图（echo）和心脏磁共振成像（MRI），并比较这些检查的预测准确性。
RHC确定49例患者中有24例（49%）患有PH。非侵入性切点为：echo显示估计右心室收缩压>47 mmHg；MRI显示主PA直径>28 mm；PFT显示用力肺活量与弥散能力之比（%FVC/%DLCO）>2.0。echo正确分类了38名受试者（24例中有14例患有PH，25例中无PH的有24例；敏感性58%，特异性96%）。echo的受试者操作特征曲线下面积（AUC）为0.84。PA直径的MRI测量敏感性为68%，特异性为71%（AUC 0.78）。PFT评估敏感性为71%，特异性为72%（AUC 0.76）。
在评估疑似PH的SSc时，echo似乎是最有用的非侵入性检查，主要是因为其高特异性、高阳性预测值和最高的AUC。然而，由于非侵入性检查的敏感性较低，RHC仍应作为金标准。
