Differences in Right Ventricular Functional Changes during Treatment between Systemic Sclerosis-associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension
Differences in Right Ventricular Functional Changes during Treatment between Systemic Sclerosis-associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension
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DOI：
10.1513/annalsats.201608-655oc
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发表时间：
2017-05-01
影响因子：
8.3
通讯作者：
Strange, Charlie
中科院分区：
文献类型：
--
作者：
Argula, Rahul G.;Karwa, Abhijit;Strange, Charlie
理由：尽管肺动脉血管扩张剂治疗取得了进展，但系统性硬化症相关性肺动脉高压（SSc-PAH）患者的死亡率仍然高得令人无法接受。目的：在超声心动图右心室功能障碍进展的基础上，我们试图确定SSc-PAH是否与特发性肺动脉高压（IPAH）相比是一种临床独特的肺血管疾病表型。方法：回顾性分析13例SSc-PAH患者和11例IPAH患者的超声心动图资料，以描述在单一或联合肺动脉血管扩张剂治疗期间RV功能障碍的进展。所有患者均有右心导管确认肺动脉高压，以及完整的基线（诊断时）和随访（最近）超声心动图。我们排除了有明显硬皮病相关间质性肺疾病的患者。调整随访时间和病程，我们进行混合模型回归分析，比较两组不同超声心动图变量的变化：三尖瓣环面收缩偏移、三尖瓣反流射流速度、右心房面积和右心室直径。结果：SSc-PAH组和IPAH组的平均年龄分别为60.8岁和48.2岁。两组的平均随访时间分别为3.8年和1.95年。SSc-PAH患者的三尖瓣环平面收缩偏移没有改善，而ipah患者的三尖瓣环平面收缩偏移增加（20.38mm, P = 0.87；对15.6 mm, P = 0.02）。其他超声心动图变量显示SSc-PAH组有恶化趋势，IPAH组有改善趋势。结论：我们的研究结果表明，与IPAH患者相比，SSc-PAH患者的超声心动图右心室功能并没有随着时间的推移而改善，尽管采用了肺动脉血管扩张剂治疗。
Rationale: Patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) continue to have an unacceptably high mortality rate despite the progress achieved with pulmonary arterial vasodilator therapies.Objectives: We sought to determine whether SSc-PAH is a clinically distinct pulmonary vascular disease phenotype when compared with idiopathic pulmonary arterial hypertension (IPAH) on the basis of progression of echocardiographic right ventricular (RV) dysfunction.Methods: Retrospective analysis of echocardiographic data in 13 patients with SSc-PAH and 11 patients with IPAH was used to delineate the progression of RV dysfunction during single or combination pulmonary arterial vasodilator therapy. All patients had right heart catheterization-confirmed pulmonary arterial hypertension as well as complete baseline (at the time of diagnosis) and follow-up (most recent) echocardiograms. We excluded patients with significant scleroderma-associated interstitial lung disease. Adjusting for time of follow-up and disease duration, we performed mixed model regression analyses comparing the changes between the two groups for different echocardiographic variables: tricuspid annular plane systolic excursion, tricuspid regurgitation jet velocity, right atrial area, and RV diameter.Results: The mean ages for the SSc-PAH and IPAH groups were 60.8 and 48.2 years, respectively. The mean follow-up periods for the two groups were 3.8 and 1.95 years, respectively. Tricuspid annular plane systolic excursion did not improve in patients with SSc-PAH, whereas it increased in the patients withIPAH(20.38mm, P = 0.87; vs. 15.6 mm, P = 0.02). The other echocardiographic variables showed a trend toward worsening in the SSc-PAH group and improvement in the IPAH group.Conclusions: Our results indicate that, in patients with SSc-PAH, echocardiographic RV function does not improve over time compared with that of patients with IPAH, despite institution of pulmonary artery vasodilator therapies.