Independence Day: Separating Right Ventricular Function From Pulmonary Arterial Hypertension in Systemic Sclerosis.
Independence Day: Separating Right Ventricular Function From Pulmonary Arterial Hypertension in Systemic Sclerosis.
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独立日：将系统性硬化症中的右心室功能与肺动脉高压分开。
DOI：
10.1161/circulationaha.116.023237
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发表时间：
2016
期刊：
影响因子：
37.8
通讯作者：
Maron,BradleyA
中科院分区：
文献类型：
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作者：
Maron,BradleyA
2346循环，2016年6月14日亚组：作为SSC更大的多器官综合征的独立贡献者的内在RV功能障碍。这些发现对心肺疾病领域有许多重要的影响。首先，现在比以往任何时候都更明显的是，RV功能必须超越其目前的角色，主要作为预后的标志。本研究的结果为仔细考虑RV本身作为终末器官损伤的不同靶点铺平了道路。RV易损性的检测可能仍然依赖于RV-肺血管力学，可能是通过RV心肌缺血负荷，这在PAH中仍未完全表现出来，但现在似乎有必要加强考虑主要疾病底物(通过炎症、神经体液心脏效应器等进行RV靶向)作为RV功能障碍的独立介质。第二，这些数据引入了一个经验基础，通过在运动中诱导RV-肺血管解偶联，潜在地解释了早期观察到的将运动耐受与SSc患者联系在一起的观察，即使在静息肺动脉压在当前正常范围内或仅轻微升高的情况下。11鉴于并存的PAH对SSC患者死亡率的重要性，最近的临床试验数据支持在该患者群体中进行早期积极治疗，12新的报告确定了传统心肺血流动力学在分配临床风险方面的局限性，13以及该领域强调PAH预防方法的总体任务，14这项研究的结果似乎证明，进一步研究RV压力-体积是在疾病病程早期将SSC-PAH风险分层的有用工具。事实上，需要更多的研究来确定可诱导的RV-肺血管解偶联与更广泛的SSC-PAH的硬临床终点的相关性，因为在这项研究中接受调查的大多数患者都有严重的心力衰竭症状，运动试验中的氧耗峰值显著降低。
2346 Circulation June 14, 2016 subgroups: intrinsic RV dysfunction as an independent contributor to the larger multiorgan syndrome of SSc. These findings have a number of important ramifications on the cardiopulmonary disease field. First, it is evident now more than ever that RV function must be regarded beyond its current role as primarily a marker of prognosis. Findings from the present study pave the way for careful consideration to the RV itself as a differential target of end-organ damage. Detecting RV vulnerability is likely still to hinge on RV–pulmonary vascular mechanics, perhaps via RV myocardial ischemic burden, which remains incompletely characterized in PAH, but enhanced consideration of the primary disease substrate (RV targeting by inflammation, neurohumoral cardiac effectors, etc) as an independent mediator of RV dysfunction now seems warranted.Second, these data introduce an empirical basis, through induced RV–pulmonary vascular uncoupling during exercise, to potentially explain earlier observations linking exercise intolerance to SSc patients even when resting pulmonary artery pressure is within the current range of normal or only mildly increased. 11 Given the importance of comorbid PAH on mortality in SSc, recent clinical trial data supporting early aggressive therapy in this patient population, 12 fresh reports identifying the limitations of conventional cardiopulmonary hemodynamics for assigning clinical risk, 13 and an overarching mandate in the field emphasizing approaches for PAH prevention, 14 it would seem that findings from this study justify further investigation of RV pressure-volume as a useful tool for SSc-PAH risk stratification early in the disease course. Indeed, additional investigations are needed to characterize the relevance of inducible RV–pulmonary vascular uncoupling to hard clinical end points across a broader spectrum of SSc-PAH because the majority of patients under investigation in this study had severe heart failure symptoms and a substantially decreased peak volume of oxygen consumption on exercise testing.