EHJ:心肺运动试验在识别和监测肺小静脉闭塞性疾病中的作用

2022-05-28 刘少飞 MedSci原创

肺小静脉闭塞 (PVOD) 疾病可能以不同的形式出现,具有不同程度的血流动力学和通气障碍。血流动力学严重程度与心肺运动试验中表现出的功能和通气障碍之间的不平衡可能会引起对潜在PVOD的怀疑。

肺静脉闭塞性疾病 (PVOD) 是肺动脉高压 (PAH) 的一个亚组,其中血管重塑主要影响毛细血管后血管。它的特点是预后特别差,并且有发生危及生命的肺水肿的风险,尤其是在 PAH 靶向治疗后。因此,怀疑 PVOD 对于指导患者的治疗至关重要。在缺乏特定的遗传或组织学发现的情况下,诊断传统上依赖于识别与 PVOD 的高可能性相关的非侵入性指标。心肺运动测试 (CPET) 是一种很有前途的附加工具,可以识别这些患者并指导他们的管理。

病例总结:

患者为一名 37 岁的女性,她在轻度至中度运动时主诉劳力性呼吸困难并伴有紫绀。她过去的病史并不显著。

体格检查,静息血氧饱和度为 92%,心脏听诊显示第二心音响亮,肺部清晰,无充血迹象。心电图显示窦性心律,QRS 电轴右偏,右胸前导联 T 波倒置。超声心动图显示右心室 (RV) 扩大,伴有中度功能障碍和收缩期室间隔变平。动脉血气检查显示低动脉氧压(PaO 2) (59 mmHg)。患者入院进行完整的诊断检查。探索性和超声心动图检查结果的结合,以及低静息 PaO 2和努力时发绀,引起了对肺动脉高压 (PAH) 的怀疑,更具体地说,是对肺静脉闭塞病 (PVOD) 的怀疑。

右心导管检查 (RHC) 证实有严重的毛细血管前肺动脉高压 (PH):平均肺动脉 (PA) 压力:51 mmHg,肺血管阻力 (PVR):8.5 WU,并保留心输出量 (CO)。验血正常,排除了 PAH 相关的原因,如 HIV 或自身免疫性疾病。肺静脉闭塞性疾病被认为是最可能的诊断,因此特别注意肺功能检查和计算机断层扫描(CT)扫描结果。

值得注意的是,一氧化碳 (DLCO) 的扩散能力严重降低 (26%),CT 扫描显示典型的 PVOD 表现。心肺运动测试 (CPET) 表现出功能能力严重下降,峰值耗氧量 (PVO 2 ) 为 9 mL/kg/min(预测值 37%),努力程度严重下降(SatO 2 : 71%),并且有严重的通气障碍,即运动开始时 VE/VCO 2值突然增加,而不是通常的初始下降;VE/VCO 2斜率高,为 57,PetCO 2值严重降低,甚至在测试中呈下降趋势 [初始 PetCO 2:23 mmHg,最终:16 mmHg]。肺动脉高压特异性治疗开始缓慢,同时监测肺充血的迹象。西地那非与利尿剂一起开始。这是非常好的耐受性,因此添加了 10 mg macitentan。患者最终出院,接受联合治疗、补充氧气和利尿剂,并进行了仔细计划的密切随访。重要的是,呼吸团队已要求对可能的肺移植进行评估,并征求了一项基因研究。

一旦确诊,她开始接受 PAH 靶向治疗,随后得到改善。患者接受了完整的重新评估,结果令人满意,包括 CPET 的结果。

病例讨论:

肺小静脉闭塞性疾病的诊断仍然是肺动脉高压医师必须处理的最困难的任务之一。准确和及时的 PVOD 诊断可能具有挑战性,因为它决定了转诊至肺移植团队的最合适时机,而 CPET 可以服务于这些目的。通过这个案例,我们想回顾一下 PVOD 可能出现的典型临床过程之一,以及如何分析诊断测试提供的信息。

启示:

1. 肺小静脉闭塞 (PVOD) 疾病可能以不同的形式出现,具有不同程度的血流动力学和通气障碍。

2. 血流动力学严重程度与心肺运动试验 (CPET) 中表现出的功能和通气障碍之间的不平衡可能会引起对潜在 PVOD 的怀疑。

3. 对 PVOD 患者的重新评估应包括 CPET,以将血流动力学发现与功能状态相关联,以便为他们的管理设计更好的路线图。

 

参考文献:

Segura de la Cal T, Pérez-Olivares C, Cristo Ropero MJ, Luna López R, Escribano-Subías P. The role of cardiopulmonary exercise testing in identifying and monitoring pulmonary veno-oclusive disease: a case report with ING. Eur Heart J Case Rep. 2022 Mar 28;6(5):ytac138. doi: 10.1093/ehjcr/ytac138. PMID: 35592750; PMCID: PMC9113370.

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JAHA:心肺运动试验对不同射血分数心衰患者的预后价值

VO2峰值和VE/VCO2斜率在临床特征和LVEF之外对预测HFpEF的结局具有额外价值。

JAHA:Fontan循环年轻患者的身体成分

Fontan循环年轻患者身体成分的特征是骨骼肌质量下降,这与运动能力的峰值相关。尽管体重指数正常,但脂肪增加很常见。

ERJ:心肺运动试验在肺静脉闭塞性疾病诊断中的作用

肺静脉闭塞性疾病 (PVOD) 是一种罕见的肺动脉高压,与特发性肺动脉高压 (PAH) 具有一些临床和血液动力学特征。然而,考虑到 PAH 特异性治疗可能会导致危及生命的肺水肿,因此怀疑 PVOD 至

如何对成人心肺运动试验进行全面认识

不明原因的劳累性呼吸困难或疲劳可由一些潜在的疾病引起,并且与静止的功能或影像学测试只有微弱的相关性。无创心肺运动测试为同时研究心肺和代谢变化提供了一个独特的、但仍未得到充分利用和认可的机会。

用心肺运动试验评估心衰患者的整体功能状态和预后有技巧

目前,心肺运动试验(CPET)是评估可活动慢性心衰(CHF )患者的整体功能状态和预后的唯一公认的量化指标。