Pulmonary heart disease: The heart-lung interaction and its impact on patient phenotypes.

Pulmonary heart disease: The heart-lung interaction and its impact on patient phenotypes.
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DOI:
10.4103/2045-8932.109910
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发表时间:
2013-01
影响因子:
2.6
通讯作者:
Wiegers SE
Wiegers SE
中科院分区:
医学4区
文献类型:
--
作者:
Forfia PR;Vaidya A;Wiegers SE

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肺心病(PHD)是指与呼吸功能异常相关的右心室结构或功能改变。尽管几乎总是与某种程度的 PH 相关,但 PH 的程度、性质、严重程度以及与 PHD 的因果关系不一定是线性和直接的。气体交换异常是 PHD 的基础,影响肺血管、心脏、肾脏和神经激素系统。慢性呼吸系统疾病的直接和间接影响可以破坏右心室-肺动脉 (RV-PA) 相互作用,同样,交感神经系统激活、血液粘度改变以及盐和水潴留等因素可以在反馈回路中发挥作用,进一步影响 RV-PA 功能。左心功能也可能受到影响,尤其是那些患有左心疾病的人。因此,异常呼吸和心血管功能之间的生理相互作用是复杂的,PHD 代表了综合多系统过程的异质终末器官效应。在本次审查中,我们建议将 PHD 分为两个不同的实体,“I 型”和“II 型”PHD。 I 型 PHD 最常见,指的是患有慢性呼吸系统疾病 (CRD) 的受试者,其中呼吸功能的紊乱超过了更轻微的心脏和循环系统紊乱。相比之下,II 型 PHD 是指肺血管和右心功能障碍较严重的一小部分患者,其表现形式通常与 PAH 患者类似。表型差异不仅仅由 PA 压力造成,而是由整体生理学和临床综合征的差异造成。因此,主要差异体现在症状、体征、心脏成像、血流动力学以及心血管和气体交换对运动的反应上。总体生理表型的这种关键基线差异可能对于预测对 PH 特异性治疗的反应至关重要。将 PHD 识别为不同的表型有助于对这些患者进行必要的区分,并且还可能为改善患者选择提供关键的临床和病理生理学框架,以便未来研究肺动脉高压特异性疗法在 PHD 中的作用。
Pulmonary heart disease (PHD) refers to altered structure or function of the right ventricle occurring in association with abnormal respiratory function. Although nearly always associated with some degree of PH, the degree, nature, severity, and causality of PH in relation to the PHD is not necessarily linear and direct. Abnormal gas exchange is a fundamental underpinning of PHD, affecting pulmonary vascular, cardiac, renal, and neurohormonal systems. Direct and indirect effects of chronic respiratory disease can disrupt the right ventricular-pulmonary arterial (RV-PA) interaction and, likewise, factors such as sympathetic nervous system activation, altered blood viscosity, and salt and water retention can function in a feedback loop to further influence RV-PA function. Left heart function may also be affected, especially in those with pre-existing left heart disease. Thus, the physiologic interactions between abnormal respiratory and cardiovascular function are complex, with PHD representing a heterogeneous end organ effect of an integrated multisystem process. In this review, we propose to separate PHD into two distinct entities, “Type I” and “Type II” PHD. Type I PHD is most common, and refers to subjects with chronic respiratory disease (CRD) where the perturbations in respiratory function dominate over more mild cardiac and circulatory disruptions. In contrast, Type II PHD refers to the smaller subset of patients with more severe pulmonary vascular and right heart dysfunction, whom often present in a fashion similar to patients with PAH. Phenotypic differences are not made by PA pressure alone, but instead by differences in the overall physiology and clinical syndrome. Thus, key differences can be seen in symptomatology, physical signs, cardiac imaging, hemodynamics, and the cardiovascular and gas exchange responses to exercise. Such key baseline differences in the overall physiologic phenotype are likely critical to predicting response to PH specific therapy. Recognizing PHD as distinct phenotypes assists in the necessary distinction of these patients, and may also provide a key clinical and pathophysiologic framework for improved patient selection for future studies investigating the role of pulmonary hypertension-specific therapies in PHD.
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