University of Iowa Carver College of Medicine, Iowa City, IA.
University of Iowa Carver College of Medicine, Iowa City, IA.
Chest. 2013 Aug;144(2):638-650. doi: 10.1378/chest.12-2114.
World Health Organization (WHO) group 2 pulmonary hypertension (PH) due to left-side heart disease (ie, heart failure or left-sided valvular heart disease) is the most common form of PH in western countries. Distinguishing patients with WHO group 2 PH, particularly the subset of patients with PH due to heart failure with preserved ejection fraction (HFpEF), from those with WHO group 1 pulmonary arterial hypertension (PAH) is challenging. Separating the two conditions is of vital importance because treatment strategies differ completely. Furthermore, therapies that are indicated for WHO group 1 PAH may be harmful in patients with WHO group 2 PH. We review the somewhat confusing PH nomenclature and the WHO classification system and rationale behind it. We then focus on left-side heart disorders that cause PH. An aging population and advances in the medical management of common cardiovascular disorders have caused the prevalence of heart failure to rise significantly, with more than one-half of patients having HFpEF. We review contemporary studies that focus on clinical and echocardiographic findings that help to distinguish HFpEF from PAH in the patient with PH. We discuss the typical, and sometimes atypical, hemodynamic profiles that characterize these two groups, review challenges in the interpretation of data obtained by right-sided heart catheterization, and highlight special maneuvers that may be required for accurate diagnosis. Finally, we review the largely disappointing studies on the use of PAH-specific therapies in patients with WHO group 2 PH, including the use of prostacyclins, endothelin receptor antagonists, and the more promising phosphodiesterase-5 inhibitors.
世界卫生组织（WHO）2 组肺动脉高压（PH）继发于左心疾病（即心力衰竭或左侧瓣膜性心脏病），是西方国家最常见的 PH 类型。区分 WHO 2 组 PH 患者，特别是射血分数保留的心力衰竭相关 PH（HFpEF）亚组患者与 WHO 1 组肺动脉高压（PAH）患者具有挑战性。将这两种情况区分开来至关重要，因为治疗策略完全不同。此外，用于 WHO 1 组 PAH 的治疗方法在 WHO 2 组 PH 患者中可能是有害的。我们回顾了有些混乱的 PH 命名法和 WHO 分类系统及其背后的原理。然后，我们将重点放在导致 PH 的左心疾病上。人口老龄化和常见心血管疾病的医疗管理进步导致心力衰竭的患病率显著上升，超过一半的患者为 HFpEF。我们回顾了当代研究，这些研究侧重于有助于在 PH 患者中区分 HFpEF 与 PAH 的临床和超声心动图发现。我们讨论了这两组的典型和有时非典型的血流动力学特征，回顾了右心导管检查数据解释的挑战，并强调了可能需要进行准确诊断的特殊操作。最后，我们回顾了在 WHO 2 组 PH 患者中使用 PAH 特异性治疗的大量令人失望的研究，包括使用前列环素、内皮素受体拮抗剂和更有前途的磷酸二酯酶-5 抑制剂。
