Rosenkranz Stephan, Gibbs J Simon R, Wachter Rolf, De Marco Teresa, Vonk-Noordegraaf Anton, Vachiéry Jean-Luc
Klinik III für Innere Medizin, Herzzentrum der Universität zu Köln, Kerpener Str. 62, 50937 Köln, Germany Cologne Cardiovascular Research Center (CCRC), Universität zu Köln, Köln, Germany
National Heart and Lung Institute (NHLI), Imperial College London, London, UK Department of Cardiology, National Pulmonary Hypertension Service, Hammersmith Hospital London, London, UK.
Eur Heart J. 2016 Mar 21;37(12):942-54. doi: 10.1093/eurheartj/ehv512. Epub 2015 Oct 27.
In patients with left ventricular heart failure (HF), the development of pulmonary hypertension (PH) and right ventricular (RV) dysfunction are frequent and have important impact on disease progression, morbidity, and mortality, and therefore warrant clinical attention. Pulmonary hypertension related to left heart disease (LHD) by far represents the most common form of PH, accounting for 65-80% of cases. The proper distinction between pulmonary arterial hypertension and PH-LHD may be challenging, yet it has direct therapeutic consequences. Despite recent advances in the pathophysiological understanding and clinical assessment, and adjustments in the haemodynamic definitions and classification of PH-LHD, the haemodynamic interrelations in combined post- and pre-capillary PH are complex, definitions and prognostic significance of haemodynamic variables characterizing the degree of pre-capillary PH in LHD remain suboptimal, and there are currently no evidence-based recommendations for the management of PH-LHD. Here, we highlight the prevalence and significance of PH and RV dysfunction in patients with both HF with reduced ejection fraction (HFrEF) and HF with preserved ejection fraction (HFpEF), and provide insights into the complex pathophysiology of cardiopulmonary interaction in LHD, which may lead to the evolution from a 'left ventricular phenotype' to a 'right ventricular phenotype' across the natural history of HF. Furthermore, we propose to better define the individual phenotype of PH by integrating the clinical context, non-invasive assessment, and invasive haemodynamic variables in a structured diagnostic work-up. Finally, we challenge current definitions and diagnostic short falls, and discuss gaps in evidence, therapeutic options and the necessity for future developments in this context.
在左心室心力衰竭(HF)患者中,肺动脉高压(PH)和右心室(RV)功能障碍很常见,对疾病进展、发病率和死亡率有重要影响,因此值得临床关注。与左心疾病(LHD)相关的肺动脉高压是目前最常见的PH形式,占病例的65%-80%。区分肺动脉高压和LHD相关性PH可能具有挑战性,但它具有直接的治疗意义。尽管在病理生理学理解和临床评估方面取得了最新进展,以及对LHD相关性PH的血流动力学定义和分类进行了调整,但毛细血管后和毛细血管前联合PH的血流动力学相互关系仍然复杂,表征LHD中毛细血管前PH程度的血流动力学变量的定义和预后意义仍不理想,目前尚无基于证据的LHD相关性PH管理建议。在此,我们强调射血分数降低的心力衰竭(HFrEF)和射血分数保留的心力衰竭(HFpEF)患者中PH和RV功能障碍的患病率及意义,并深入探讨LHD中心肺相互作用的复杂病理生理学,这可能导致HF自然病程中从“左心室表型”向“右心室表型”的演变。此外,我们建议通过在结构化诊断检查中整合临床背景、非侵入性评估和侵入性血流动力学变量,更好地定义PH的个体表型。最后,我们对当前的定义和诊断不足提出质疑,并讨论证据空白、治疗选择以及在此背景下未来发展的必要性。