Department of Pulmonology, Amsterdam Cardiovascular Sciences, VU University Medical Center Amsterdam, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands.
Division of Cardiology, Department of Medicine, Medical University of South Carolina, Charleston, SC, USA.
Cardiovasc Res. 2017 Oct 1;113(12):1423-1432. doi: 10.1093/cvr/cvx145.
In pulmonary arterial hypertension (PAH), right ventricular (RV) adaptation is essential to overcome the chronic increases in RV pressure overload. Ultimately, RV compensatory mechanisms are not sufficient and patients succumb to RV failure. The processes underlying the transition of RV adaptation to RV failure are not well understood. In this review, we propose that important insights in RV adaptation processes can be obtained by comparing different etiologies of PAH, namely patients with PAH secondary to Eisenmenger syndrome, patients with PAH secondary to systemic sclerosis and patients where no cause is identified: idiopathic PAH. Although the amount of RV afterload does not differ between these patient groups, their prognosis is distinctly different. We will show that an adaptive RV phenotype, as is observed in Eisenmenger patients, coincides with RV hypertrophy, increased RV contractility, low RV fibrosis and low RV diastolic stiffness. Whereas a phenotype of RV failure, as is observed in patients with PAH-secondary to systemic sclerosis, is characterized by impaired contractile reserve, RV fibrosis and RV diastolic stiffness.
在肺动脉高压（PAH）中，右心室（RV）的适应性对于克服慢性RV 压力超负荷至关重要。最终，RV 代偿机制不足以使患者免于 RV 衰竭。RV 适应性向 RV 衰竭转变的过程尚不清楚。在这篇综述中，我们提出通过比较 PAH 的不同病因，即艾森曼格综合征继发 PAH 患者、系统性硬化症继发 PAH 患者和未明确病因的特发性 PAH 患者，可以获得 RV 适应性过程的重要见解。尽管这些患者群体的 RV 后负荷量没有差异，但他们的预后却截然不同。我们将表明，适应性 RV 表型，如艾森曼格患者所观察到的那样，与 RV 肥大、RV 收缩力增加、RV 纤维化程度低和 RV 舒张僵硬度低有关。而 RV 衰竭表型，如系统性硬化症继发 PAH 患者所观察到的那样，其特征为收缩储备功能受损、RV 纤维化和 RV 舒张僵硬度增加。
