Right Ventricular Adaptation and Failure in Pulmonary Arterial Hypertension

摘要: Pulmonary arterial hypertension (PAH) is an obstructive pulmonary vasculopathy, characterized by excess proliferation, apoptosis resistance, inflammation, fibrosis, and vasoconstriction. Although PAH therapies target some of these vascular abnormalities (primarily vasoconstriction), most do not directly benefit the right ventricle (RV). This suboptimal because a patient's functional state prognosis are largely determined success adaptation RV to increased afterload. The initially hypertrophies but might ultimately decompensate, becoming dilated, hypokinetic, fibrotic. A number pathophysiologic have been identified in RV, including: ischemia hibernation (partially reflecting capillary rarefaction), autonomic activation (due G protein receptor kinase 2-mediated downregulation desensitization β-adrenergic receptors), mitochondrial-metabolic (notably uncoupled glycolysis glutaminolysis), fibrosis. Many detectable using molecular imaging serve as biomarkers. Some pathways, such those regulating angiogenesis, metabolism, mitochondrial dynamics, similarly deranged vasculature, offering possibility that treat circulation. An important paradigm circulation constitute unified cardiopulmonary unit. Clinical trials pharmacotherapies should assess both components