Adaptation and Maladaptation of the Right Ventricle in Pulmonary Vascular Diseases

The right ventricle is coupled to the low-pressure pulmonary circulation. In pulmonary vascular diseases, right ventricular (RV) adaptation is key to maintain ventriculoarterial coupling. RV hypertrophy is the first adaptation to diminish RV wall tension, increase contractility, and protect cardiac output. Unfortunately, RV hypertrophy cannot be sustained and progresses toward a maladaptive phenotype, characterized by dilation and ventriculoarterial uncoupling. The mechanisms behind the transition from RV adaptation to RV maladaptation and right heart failure are unraveled. Therefore, in this article, we explain the main traits of each phenotype, and how some early beneficial adaptations become prejudicial in the long-term.