Key Points
- To review the pathophysiology, diagnostic evaluation, and current therapeutic strategies associated with right ventricular adaptation and failure in pulmonary arterial hypertension.
- Synthesized pathophysiological mechanisms governing right ventricular-arterial uncoupling, chamber dilatation, and diastolic stiffening in pulmonary arterial hypertension.
- Summarized diagnostic modalities including echocardiography, magnetic resonance imaging, and right heart catheterization.
- Evaluated current clinical management strategies focused on pulmonary afterload reduction, fluid optimization, and inotropic support.
- Early disease exhibits compensatory right ventricular contractility but suboptimal right ventricular-arterial coupling that curtails maximum cardiac output and aerobic capacity.
- Advanced disease triggers right ventricular dilatation, myocardial fibrosis, and sarcomeric stiffening, causing elevated right-sided filling pressures, left ventricular under-filling, systemic hypotension, and exertional dyspnea.
- Current standard therapies effectively lower pulmonary afterload and optimize fluid status, but no pharmacological treatments directly target right ventricular function.
Structured PICO
PPopulationPatients with pulmonary arterial hypertension (PAH)
This review outlines the pathophysiology of right ventricular failure in pulmonary arterial hypertension and highlights the current lack of therapies specifically targeting the right ventricle.