Abstract

Neuro-hormonal activation may lead to or be associated with pulmonary arterial hypertension (PAH) and right ventricular dysfunction. Notwithstanding whether it is the cause or the consequence of PAH-related right ventricle (RV) dysfunction neurohormonal activation contributes to significant morbidity and mortality in patients with PAH and the progression of RV dysfunction. Experimental data regarding the use of beta adrenergic blockade and renin-angiotensin aldosterone system modulation are encouraging. However, clinical studies have largely been negative or neutral; and, neuro-hormonal modulation is discouraged in patients with PAH related RV dysfunction for fear of systemic hypotension. Herein, we summarize the pathophysiological background that supports the potential role of neuro-hormonal modulation in the management of PAH related RV dysfunction; also present current clinical experience; and, discuss the need for controlled studies to move forward. Lastly, we review potential non- pharmacological modalities for neuro-hormonal modulations in PAH patients with RV dysfunction.

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