• Fabrice Antigny, Olaf Mercier, Marc Humbert, and Jessica Sabourin.
• Faculté de médecine, Université Paris-Saclay, 94270 Le Kremlin Bicêtre, France; Service de pneumologie, Centre de référence de l'hypertension pulmonaire, Hôpital Bicêtre, AP-HP, 94270 Le Kremlin Bicêtre, France; Inserm UMR-S 999, Hôpital Marie Lannelongue, 92350 Le Plessis Robinson, France. Electronic address: fabrice.antigny@u-psud.fr.
• Arch Cardiovasc Dis. 2020 Jan 1; 113 (1): 70-84.

AbstractPulmonary arterial hypertension is a progressive and lethal cardiopulmonary disease. The rise in right ventricular afterload leads to right ventricular hypertrophy and failure. Right ventricular failure is the most important prognostic factor for morbidity and mortality in pulmonary arterial hypertension or pulmonary hypertension caused by left heart diseases. Surprisingly, the right ventricle is not targeted by pulmonary arterial hypertension-specific therapies. The current profound lack of basic understanding of pulmonary arterial hypertension-related right ventricular remodelling can explain, at least in part, this paradox. The physiology and haemodynamic function of the right ventricle in the normal state differ considerably from those of the left ventricle, and the known mechanisms of left ventricular dysfunction cannot be generalized to right ventricular dysfunction. Ion channel activities and calcium homeostasis tightly regulate cardiac function, and their dysfunction contributes to the pathogenesis of cardiac diseases. This review focuses on the ion channels (potassium, calcium) and intracellular calcium handling remodelling involved in right ventricular hypertrophy and dysfunction caused by pulmonary arterial hypertension.Copyright © 2019 Elsevier Masson SAS. All rights reserved.

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