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New insights into ventricular arrhythmogenesis in a pure model of pulmonary arterial hypertension

  • K. Lockhart Jamieson
    Affiliations
    Libin Cardiovascular Institute, Department of Cardiac Sciences, Department of Physiology and Pharmacology, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada
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  • Robert A. Rose
    Correspondence
    Address reprint requests and correspondence: Dr Robert A. Rose, Libin Cardiovascular Institute, Cumming School of Medicine, University of Calgary, GAC66, Health Research Innovation Centre, 3280 Hospital Drive NW, Calgary, Alberta, Canada T2N 4Z6.
    Affiliations
    Libin Cardiovascular Institute, Department of Cardiac Sciences, Department of Physiology and Pharmacology, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada
    Search for articles by this author
Published:October 07, 2021DOI:https://doi.org/10.1016/j.hrthm.2021.10.002
      Pulmonary arterial hypertension, defined as a resting mean pulmonary arterial pressure over 25 mm Hg, is characterized by extensive vasoconstriction, adverse vascular remodeling, and vascular noncompliance due to extensive fibrosis and vascular stiffening.
      • Thenappan T.
      • Ormiston M.L.
      • Ryan J.J.
      • Archer S.L.
      Pulmonary arterial hypertension: pathogenesis and clinical management.
      • Spiekerkoetter E.
      • Kawut S.M.
      • de Jesus Perez V.A.
      New and emerging therapies for pulmonary arterial hypertension.
      • Cirulis M.M.
      • Ryan J.J.
      • Archer S.L.
      Pathophysiology, incidence, management, and consequences of cardiac arrhythmia in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.
      Pulmonary arterial hypertension (PAH) is further characterized by progressive endothelial damage, significant neointimal proliferation, and complex plexogenic arterial lesions.
      • Thenappan T.
      • Ormiston M.L.
      • Ryan J.J.
      • Archer S.L.
      Pulmonary arterial hypertension: pathogenesis and clinical management.
      ,
      • Cirulis M.M.
      • Ryan J.J.
      • Archer S.L.
      Pathophysiology, incidence, management, and consequences of cardiac arrhythmia in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.
      As PAH progresses, increased afterload on the right ventricle (RV) results in ventricular hypertrophy and eventual RV failure and death. Despite significant advances in pharmacotherapies to treat PAH, there is no cure and 5-year mortality remains high.
      • Thenappan T.
      • Ormiston M.L.
      • Ryan J.J.
      • Archer S.L.
      Pulmonary arterial hypertension: pathogenesis and clinical management.
      ,
      • Cirulis M.M.
      • Ryan J.J.
      • Archer S.L.
      Pathophysiology, incidence, management, and consequences of cardiac arrhythmia in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.
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