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Onset of systemic arterial hypertension after initiation of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis: A case series

  • Andrea Gramegna
    Correspondence
    Corresponding author at: Fond. IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Padiglione Sacco, Via Francesco Sforza 35, Milano 20121, Italy.
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Italy

    Internal Medicine Department, Respiratory Unit and Cystic Fibrosis Adult Center. Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
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  • Claudia De Petro
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Italy

    Internal Medicine Department, Respiratory Unit and Cystic Fibrosis Adult Center. Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
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  • Gloria Leonardi
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Italy

    Internal Medicine Department, Respiratory Unit and Cystic Fibrosis Adult Center. Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
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  • Martina Contarini
    Affiliations
    Internal Medicine Department, Respiratory Unit and Cystic Fibrosis Adult Center. Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
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  • Francesco Amati
    Affiliations
    Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy

    IRCCS Humanitas Research Hospital, Respiratory Unit, Rozzano, Italy
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  • Roberto Meazza
    Affiliations
    Cardiology Unit, Internal Medicine Department, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
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  • Stefano Carugo
    Affiliations
    Cardiology Unit, Internal Medicine Department, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
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  • Francesco Blasi
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Italy

    Internal Medicine Department, Respiratory Unit and Cystic Fibrosis Adult Center. Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
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Published:April 18, 2022DOI:https://doi.org/10.1016/j.jcf.2022.04.010

      Highlights

      • Initiation with elexacaftor/tezacaftor/ivacaftor treatment might be associated with acute onset of systemic arterial hypertension.
      • Careful monitoring of cardiovascular paramenters is recommended in patients starting with elexacaftor/tezacaftor/ivacaftor treatment.
      • Referral may be made to cardiologists when patients present elevated blood pressure after the start with elexacaftor/tezacaftor/ivacaftor.

      Abstract

      Elexacaftor/tezacaftor/ivacaftor (ETI) is associated with major improvements in respiratory outcomes of individuals with cystic fibrosis (CF) and at least one Phe508del mutation. Although ETI was well tolerated in registration studies, the attention on adverse events not previously described is very high in the post-marketing phase. In this case series we report the onset of systemic arterial hypertension in 4 individuals with CF within the first weeks of starting therapy. All patients needed cardiac evaluation and started chronic anti-hypertensive therapy. Until more data is available, this report could foster the attention of CF physicians towards careful monitoring of cardiovascular parameters in patients starting ETI.

      Keywords

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