{
  "abstract": "Background Cardiac resynchronisation therapy (CRT) is a key treatment for heart failure (HF) in acquired heart disease, but its benefits in adults with congenital heart disease and a systemic right ventricle (sRV) remain unclear. This study aimed to assess whether CRT improves outcomes in patients with sRV.Methods This is an international, retrospective study including patients >18 years from 33 centres with transposition of the great arteries (TGA) following atrial switch operation and congenitally corrected TGA. The primary endpoint included overall survival and survival free from HF. The secondary endpoint was a composite of death, hospitalisation for HF, heart transplant, mechanical support and ventricular tachycardia/implantable cardioverter-defibrillator therapies.Results We identified 105 out of 1721 patients (3.5%) who underwent CRT. Median follow-up after CRT implant was 4.6 (1.6–8) years. QRS improvement was limited to those with previous pacing (167±35 vs 154±28 ms; p=0.002). Following CRT, there was no significant change in B-type natriuretic peptide values, peak VO 2 and tricuspid regurgitation severity by echocardiography. CRT complications occurred in 10 (9.5%), though they were usually minor. Patients with CRT were propensity-matched to controls according to age, sex, anatomy, presence of complex disease, previous HF and sRV dysfunction at baseline. At univariable analysis, CRT (HR 4.39–95%, CI 1.6 to 11.9; p=0.003), older age and moderate-to-severe sRV dysfunction at baseline were predictive of death, while CRT (HR 3–95%, CI 1.3 to 7; p=0.01) and sRV dysfunction were associated with HF admission. By multivariable analysis, CRT (HR 8.8–95%, CI 2.9 to 26.6; p=0.0001) and age (HR 1.1%–95%, CI 1.01 to 1.15; p<0.0001) were independently associated with poorer outcome.Conclusion In this retrospective study in the largest population thus far described with an sRV, CRT implant was not associated with improved survival, even after controlling for key confounders.",
  "authors": [
    {
      "affiliations": [
        "Adult Congenital Heart Disease Unit, Monaldi Hospital, Naples, Campania, Italy"
      ],
      "name": "Flavia Fusco"
    },
    {
      "affiliations": [
        "Cardiology, Monaldi Hospital-Second University of Naples, Naples, Italy"
      ],
      "name": "Giancarlo Scognamiglio"
    },
    {
      "affiliations": [
        "Cardiology, Sahlgrenska University Hospital, Gothenburg, Sweden"
      ],
      "name": "Mikael Dellborg"
    },
    {
      "affiliations": [
        "Interventional Cardiology, Regina General Hospital, Regina, Saskatchewan, Canada"
      ],
      "name": "Payam Dehghani"
    },
    {
      "affiliations": [
        "Lucile Packard Children’s Hospital, Stanford, Palo Alto, California, USA"
      ],
      "name": "Susan M Jameson"
    },
    {
      "affiliations": [
        "Indiana University Krannert Institute of Cardiology, Indianapolis, Indiana, USA"
      ],
      "name": "W Aaron Kay"
    },
    {
      "affiliations": [
        "Pediatric Cardiology, Children’s Hospital and Medical Center, Omaha, Nebraska, USA",
        "Internal Medicine",
        "Cardiology, University of Nebraska Medical Center, Omaha, Nebraska, USA"
      ],
      "name": "Jonathan W Cramer"
    },
    {
      "affiliations": [
        "Department of Medicine, University of Alberta, Edmonton, Alberta, Canada"
      ],
      "name": "Isabelle Vonder Muhll"
    },
    {
      "affiliations": [
        "Division of Cardiology, Department of Medicine, University of Washington Medical Center, Seattle, Washington, USA"
      ],
      "name": "Eric V Krieger"
    },
    {
      "affiliations": [
        "Emory University, Atlanta, Georgia, USA"
      ],
      "name": "Fred H Rodriguez"
    },
    {
      "affiliations": [
        "The Royal Melbourne Hospital, Melbourne, Victoria, Australia"
      ],
      "name": "Luke J Burchill"
    },
    {
      "affiliations": [
        "Providence, Renton, Washington, USA"
      ],
      "name": "Jeremy Nicolarsen"
    },
    {
      "affiliations": [
        "University of Colorado System, Denver, Colorado, USA"
      ],
      "name": "Joseph Kay"
    },
    {
      "affiliations": [
        "Cardiology, Erasmus MC, Rotterdam, Zuid-Holland, Netherlands"
      ],
      "name": "Robert M Kauling"
    },
    {
      "affiliations": [
        "Ochsner Health System, New Orleans, Louisiana, USA"
      ],
      "name": "Sangeeta Shah"
    },
    {
      "affiliations": [
        "Missouri State University, Springfield, Missouri, USA"
      ],
      "name": "Anthony Magalski"
    },
    {
      "affiliations": [
        "The Royal Melbourne Hospital, Melbourne, Victoria, Australia"
      ],
      "name": "Joshua Wong"
    },
    {
      "affiliations": [
        "Cardiology, Royal Prince Alfred Hospital Department of Radiology and Medical Imaging, Camperdown, New South Wales, Australia"
      ],
      "name": "David S Celermajer"
    },
    {
      "affiliations": [
        "Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia"
      ],
      "name": "David William Baker"
    },
    {
      "affiliations": [
        "Cardiology, ErasmusMC, Rotterdam, Netherlands"
      ],
      "name": "Jolien W Roos-Hesselink"
    },
    {
      "affiliations": [
        "Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA"
      ],
      "name": "Salil Ginde"
    },
    {
      "affiliations": [
        "Cardiology, UCLA, Los Angeles, California, USA"
      ],
      "name": "Jamil Aboulhosn"
    },
    {
      "affiliations": [
        "Emory University, Atlanta, Georgia, USA"
      ],
      "name": "Marissa Kuo"
    },
    {
      "affiliations": [
        "Missouri State University, Springfield, Missouri, USA"
      ],
      "name": "Christopher DeZorzi"
    },
    {
      "affiliations": [
        "Montreal Heart Institute, Montreal, Quebec, Canada"
      ],
      "name": "Paul Khairy"
    },
    {
      "affiliations": [
        "Boston Children’s Hospital, Boston, Massachusetts, USA"
      ],
      "name": "Carla P Rodriguez-Monserrate"
    },
    {
      "affiliations": [
        "Children’s Hospital and Medical Center, Omaha, Nebraska, USA"
      ],
      "name": "Shelby Kutty"
    },
    {
      "affiliations": [
        "Cardiology, Royal Melbourne Hospital, Melbourne, Victoria, Australia"
      ],
      "name": "William Wilson"
    },
    {
      "affiliations": [
        "Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio, USA"
      ],
      "name": "Adam M Lubert"
    },
    {
      "affiliations": [
        "The University of British Columbia, Vancouver, British Columbia, Canada"
      ],
      "name": "Jasmine Grewal"
    },
    {
      "affiliations": [
        "Mississippi Department of Mental Health, Jackson, Mississippi, USA"
      ],
      "name": "Frank Han"
    },
    {
      "affiliations": [
        "Michigan Medicine, Ann Arbor, Michigan, USA"
      ],
      "name": "Timothy Cotts"
    },
    {
      "affiliations": [
        "University of Alberta, Edmonton, Alberta, Canada"
      ],
      "name": "Stephen Pylypchuk"
    },
    {
      "affiliations": [
        "Vanderbilt University, Nashville, Tennessee, USA"
      ],
      "name": "Tripti Gupta"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Surgery, 2nd Medical Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic"
      ],
      "name": "Petra Antonová"
    },
    {
      "affiliations": [
        "Cardiology, Auckland City Hospital, Auckland, New Zealand"
      ],
      "name": "Clare O’Donnell"
    },
    {
      "affiliations": [
        "Cardiology, Children’s National Medical Center, Washinton, District of Columbia, USA"
      ],
      "name": "Anitha John"
    },
    {
      "affiliations": [
        "Adult Congenital Heart Disease Unit, Virgen del Rocio University Hospital Cardiology and Cardiovascular Surgery Service, Sevilla, Spain"
      ],
      "name": "Pastora Gallego"
    },
    {
      "affiliations": [
        "Cardiology, Amsterdam UMC - Locatie AMC, Amsterdam, Netherlands"
      ],
      "name": "Alexandra van Dissel"
    },
    {
      "affiliations": [
        "Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA"
      ],
      "name": "Alexander R Opotowsky"
    },
    {
      "affiliations": [
        "University of Colorado System, Denver, Colorado, USA"
      ],
      "name": "Elizabeth Yeung"
    },
    {
      "affiliations": [
        "Oregon Health and Science University, Portland, Oregon, USA"
      ],
      "name": "Craig S Broberg"
    },
    {
      "affiliations": [
        "Adult Congenital Heart Disease Unit, Monaldi Hospital, Napoli, Italy"
      ],
      "name": "Berardo Sarubbi"
    }
  ],
  "title": "Cardiac resynchronisation therapy among adults with a systemic right ventricle: a multicentre experience",
  "uid": "b5e0c13e-8db5-5bfb-bd73-55a976b9b83c"
}