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Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort
  1. Anne Marie Valente1,
  2. Kimberlee Gauvreau1,
  3. Gabriele Egidy Assenza1,
  4. Sonya V Babu-Narayan2,
  5. Jenna Schreier1,
  6. Michael A Gatzoulis2,
  7. Maarten Groenink3,
  8. Ryo Inuzuka2,
  9. Philip J Kilner2,
  10. Zeliha Koyak3,
  11. Michael J Landzberg1,
  12. Barbara Mulder3,
  13. Andrew J Powell1,
  14. Rachel Wald4,
  15. Tal Geva1
  1. 1Departments of Cardiology, Boston Children's Hospital and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA
  2. 2NIHR Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College, London, UK
  3. 3Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands
  4. 4Division of Cardiology, University of Toronto, Peter Munk Cardiac Centre, Toronto, Ontario, Canada
  1. Correspondence to Dr Anne Marie Valente, Department of Cardiology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, UK; anne.valente{at}cardio.chboston.org

Abstract

Objective Patients with repaired tetralogy of Fallot (TOF) experience increased rates of mortality and morbidity in adulthood. This study was designed to identify risk factors for death and ventricular tachycardia (VT) in a large contemporary cohort of patients with repaired TOF.

Methods Subjects with repaired TOF from four large congenital heart centres in the USA, Canada and Europe were enrolled. Clinical, ECG, exercise, cardiac magnetic resonance (CMR) and outcome data were analysed.

Results Of the 873 patients (median age 24.4 years), 32 (3.7%) reached the primary outcome (28 deaths, 4 sustained VT; median age at outcome 38 years; median time from CMR to outcome 1.9 years). Cox proportional-hazards regression identified RV mass-to-volume ratio ≥0.3 g/mL (HR, 5.04; 95% CI 2.3 to 11.0; p<0.001), LV EF z score<−2.0 (HR, 3.34; 95% CI 1.59 to 7.01; p=0.001), and history of atrial tachyarrhythmia (HR, 3.65; 95% CI 1.75 to 7.62; p=0.001) as outcome predictors. RV dysfunction was predictive of the outcome similar to LV dysfunction. In subgroup analysis of 315 subjects with echocardiographic assessment of RV systolic pressure, higher pressure (HR 1.39; 95% CI 1.19 to 1.62; p<0.001) was associated with death and sustained VT independent of RV hypertrophy and LV dysfunction.

Conclusions RV hypertrophy, ventricular dysfunction and atrial tachyarrhythmias are predictive of death and sustained VT in adults with repaired TOF. These findings may inform risk stratification and the design of future therapeutic trials.

  • Congenital Heart Disease

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