Constantinos O'Mahony1,2,3, Fatima Jichi4, Steve R Ommen5, Imke Christiaans3,6,7, Eloisa Arbustini8, Pablo Garcia-Pavia3,9,10, Franco Cecchi11, Iacopo Olivotto11, Hiroaki Kitaoka12, Israel Gotsman13, Gerald Carr-White14, Jens Mogensen15, Loizos Antoniades16, Saidi A Mohiddin17,3,15, Mathew S Maurer18, Hak Chiaw Tang19, Jeffrey B Geske5, Konstantinos C Siontis5,20, Karim D Mahmoud5,21, Alexa Vermeer3,6,7, Arthur Wilde3,6, Valentina Favalli3,8, Oliver P Guttmann17,22,3, Maria Gallego-Delgado9, Fernando Dominguez9, Ilaria Tanini11, Toru Kubo12, Andre Keren13,23, Teofila Bueser15,24, Sarah Waters14, Issa F Issa25, James Malcolmson17,3,25, Tom Burns14, Neha Sekhri17,3,15, Christopher W Hoeger18, Rumana Z Omar26, Perry M Elliott17,2,22,3. 1. St. Bartholomew's Centre for Inherited Cardiovascular Disease, St Bartholomew's Hospital, West Smithfield, London, United Kingdom (C.O., S.A.M., O.P.G., J.M., N.S., P.M.E.). drcostasomahony@gmail.com. 2. Centre for Heart Muscle Disease, Institute of Cardiovascular Science (C.O., P.M.E.). 3. University College London, United Kingdom. European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart (ERN GUARDHEART; http://guardheart.ern-net.eu) (C.O., I.C., P.G.-P., S.A.M., A.V., A.W., V.F., O.P.G., J.M., N.S., P.M.E.). 4. Biostatistics Group, University College London Hospitals/University College London Joint Research Office (F.J.). 5. Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN (S.R.O., J.B.G., K.C.S., K.D.M.). 6. Heart Center, Department of Clinical and Experimental Cardiology (I.C., A.V., A.W.). 7. Department of Clinical Genetics (I.C., A.V.). 8. Academic Medical Center, Amsterdam, Netherlands. Centre for Inherited Cardiovascular Diseases, Transplant Research Area, Istituto di Ricovero e Cura a Carattere Scientifico Foundation, Policlinico San Matteo, Pavia, Italy (E.A., V.F.). 9. Heart Failure and Inherited Cardiac Diseases Unit, Department of Cardiology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain (P.G.-P., M.G.-D., F.D.). 10. Centro de Investigacion Biomedica en Red en Enfermedades Cardiovasculares, Madrid, Spain (P.G.-P.). University Francisco de Vitoria, Pozuelo de Alarcón, Madrid, Spain (P.G.-P.). 11. Department of Cardiology, Careggi University Hospital, Florence, Italy (F.C., I.O., I.T.). 12. Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kohasu, Oko-cho, Nankoku-shi, Japan (H.K., T.K.). 13. Heart Institute, Hadassah University Hospital, Jerusalem, Israel (I.G., A.K.). 14. Guy's and St. Thomas' Hospital National Health Service Foundation Trust, London, United Kingdom (G.C.-W., T.B., S.W.). 15. London Chest Hospital, United Kingdom (S.A.M., J.M., T.B., N.S.). 16. Inherited Cardiovascular Disease Unit, Department of Cardiology, Nicosia General Hospital, Latsia, Cyprus (L.A.). 17. St. Bartholomew's Centre for Inherited Cardiovascular Disease, St Bartholomew's Hospital, West Smithfield, London, United Kingdom (C.O., S.A.M., O.P.G., J.M., N.S., P.M.E.). 18. Columbia University Medical Center, New York, NY (M.S.M., C.W.H.). 19. Department of Cardiology, National Heart Centre Singapore (H.C.T.). 20. Division of Cardiovascular Medicine, University of Michigan, Ann Arbor (K.C.S.). 21. Thorax Center, Department of Cardiology, Erasmus Medical Center, Rotterdam, Netherlands (K.D.M.). 22. The Inherited Cardiac Diseases Unit, The Heart Hospital (O.P.G., P.M.E.). 23. Clalit Health Services Beit Hadfus 20, Jerusalem, Israel (A.K.). Assuta Hospitals, Tel Aviv, Israel (A.K.). 24. King's College London, United Kingdom (T.B.). St George's, University of London, United Kingdom (T.B.). 25. Department of Cardiology, Odense University Hospital, Denmark (J.M., I.F.I.). 26. Department of Statistical Science (R.Z.O.).
Abstract
BACKGROUND: Identification of people with hypertrophic cardiomyopathy (HCM) who are at risk of sudden cardiac death (SCD) and require a prophylactic implantable cardioverter defibrillator is challenging. In 2014, the European Society of Cardiology proposed a new risk stratification method based on a risk prediction model (HCM Risk-SCD) that estimates the 5-year risk of SCD. The aim was to externally validate the 2014 European Society of Cardiology recommendations in a geographically diverse cohort of patients recruited from the United States, Europe, the Middle East, and Asia. METHODS: This was an observational, retrospective, longitudinal cohort study. RESULTS: The cohort consisted of 3703 patients. Seventy three (2%) patients reached the SCD end point within 5 years of follow-up (5-year incidence, 2.4% [95% confidence interval {CI}, 1.9-3.0]). The validation study revealed a calibration slope of 1.02 (95% CI, 0.93-1.12), C-index of 0.70 (95% CI, 0.68-0.72), and D-statistic of 1.17 (95% CI, 1.05-1.29). In a complete case analysis (n= 2147; 44 SCD end points at 5 years), patients with a predicted 5-year risk of <4% (n=1524; 71%) had an observed 5-year SCD incidence of 1.4% (95% CI, 0.8-2.2); patients with a predicted risk of ≥6% (n=297; 14%) had an observed SCD incidence of 8.9% (95% CI, 5.96-13.1) at 5 years. For every 13 (297/23) implantable cardioverter defibrillator implantations in patients with an estimated 5-year SCD risk ≥6%, 1 patient can potentially be saved from SCD. CONCLUSIONS: This study confirms that the HCM Risk-SCD model provides accurate prognostic information that can be used to target implantable cardioverter defibrillator therapy in patients at the highest risk of SCD.
BACKGROUND: Identification of people with hypertrophic cardiomyopathy (HCM) who are at risk of sudden cardiac death (SCD) and require a prophylactic implantable cardioverter defibrillator is challenging. In 2014, the European Society of Cardiology proposed a new risk stratification method based on a risk prediction model (HCM Risk-SCD) that estimates the 5-year risk of SCD. The aim was to externally validate the 2014 European Society of Cardiology recommendations in a geographically diverse cohort of patients recruited from the United States, Europe, the Middle East, and Asia. METHODS: This was an observational, retrospective, longitudinal cohort study. RESULTS: The cohort consisted of 3703 patients. Seventy three (2%) patients reached the SCD end point within 5 years of follow-up (5-year incidence, 2.4% [95% confidence interval {CI}, 1.9-3.0]). The validation study revealed a calibration slope of 1.02 (95% CI, 0.93-1.12), C-index of 0.70 (95% CI, 0.68-0.72), and D-statistic of 1.17 (95% CI, 1.05-1.29). In a complete case analysis (n= 2147; 44 SCD end points at 5 years), patients with a predicted 5-year risk of <4% (n=1524; 71%) had an observed 5-year SCD incidence of 1.4% (95% CI, 0.8-2.2); patients with a predicted risk of ≥6% (n=297; 14%) had an observed SCD incidence of 8.9% (95% CI, 5.96-13.1) at 5 years. For every 13 (297/23) implantable cardioverter defibrillator implantations in patients with an estimated 5-year SCD risk ≥6%, 1 patient can potentially be saved from SCD. CONCLUSIONS: This study confirms that the HCM Risk-SCD model provides accurate prognostic information that can be used to target implantable cardioverter defibrillator therapy in patients at the highest risk of SCD.
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