title: Inverted U-Shaped Relation Between the Risk of Sudden Cardiac Death and Maximal Left Ventricular Wall Thickness in Hypertrophic Cardiomyopathy.
abstract: Hypertrophic cardiomyopathy is associated with sudden cardiac death (SCD). Some studies have shown an association between risk of sudden death and left ventricular maximal wall thickness (MWT), but there are few data in patients with extreme hypertrophy. The aim of this study was to determine the relation between MWT and the risk of SCD.
  
  
  
  This is a multicenter, retrospective, longitudinal cohort study of 3673 adult (≥16 years) patients, previously used to develop and validate a risk prediction model for SCD (HCM Risk-SCD [hypertrophic cardiomyopathy risk-SCD]). There was an inverted U-shaped relation between MWT and the estimated 5-year risk of SCD. In patients with MWT≥35 mm (n=47; mean age, 33 years; 81% men), there was a single SCD end point (annual rate, 0.2%; 95% confidence interval, 0.03-1.60) and 3 additional cardiovascular events during a median follow-up of 9.5 years. Compared with patients with MWT≤14 mm, those with MWT≥35 mm did not have a higher risk for SCD (hazard ratio, 0.22; 95% confidence interval, 0.03-1.65), cardiovascular death (hazard ratio, 0.66; 95% confidence interval, 0.26-1.67), or all-cause mortality (hazard ratio, 0.73; 95% confidence interval, 0.32-1.69).
  
  
  
  The risk of SCD has a complex, nonlinear relationship to MWT. The pathophysiological mechanisms behind this observation require further study but implantable cardioverter defibrillator implantation should not be guided solely on the severity of left ventricular hypertrophy.
authors.affiliation: From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.). c.omahony@doctors.org.uk.
authors.forename: Constantinos
authors.initials: C
authors.lastname: O'Mahony
authors.affiliation: From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.).
authors.forename: Fatima
authors.initials: F
authors.lastname: Jichi
authors.affiliation: From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.).
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Monserrat
authors.affiliation: From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.).
authors.forename: Martin
authors.initials: M
authors.lastname: Ortiz-Genga
authors.affiliation: From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.).
authors.forename: Aristides
authors.initials: A
authors.lastname: Anastasakis
authors.affiliation: From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.).
authors.forename: Claudio
authors.initials: C
authors.lastname: Rapezzi
authors.affiliation: From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.).
authors.forename: Elena
authors.initials: E
authors.lastname: Biagini
authors.affiliation: From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.).
authors.forename: Juan Ramon
authors.initials: JR
authors.lastname: Gimeno
authors.affiliation: From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.).
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Limongelli
authors.affiliation: From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.).
authors.forename: William J
authors.initials: WJ
authors.lastname: McKenna
authors.affiliation: From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.).
authors.forename: Rumana Z
authors.initials: RZ
authors.lastname: Omar
authors.affiliation: From the Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom (C.O'M., W.J.M., P.M.E.); Biostatistics Group, University College London Hospitals/University College London Research Support Centre, London, United Kingdom (F.J., R.Z.O.); Department of Statistical Science, University College London, London, United Kingdom (R.Z.O.); Research Unit, Department of Cardiology, A Coruña University Hospital, and Galician Health Service, A Coruña, Spain (L.M., M.O.-G.); Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece (A.A.); Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy (C.R., E.B.); Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain (J.R.G.); and Monaldi Hospital, Second University of Naples, Naples, Italy (G.L.).
authors.forename: Perry M
authors.initials: PM
authors.lastname: Elliott
mesh_terms: D000328:Adult; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications*; D016757:Death, Sudden, Cardiac / Q000209:etiology* / Q000517:prevention & control; D017147:Defibrillators, Implantable; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000150:complications*; D008137:Longitudinal Studies; D008297:Male; D012189:Retrospective Studies
keywords: cardiomyopathy, hypertrophic; death, sudden; defibrillators, implantable; follow-up studies; hypertrophy, left ventricular