Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy
Por:
Norrish G, Ding T, Field E, Cervi E, Ziólkowska L, Olivotto I, Khraiche D, Limongelli G, Anastasakis A, Weintraub R, Biagini E, Ragni L, Prendiville T, Duignan S, McLeod K, Ilina M, Fernandez A, Marrone C, Bökenkamp R, Baban A, Kubus P, Daubeney PEF, Sarquella-Brugada G, César-Díaz S, Klaassen S, Ojala TH, Bhole V, Medrano C, Uzun O, Brown E, Gran F, Sinagra G, Castro FJ, Stuart G, Vignati G, Yamazawa H, Barriales-Villa R, Garcia-Guereta L, Adwani S, Linter K, Bharucha T, Garcia-Pavia P, Siles A, Rasmussen TB, Calcagnino M, Jones CB, De Wilde H, Kubo T, Felice T, Popoiu A, Mogensen J, Mathur S, Centeno F, Reinhardt Z, Schouvey S, O'Mahony C, Omar RZ, Elliott PM and Kaski JP
Publicada:
1 may 2022
Ahead of Print:
2 may 2022
Resumen:
Background: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. Methods: The study cohort comprised 1075 children (mean age, 10.2 years [+/- 4.4]) diagnosed with HCM (1-16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). Results: MLVWT Z score was <10 in 598 (58.1%), >= 10 to <20 in 334 (31.1%), and >= 20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score >= 20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3-9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores <10, >= 10 to <20, and >= 20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. Conclusions: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM.
Filiaciones:
Norrish G:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom (G.N., E.F., E.C., J.P.K.)
Institute of Cardiovascular Sciences, University College London, United Kingdom. (G.N., C.O., P.M.E., J.P.K.)
Ding T:
Department of Statistical Science, University College London, United Kingdom. (T.D., R.Z.O.)
Field E:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom (G.N., E.F., E.C., J.P.K.)
Cervi E:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom (G.N., E.F., E.C., J.P.K.)
Ziólkowska L:
The Children's Memorial Health Institute, Warsaw, Poland (L.Z.)
Olivotto I:
Careggi University Hopsital, Florence, Italy (I.O.)
Khraiche D:
Necker-Enfants Malades Hospital, Paris, France (D.K.)
Limongelli G:
Monaldi Hospital, Naples, Italy (G.L.)
Anastasakis A:
Onassis Cardiac Surgery Center, Athens, Greece (A.A.)
Weintraub R:
Royal Children's Hospital, Melbourne, Australia (R.W.)
Biagini E:
Cardiology Unit, S. Orsola-Malpighi Hospital, IRCCS Azienda Ospedalierao-Universitaria di Bologna, Italy (E.B., L.R.)
Ragni L:
Cardiology Unit, S. Orsola-Malpighi Hospital, IRCCS Azienda Ospedalierao-Universitaria di Bologna, Italy (E.B., L.R.)
Prendiville T:
Our Lady's Children's Hospital, Dublin, Ireland (T.P., S.D.)
Duignan S:
Our Lady's Children's Hospital, Dublin, Ireland (T.P., S.D.)
McLeod K:
Royal Hospital for Children, Glasgow, United Kingdom (K.M., M.I.)
Ilina M:
Royal Hospital for Children, Glasgow, United Kingdom (K.M., M.I.)
Fernandez A:
Fundación Favaloro University Hospital, Buenos Aires, Argentina (A.F.)
Marrone C:
Papa Giovanni XXIII Hospital, Bergamo (C.M.)
Fondazione Toscana G. Monasterio, Massa-Pisa, Italy (C.M.)
Bökenkamp R:
Leiden University Medical Center, the Netherlands (R.B.)
Baban A:
Bambino Gesu Hospital, Rome, Italy (A.B.)
Kubus P:
University Hospital Motol, Prague, Czech Republic (P.K.)
Daubeney PEF:
Royal Brompton and Harefield NHS Trust, London, United Kingdom (P.E.F.D.)
Sarquella-Brugada G:
Sant Joan de Deu, Barcelona, Spain (G.S.-B., S.C.)
César-Díaz S:
Sant Joan de Deu, Barcelona, Spain (G.S.-B., S.C.)
Klaassen S:
Department of Pediatric Cardiology, Charite-Universitatsmedizin Berlin, Germany. (S.K.)
Experimental and Clinical Research Center, a joint cooperation between the Charité Medical Faculty and the Max-Delbrück-Center for Molecular Medicine, Charite-Universitatsmedizin Berlin, Germany.(S.K.)
German Centre for Cardiovascular Research, Partner Site Berlin, Germany (S.K.)
Ojala TH:
Department of Pediatric Cardiology, Pediatric Research Center, New Children's Hospital, University of Helsinki, Finland (T.H.O.)
Bhole V:
Birmingham Children's Hospital, United Kingdom (V.B.)
Medrano C:
Hospital General Universitario Gregorio Marañón, Madrid, Spain (C.M.)
Uzun O:
University Hospital of Wales, Cardiff (O.U.)
Brown E:
Leeds General Infirmary, United Kingdom (E.B.)
Gran F:
Val d'Hebron University Hospital, Barcelona, Spain (F.G.)
Sinagra G:
Heart Muscle Disease Registry Trieste, University of Trieste, Italy (G.S.)
Castro FJ:
University Hospital Virgen de la Arrixaca, Murcia, Spain (F.J.C.)
Stuart G:
Bristol Royal Hospital for Children, United Kingdom (G.S.)
Vignati G:
Niguarda Hospital, Milan, Italy (G.V.)
Yamazawa H:
Department of Pediatrics, Faculty of Medicine and Graduate School of Medicine, Hokkaido University Hospital, Sapporo, Japan (H.Y.)
Barriales-Villa R:
Complexo Hospitalario Universitario A Coruna, INIBIC, CIBERCV (R.B.-V.)
Garcia-Guereta L:
University Hospital La Paz, Madrid, Spain (L.G.-G.)
Adwani S:
John Radcliffe Hospital, Oxford (S.A.)
Linter K:
Glenfield Hospital, Leicester (K.L.)
Bharucha T:
Southampton General Hospital, Southampton, United Kingdom (T.B.)
Garcia-Pavia P:
Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain (P.G.-P., A.S.)
Siles A:
Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain (P.G.-P., A.S.)
Rasmussen TB:
Aarhus University Hospital, Denmark (T.B.R.)
Calcagnino M:
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milano, Dept di Medicina Interna, UOC Cardiologica (M.C.)
Jones CB:
Alder Hey Children's Hospital, Liverpool, United Kingdom (C.B.J.)
De Wilde H:
Ghent University Hospital, Belgium (H.D.W.)
Kubo T:
Kochi Medical School Hospital, Japan (T.K.)
Felice T:
Mater Dei Hospital, Malta (T.F.)
Popoiu A:
Department of Pediatrics, University of Medicine and Pharmacy "Victor Babes" Timisoara, Children's Hospital 'Louis Turcanu,' Romania (A.P.)
Mogensen J:
Aalborg University Hospital, Denmark (J.M.)
Mathur S:
Evelina Children's Hospital, London, United Kingdom (S.M.)
Centeno F:
Rio Hortega University Hospital, Valladolid, Spain (F.C.)
Reinhardt Z:
The Freeman Hospital, Newcastle, United Kingdom (Z.R.)
Schouvey S:
Hospital Saint Joseph, Marseille, France (S.S.)
O'Mahony C:
Institute of Cardiovascular Sciences, University College London, United Kingdom. (G.N., C.O., P.M.E., J.P.K.)
St Bartholomew's Centre for Inherited Cardiovascular Diseases, St Bartholomew's Hospital, West Smithfield, London, United Kingdom (C.O., P.M.E.)
Omar RZ:
Department of Statistical Science, University College London, United Kingdom. (T.D., R.Z.O.)
Elliott PM:
Institute of Cardiovascular Sciences, University College London, United Kingdom. (G.N., C.O., P.M.E., J.P.K.)
St Bartholomew's Centre for Inherited Cardiovascular Diseases, St Bartholomew's Hospital, West Smithfield, London, United Kingdom (C.O., P.M.E.)
Kaski JP:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom (G.N., E.F., E.C., J.P.K.)
Institute of Cardiovascular Sciences, University College London, United Kingdom. (G.N., C.O., P.M.E., J.P.K.)
Green Published, hybrid, Green Accepted
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