Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy
Por:
Norrish G, Cleary A, Field E, Cervi E, Boleti O, 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, Yamazawa H, Barriales-Villa R, Garcia-Guereta L, Adwani S, Linter K, Bharucha T, Gonzales-Lopez E, 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, Elliott PM and Kaski JP
Publicada:
24 may 2022
Ahead of Print:
1 may 2022
Categoría:
Cardiology and cardiovascular medicine
Resumen:
BACKGROUND Up to one-half of childhood sarcomeric hypertrophic
cardiomyopathy (HCM) presents before the age of 12 years, but this
patient group has not been systematically characterized.
OBJECTIVES The aim of this study was to describe the clinical
presentation and natural history of patients presenting with
nonsyndromic HCM before the age of 12 years.
METHODS Data from the International Paediatric Hypertrophic
Cardiomyopathy Consortium on 639 children diagnosed with HCM younger
than 12 years were collected and compared with those from 568 children
diagnosed between 12 and 16 years.
RESULTS At baseline, 339 patients (53.6%) had family histories of HCM,
132 (20.9%) had heart failure symptoms, and 250 (39.2%) were prescribed
cardiac medications. The median maximal left ventricular wall thickness
z-score was 8.7 (IQR: 5.3-14.4), and 145 patients (27.2%) had left
ventricular outflow tract obstruction. Over a median follow-up period of
5.6 years (IQR: 2.3-10.0 years), 42 patients (6.6%) died, 21 (3.3%)
underwent cardiac transplantation, and 69 (10.8%) had life-threatening
arrhythmic events. Compared with those presenting after 12 years, a
higher proportion of younger patients underwent myectomy (10.5% vs 7.2%;
P = 0.045), but fewer received primary prevention implantable
cardioverter-defibrillators (18.9% vs 30.1%; P = 0.041). The incidence
of mortality or life-threatening arrhythmic events did not differ, but
events occurred at a younger age.
CONCLUSIONS Early-onset childhood HCM is associated with a comparable
symptom burden and cardiac phenotype as in patients presenting later in
childhood. Long-term outcomes including mortality did not differ by age
of presentation, but patients presenting at younger than 12 years
experienced adverse events at younger ages. (C) 2022 The Authors.
Published by Elsevier on behalf of the American College of Cardiology
Foundation.
Filiaciones:
Norrish G:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom
Institute of Cardiovascular Sciences, University College London, London, United Kingdom
Cleary A:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom
Field E:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom
Cervi E:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom
Boleti O:
Institute of Cardiovascular Sciences, University College London, London, United Kingdom
Ziólkowska L:
The Children's Memorial Health Institute, Warsaw, Poland
Olivotto I:
Careggi University Hospital, Florence, Italy
Khraiche D:
Necker-Enfants Malades Hospital, Paris, France
Limongelli G:
Inherited and Rare Cardiovascular Disease Unit, AO dei Colli Monaldi Hospital, Universita della Campania "Luigi Vanvitelli," Naples, Italy
Anastasakis A:
Onassis Cardiac Surgery Center, Athens, Greece
Weintraub R:
The Royal Children's Hospital, Melbourne, Victoria, Australia
Biagini E:
Cardiology Unit, St Orsola Hospital, IRCCS Azienda Ospedalierao-Universitaria di Bologna, Bologna, Italy
Ragni L:
Cardiology Unit, St Orsola Hospital, IRCCS Azienda Ospedalierao-Universitaria di Bologna, Bologna, Italy
Prendiville T:
Our Lady's Children's Hospital, Dublin, Ireland
Duignan S:
Our Lady's Children's Hospital, Dublin, Ireland
McLeod K:
Royal Hospital for Children, Glasgow, United Kingdom
Ilina M:
Royal Hospital for Children, Glasgow, United Kingdom
Fernandez A:
Fundación Favaloro University Hospital, Buenos Aires, Argentina
Marrone C:
Papa Giovanni XXIII hospital, Bergamo, Italy
Fondazione Toscana G. Monasterio, Massa-Pisa, Italy
Bökenkamp R:
Leiden University Medical Center, Leiden, the Netherlands
Baban A:
Bambino Gesu Hospital, Rome, Italy
Kubus P:
University Hospital Motol, Prague, Czech Republic
Daubeney PEF:
Royal Brompton and Harefield NHS Trust, London, United Kingdom
Sarquella-Brugada G:
Sant Joan de Déu, Barcelona, Spain
César-Díaz S:
Sant Joan de Déu, Barcelona, Spain
Klaassen S:
Department of Pediatric Cardiology, Charite-Universitatsmedizin Berlin, Berlin, Germany
Experimental and Clinical Research Center, a joint cooperation between the Charité Medical Faculty and the Max-Delbrück-Center for Molecular Medicine, Charite-Universitatsmedizin Berlin, Berlin, Germany
DZHK (German Center for Cardiovascular Research), partner site Berlin, Berlin, Germany
Ojala TH:
Department of Pediatric Cardiology, Pediatric Research Center, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
Bhole V:
Birmingham Children's Hospital, Birmingham, United Kingdom
Medrano C:
Hospital General Universitario Gregorio Marañón, Madrid, Spain
Uzun O:
University Hospital of Wales, Cardiff, United Kingdom
Brown E:
Leeds General Infirmary, Leeds, United Kingdom
Gran F:
Vall d'Hebron University Hospital, Barcelona, Spain
Sinagra G:
Cardiothoracovascular Department, University of Trieste, Trieste, Italy
Castro FJ:
University Hospital Virgen de la Arrixaca, Murcia, Spain
Stuart G:
Bristol Royal Hospital for Children, Bristol, United Kingdom
Yamazawa H:
Department of Pediatrics, Faculty of Medicine and Graduate School of Medicine, Hokkaido University Hospital, Sapporo, Japan
Barriales-Villa R:
Complexo Hospitalario Universitario A Coruña, INIBIC, CIBERCV, La Coruña, Spain
Garcia-Guereta L:
University Hospital La Paz, Madrid, Spain
Adwani S:
John Radcliffe Hospital, Oxford, United Kingdom
Linter K:
Glenfield Hospital, Leicester, United Kingdom
Bharucha T:
Southampton General Hospital, Southampton, United Kingdom
Gonzales-Lopez E:
Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain
Siles A:
Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain
Rasmussen TB:
Aarhus University Hospital, Aarhus, Denmark
Calcagnino M:
Fondazione IRCCS Ca Granda - Ospedale Maggiore Policlinico Milano, Department di Medicina Interna - UOC Cardiologica, Milan, Italy
Jones CB:
Alder Hey Children's Hospital, Liverpool, United Kingdom
De Wilde H:
Ghent University Hospital, Ghent, Belgium
Kubo T:
Kochi Medical School Hospital, Kochi, Japan
Felice T:
Mater Dei Hospital, Msida, Malta
Popoiu A:
University of Medicine and Pharmacy "Victor Babes" Timisoara, Department of Pediatrics, Children's Hospital "Louis Turcanu," Timisoara, Romania
Mogensen J:
Aalborg University Hospital, Aalborg, Denmark
Mathur S:
Evelina Children's Hospital, London, United Kingdom
Centeno F:
Rio Hortega University Hospital, Valladolid, Spain
Reinhardt Z:
The Freeman Hospital, Newcastle, United Kingdom
Schouvey S:
Hospital Saint Joseph, Marseille, France
Elliott PM:
Institute of Cardiovascular Sciences, University College London, London, United Kingdom
St Bartholomew's Centre for Inherited Cardiovascular Diseases, St Bartholomew's Hospital, West Smithfield, London, United Kingdom
Kaski JP:
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom
Institute of Cardiovascular Sciences, University College London, London, United Kingdom
Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom; Institute of Cardiovascular Sciences, University College London, London, United Kingdom.; Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom.; Institute of Cardiovascular Sciences, University College London, London, United Kingdom.; The Children's Memorial Health Institute, Warsaw, Poland.; Careggi University Hospital, Florence, Italy.; Necker-Enfant
Green Submitted, hybrid
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