Your browser doesn't support javascript.
loading
Risk of Sudden Death in Patients With RASopathy Hypertrophic Cardiomyopathy.
Lynch, Aine; Tatangelo, Mark; Ahuja, Sachin; Steve Fan, Chun-Po; Min, Sandar; Lafreniere-Roula, Myriam; Papaz, Tanya; Zhou, Vivian; Armstrong, Kathryn; Aziz, Peter F; Benson, Lee N; Butts, Ryan; Dragulescu, Andreea; Gardin, Letizia; Godown, Justin; Jeewa, Aamir; Kantor, Paul F; Kaufman, Beth D; Lal, Ashwin K; Parent, John J; Richmond, Marc; Russell, Mark W; Balaji, Seshadri; Stephenson, Elizabeth A; Villa, Chet; Jefferies, John L; Whitehill, Robert; Conway, Jennifer; Howard, Taylor S; Nakano, Stephanie J; Rossano, Joseph; Weintraub, Robert G; Mital, Seema.
Afiliación
  • Lynch A; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto Ontario, Canada.
  • Tatangelo M; Ted Rogers Computational Program, Ted Rogers Centre for Heart Research, Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada.
  • Ahuja S; Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada.
  • Steve Fan CP; Ted Rogers Computational Program, Ted Rogers Centre for Heart Research, Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada.
  • Min S; Genetics and Genome Biology, Hospital for Sick Children, Toronto, Ontario, Canada.
  • Lafreniere-Roula M; Applied Health Research Centre, St Michael's Hospital of Unity Health Toronto, Toronto, Ontario, Canada.
  • Papaz T; Genetics and Genome Biology, Hospital for Sick Children, Toronto, Ontario, Canada; Ted Rogers Centre for Heart Research, Toronto, Ontario, Canada.
  • Zhou V; Genetics and Genome Biology, Hospital for Sick Children, Toronto, Ontario, Canada.
  • Armstrong K; Department of Pediatrics, BC Children's Hospital, Vancouver, British Columbia, Canada.
  • Aziz PF; Department of Pediatrics, Cleveland Clinic Children's Hospital, Cleveland, Ohio, USA.
  • Benson LN; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto Ontario, Canada.
  • Butts R; Department of Pediatrics, UT Southwestern Medical Center, Dallas, Texas, USA.
  • Dragulescu A; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto Ontario, Canada.
  • Gardin L; Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
  • Godown J; Department of Pediatrics, Monroe Carrell Jr Children's Hospital at Vanderbilt University, Nashville, Tennessee, USA.
  • Jeewa A; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto Ontario, Canada.
  • Kantor PF; Department of Pediatrics, Children's Hospital of Los Angeles, Los Angeles, California, USA.
  • Kaufman BD; Department of Pediatrics, Lucile Packard Children's Hospital, Stanford University, Palo Alto, California, USA.
  • Lal AK; Department of Pediatrics, Primary Children's Hospital, University of Utah, Salt Lake City, Utah, USA.
  • Parent JJ; Department of Pediatrics, Riley Children's Hospital, Indianapolis, Indiana, USA.
  • Richmond M; Department of Pediatrics, Morgan Stanley Children's Hospital, Columbia University Medical Center, New York, New York, USA.
  • Russell MW; Department of Pediatrics, University of Michigan Health System, Ann Arbor, Michigan, USA.
  • Balaji S; Department of Pediatrics, Oregon Health and Science University, Portland, Oregon, USA.
  • Stephenson EA; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto Ontario, Canada.
  • Villa C; Department of Pediatrics, Cincinnati Children's Hospital, Cincinnati, Ohio, USA.
  • Jefferies JL; Department of Pediatrics, University of Tennessee Health Sciences Centre, Memphis, Tennessee, USA.
  • Whitehill R; Department of Pediatrics, Children's Healthcare of Atlanta, Atlanta, Georgia, USA.
  • Conway J; Department of Pediatrics, Stollery Children's Hospital, Edmonton, Alberta, Canada.
  • Howard TS; Department of Pediatrics, Texas Children's Hospital, Houston, Texas, USA.
  • Nakano SJ; Department of Pediatrics, Children's Hospital Colorado, Aurora, Colorado, USA.
  • Rossano J; Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
  • Weintraub RG; Department of Cardiology, The Royal Children's Hospital of Melbourne, Melbourne, Victoria, Australia.
  • Mital S; Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto Ontario, Canada; Ted Rogers Computational Program, Ted Rogers Centre for Heart Research, Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada; Ted Rogers Centre for Heart Research, Toronto,
J Am Coll Cardiol ; 81(11): 1035-1045, 2023 03 21.
Article en En | MEDLINE | ID: mdl-36922089
BACKGROUND: Genetic defects in the RAS/mitogen-activated protein kinase pathway are an important cause of hypertrophic cardiomyopathy (RAS-HCM). Unlike primary HCM (P-HCM), the risk of sudden cardiac death (SCD) and long-term survival in RAS-HCM are poorly understood. OBJECTIVES: The study's objective was to compare transplant-free survival, incidence of SCD, and implantable cardioverter-defibrillator (ICD) use between RAS-HCM and P-HCM patients. METHODS: In an international, 21-center cohort study, we analyzed phenotype-positive pediatric RAS-HCM (n = 188) and P-HCM (n = 567) patients. The between-group differences in cumulative incidence of all outcomes from first evaluation were compared using Gray's tests, and age-related hazard of all-cause mortality was determined. RESULTS: RAS-HCM patients had a lower median age at diagnosis compared to P-HCM (0.9 years [IQR: 0.2-5.0 years] vs 9.8 years [IQR: 2.0-13.9 years], respectively) (P < 0.001). The 10-year cumulative incidence of SCD from first evaluation was not different between RAS-HCM and P-HCM (4.7% vs 4.2%, respectively; P = 0.59). The 10-year cumulative incidence of nonarrhythmic deaths or transplant was higher in RAS-HCM compared with P-HCM (11.0% vs 5.4%, respectively; P = 0.011). The 10-year cumulative incidence of ICD insertions, however, was 5-fold lower in RAS-HCM compared with P-HCM (6.9% vs 36.6%; P < 0.001). Nonarrhythmic deaths occurred primarily in infancy and SCD primarily in adolescence. CONCLUSIONS: RAS-HCM was associated with a higher incidence of nonarrhythmic death or transplant but similar incidence of SCD as P-HCM. However, ICDs were used less frequently in RAS-HCM compared to P-HCM. In addition to monitoring for heart failure and timely consideration of advanced heart failure therapies, better risk stratification is needed to guide ICD practices in RAS-HCM.
Asunto(s)
Palabras clave
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Cardiomiopatía Hipertrófica / Desfibriladores Implantables / Insuficiencia Cardíaca Tipo de estudio: Diagnostic_studies / Etiology_studies / Incidence_studies / Observational_studies / Risk_factors_studies Límite: Humans Idioma: En Revista: J Am Coll Cardiol Año: 2023 Tipo del documento: Article País de afiliación: Canadá Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Cardiomiopatía Hipertrófica / Desfibriladores Implantables / Insuficiencia Cardíaca Tipo de estudio: Diagnostic_studies / Etiology_studies / Incidence_studies / Observational_studies / Risk_factors_studies Límite: Humans Idioma: En Revista: J Am Coll Cardiol Año: 2023 Tipo del documento: Article País de afiliación: Canadá Pais de publicación: Estados Unidos