title: Phenotypic expression in hypertrophic cardiomyopathy and late gadolinium enhancement on cardiac magnetic resonance.
abstract: OBJECTIVE
  The prognostic value of late gadolinium enhancement (LGE) for risk stratification of hypertrophic cardiomyopathy (HCM) patients is the subject of disagreement. We set out to examine the association between clinical and morphological variables, risk factors for sudden cardiac death and LGE in HCM patients.
  
  
  METHODS
  From a population of 78 patients with HCM, we studied 53 who underwent cardiac magnetic resonance. They were divided into two groups according to the presence or absence of LGE. Ventricular arrhythmias and morbidity and mortality during follow-up were analyzed.
  
  
  RESULTS
  Patients with LGE were younger at the time of diagnosis (p=0.046) and more often had a family history of sudden death (p=0.008) and known coronary artery disease (p=0.086). On echocardiography they had greater maximum wall thickness (p=0.007) and left atrial area (p=0.037) and volume (p=0.035), and more often presented a restrictive pattern of diastolic dysfunction (p=0.011) with a higher E/É ratio (p=0.003) and left ventricular systolic dysfunction (p=0.038). Cardiac magnetic resonance supported the association between LGE and previous echocardiographic findings: greater left atrial area (p=0.029) and maximum wall thickness (p<0.001) and lower left ventricular ejection fraction (p=0.056). Patients with LGE more often had an implantable cardioverter-defibrillator (ICD) (p=0.015). At follow-up, no differences were found in the frequency of ventricular arrhythmias, appropriate ICD therapies or mortality.
  
  
  CONCLUSIONS
  The presence of LGE emerges as a risk marker, associated with the classical predictors of sudden cardiac death in this population. However, larger studies are required to confirm its independent association with clinical events.
authors.affiliation: Serviço de Cardiologia, Hospital Geral, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. Electronic address: franciscacaetano@sapo.pt.
authors.forename: Francisca
authors.initials: F
authors.lastname: Caetano
authors.affiliation: Serviço de Cardiologia, Hospital Geral, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Ana
authors.initials: A
authors.lastname: Botelho
authors.affiliation: Serviço de Cardiologia, Hospital Geral, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Joana
authors.initials: J
authors.lastname: Trigo
authors.affiliation: Serviço de Cardiologia, Hospital Geral, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Joana
authors.initials: J
authors.lastname: Silva
authors.affiliation: Serviço de Cardiologia, Hospital Geral, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Inês
authors.initials: I
authors.lastname: Almeida
authors.affiliation: Serviço de Genética Médica, Hospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Margarida
authors.initials: M
authors.lastname: Venâncio
authors.affiliation: Serviço de Cardiologia, Hospital Geral, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: João
authors.initials: J
authors.lastname: Pais
authors.affiliation: Serviço de Radiologia, Hospital Geral, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Conceição
authors.initials: C
authors.lastname: Sanches
authors.affiliation: Serviço de Cardiologia, Hospital Geral, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
authors.forename: António
authors.initials: A
authors.lastname: Leitão Marques
mesh_terms: D057791:Cardiac Imaging Techniques / Q000379:methods*; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications / Q000175:diagnosis* / Q000235:genetics*; D003287:Contrast Media; D016757:Death, Sudden, Cardiac / Q000209:etiology; D005260:Female; D005682:Gadolinium; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012189:Retrospective Studies; D012307:Risk Factors
chemical_list: D003287:Contrast Media; D005682:Gadolinium
keywords: Cardiac magnetic resonance; Hypertrophic cardiomyopathy; Late gadolinium enhancement; Miocardiopatia hipertrófica; Prognosis; Prognóstico; Realce tardio; Ressonância magnética cardíaca