title: Prevalence and Impact of Coexistent Bicuspid Aortic Valve in Hypertrophic Cardiomyopathy.
abstract: BACKGROUND
  The association between bicuspid aortic valve (BAV) and hypertrophic cardiomyopathy (HCM) has been reported but its true prevalence is unknown. This study investigated the prevalence and clinical impact of coexistent BAV in a large referral HCM population.
  
  
  METHODS
  Retrospective analysis of 3765 echocardiograms between 2004 and 2014 in 2640 consecutive patients with HCM was performed to assess for BAV. Patients with coexistent conditions were studied.
  
  
  RESULTS
  Twenty-three patients (0.9%) were identified with coexisting BAV and HCM. Mean age was 52±16years, 18 males (78%), 16 with NYHA functional class I/II at initial evaluation (70%). A family history of HCM was present in five patients (22%); none had a family history of BAV or aortopathy. Maximal left ventricular wall thickness was 24±6mm; the majority had either reverse curve or sigmoid septal morphology. Moderate or greater aortic valve dysfunction was present in seven patients (30%), BAV-related aortopathy in 18 patients (78%) and dynamic left ventricular outflow tract (LVOT) obstruction in nine patients (39%). Three patients had combined LVOT obstruction and aortic stenosis. Median time from diagnosis of BAV or HCM to last follow-up was 11±12.5years. At last follow-up, 22% had undergone BAV-related surgeries, 30% had septal reduction therapy (SRT), and 17% had combined SRT and BAV-related surgeries. Overall survival was 95% at 10 years.
  
  
  CONCLUSIONS
  This study reported a 0.9% prevalence of BAV among HCM population, similar to the general population. Aortopathy and LVOT obstruction were common, necessitating cardiac interventions in over one-third of cases. Long-term survival appeared favourable.
authors.affiliation: Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Ratnasari
authors.initials: R
authors.lastname: Padang
authors.affiliation: Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Bernard J
authors.initials: BJ
authors.lastname: Gersh
authors.affiliation: Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Steve R
authors.initials: SR
authors.lastname: Ommen
authors.affiliation: Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA. Electronic address: Geske.Jeffrey@mayo.edu.
authors.forename: Jeffrey B
authors.initials: JB
authors.lastname: Geske
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001021:Aortic Valve / Q000002:abnormalities* / Q000503:physiopathology; D000082882:Bicuspid Aortic Valve Disease; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology; D015897:Comorbidity / Q000639:trends; D004452:Echocardiography; D005260:Female; D006349:Heart Valve Diseases / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D014481:United States / Q000453:epidemiology; D055815:Young Adult
keywords: Bicuspid aortic valve; Hypertrophic cardiomyopathy; Outcome; Prevalence