title: Pulmonary hypertension is associated with worse survival in hypertrophic cardiomyopathy.
abstract: OBJECTIVE
  Pulmonary hypertension (PH) is associated with increased mortality in various forms of left-sided heart disease. However, the implications of PH in hypertrophic cardiomyopathy (HCM) have not been elucidated. The objective of this study was to determine the prevalence and prognostic implications of PH in HCM.
  
  
  RESULTS
  The study cohort consisted of 1570 (54 ± 15 years; 53% male) adults with HCM followed for a median of 3.3 years. PH [pulmonary artery systolic pressure (PASP) >36 mmHg] was identified in 38% of patients who were older (57 ± 15 vs. 52 ± 15 years, P < 0.0001), more likely to be female (59 vs. 40%, P < 0.0001), and were characterized by a higher prevalence of New York Heart Association (NYHA) class 3 or 4 symptoms (61 vs. 45%, P < 0.0001) and atrial fibrillation (29 vs. 15%, P < 0.0001) vs. those without PH. Only 12% had moderate or severe PH (PASP >50 mmHg). In multivariate Cox regression analyses adjusted for age, sex, NYHA class 3 or 4 symptoms, and atrial fibrillation, PASP was an independent predictor of all-cause mortality in patients with non-obstructive HCM (HR 1.59 per 10 mmHg PASP increase, 95% CI 1.28-1.96, P < 0.0001) and in those with obstructive physiology who did not undergo septal reduction therapy (SRT) (HR 1.15 per 10 mmHg PASP, 95% CI 1.01-1.31, P = 0.035). However, PH was not predictive of outcomes in patients with obstructive HCM who underwent SRT.
  
  
  CONCLUSIONS
  Over one-third of adults evaluated in our referral HCM clinic demonstrated concomitant PH, and this was associated with increased mortality except in those with obstructive HCM who underwent SRT.
authors.affiliation: Division of Cardiovascular Diseases and Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA gersh.bernard@mayo.edu.
authors.forename: Kevin C
authors.initials: KC
authors.lastname: Ong
authors.affiliation: Division of Cardiovascular Diseases and Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Jeffrey B
authors.initials: JB
authors.lastname: Geske
authors.affiliation: Division of Cardiovascular Diseases and Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Virginia B
authors.initials: VB
authors.lastname: Hebl
authors.affiliation: Division of Cardiovascular Diseases and Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Rick A
authors.initials: RA
authors.lastname: Nishimura
authors.affiliation: Division of Cardiovascular Diseases and Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Hartzell V
authors.initials: HV
authors.lastname: Schaff
authors.affiliation: Division of Cardiovascular Diseases and Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Ackerman
authors.affiliation: Division of Cardiovascular Diseases and Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Kyle W
authors.initials: KW
authors.lastname: Klarich
authors.affiliation: Division of Cardiovascular Diseases and Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Konstantinos C
authors.initials: KC
authors.lastname: Siontis
authors.affiliation: Division of Cardiovascular Diseases and Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Thais
authors.initials: T
authors.lastname: Coutinho
authors.affiliation: Division of Cardiovascular Diseases and Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Dearani
authors.affiliation: Division of Cardiovascular Diseases and Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Steve R
authors.initials: SR
authors.lastname: Ommen
authors.affiliation: Division of Cardiovascular Diseases and Cardiovascular Surgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Bernard J
authors.initials: BJ
authors.lastname: Gersh
mesh_terms: D000328:Adult; D000368:Aged; D006348:Cardiac Surgical Procedures / Q000379:methods; D002312:Cardiomyopathy, Hypertrophic / Q000000981:diagnostic imaging* / Q000453:epidemiology* / Q000601:surgery; D002423:Cause of Death; D015331:Cohort Studies; D015897:Comorbidity; D004452:Echocardiography; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000453:epidemiology* / Q000601:surgery; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D011669:Pulmonary Wedge Pressure; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D016019:Survival Analysis
keywords: echocardiography; hypertrophic cardiomyopathy; pulmonary hypertension; surgery; survival