title: Mechanism of Progressive Heart Failure and Significance of Pulmonary Hypertension in Obstructive Hypertrophic Cardiomyopathy.
abstract: BACKGROUND
  There are limited data on the prevalence, pathophysiology, and management implications of pulmonary hypertension in patients with obstructive hypertrophic cardiomyopathy and advanced heart failure.
  
  
  RESULTS
  To assess the clinical significance of measured cardiopulmonary hemodynamics in hypertrophic cardiomyopathy patients with heart failure, we retrospectively assessed right heart catheterization data in 162 consecutive patients with outflow tract gradients (median [interquartile range], 90 mm Hg [70-110 mm Hg]), 59±11 years old, and 49% men, predominately New York Heart Association class III/IV status. Pulmonary hypertension (mean pulmonary artery pressure, ≥25 mm Hg) was present in 82 patients (51%), including 29 (18%) regarded as moderate-severe (mean pulmonary artery pressure, ≥35 mm Hg) and 28 (34%) also had increased pulmonary vascular resistance >3.0 WU. The pulmonary artery wedge pressure was ≤15 mm Hg in 54%, indicating that left atrial hypertension was absent in a majority of patients. Notably, 9 patients (11%) met hemodynamic criteria for precapillary pulmonary hypertension (mean pulmonary artery pressure, ≥25 mm Hg; pulmonary vascular resistance, >3.0 WU; pulmonary artery wedge pressure, ≤15 mm Hg). Over a median follow-up of 327 days (90-743 days) after surgical myectomy (or alcohol septal ablation), 92% and 95% of patients with or without preoperative pulmonary hypertension, respectively, were asymptomatic or mildly symptomatic. One postoperative death occurred in a 59-year-old woman with acute respiratory failure and mean pulmonary artery pressure of 65 mm Hg.
  
  
  CONCLUSIONS
  Pulmonary hypertension was common in obstructive hypertrophic cardiomyopathy patients with advanced heart failure. Although possibly a contributor to preoperative heart failure, pulmonary hypertension did not significantly influence clinical and surgical outcome. Notably, a novel patient subgroup was identified with resting invasive hemodynamics consistent with pulmonary vascular disease.
authors.affiliation: From the Division of Cardiology, Hypertrophic Cardiomyopathy Institute (M.C., E.J.R., B.J.M., M.S.M.) and Division of Pulmonary, Critical Care and Sleep Medicine (N.S.H., I.R.P.), Tufts Medical Center, Boston, MA; Division of Internal Medicine, Department of Medical Sciences, University of Torino, Italy (M.C., A.M.); Department of Pediatric Cardiology, Boston Children's Hospital, MA (A.R.O.); Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA (B.A.M.); and Department of Cardiology, Boston VA Healthcare System, MA (B.A.M.).
authors.forename: Michele
authors.initials: M
authors.lastname: Covella
authors.affiliation: From the Division of Cardiology, Hypertrophic Cardiomyopathy Institute (M.C., E.J.R., B.J.M., M.S.M.) and Division of Pulmonary, Critical Care and Sleep Medicine (N.S.H., I.R.P.), Tufts Medical Center, Boston, MA; Division of Internal Medicine, Department of Medical Sciences, University of Torino, Italy (M.C., A.M.); Department of Pediatric Cardiology, Boston Children's Hospital, MA (A.R.O.); Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA (B.A.M.); and Department of Cardiology, Boston VA Healthcare System, MA (B.A.M.).
authors.forename: Ethan J
authors.initials: EJ
authors.lastname: Rowin
authors.affiliation: From the Division of Cardiology, Hypertrophic Cardiomyopathy Institute (M.C., E.J.R., B.J.M., M.S.M.) and Division of Pulmonary, Critical Care and Sleep Medicine (N.S.H., I.R.P.), Tufts Medical Center, Boston, MA; Division of Internal Medicine, Department of Medical Sciences, University of Torino, Italy (M.C., A.M.); Department of Pediatric Cardiology, Boston Children's Hospital, MA (A.R.O.); Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA (B.A.M.); and Department of Cardiology, Boston VA Healthcare System, MA (B.A.M.).
authors.forename: Nicholas S
authors.initials: NS
authors.lastname: Hill
authors.affiliation: From the Division of Cardiology, Hypertrophic Cardiomyopathy Institute (M.C., E.J.R., B.J.M., M.S.M.) and Division of Pulmonary, Critical Care and Sleep Medicine (N.S.H., I.R.P.), Tufts Medical Center, Boston, MA; Division of Internal Medicine, Department of Medical Sciences, University of Torino, Italy (M.C., A.M.); Department of Pediatric Cardiology, Boston Children's Hospital, MA (A.R.O.); Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA (B.A.M.); and Department of Cardiology, Boston VA Healthcare System, MA (B.A.M.).
authors.forename: Ioana R
authors.initials: IR
authors.lastname: Preston
authors.affiliation: From the Division of Cardiology, Hypertrophic Cardiomyopathy Institute (M.C., E.J.R., B.J.M., M.S.M.) and Division of Pulmonary, Critical Care and Sleep Medicine (N.S.H., I.R.P.), Tufts Medical Center, Boston, MA; Division of Internal Medicine, Department of Medical Sciences, University of Torino, Italy (M.C., A.M.); Department of Pediatric Cardiology, Boston Children's Hospital, MA (A.R.O.); Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA (B.A.M.); and Department of Cardiology, Boston VA Healthcare System, MA (B.A.M.).
authors.forename: Alberto
authors.initials: A
authors.lastname: Milan
authors.affiliation: From the Division of Cardiology, Hypertrophic Cardiomyopathy Institute (M.C., E.J.R., B.J.M., M.S.M.) and Division of Pulmonary, Critical Care and Sleep Medicine (N.S.H., I.R.P.), Tufts Medical Center, Boston, MA; Division of Internal Medicine, Department of Medical Sciences, University of Torino, Italy (M.C., A.M.); Department of Pediatric Cardiology, Boston Children's Hospital, MA (A.R.O.); Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA (B.A.M.); and Department of Cardiology, Boston VA Healthcare System, MA (B.A.M.).
authors.forename: Alexander R
authors.initials: AR
authors.lastname: Opotowsky
authors.affiliation: From the Division of Cardiology, Hypertrophic Cardiomyopathy Institute (M.C., E.J.R., B.J.M., M.S.M.) and Division of Pulmonary, Critical Care and Sleep Medicine (N.S.H., I.R.P.), Tufts Medical Center, Boston, MA; Division of Internal Medicine, Department of Medical Sciences, University of Torino, Italy (M.C., A.M.); Department of Pediatric Cardiology, Boston Children's Hospital, MA (A.R.O.); Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA (B.A.M.); and Department of Cardiology, Boston VA Healthcare System, MA (B.A.M.).
authors.forename: Barry J
authors.initials: BJ
authors.lastname: Maron
authors.affiliation: From the Division of Cardiology, Hypertrophic Cardiomyopathy Institute (M.C., E.J.R., B.J.M., M.S.M.) and Division of Pulmonary, Critical Care and Sleep Medicine (N.S.H., I.R.P.), Tufts Medical Center, Boston, MA; Division of Internal Medicine, Department of Medical Sciences, University of Torino, Italy (M.C., A.M.); Department of Pediatric Cardiology, Boston Children's Hospital, MA (A.R.O.); Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA (B.A.M.); and Department of Cardiology, Boston VA Healthcare System, MA (B.A.M.).
authors.forename: Martin S
authors.initials: MS
authors.lastname: Maron
authors.affiliation: From the Division of Cardiology, Hypertrophic Cardiomyopathy Institute (M.C., E.J.R., B.J.M., M.S.M.) and Division of Pulmonary, Critical Care and Sleep Medicine (N.S.H., I.R.P.), Tufts Medical Center, Boston, MA; Division of Internal Medicine, Department of Medical Sciences, University of Torino, Italy (M.C., A.M.); Department of Pediatric Cardiology, Boston Children's Hospital, MA (A.R.O.); Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA (B.A.M.); and Department of Cardiology, Boston VA Healthcare System, MA (B.A.M.). bmaron@partners.org.
authors.forename: Bradley A
authors.initials: BA
authors.lastname: Maron
mesh_terms: D055011:Ablation Techniques; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D006328:Cardiac Catheterization; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology* / Q000601:surgery; D015150:Echocardiography, Doppler; D000431:Ethanol / Q000008:administration & dosage; D005260:Female; D006333:Heart Failure / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology*; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D011651:Pulmonary Artery / Q000503:physiopathology*; D011669:Pulmonary Wedge Pressure; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D016896:Treatment Outcome; D014655:Vascular Resistance
chemical_list: D000431:Ethanol
keywords: cardiomyopathy, hypertrophic; hypertension, pulmonary; pulmonary artery; pulmonary heart disease