title: Advanced heart failure with preserved systolic function in nonobstructive hypertrophic cardiomyopathy: under-recognized subset of candidates for heart transplant.
abstract: BACKGROUND
  In hypertrophic cardiomyopathy (HCM), heart transplant has been predominantly confined to patients with systolic dysfunction. An underappreciated HCM subset comprises patients with preserved left ventricular (LV) systolic function who may also require consideration for transplantation. Therefore, we sought to define the clinical profile and occurrence of advanced heart failure among patients with nonobstructive HCM and preserved systolic function.
  
  
  RESULTS
  Databases from 2 referral centers comprising 2100 HCM patients were interrogated. Forty-six nonobstructive HCM patients (2.2%) either received or were listed for heart transplant, including 20 with normal systolic function (ejection fraction ≥50%). At transplant listing, these 20 patients were 42±13 years old, each in New York Heart Association functional class III/IV with ejection fraction of 62±7%. LV was hypertrophied with maximum wall thickness of 22±4 mm and nondilated (end-diastolic dimension, 39±7 mm). Cardiovascular magnetic resonance in 10 (of 15) patients showed no or minimal fibrosis (≤5% LV mass). Elevated LV end-diastolic or pulmonary capillary wedge pressure, consistent with diastolic dysfunction, was present in 15 patients (75%). LV filling was impaired by echocardiographic measures in all patients, including a restrictive inflow pattern in 8 (40%). In 2 patients, traditional criteria for transplant were absent, including peak VO2 >14 mL/kg/min. Heart transplantation was performed in 12 patients with each alive and without cardiovascular symptoms, 2.3±1.7 years later.
  
  
  CONCLUSIONS
  A previously under-recognized segment of the broad HCM clinical spectrum consists of nonobstructive patients with advanced heart failure, in the presence of preserved systolic function, for whom heart transplant is the sole definitive therapeutic option.
authors.affiliation: From the Department of Medicine, Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (E.J.R., M.S.K., J.E.U., D.D., M.S.M.); Department of Medicine, the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M., S.A.C., D.S.F., K.M.H., K.M.H.); Departments of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Boston, MA (R.H.C.); and Department of Pathology and Medicine, Baylor University Medical Center, Dallas, TX (W.C.R.).
authors.forename: Ethan J
authors.initials: EJ
authors.lastname: Rowin
authors.affiliation: From the Department of Medicine, Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (E.J.R., M.S.K., J.E.U., D.D., M.S.M.); Department of Medicine, the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M., S.A.C., D.S.F., K.M.H., K.M.H.); Departments of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Boston, MA (R.H.C.); and Department of Pathology and Medicine, Baylor University Medical Center, Dallas, TX (W.C.R.).
authors.forename: Barry J
authors.initials: BJ
authors.lastname: Maron
authors.affiliation: From the Department of Medicine, Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (E.J.R., M.S.K., J.E.U., D.D., M.S.M.); Department of Medicine, the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M., S.A.C., D.S.F., K.M.H., K.M.H.); Departments of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Boston, MA (R.H.C.); and Department of Pathology and Medicine, Baylor University Medical Center, Dallas, TX (W.C.R.).
authors.forename: Michael S
authors.initials: MS
authors.lastname: Kiernan
authors.affiliation: From the Department of Medicine, Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (E.J.R., M.S.K., J.E.U., D.D., M.S.M.); Department of Medicine, the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M., S.A.C., D.S.F., K.M.H., K.M.H.); Departments of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Boston, MA (R.H.C.); and Department of Pathology and Medicine, Baylor University Medical Center, Dallas, TX (W.C.R.).
authors.forename: Susan A
authors.initials: SA
authors.lastname: Casey
authors.affiliation: From the Department of Medicine, Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (E.J.R., M.S.K., J.E.U., D.D., M.S.M.); Department of Medicine, the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M., S.A.C., D.S.F., K.M.H., K.M.H.); Departments of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Boston, MA (R.H.C.); and Department of Pathology and Medicine, Baylor University Medical Center, Dallas, TX (W.C.R.).
authors.forename: David S
authors.initials: DS
authors.lastname: Feldman
authors.affiliation: From the Department of Medicine, Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (E.J.R., M.S.K., J.E.U., D.D., M.S.M.); Department of Medicine, the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M., S.A.C., D.S.F., K.M.H., K.M.H.); Departments of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Boston, MA (R.H.C.); and Department of Pathology and Medicine, Baylor University Medical Center, Dallas, TX (W.C.R.).
authors.forename: Katarzyna M
authors.initials: KM
authors.lastname: Hryniewicz
authors.affiliation: From the Department of Medicine, Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (E.J.R., M.S.K., J.E.U., D.D., M.S.M.); Department of Medicine, the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M., S.A.C., D.S.F., K.M.H., K.M.H.); Departments of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Boston, MA (R.H.C.); and Department of Pathology and Medicine, Baylor University Medical Center, Dallas, TX (W.C.R.).
authors.forename: Raymond H
authors.initials: RH
authors.lastname: Chan
authors.affiliation: From the Department of Medicine, Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (E.J.R., M.S.K., J.E.U., D.D., M.S.M.); Department of Medicine, the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M., S.A.C., D.S.F., K.M.H., K.M.H.); Departments of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Boston, MA (R.H.C.); and Department of Pathology and Medicine, Baylor University Medical Center, Dallas, TX (W.C.R.).
authors.forename: Kevin M
authors.initials: KM
authors.lastname: Harris
authors.affiliation: From the Department of Medicine, Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (E.J.R., M.S.K., J.E.U., D.D., M.S.M.); Department of Medicine, the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M., S.A.C., D.S.F., K.M.H., K.M.H.); Departments of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Boston, MA (R.H.C.); and Department of Pathology and Medicine, Baylor University Medical Center, Dallas, TX (W.C.R.).
authors.forename: James E
authors.initials: JE
authors.lastname: Udelson
authors.affiliation: From the Department of Medicine, Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (E.J.R., M.S.K., J.E.U., D.D., M.S.M.); Department of Medicine, the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M., S.A.C., D.S.F., K.M.H., K.M.H.); Departments of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Boston, MA (R.H.C.); and Department of Pathology and Medicine, Baylor University Medical Center, Dallas, TX (W.C.R.).
authors.forename: David
authors.initials: D
authors.lastname: DeNofrio
authors.affiliation: From the Department of Medicine, Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (E.J.R., M.S.K., J.E.U., D.D., M.S.M.); Department of Medicine, the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M., S.A.C., D.S.F., K.M.H., K.M.H.); Departments of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Boston, MA (R.H.C.); and Department of Pathology and Medicine, Baylor University Medical Center, Dallas, TX (W.C.R.).
authors.forename: William C
authors.initials: WC
authors.lastname: Roberts
authors.affiliation: From the Department of Medicine, Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (E.J.R., M.S.K., J.E.U., D.D., M.S.M.); Department of Medicine, the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, MN (B.J.M., S.A.C., D.S.F., K.M.H., K.M.H.); Departments of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Boston, MA (R.H.C.); and Department of Pathology and Medicine, Baylor University Medical Center, Dallas, TX (W.C.R.). mmaron@tuftsmedialcenter.org.
authors.forename: Martin S
authors.initials: MS
authors.lastname: Maron
mesh_terms: D000293:Adolescent; D000328:Adult; D002312:Cardiomyopathy, Hypertrophic / Q000453:epidemiology*; D002648:Child; D005260:Female; D006333:Heart Failure / Q000453:epidemiology*; D016027:Heart Transplantation; D006801:Humans; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D011669:Pulmonary Wedge Pressure; D013599:Systole; D016277:Ventricular Function, Left; D055815:Young Adult
keywords: diastolic dysfunction; heart transplantation; hypertrophic cardiomyopathy