title: Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction.
abstract: BACKGROUND
  Nonobstructive hypertrophic cardiomyopathy (HCM) has been regarded as the predominant hemodynamic form of the disease on the basis of assessment of outflow gradient under resting conditions. We sought to prospectively define the prevalence, clinical profile, and significance of left ventricular (LV) outflow tract obstruction under resting conditions and with physiological exercise in a large HCM cohort.
  
  
  RESULTS
  We prospectively analyzed 320 consecutive HCM patients (age, 47+/-17 years), measuring LV outflow gradient at rest, with Valsalva maneuver, and with exercise echocardiography. LV outflow obstruction was present at rest and/or with exercise in 225 patients (70%); 119 had rest gradients > or = 50 mm Hg and were not exercised. Of the other 201 patients with gradients < 50 mm Hg at rest (average, 4+/-9 mm Hg), 106 developed mechanical obstruction to LV outflow resulting from mitral valve-septal contact after exercise (80+/-43 mm Hg), including 76 with marked gradients > or = 50 mm Hg and 46 with heart failure symptoms. The remaining 95 patients (30%) had no or small gradients (< 30 mm Hg) both at rest and with exercise. Valsalva maneuver underestimated the presence and magnitude of exercise-induced obstruction.
  
  
  CONCLUSIONS
  Among those patients who come to clinical evaluation, HCM is a predominantly obstructive disease in which LV outflow gradients, frequently associated with heart failure symptoms and often identified only with exercise, are evident in most patients (ie, 70%). Identification of LV outflow obstruction with exercise echocardiography may broaden management options in HCM by identifying symptomatic patients not otherwise regarded as potential candidates for septal reduction therapy. Assessment of subaortic gradients with exercise should be a routine component of the evaluation of HCM patients without outflow obstruction under resting conditions.
authors.affiliation: Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts-New England Medical Center, Boston, MA 02111, USA. mmaron@tufts-nemc.org
authors.forename: Martin S
authors.initials: MS
authors.lastname: Maron
authors.forename: Iacopo
authors.initials: I
authors.lastname: Olivotto
authors.forename: Andrey G
authors.initials: AG
authors.lastname: Zenovich
authors.forename: Mark S
authors.initials: MS
authors.lastname: Link
authors.forename: Natesa G
authors.initials: NG
authors.lastname: Pandian
authors.forename: Jeffery T
authors.initials: JT
authors.lastname: Kuvin
authors.forename: Stefano
authors.initials: S
authors.lastname: Nistri
authors.forename: Franco
authors.initials: F
authors.lastname: Cecchi
authors.forename: James E
authors.initials: JE
authors.lastname: Udelson
authors.forename: Barry J
authors.initials: BJ
authors.lastname: Maron
mesh_terms: D000328:Adult; D000369:Aged, 80 and over; D002303:Cardiac Output, Low / Q000175:diagnosis / Q000209:etiology; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications*; D025401:Echocardiography, Stress; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011446:Prospective Studies; D012720:Severity of Illness Index; D014636:Valsalva Maneuver; D014694:Ventricular Outflow Obstruction / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology / Q000503:physiopathology*