Published online before print November 6, 2006, doi: 10.1161/CIRCULATIONAHA.106.644682
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
(Circulation. 2006;114:2232-2239.)
© 2006 American Heart Association, Inc.
Heart Failure |
Hypertrophic Cardiomyopathy Is Predominantly a Disease of Left Ventricular Outflow Tract Obstruction
From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts-New England Medical Center, Boston, Mass (M.S.M., J.E.U., M.S.L., J.T.K., N.G.P.); Regional Referral Center for Myocardial Diseases, Azienda Ospedaliera Careggi, Florence, Italy (I.O., S.N., F.C.); and Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minn (A.G.Z., B.J.M.).
Correspondence to Martin S. Maron, MD, Tufts-New England Medical Center, #70, 750 Washington St, Boston, MA 02111. E-mail mmaron{at}tufts-nemc.org
Received March 21, 2006; de novo received June 8, 2006; revision received August 22, 2006; accepted August 23, 2006.
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.
Methods and 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 
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 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.
CLINICAL PERSPECTIVE
This article has been cited by other articles:
 |
|
 |
|
  P P Dimitrow, A Undas, M Bober, W Tracz, and J S Dubiel Obstructive hypertrophic cardiomyopathy is associated with enhanced thrombin generation and platelet activation Heart, June 1, 2008; 94(6): e21 - e21. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. K. Kaple, R. T. Murphy, L. M. DiPaola, P. L. Houghtaling, H. M. Lever, B. W. Lytle, E. H. Blackstone, and N. G. Smedira Mitral Valve Abnormalities in Hypertrophic Cardiomyopathy: Echocardiographic Features and Surgical Outcomes Ann. Thorac. Surg., May 1, 2008; 85(5): 1527 - 1535. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. J. Mittnacht, M. Fanshawe, and S. Konstadt Anesthetic Considerations in the Patient With Valvular Heart Disease Undergoing Noncardiac Surgery Seminars in Cardiothoracic and Vascular Anesthesia, March 1, 2008; 12(1): 33 - 59. [Abstract] [PDF]
|
|
|
|
 |
|
 M. Togni, M. Billinger, S. Cook, and O. M. Hess Septal myectomy: cut, coil, or boil? Eur. Heart J., February 1, 2008; 29(3): 296 - 298. [Full Text] [PDF]
|
|
|
|
 |
|
 M. A. Fifer and G. J. Vlahakes Management of Symptoms in Hypertrophic Cardiomyopathy Circulation, January 22, 2008; 117(3): 429 - 439. [Full Text] [PDF]
|
|
|
|
 |
|
 A. J. C. Mittnacht, C. Moung, and W. W. Lai Massive Cardiac Hypertrophy in a Patient with Danon Disease: An Intraoperative Transesophageal Echocardiographic Evaluation Anesth. Analg., October 1, 2007; 105(4): 963 - 965. [Full Text] [PDF]
|
|
|
|
|
|
G. K. Efthimiadis, S. Meditskou, N. E. Mezilis, I. Styliadis, A. Manthos, and G. E. Parcharidis Can septal myectomy prevent sudden cardiac death in hypertrophic obstructive cardiomyopathy? Eur. Heart J., September 1, 2007; 28(17): 2177 - 2177. [Full Text] [PDF]
|
|
|
|
 |
|
 I. Olivotto, S. R. Ommen, M. S. Maron, F. Cecchi, and B. J. Maron Surgical Myectomy Versus Alcohol Septal Ablation for Obstructive Hypertrophic Cardiomyopathy: Will There Ever Be a Randomized Trial? J. Am. Coll. Cardiol., August 28, 2007; 50(9): 831 - 834. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Parodi, S. Del Pace, C. Salvadori, N. Carrabba, I. Olivotto, G. F. Gensini, and for the Tuscany Registry of Tako-Tsubo Cardiomyopa Left Ventricular Apical Ballooning Syndrome as a Novel Cause of Acute Mitral Regurgitation J. Am. Coll. Cardiol., August 14, 2007; 50(7): 647 - 649. [Full Text] [PDF]
|
|
|
|
|
|
B. J. Maron Surgical Myectomy Remains the Primary Treatment Option for Severely Symptomatic Patients With Obstructive Hypertrophic Cardiomyopathy Circulation, July 10, 2007; 116(2): 196 - 206. [Full Text] [PDF]
|
|
|
|
|
|
M. A. Fifer Most Fully Informed Patients Choose Septal Ablation Over Septal Myectomy Circulation, July 10, 2007; 116(2): 207 - 216. [Full Text] [PDF]
|
|
|
|
|
|
C. Geier, A. Perrot, and C. Ozcelik Letter by Geier et al Regarding Article, "Hypertrophic Cardiomyopathy Is Predominantly a Disease of Left Ventricular Outflow Tract Obstruction" Circulation, June 12, 2007; 115(23): e622 - e622. [Full Text] [PDF]
|
|
|
|
|
|
M. S. Maron, M. S. Link, J. E. Udelson, J. T. Kuvin, N. G. Pandian, I. Olivotto, S. Nistri, F. Cecchi, and B. J. Maron Response to Letter Regarding Article, "Hypertrophic Cardiomyopathy Is Predominantly a Disease of Left Ventricular Outflow Tract Obstruction" Circulation, June 12, 2007; 115(23): e623 - e623. [Full Text] [PDF]
|
|
|
|
|
|
B. J. Maron, P. D. Thompson, M. J. Ackerman, G. Balady, S. Berger, D. Cohen, R. Dimeff, P. S. Douglas, D. W. Glover, A. M. Hutter Jr, et al. Recommendations and Considerations Related to Preparticipation Screening for Cardiovascular Abnormalities in Competitive Athletes: 2007 Update: A Scientific Statement From the American Heart Association Council on Nutrition, Physical Activity, and Metabolism: Endorsed by the American College of Cardiology Foundation Circulation, March 27, 2007; 115(12): 1643 - 1655. [Full Text] [PDF]
|
|
|
|
|
|
R. A. Nishimura and S. R. Ommen Hypertrophic Cardiomyopathy: The Search for Obstruction Circulation, November 21, 2006; 114(21): 2200 - 2202. [Full Text] [PDF]
|
|