This Article Figures Only Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Web of Science (3) Citing Articles via Google Scholar Google Scholar Articles by Maron, M. S. Articles by Camici, P. G. Search for Related Content PubMed PubMed Citation Articles by Maron, M. S. Articles by Camici, P. G. Related Collections Related Article

VIEWPOINT: CARDIOMYOPATHY

The Case for Myocardial Ischemia in Hypertrophic Cardiomyopathy

Martin S. Maron, MD^_*,_*, Iacopo Olivotto, MD, Barry J. Maron, MD, Sanjay K. Prasad, MD, Franco Cecchi, MD, James E. Udelson, MD^_* and Paolo G. Camici, MD^||

^_* Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, Massachusetts
Regional Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy
Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota
Center for Advanced MR in Cardiology and Department of Cardiology, Royal Brompton Hospital, London, United Kingdom
^|| Medical Research Council Clinical Sciences Centre and National Heart and Lung Institute, Imperial College, London, United Kingdom

Manuscript received February 25, 2009; revised manuscript received April 20, 2009, accepted April 21, 2009.

^_* Reprint requests and correspondence: Dr. Martin S. Maron, Tufts Medical Center, No. 70, 800 Washington Street, Boston, Massachusetts 02111 (Email: mmaron{at}tuftsmedicalcenter.org).

Since its original description 50 years ago, myocardial ischemia has been a recognized but underappreciated aspect of the pathophysiology of hypertrophic cardiomyopathy (HCM). Nevertheless, the assessment of myocardial ischemia is still not part of routine clinical diagnostic or management strategies. Morphologic abnormalities of the intramural coronary arterioles represent the primary morphologic substrate for microvascular dysfunction and its functional consequence—that is, blunted myocardial blood flow (MBF) during stress. Recently, a number of studies using contemporary cardiovascular imaging modalities such as positron emission tomography (PET) and cardiovascular magnetic resonance (CMR) have led to an enhanced understanding of the role that myocardial ischemia and its sequelae fibrosis play on clinical outcome. In this regard, studies with PET have shown that HCM patients have impaired MBF after dipyridamole infusion and that this blunted MBF is a powerful independent predictor of cardiovascular mortality and adverse LV remodeling associated with LV systolic dysfunction. Stress CMR with late gadolinium enhancement (LGE) has also shown that MBF is reduced in relation to magnitude of wall thickness and in those LV segments occupied by LGE (i.e., fibrosis). These CMR observations show an association between ischemia, myocardial fibrosis, and LV remodeling, providing support that abnormal MBF caused by microvascular dysfunction is responsible for myocardial ischemia-mediated myocyte death, and ultimately replacement fibrosis. Efforts should now focus on detecting myocardial ischemia before adverse LV remodeling begins, so that interventional treatment strategies can be initiated earlier in the clinical course to mitigate ischemia and beneficially alter the natural history of HCM.

Key Words: hypertrophic cardiomyopathy • myocardial ischemia • fibrosis • MRI • PET

Abbreviations and Acronyms   CMR = cardiovascular magnetic resonance   HCM = hypertrophic cardiomyopathy   ICD = implantable cardioverter-defibrillator   LGE = late gadolinium enhancement   LV = left ventricular   MBF = myocardial blood flow   PET = positron emission tomography   SPECT = single-photon emission computed tomography

Related Article

Inside This Issue
J. Am. Coll. Cardiol. 2009 54: A28. [Full Text] [PDF]

This article has been cited by other articles:

				B. J. Gersh, B. J. Maron, R. O. Bonow, J. A. Dearani, M. A. Fifer, M. S. Link, S. S. Naidu, R. A. Nishimura, S. R. Ommen, H. Rakowski, et al. 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons J. Am. Coll. Cardiol., December 13, 2011; 58(25): e212 - e260. [Full Text] [PDF]

				Writing Committee Members, B. J. Gersh, B. J. Maron, R. O. Bonow, J. A. Dearani, M. A. Fifer, M. S. Link, S. S. Naidu, R. A. Nishimura, S. R. Ommen, et al. 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Circulation, December 13, 2011; 124(24): e783 - e831. [Full Text] [PDF]

				Writing Committee Members:*, B. J. Gersh, B. J. Maron, R. O. Bonow, J. A. Dearani, M. A. Fifer, M. S. Link, S. S. Naidu, R. A. Nishimura, S. R. Ommen, et al. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines J. Thorac. Cardiovasc. Surg., December 1, 2011; 142(6): e153 - e203. [Full Text] [PDF]

				I. Olivotto, F. Girolami, R. Sciagra, M. J. Ackerman, B. Sotgia, J. M. Bos, S. Nistri, A. Sgalambro, C. Grifoni, F. Torricelli, et al. Microvascular Function Is Selectively Impaired in Patients With Hypertrophic Cardiomyopathy and Sarcomere Myofilament Gene Mutations J. Am. Coll. Cardiol., August 16, 2011; 58(8): 839 - 848. [Abstract] [Full Text] [PDF]

				S. A. J. Timmer, P. Knaapen, T. Germans, P. A. Dijkmans, M. Lubberink, J. M. ten Berg, F. J. ten Cate, I. K. Russel, M. J. W. Gotte, A. A. Lammertsma, et al. Effects of alcohol septal ablation on coronary microvascular function and myocardial energetics in hypertrophic obstructive cardiomyopathy Am J Physiol Heart Circ Physiol, July 1, 2011; 301(1): H129 - H137. [Abstract] [Full Text] [PDF]

				P. G. Camici Advances in SPECT and PET for the management of heart failure Heart, December 1, 2010; 96(23): 1932 - 1937. [Full Text] [PDF]

				T. Force, R. O. Bonow, S. R. Houser, R. J. Solaro, R. E. Hershberger, B. Adhikari, M. E. Anderson, R. Boineau, B. J. Byrne, T. P. Cappola, et al. Research Priorities in Hypertrophic Cardiomyopathy: Report of a Working Group of the National Heart, Lung, and Blood Institute Circulation, September 14, 2010; 122(11): 1130 - 1133. [Full Text] [PDF]

				T. Tarumi, S. Takebayashi, M. Fujita, T. Nakano, M. Ito, and T. Yamakado Pacing tachycardia exaggerates left ventricular diastolic dysfunction but not systolic function and regional asynergy or asynchrony in patients with hypertrophic cardiomyopathy Europace, September 1, 2010; 12(9): 1308 - 1315. [Abstract] [Full Text] [PDF]

				B. J. Maron Contemporary Insights and Strategies for Risk Stratification and Prevention of Sudden Death in Hypertrophic Cardiomyopathy Circulation, January 26, 2010; 121(3): 445 - 456. [Full Text] [PDF]