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J Am Coll Cardiol, 2002; 40:2156-2164
© 2002 by the American College of Cardiology Foundation
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CLINICAL STUDY

Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy

Lubna Choudhury, MD, MRCP*, Heiko Mahrholdt, MD*, Anja Wagner, MD*, Kelly M. Choi, MD*, Michael D. Elliott, MD*, Francis J. Klocke, MD, MACC*, Robert O. Bonow, MD, FACC*, Robert M. Judd, PhD* and Raymond J. Kim, MD, FACC*,*

* Feinberg Cardiovascular Research Institute, Department of Medicine, Northwestern University, Chicago, Illinois, USA

Manuscript received April 1, 2002; revised manuscript received June 18, 2002, accepted August 26, 2002.

* Reprint requests and correspondence: Dr. Raymond J. Kim, Duke Cardiovascular MRI Center, DUMC-3934, Durham, North Carolina, USA 27710.
Raymond.kim{at}dcmrc.mc.duke.edu

OBJECTIVES: We sought to ascertain whether myocardial scarring occurs in living unselected patients with hypertrophic cardiomyopathy (HCM).

BACKGROUND: Myocardial scarring is known to occur in select HCM patients, who were highly symptomatic prior to death or who died suddenly. The majority of HCM patients, however, are minimally symptomatic and have not suffered sudden death.

METHODS: Cine and gadolinium-enhanced magnetic resonance imaging was performed in 21 HCM patients who were predominantly asymptomatic. Gadolinium hyperenhancement was assumed to represent myocardial scar, and the extent of scar was compared to left ventricular (LV) morphology and function.

RESULTS: Scarring was present in 17 patients (81%). Scarring occurred only in hypertrophied regions (≥10 mm), was patchy with multiple foci, and predominantly involved the middle third of the ventricular wall. All 17 patients had scarring at the junction of the interventricular septum and the right ventricular (RV) free wall. On a regional basis, the extent of scarring correlated positively with wall thickness (r = 0.36, p < 0.0001), and inversely with wall thickening (r = –0.21, p < 0.0001). On a per patient basis, the extent of scarring (mean, 8 ± 9% of LV mass) was minimally related to maximum wall thickness (r = 0.40, p = 0.07) and LV mass (r = 0.33, p = 0.15), and correlated inversely with ejection fraction (r = –0.46, p = 0.04).

CONCLUSIONS: Myocardial scarring is common in asymptomatic or mildly symptomatic HCM patients who have not suffered sudden death. When present, scarring occurs in hypertrophied regions, is consistently localized to the junctions of the septum and RV free wall, and correlates positively with regional hypertrophy and inversely with regional contraction.

Abbreviations and Acronyms
  CAD
  coronary artery disease
  HCM
  hypertrophic cardiomyopathy
  LV
  left ventricular
  LVOT
  left ventricular outflow tract
  MRI
  magnetic resonance imaging
  RV
  right ventricular
  SAM
  systolic anterior motion of the mitral valve




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[Abstract] [Full Text] [PDF]

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G. P. McCann, C. T. Gan, A. M. Beek, H. W. M. Niessen, A. V. Noordegraaf, and A. C. van Rossum
Extent of MRI Delayed Enhancement of Myocardial Mass Is Related to Right Ventricular Dysfunction in Pulmonary Artery Hypertension
Am. J. Roentgenol., February 1, 2007; 188(2): 349 - 355.
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U. S. Valeti, R. A. Nishimura, D. R. Holmes, P. A. Araoz, J. F. Glockner, J. F. Breen, S. R. Ommen, B. J. Gersh, A. J. Tajik, C. S. Rihal, et al.
Comparison of Surgical Septal Myectomy and Alcohol Septal Ablation With Cardiac Magnetic Resonance Imaging in Patients With Hypertrophic Obstructive Cardiomyopathy
J. Am. Coll. Cardiol., January 23, 2007; 49(3): 350 - 357.
[Abstract] [Full Text] [PDF]

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J. Frielingsdorf, M. Genoni, O. M. Hess, and F. A. Flachskampf
Do women have impaired regional systolic function in hypertensive heart disease? A 3-dimensional echocardiography study
Eur Heart J Cardiovasc Imaging, January 1, 2007; 8(1): 42 - 47.
[Abstract] [Full Text] [PDF]

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S. F. Nagueh and J. J. Mahmarian
Noninvasive Cardiac Imaging in Patients With Hypertrophic Cardiomyopathy
J. Am. Coll. Cardiol., December 19, 2006; 48(12): 2410 - 2422.
[Abstract] [Full Text] [PDF]

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C. M. Kramer
The Expanding Prognostic Role of Late Gadolinium Enhanced Cardiac Magnetic Resonance
J. Am. Coll. Cardiol., November 21, 2006; 48(10): 1986 - 1987.
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C. B. Marcu, A. M. Beek, and A. C. van Rossum
Clinical applications of cardiovascular magnetic resonance imaging.
Can. Med. Assoc. J., October 10, 2006; 175(8): 911 - 917.
[Abstract] [Full Text] [PDF]

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K Debl, B Djavidani, S Buchner, C Lipke, W Nitz, S Feuerbach, G Riegger, and A Luchner
Delayed hyperenhancement in magnetic resonance imaging of left ventricular hypertrophy caused by aortic stenosis and hypertrophic cardiomyopathy: visualisation of focal fibrosis
Heart, October 1, 2006; 92(10): 1447 - 1451.
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P. Sipola, K Peuhkurinen, K Lauerma, M Husso, P Jaaskelainen, M Laakso, H J Aronen, J Risteli, and J Kuusisto
Myocardial late gadolinium enhancement is associated with raised serum amino-terminal propeptide of type III collagen concentrations in patients with hypertrophic cardiomyopathy attributable to the Asp175Asn mutation in the {alpha} tropomyosin gene: magnetic resonance imaging study.
Heart, September 1, 2006; 92(9): 1321 - 1322.
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C. A. Dumont, L. Monserrat, R. Soler, E. Rodriguez, X. Fernandez, J. Peteiro, A. Bouzas, B. Bouzas, and A. Castro-Beiras
Interpretation of electrocardiographic abnormalities in hypertrophic cardiomyopathy with cardiac magnetic resonance
Eur. Heart J., July 2, 2006; 27(14): 1725 - 1731.
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I. Klem, J. F. Heitner, D. J. Shah, M. H. Sketch Jr, V. Behar, J. Weinsaft, P. Cawley, M. Parker, M. Elliott, R. M. Judd, et al.
Improved Detection of Coronary Artery Disease by Stress Perfusion Cardiovascular Magnetic Resonance With the Use of Delayed Enhancement Infarction Imaging
J. Am. Coll. Cardiol., April 18, 2006; 47(8): 1630 - 1638.
[Abstract] [Full Text] [PDF]

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E Perugini, C Rapezzi, T Piva, O Leone, L Bacchi-Reggiani, L Riva, F Salvi, L Lovato, A Branzi, and R Fattori
Non-invasive evaluation of the myocardial substrate of cardiac amyloidosis by gadolinium cardiac magnetic resonance
Heart, March 1, 2006; 92(3): 343 - 349.
[Abstract] [Full Text] [PDF]

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S. V. Babu-Narayan, P. J. Kilner, W. Li, J. C. Moon, O. Goktekin, P. A. Davlouros, M. Khan, S. Y. Ho, D. J. Pennell, and M. A. Gatzoulis
Ventricular Fibrosis Suggested by Cardiovascular Magnetic Resonance in Adults With Repaired Tetralogy of Fallot and Its Relationship to Adverse Markers of Clinical Outcome
Circulation, January 24, 2006; 113(3): 405 - 413.
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T. Oosterhof, B. J. M. Mulder, H. W. Vliegen, and A. de Roos
Corrected Tetralogy of Fallot: Delayed Enhancement in Right Ventricular Outflow Tract
Radiology, December 1, 2005; 237(3): 868 - 871.
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B. Schumacher, F. H. Gietzen, H. Neuser, J. Schummelfeder, M. Schneider, S. Kerber, R. Schimpf, C. Wolpert, and M. Borggrefe
Electrophysiological Characteristics of Septal Hypertrophy in Patients With Hypertrophic Obstructive Cardiomyopathy and Moderate to Severe Symptoms
Circulation, October 4, 2005; 112(14): 2096 - 2101.
[Abstract] [Full Text] [PDF]

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K. G. Blyth, B. A. Groenning, T. N. Martin, J. E. Foster, P. B. Mark, H. J. Dargie, and A. J. Peacock
Contrast enhanced-cardiovascular magnetic resonance imaging in patients with pulmonary hypertension
Eur. Heart J., October 1, 2005; 26(19): 1993 - 1999.
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P. Sipola, K. Lauerma, P. Jaaskelainen, M. Laakso, K. Peuhkurinen, H. Manninen, H. J. Aronen, and J. Kuusisto
Cine MR Imaging of Myocardial Contractile Impairment in Patients with Hypertrophic Cardiomyopathy Attributable to Asp175Asn Mutation in the {alpha}-Tropomyosin Gene
Radiology, September 1, 2005; 236(3): 815 - 824.
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C. Rickers, N. M. Wilke, M. Jerosch-Herold, S. A. Casey, P. Panse, N. Panse, J. Weil, A. G. Zenovich, and B. J. Maron
Utility of Cardiac Magnetic Resonance Imaging in the Diagnosis of Hypertrophic Cardiomyopathy
Circulation, August 9, 2005; 112(6): 855 - 861.
[Abstract] [Full Text] [PDF]

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 Eur Heart JHome page
H. Mahrholdt, A. Wagner, R. M. Judd, U. Sechtem, and R. J. Kim
Delayed enhancement cardiovascular magnetic resonance assessment of non-ischaemic cardiomyopathies
Eur. Heart J., August 1, 2005; 26(15): 1461 - 1474.
[Abstract] [Full Text] [PDF]

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J C Moon, J Mogensen, P M Elliott, G C Smith, A G Elkington, S K Prasad, D J Pennell, and W J McKenna
Myocardial late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy caused by mutations in troponin I
Heart, August 1, 2005; 91(8): 1036 - 1040.
[Abstract] [Full Text] [PDF]

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 Am. J. Roentgenol.Home page
Y. Amano, S. Kumita, M. Takayama, and T. Kumazaki
Comparison of Contrast-Enhanced MRI with Iodine-123 BMIPP for Detection of Myocardial Damage in Hypertrophic Cardiomyopathy
Am. J. Roentgenol., August 1, 2005; 185(2): 312 - 318.
[Abstract] [Full Text] [PDF]

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P. Knaapen, W. G. van Dockum, O. Bondarenko, W. E.M. Kok, M. J.W. Gotte, R. Boellaard, A. M. Beek, C. A. Visser, A. C. van Rossum, A. A. Lammertsma, et al.
Delayed Contrast Enhancement and Perfusable Tissue Index in Hypertrophic Cardiomyopathy: Comparison Between Cardiac MRI and PET
J. Nucl. Med., June 1, 2005; 46(6): 923 - 929.
[Abstract] [Full Text] [PDF]

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C. J. Soriano, F. Ridocci, J. Estornell, J. Jimenez, V. Martinez, and J. A. De Velasco
Noninvasive diagnosis of coronary artery disease in patients with heart failure and systolic dysfunction of uncertain etiology, using late gadolinium-enhanced cardiovascular magnetic resonance
J. Am. Coll. Cardiol., March 1, 2005; 45(5): 743 - 748.
[Abstract] [Full Text] [PDF]

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R. R. Edelman
Contrast-enhanced MR Imaging of the Heart: Overview of the Literature
Radiology, September 1, 2004; 232(3): 653 - 668.
[Abstract] [Full Text] [PDF]

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 J Am Coll CardiolHome page
J. C. C. Moon, E. Reed, M. N. Sheppard, A. G. Elkington, S. Ho, M. Burke, M. Petrou, and D. J. Pennell
The histologic basis of late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy
J. Am. Coll. Cardiol., June 16, 2004; 43(12): 2260 - 2264.
[Abstract] [Full Text] [PDF]

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 CirculationHome page
K. Shan, G. Constantine, M. Sivananthan, and S. D. Flamm
Role of Cardiac Magnetic Resonance Imaging in the Assessment of Myocardial Viability
Circulation, March 23, 2004; 109(11): 1328 - 1334.
[Full Text] [PDF]

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 CirculationHome page
H. Mahrholdt, C. Goedecke, A. Wagner, G. Meinhardt, A. Athanasiadis, H. Vogelsberg, P. Fritz, K. Klingel, R. Kandolf, and U. Sechtem
Cardiovascular Magnetic Resonance Assessment of Human Myocarditis: A Comparison to Histology and Molecular Pathology
Circulation, March 16, 2004; 109(10): 1250 - 1258.
[Abstract] [Full Text] [PDF]

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W. G. van Dockum, F. J. ten Cate, J. M. ten Berg, A. M. Beek, J. W. R. Twisk, J. Vos, M. B. M. Hofman, C. A. Visser, and A. C. van Rossum
Myocardial infarction after percutaneous transluminal septal myocardial ablation in hypertrophic obstructive cardiomyopathy: evaluation by contrast-enhanced magnetic resonance imaging
J. Am. Coll. Cardiol., January 7, 2004; 43(1): 27 - 34.
[Abstract] [Full Text] [PDF]

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 J Am Coll CardiolHome page
A. N. DeMaria, O. Ben-Yehuda, D. Berman, G. K. Feld, B. H. Greenberg, J. D. Knoke, K. U. Knowlton, W. Y. W. Lew, and S. Tsimikas
Highlights of the year in JACC 2003
J. Am. Coll. Cardiol., December 17, 2003; 42(12): 2156 - 2166.
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 Eur Heart JHome page
J. C.C. Moon, B. Sachdev, A. G. Elkington, W. J. McKenna, A. Mehta, D. J. Pennell, P. J. Leed, and P. M. Elliott
Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease: Evidence for a disease specific abnormality of the myocardial interstitium
Eur. Heart J., December 1, 2003; 24(23): 2151 - 2155.
[Abstract] [Full Text] [PDF]


 
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