Hypertrophic cardiomyopathy MRI

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Editors-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]

Overview

Late myocardial enhancement has been associated with myocardial fibrosis and may allow for earlier detection of hypertrophic cardiomyopathy (HCM) than is currently available with echocardiography and ECG. MRI is helpful in visualizing the asymmetric thickening of the interventricular septum in patients with HCM. However, it may be more helpful than other forms of imaging to differentiate the variant types of hypertrophic cardiomyopathy. MRI can be helpful in evaluating the extent of systolic anterior motion of the mitral valve. MRI can help visualize turbulence in the left ventricular outflow tract created by an obstruction in patients with obstructive hypertrophic cardiomyopathy.

Cardiac MRI

Late myocardial enhancement

Late myocardial enhancement has been associated with myocardial fibrosis and may allow for earlier detection of hypertrophic cardiomyopathy than is currently available with echocardiography and ECG.

  • It has been shown that in patients with previously diagnosed hypertrophic cardiomyopathy[1]:
  • Moon et al looked at whether the extent of hyperenhancement on MRI in patients with HCM is associated with an elevated risk of heart failure and sudden death [2] The study involved 53 patient were selected for the presence or absence of an increased clinical risk of sudden death and/or progressive adverse left ventricular remodeling.
  • Myocardial hyperenhancement was present in 79% of patients.
  • They found no evidence of abnormal myocardium on non-contrast images.
  • There was more hyperenhancement in patients with the progressive disease than without.
  • There was greater hyperenhancement in patients with ≥ 2 risk factors for sudden death.
  • Patients with diffuse hyperenhancement had ≥ 2 risk factors for sudden death vs patients with confluent hyperenhancement.

Of note, other investigators have discovered that in carriers without signs of hypertrophy on EKG or echocardiography, Cardiac MR can detect the presence of crypts in the LV wall which may progress to hypertrophy.

Left Ventricular Hypertrophy

MRI is helpful in visualizing the asymmetric thickening of the interventricular septum in patients with HCM. However, it may be more helpful than other forms of imaging to differentiate the variant types of hypertrophic cardiomyopathy.[3]

Systolic Anterior Motion

MRI can be helpful in evaluating the extent of systolic anterior motion of the mitral valve.

Obstruction

MRI can help visualize turbulence in the left ventricular outflow tract created by an obstruction in patients with obstructive hypertrophic cardiomyopathy.

ACC/AHA Guidelines- ACCF/ACR/AHA/NASCI/SCMR 2010 Expert Consensus Document on Cardiovascular Magnetic Resonance (DO NOT EDIT)[4]

CMR may be used for assessment of patients with LV dysfunction or hypertrophy or suspected forms of cardiac injury not related to ischemic heart disease. When the diagnosis is unclear, CMR may be considered to identify the etiology of cardiac dysfunction in patients presenting with heart failure, including

  • Evaluation of dilated cardiomyopathy in the setting of normal coronary arteries,
  • Patients with positive cardiac enzymes without obstructive atherosclerosis on angiography,
  • Patients suspected of amyloidosis or other infiltrative diseases,
  • Hypertrophic cardiomyopathy,
  • Arrhythmogenic right ventricular dysplasia, or
  • Syncope or ventricular arrhythmia.

2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy (DO NOT EDIT)[5]

Cardiac Magnetic Resonance (DO NOT EDIT)[5]

Class I
"1. CMR imaging is indicated in patients with suspected HCM when echocardiography is inconclusive for diagnosis.[6][7] (Level of Evidence: B) "
"2. CMR imaging is indicated in patients with known HCM when additional information that may have an impact on management or decision making regarding invasive management, such as magnitude and distribution of hypertrophy or anatomy of the mitral valve apparatus or papillary muscles, is not adequately defined with echocardiography.[6][7][8][9][10] (Level of Evidence: B) "
Class IIa
"1. CMR imaging is reasonable in patients with HCM to define apical hypertrophy and/or aneurysm if echocardiography is inconclusive.[6][8] (Level of Evidence: B) "
Class IIb
"1. In selected patients with known HCM, when SCD risk stratification is inconclusive after documentation of the conventional risk factors, CMR imaging with assessment of late gadolinium enhancement (LGE) may be considered in resolving clinical decision making.[11][12][13][14][15] (Level of Evidence: C) "
"2. CMR imaging may be considered in patients with LV hypertrophy and the suspicion of alternative diagnoses to HCM, including cardiac amyloidosis, Fabry disease, and genetic phenocopies such as LAMP2 cardiomyopathy.[16][17][18] (Level of Evidence: C) "

References

  1. Choudhury et al. Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy. J. Am Coll Card. 2002; 40: 2156.
  2. Moon et al., Toward Clinical Risk Assessment in Hypertrophic Cardiomyopathy with Gadolinium Cardiovascular Magnetic Resonance. J Am Coll Card. 2003; 41; 1561.
  3. Germans, T et al. Structural Abnormalities of the inferoseptal left ventricular wall detected by Cardiac Magnetic Resonance Imaging in carriers of Hypertrophic Cardiomyopathy mutations. J Am Coll Cardiol. 2006: 48; 2518.
  4. American College of Cardiology Foundation Task Force on Expert Consensus Documents. Hundley WG, Bluemke DA, Finn JP, Flamm SD, Fogel MA; et al. (2010). "ACCF/ACR/AHA/NASCI/SCMR 2010 expert consensus document on cardiovascular magnetic resonance: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents". Circulation. 121 (22): 2462–508. doi:10.1161/CIR.0b013e3181d44a8f. PMC 3034132. PMID 20479157.
  5. 5.0 5.1 Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW (2011). "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". Journal of the American College of Cardiology. 58 (25): e212–60. doi:10.1016/j.jacc.2011.06.011. PMID 22075469. Retrieved 2011-12-19. Unknown parameter |month= ignored (help)
  6. 6.0 6.1 6.2 Moon JC, Fisher NG, McKenna WJ, Pennell DJ (2004). "Detection of apical hypertrophic cardiomyopathy by cardiovascular magnetic resonance in patients with non-diagnostic echocardiography". Heart (British Cardiac Society). 90 (6): 645–9. PMC 1768283. PMID 15145868. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)
  7. 7.0 7.1 Rickers C, Wilke NM, Jerosch-Herold M, Casey SA, Panse P, Panse N, Weil J, Zenovich AG, Maron BJ (2005). "Utility of cardiac magnetic resonance imaging in the diagnosis of hypertrophic cardiomyopathy". Circulation. 112 (6): 855–61. doi:10.1161/CIRCULATIONAHA.104.507723. PMID 16087809. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)
  8. 8.0 8.1 Maron MS, Finley JJ, Bos JM, Hauser TH, Manning WJ, Haas TS, Lesser JR, Udelson JE, Ackerman MJ, Maron BJ (2008). "Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy". Circulation. 118 (15): 1541–9. doi:10.1161/CIRCULATIONAHA.108.781401. PMID 18809796. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)
  9. Maron MS, Lesser JR, Maron BJ (2010). "Management implications of massive left ventricular hypertrophy in hypertrophic cardiomyopathy significantly underestimated by echocardiography but identified by cardiovascular magnetic resonance". The American Journal of Cardiology. 105 (12): 1842–3. doi:10.1016/j.amjcard.2010.01.367. PMID 20538141. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)
  10. Maron MS, Maron BJ, Harrigan C, Buros J, Gibson CM, Olivotto I, Biller L, Lesser JR, Udelson JE, Manning WJ, Appelbaum E (2009). "Hypertrophic cardiomyopathy phenotype revisited after 50 years with cardiovascular magnetic resonance". Journal of the American College of Cardiology. 54 (3): 220–8. doi:10.1016/j.jacc.2009.05.006. PMID 19589434. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)
  11. Adabag AS, Maron BJ, Appelbaum E, Harrigan CJ, Buros JL, Gibson CM, Lesser JR, Hanna CA, Udelson JE, Manning WJ, Maron MS (2008). "Occurrence and frequency of arrhythmias in hypertrophic cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic resonance". Journal of the American College of Cardiology. 51 (14): 1369–74. doi:10.1016/j.jacc.2007.11.071. PMID 18387438. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)
  12. Maron MS, Appelbaum E, Harrigan CJ, Buros J, Gibson CM, Hanna C, Lesser JR, Udelson JE, Manning WJ, Maron BJ (2008). "Clinical profile and significance of delayed enhancement in hypertrophic cardiomyopathy". Circulation. Heart Failure. 1 (3): 184–91. doi:10.1161/CIRCHEARTFAILURE.108.768119. PMID 19808288. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)
  13. Rubinshtein R, Glockner JF, Ommen SR, Araoz PA, Ackerman MJ, Sorajja P, Bos JM, Tajik AJ, Valeti US, Nishimura RA, Gersh BJ (2010). "Characteristics and clinical significance of late gadolinium enhancement by contrast-enhanced magnetic resonance imaging in patients with hypertrophic cardiomyopathy". Circulation. Heart Failure. 3 (1): 51–8. doi:10.1161/CIRCHEARTFAILURE.109.854026. PMID 19850699. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)
  14. O'Hanlon R, Grasso A, Roughton M, Moon JC, Clark S, Wage R, Webb J, Kulkarni M, Dawson D, Sulaibeekh L, Chandrasekaran B, Bucciarelli-Ducci C, Pasquale F, Cowie MR, McKenna WJ, Sheppard MN, Elliott PM, Pennell DJ, Prasad SK (2010). "Prognostic significance of myocardial fibrosis in hypertrophic cardiomyopathy". Journal of the American College of Cardiology. 56 (11): 867–74. doi:10.1016/j.jacc.2010.05.010. PMID 20688032. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)
  15. Moon JC, McKenna WJ, McCrohon JA, Elliott PM, Smith GC, Pennell DJ (2003). "Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance". Journal of the American College of Cardiology. 41 (9): 1561–7. PMID 12742298. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)
  16. Gange CA, Link MS, Maron MS (2009). "Utility of cardiovascular magnetic resonance in the diagnosis of Anderson-Fabry disease". Circulation. 120 (13): e96–7. doi:10.1161/CIRCULATIONAHA.109.849828. PMID 19786638. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)
  17. Maceira AM, Joshi J, Prasad SK, Moon JC, Perugini E, Harding I, Sheppard MN, Poole-Wilson PA, Hawkins PN, Pennell DJ (2005). "Cardiovascular magnetic resonance in cardiac amyloidosis". Circulation. 111 (2): 186–93. doi:10.1161/01.CIR.0000152819.97857.9D. PMID 15630027. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)
  18. Moon JC, Sachdev B, Elkington AG, McKenna WJ, Mehta A, Pennell DJ, Leed PJ, Elliott PM (2003). "Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease. Evidence for a disease specific abnormality of the myocardial interstitium". European Heart Journal. 24 (23): 2151–5. PMID 14643276. Retrieved 2011-12-23. Unknown parameter |month= ignored (help)

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