Pediatric Cardiology

, Volume 38, Issue 5, pp 1024–1031 | Cite as

Delayed Myocardial Enhancement in Pediatric Hypertrophic Cardiomyopathy: Correlation with LV Function, Echocardiography, and Demographic Parameters

  • Sonia El Saiedi
  • Noha Hossam Behairy
  • Ahmed Kharabish
  • Reem Esmail
  • Zeinab Salah Seliem
  • Mervat Shafik
  • Wesam El Mozy
Original Article


Our aim was (1) to detect the presence of fibrosis by Cardiac magnetic resonance imaging (CMR) in the pediatric age group. (2) Correlate CMR findings with demographic data, LV function, and other echocardiographic parameters. We studied 40 pediatric patients diagnosed as HCM by echocardiography. All patients were subjected to clinical examination (in which the NYHA classification was determined for each patient), echocardiography, and CMR. CMR was done on a 1.5T Philips Achieva scanner in SSFP with delayed myocardial enhancement (DE-MRI). All demographic and functional parameters as well as pressure gradient across left ventricular outflow tract (LVOT) were correlated with the percentage of myocardial enhancement. We studied 13 female and 27 male patients from 45 days up to 18 years. The mean percentage of DE-MRI was 9.7 ± 9%. We found significant correlation between the NYHA classification and the pressure gradient across the LVOT (P = < 0.001) as well as the percentage of DE-MRI (P = 0.004). The percentage of DE-MRI showed positive correlation with LV myocardial mass index (P = 0.042). It didn’t correlate with any other demographic or LV functional cardiac parameters. A good positive correlation was detected between the percentage of DE-MRI and the severity of pressure gradient across LVOT measured by echocardiography (r = 0.69 and P = <0.001). We found a significant correlation between the percentage of DE-MRI in children with HCM and the pressure gradient across LVOT, NYHA classification, and LV myocardial mass. This may help in the further management of those patients, planning for follow-up, and prognosis of the disease.


Hypertrophic cardiomyopathy LV function Delayed enhancement Cardiac magnetic resonance 



This study was not funded.

Compliance with Ethical Standards

Conflict of Interest

All authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.


  1. 1.
    Authors/Task Force members, Elliott PM, Anastasakis A, Borger MA et al (2014) ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology(ESC). Eur Heart J 2014(35):2733–2779Google Scholar
  2. 2.
    Mahrholdt H, Wagner A, Judd RM, Sechtem U, Kim RJ (2005) Delayed enhancement cardiovascular magnetic resonance assessment of non-ischaemic cardiomyopathies. Eur Heart J 26:1461–1474CrossRefPubMedGoogle Scholar
  3. 3.
    Rickers C, Wilke N, 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 112:855–861CrossRefPubMedGoogle Scholar
  4. 4.
    Maron MS, Hauser TH, Dubrow E, Horst TA, Kissinger KV, Udelson JE, Manning WJ (2007) Right ventricular involvement in hypertrophic cardiomyopathy. Am J Cardiol 100:1293–1298CrossRefPubMedGoogle Scholar
  5. 5.
    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. J Am Coll Cardiol 54:220–228CrossRefPubMedGoogle Scholar
  6. 6.
    Hudsmith LE, Neubauer S (2008) Detection of myocardial disorders by magnetic resonance spectroscopy. Nat Clin Pract Cardiovasc Med 5(Suppl 2):S49–S56CrossRefPubMedGoogle Scholar
  7. 7.
    Sipola P, Lauerma K, Jääskeläinen P, Laakso M, Peuhkurinen K, Manninen H, Aronen HJ, Kuusisto J (2005) Cine MR imaging of myocardial contractile impairment in patients with hypertrophic cardiomyopathy attributable to Asp175Asn mutation in the alpha tropomyosin gene. Radiology 236(3):815–824CrossRefPubMedGoogle Scholar
  8. 8.
    Hansen MW, Merchant N (2007) MRI of hypertrophic cardiomyopathy: part 2, differential diagnosis, risk stratification, and posttreatment MRI appearances. AJR Am J Roentgenol. 189(6):1344–1352CrossRefPubMedGoogle Scholar
  9. 9.
    The Criteria Committee of the New York Heart Association (1994) Nomenclature and criteria for diagnosis of diseases of the heart and great vessels, 9th edn. Little, Brown & Co., Boston, pp 253–256Google Scholar
  10. 10.
    Gersh BJ, Maron BJ, Bonow RO, et al. (2011) for the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines (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 58:e212–e260Google Scholar
  11. 11.
    Elliott P, McKenna WJ (2004) Hypertrophic cardiomyopathy. Lancet 363:1881–1891CrossRefPubMedGoogle Scholar
  12. 12.
    Kwon DH, Smedira NG, Rodriguez ER, Tan C, Setser R, Thamila M et al (2009) Cardiac magnetic resonance detection of myocardial scarring in hypertrophic cardiomyopathy: correlation with histopathology and prevalence or ventricular tachycardia. J Am Coll Cardiol 54:242–249CrossRefPubMedGoogle Scholar
  13. 13.
    Gerdts E, Bjornstadt H, Toft S, Devereux RB, Omvik P (2002) Impact of diastolic Doppler indices on exercise capacity in hypertensive patients with electrocardiographic left ventricular hypertrophy (a LIFE substudy). J Hypertens 20:1223–1229CrossRefPubMedGoogle Scholar
  14. 14.
    Harris KM, Spirito P, Maron MS, Zenovich AG, Formisano F, Lesser JR, Mackey-Bojack S, Manning WJ, Udelson JE, Maron BJ (2006) Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage of hypertrophic cardiomyopathy. Circulation 114:216–225CrossRefPubMedGoogle Scholar
  15. 15.
    Aryana A, d’Avila A, Heist EK, Mela T, Singh JP, Ruskin JN, Reddy VY (2007) Remote magnetic navigation to guide endocardial and epicardial catheter mapping of scar-related ventricular tachycardia. Circulation 115:1191–1200PubMedGoogle Scholar
  16. 16.
    Mavrogeni S, Petrou E, Kolovou G, Theodorakis G, Iliodromitis E (2013) Prediction of ventricular arrhythmias using cardiovascular magnetic resonance. Eur Heart J Cardiovasc Imaging 14(6):518–525CrossRefPubMedGoogle Scholar
  17. 17.
    Moon JC, Reed E, Sheppard MN et al (2004) The histologic basis of late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy. J Am Coll Cardiol 43(12):2260–2264CrossRefPubMedGoogle Scholar
  18. 18.
    Dimitrow PP, Klimeczek P, Vliegenthart R et al (2008) Late hyperenhancement in gadolinium-enhance magnetic resonance imaging: comparison of hypertrophic cardiomyopathy patients with and without nonsustained ventricular tachycardia. Int J Cardiovasc Imaging 24(1):77–83CrossRefPubMedGoogle Scholar
  19. 19.
    Kwon DH, Smedira NG, Rodriguez ER et al (2009) Cardiac magnetic resonance detection of myocardial scarring in hypertrophic cardiomyopathy: correlation with histopathology and prevalence of ventricular tachycardia. J Am Coll Cardiol 54(3):242–249CrossRefPubMedGoogle Scholar
  20. 20.
    Nazarian S, Lima JA (2008) Cardiovascular magnetic resonance for risk stratification of arrhythmia in hypertrophic cardiomyopathy. J Am Coll Cardiol 51(14):1375–1376CrossRefPubMedGoogle Scholar
  21. 21.
    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. J Am Coll Cardiol 56:867–874CrossRefPubMedGoogle Scholar
  22. 22.
    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. Circ Heart Fail. 1:184–191CrossRefPubMedGoogle Scholar
  23. 23.
    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. Circ Heart Fail. 3:51–58CrossRefPubMedGoogle Scholar
  24. 24.
    Colan SD, Lipshultz SE, Lowe AM et al (2007) Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children: findings from the Pediatric Cardiomyopathy Registry. Circulation 115:773–781CrossRefPubMedGoogle Scholar
  25. 25.
    El-Saiedi SA, Seliem ZS, Esmail RI (2013) Hypertrophic cardiomyopathy: prognostic factors and survival analysis in 128 Egyptian patients. Cardiol Young 29:1–7Google Scholar
  26. 26.
    Choudhury L, Mahrholdt H, Wagner A et al (2002) Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 40:2156–2164CrossRefPubMedGoogle Scholar
  27. 27.
    Moon JC, McKenna W, McCrohon JA, Elliott PM, Smith GC, Pennell DJ (2003) Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. J Am Coll Cardiol 41:1561–1567CrossRefPubMedGoogle Scholar
  28. 28.
    Moon JC, Mogensen J, Elliott PM, Smith GC, Elkington AG, Prasad SK, Pennell DJ, McKenna WJ (2005) Myocardial late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy caused by mutations in troponin I. Heart 91:1036–1040CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Wilson JM, Villareal RP, Hariharan R, Massumi A, Muthupillai R, Flamm SD (2002) Magnetic resonance imaging of myocardial fibrosis in hypertrophic cardiomyopathy. Tex Heart Inst J 29:176–180PubMedPubMedCentralGoogle Scholar
  30. 30.
    Teraoka K, Hirano M, Ookubo H, Sasaki K, Katsuyama H, Amino M, Abe Y, Yamashina A (2004) Delayed contrast enhancement of MRI in hypertrophic cardiomyopathy. Magn Reson Imaging 22:155–161CrossRefPubMedGoogle Scholar
  31. 31.
    Noureldin RA, Liu S, Nacif MS, Judge DP, Halushka MK, Abraham TP, Ho C, Bluemke DA (2012) The diagnosis of hypertrophic cardiomyopathy by cardiovascular magnetic resonance. J Cardiovasc Magn Reson 14:17CrossRefPubMedPubMedCentralGoogle Scholar
  32. 32.
    Dumont C, Monserrat L, Soler R, Rodríguez E, Fernández X, Peteiro J, Bouzas B, Piñón P, Castro-Beirasa A (2007) Clinical significance of late gadolinium enhancement on cardiac magnetic resonance in patients with hypertrophic cardiomyopathy. Rev Esp Cardiol 60(1):15–23CrossRefPubMedGoogle Scholar
  33. 33.
    Monserrat L, Elliott PM, Gimeno JR, Sharma S, Penas-Lado M, McKenna WJ (2003) Nonsustained ventricular tachycardia in hypertrophic cardiomyopathy: an independent marker of sudden death risk in young patients. J Am Coll Cardiol 42:873–879CrossRefPubMedGoogle Scholar
  34. 34.
    Adabag AS, Casey SA, Kuskowski MA, Zenovich AG, Maron BJ (2005) Spectrum and prognostic significance of arrhythmias on ambulatory Holter electrocardiogram in hypertrophic cardiomyopathy. J Am Coll Cardiol 45:697–704CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  • Sonia El Saiedi
    • 1
  • Noha Hossam Behairy
    • 1
  • Ahmed Kharabish
    • 1
  • Reem Esmail
    • 1
  • Zeinab Salah Seliem
    • 1
  • Mervat Shafik
    • 1
  • Wesam El Mozy
    • 1
  1. 1.Cairo University, Kasr El Aini HospitalGizaEgypt

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