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

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Abstract

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.

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References

  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):2733–2779

    Google Scholar 

  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 26:1461–1474

    Article  PubMed  Google Scholar 

  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 112:855–861

    Article  PubMed  Google Scholar 

  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 100:1293–1298

    Article  PubMed  Google Scholar 

  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 54:220–228

    Article  PubMed  Google Scholar 

  6. Hudsmith LE, Neubauer S (2008) Detection of myocardial disorders by magnetic resonance spectroscopy. Nat Clin Pract Cardiovasc Med 5(Suppl 2):S49–S56

    Article  CAS  PubMed  Google Scholar 

  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):815–824

    Article  PubMed  Google Scholar 

  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):1344–1352

    Article  PubMed  Google Scholar 

  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 253–256

    Google Scholar 

  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–e260

  11. Elliott P, McKenna WJ (2004) Hypertrophic cardiomyopathy. Lancet 363:1881–1891

    Article  CAS  PubMed  Google Scholar 

  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 54:242–249

    Article  PubMed  Google Scholar 

  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 20:1223–1229

    Article  CAS  PubMed  Google Scholar 

  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 114:216–225

    Article  PubMed  Google Scholar 

  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 115:1191–1200

    PubMed  Google Scholar 

  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):518–525

    Article  PubMed  Google Scholar 

  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):2260–2264

    Article  PubMed  Google Scholar 

  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):77–83

    Article  PubMed  Google Scholar 

  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):242–249

    Article  PubMed  Google Scholar 

  20. Nazarian S, Lima JA (2008) Cardiovascular magnetic resonance for risk stratification of arrhythmia in hypertrophic cardiomyopathy. J Am Coll Cardiol 51(14):1375–1376

    Article  PubMed  Google Scholar 

  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 56:867–874

    Article  PubMed  Google Scholar 

  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. 1:184–191

    Article  PubMed  Google Scholar 

  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. 3:51–58

    Article  PubMed  Google Scholar 

  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 115:773–781

    Article  PubMed  Google Scholar 

  25. El-Saiedi SA, Seliem ZS, Esmail RI (2013) Hypertrophic cardiomyopathy: prognostic factors and survival analysis in 128 Egyptian patients. Cardiol Young 29:1–7

    Google Scholar 

  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 40:2156–2164

    Article  PubMed  Google Scholar 

  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 41:1561–1567

    Article  PubMed  Google Scholar 

  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 91:1036–1040

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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 29:176–180

    PubMed  PubMed Central  Google Scholar 

  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 22:155–161

    Article  PubMed  Google Scholar 

  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 14:17

    Article  PubMed  PubMed Central  Google Scholar 

  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):15–23

    Article  PubMed  Google Scholar 

  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 42:873–879

    Article  PubMed  Google Scholar 

  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 45:697–704

    Article  PubMed  Google Scholar 

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Correspondence to Noha Hossam Behairy.

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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.

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

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El Saiedi, S., Behairy, N.H., Kharabish, A. et al. Delayed Myocardial Enhancement in Pediatric Hypertrophic Cardiomyopathy: Correlation with LV Function, Echocardiography, and Demographic Parameters. Pediatr Cardiol 38, 1024–1031 (2017). https://doi.org/10.1007/s00246-017-1612-y

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  • DOI: https://doi.org/10.1007/s00246-017-1612-y

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