Challenges in diagnosis and prognosis of Dilated cardiomyopathy with cardiac MRI
Abstract
Dilated Cardiomyopathy (DCM) is the final response of the myocardium to several conditions, in most cases related to genetic or inflammatory factors, which lead to progressive left ventricular wall thinning and dilation, myocardial fibrosis, and cardiac dysfunction.
DCM is the third most common cause of heart failure (after ischemia and valvular heart disease) and the most common cardiomyopathy (other types: hypertrophic, restrictive, and arrhythmogenic right ventricular cardiomyopathies). It is end-stage cardiomyopathy, predominantly involving the left ventricle.
Evaluation of the etiology by excluding other causes of LV dilation and dysfunction, may lead to the appropriate therapeutic protocol and contribute to prognosis.
Detection of pre-DCM phenotype may reduce morbidity and mortality.
The aim of surveillance and therapy in each stage of DCM is to prevent remodeling, assess reverse remodeling, and avoid adverse events, especially sudden cardiac death.
Cardiac MRI (CMR) has a major role in diagnosis, prognosis, and selection of therapeutic schema. It is the gold-standard technique to measure chamber dimensions, to assess morphological features, systolic function, and valve disease. Also, tissue characterization may exclude ischemia as well as detect myocardial fibrosis which leads to arrhythmias and heart failure.
In this article, we discuss the predictive value of Cardiac Magnetic Resonance (CMR) for the evaluation of etiology, detection of pre-clinical DCM, assessment of left ventricular dysfunction, cardiac remodeling, and myocardial fibrosis, aspects that give a prognosis of sudden cardiac death and also guidance in the decision for device therapy.
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(1) Elliott P, Andersson B, Arbustini E et al (2008) Classification of the cardiomyopathies: a position statement from the European Society of Cardiology working group on myocardial and pericardial diseases. Eur Heart J 29:270–276
(2) Jefferies J. L., Towbin J. A. (2010). Dilated cardiomyopathy. Lancet 375 752–762.
(3) J. A. Towbin, A. M. Lowe, S. D. Colan et al., “Incidence, causes, and outcomes of dilated cardiomyopathy in children,” The Journal of the American Medical Association, vol. 296, no. 15, pp. 1867–1876, 2006
(4) Mestroni L, Rocco C, Gregori D, et al. Familial dilated cardiomyopathy. J Am Coll Cardiol. (1999) 34:181–90. doi: 10.1016/S0735-1097(99)00172-2
(5) Sweet ME, Taylor MR, Mestroni L. Diagnosis, prevalence, and screening of familial dilated cardiomyopathy. Expert Opin Orphan Drugs. (2015) 3:869– 76. doi: 10.1517/21678707.2015.1057498
(6) Ganesh SK, Arnett DK, Assimes TL, et al. Genetics and genomics for the prevention and treatment of cardiovascular disease: update. Circulation. (2013) 128:2813–51. doi: 10.1161/01.cir.0000437913.98912.1d
(7) Ghosh N., Haddad H. Recent progress in the genetics of cardiomyopathy and its role in the clinical evaluation of patients with cardiomyopathy. Curr Opin Cardiol. 2011 Mar;26(2):155-64. doi: 10.1097/HCO.0b013e3283439797
(8) Japp A., Gulati A., Cook S.A., et al. The Diagnosis and Evaluation of Dilated Cardiomyopathy. J Am College of Radiol (2016) doi.org/10.1016/j.jacc.2016.03.590
(9) Sun JP, James KB, Yang XS et al (1997) Comparison of mortality rates and progression of left ventricular dysfunction in patients with idiopathic dilated cardiomyopathy and dilated versus nondilated right ventricular cavities. Am J Cardiol 80:1583–1587
(10) HombachV MerkleN, Torzewski J et al (2009) Electrocardiographic and cardiac magnetic resonance imaging parameters as predictors of a worse outcome in patients with idiopathic dilated cardiomyopathy. Eur Heart J 30:2011–2018
(11) Francone M. Role of Cardiac Magnetic Resorance in the Evaluation of Dilated Cardiomyopathy: Diagnostic Contribution and Prognostic Significance. (2014) Hindawi Publishing Corporation ISRN Radiology, ID 365404, doi.org/10.1155/2014/365404
(12) Beltrami CA, Finato N, Rocco M et al (1995) The cellular basis of dilated cardiomyopathy in humans. J Mol Cell Cardiol 27:291–305
(13) G. W. Dorn II, “Apoptotic and non-apoptotic programmed cardiomyocyte death in ventricular remodelling,” Cardiovascular Research, vol. 81, no. 3, pp. 465–473, 2009
(14) A. Barison, P. G. Masci, and M. Emdin, “Fibrosis and mortality in patients with dilated cardiomyopathy,” The Journal of The American Medical Association, vol. 309, no. 24, pp. 2547–2549, 2013
(15) Buser PT, Wagner S, Auffermann W et al (1990) Threedimensional analysis of the regional contractility of the normal and the cardiomyopathic left ventricle using cinemagnetic resonance imaging (in German). Z Kardiol 79:573–579
(16) Imai H, Kumai T, Sekiya M et al (1992) Left ventricular trabeculae evaluated with MRI in dilated cardiomyopathy and old myocardial infarction. J Cardiol 22:83–90
(17) Soriano CJ, Ridocci F, Estornell J, Jimenez J, Martinez V, De Velasco JA (2005) Noninvasive diagnosis of coronary artery disease in patients with heart failure and systolic dysfunction of uncertain etiology, using late gadoliniumenhanced cardiovascular magnetic resonance. J Am Coll Cardiol 45:743–748
(18) McCrohon JA, Moon JCC, Prasad SK et al (2003) Differentiation of heart failure related to dilated cardiomyopathy and coronary artery disease using gadolinium-enhanced cardiovascular magnetic resonance. Circulation 108:54–59
(19) Wu E, Judd RM, Vargas JD et al (2001) Visualisation of the presence, location and transmural extent of healed Q-wave and non-Q-wave myocardial infarction. Lancet 357:21–28
(20) Semelka RC, Tomei E, Wagner S et al (1990) Interstudy reproducibility of dimensional and functional measurements between cine magnetic resonance studies in the morphologically abnormal left ventricle. Am Heart J 119: 1367–1373
(21) Strohm O, Schulz-Menger J, Pilz B, Osterziel KJ, Dietz R, Friedrich MG (2001) Measurement of left ventricular dimensions and function in patients with dilated cardiomyopathy. J Magn Reson Imaging 13:367–371
(22) Gaudio C, Tanzilli G, Mazzarotto P et al (1991) Comparison of left ventricular ejection fraction by magnetic resonance imaging and radionuclide ventriculography in idiopathic dilated cardiomyopathy. Am J Cardiol 67:411–415
(23) Buss SJ, Breuninger K, Lehrke S, et al. Assessment of myocardial deformation with cardiac magnetic resonance strain imaging improves risk stratification in patients with dilated cardiomyopathy. Eur Hear J Cardiovasc Imaging. (2015) 16:307–15. doi: 10.1093/ehjci/jeu181
(24) aus dem Siepen F, Buss SJ, Messroghli D, et al. T1 mapping in dilated cardiomyopathy with cardiac magnetic resonance: quantification of diffuse myocardial fibrosis and comparison with endomyocardial biopsy. Eur Heart J Cardiovasc Imaging 2015;16:210–6
(25) Puntmann VO, Voigt T, Chen Z, et al. Native T1 mapping in differentiation of normal myocardium from diffuse disease in hypertrophic and dilated cardiomyopathy. J Am Coll Cardiol Img 2013;6: 475–84
(26) Fontana M, Barison A, Botto N, et al. CMR-verified interstitial myocardial fibrosis as a marker of subclinical cardiac involvement in LMNA mutation carriers. J Am Coll Cardiol Img 2013;6: 124–6
(27) Assomull RG, Shakespeare C, Kalra PR, Lloyd G, Gulati A, Strange J, et al. Role of cardiovascular magnetic resonance as a gatekeeper to invasive coronary angiography in patients presenting with heart failure of unknown etiology. Circulation. (2011) 124:1351–60. doi: 10.1161/CIRCULATIONAHA.110.011346
(28) Friedrich MG, Sechtem U, Schulz-Menger J, et al. Cardiovascular magnetic resonance in myocarditis: a JACC White Paper. J Am Coll Cardiol 2009;53:1475–87
(29) Caforio ALP, Pankuweit S, Arbustini E, et al. Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J. (2013) 34:2636–48. doi: 10.1093/eurheartj/eht210
(30) Fauchier L, Babuty D, Poret P, et al. Comparison of long-term outcome of alcoholic and idiopathic dilated cardiomyopathy. Eur Heart J 2000;21: 306–14
(31) Cerrato E, D’Ascenzo F, Biondi-Zoccai G, et al. Cardiac dysfunction in pauci symptomatic human immunodeficiency virus patients: a meta-analysis in the highly active antiretroviral therapy era. Eur Heart J 2013;34:1432–6
(32) Mitropoulou P, Georgiopoulos G, Figliozzi S, et al. (2020) Multi-Modality Imaging in Dilated Cardiomyopathy: With a Focus on the Role of Cardiac Magnetic Resonance. Front. Cardiovasc. Med. 7:97. doi: 10.3389/fcvm.2020.00097
(33) Charron P, Arad M, Arbustini E et al (2010) Genetic counseling and testing in cardiomyopathies: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 31:2715–2726
(34) Dec GW, Fuster V (1994) Idiopathic dilated cardiomyopathy. N Engl J Med 331:1564–1574
(35) Rubis P. The diagnostic work up of genetic and inflammatory dilated cardiomyopathy. e-Journal of Cardiology Practice. (2015)
(36) Mestroni L, Maisch B, McKenna WJ, et al. Guidelines for the study of familial dilated cardiomyopathies. Collaborative Research Group of the European Human and Capital Mobility Project on Familial Dilated Cardiomyopathy. Eur Heart J. 1999;20:93–102
(37) Gigli M, Merlo M, Graw SL, et al. Genetic risk of arrhythmic phenotypes in patients with dilated cardiomyopathy. J Am Coll Cardiol. (2019) 74:1480–90. doi: 10.1016/j.jacc.2019.06.072 (article 1, page 2999, ref 16)
(38) Mosterd A, Hoes AW. Clinical epidemiology of heart failure. Heart. (2007) 93:1137–46. doi: 10.1136/hrt.2003.025270
(39) Sicari R, Nihoyannopoulos P, Evangelista A, et al. Stress echocardiography expert consensus statement: European Association of Echocardiography (EAE) (a registered branch of the ESC). Eur J Echocardiogr. (2008) 9:415–37. doi: 10.1093/ejechocard/jen175
(40) Hershberger RE, Lindenfeld J, Mestroni L, et al. Genetic evaluation of cardiomyopathy—a Heart Failure Society of America practice guideline. J Card Fail 2009;15:83–97
(41) Fatkin D, Yeoh T, Hayward CS, et al. Evaluation of left ventricular enlargement as a marker of early disease in familial dilated cardiomyopathy. Circ Cardiovasc Genet 2011;4:342–8
(42) Mahon NG, Murphy RT, MacRae CA, et al. Echocardiographic evaluation in asymptomatic relatives of patients with dilated cardiomyopathy reveals preclinical disease. Ann Intern Med. (2005) 143:108–15. doi: 10.7326/0003-4819-143-2-200507190-00009
(43) Lakdawala NK, Thune JJ, Colan SD, et al. Subtle abnormalities in contractile function are an early manifestation of sarcomere mutations in dilated cardiomyopathy. Circ Cardiovasc Genet 2012;5:503–10
(44) Thavendiranathan P, Poulin F, Lim KD, et al. Use of myocardial strain imaging by echocardiography for the early detection of cardiotoxicity in patients during and after cancer chemotherapy: a systematic review. J Am Coll Cardiol 2014;63: 2751–68
(45) van Rijsingen IA, Arbustini E, Elliott PM, et al. Risk factors for malignant ventricular arrhythmias in lamin a/c mutation carriers a European cohort study. J Am Coll Cardiol. 2012;59:493–500. doi: 10.1016/j.jacc.2011.08.078
(46) Rossi A, Dini FL, Faggiano P, et al. Independent prognostic value of functional mitral regurgitation in patients with heart failure. A quantitative analysis of 1256 patients with ischaemic and nonischaemic dilated cardiomyopathy. Heart 2011;97: 1675–80
(47) Stolfo D, Merlo M, Pinamonti B, et al. Early improvement of functional mitral regurgitation in patients with idiopathic dilated cardiomyopathy. Am J Cardiol 2015;115:1137–43
(48) De Bonis M, Taramasso M, Verzini A, et al. Long-term results of mitral repair for functional mitral regurgitation in idiopathic dilated cardiomyopathy. Eur J Cardiothorac Surg 2012;42: 640–6
(49) van Bommel RJ, Marsan NA, Delgado V, et al. Cardiac resynchronization therapy as a therapeutic option in patients with moderatesevere functional mitral regurgitation and high operative risk. Circulation 2011;124: 912–9
(50) de Leeuw N, Ruiter DJ, Balk AH, et al. Histopathologic findings in explanted heart tissue from patients with end-stage idiopathic dilated cardiomyopathy. Transpl Int 2001;14:299–306
(51) Iles L, Pfluger H, Lefkovits L, et al. Myocardial fibrosis predicts appropriate device therapy in patients with implantable cardioverterdefibrillators for primary prevention of sudden cardiac death. J Am Coll Cardiol 2011;57:821–8
(52) Souders CA, Bowers SLK, Baudino TA. Cardiac fibroblast. Circ Res. (2009) 105:1164–76. doi: 10.1161/CIRCRESAHA.109.209809
(53) Masci PG, Schuurman R, Andrea B, et al. Myocardial fibrosis as a key determinant of left ventricular remodeling in idiopathic dilated cardiomyopathy. Circ Cardiovasc Imaging. (2013) 6:790–9. doi: 10.1161/CIRCIMAGING.113.000438
(54) Leong DP, Chakrabarty A, Shipp N, et al. Effects of myocardial fibrosis and ventricular dyssynchrony on response to therapy in newpresentation idiopathic dilated cardiomyopathy: insights from cardiovascular magnetic resonance and echocardiography. Eur Heart J 2012;33:64–8
(55) Leyva F, Taylor RJ, Foley PW, et al. Left ventricular midwall fibrosis as a predictor of mortality and morbidity after cardiac resynchronization therapy in patients with nonischemic cardiomyopathy. J Am Coll Cardiol 2012;60:1659–67
(56) A. Gulati, A. Jabbour, T. F. Ismail et al., “Association of fibrosis with mortality and sudden cardiac death in patients with nonischemic dilated cardiomyopathy,” The Journal of the American Medical Association, vol. 309, no. 9, pp. 896–908, 2013
(57) Assomull RG, Prasad SK, Lyne J, et al. Cardiovascular magnetic resonance, fibrosis, and prognosis in dilated cardiomyopathy. J Am Coll Cardiol. (2006) 48:1977–85. doi: 10.1016/j.jacc.2006.07.049
(58) Gao P, Yee R, Gula L, et al. Prediction of arrhythmic events in ischemic and dilated cardiomyopathy patients referred for implantable cardiac defibrillator. Circ Cardiovasc Imaging. (2012) 5:448– 56. doi: 10.1161/CIRCIMAGING.111.971549
(59) Perazzolo Marra M, De Lazzari M, Zorzi A, et al. Impact of the presence and amount of myocardial fibrosis by cardiac magnetic resonance on arrhythmic outcome and sudden cardiac death in nonischemic dilated cardiomyopathy. Hear Rhythm. (2014) 11:856–63. doi: 10.1016/j.hrthm.2014.01.014
(60) Flett AS, Hayward MP, Ashworth MT, et al. Equilibrium contrast cardiovascular magnetic resonance for the measurement of diffuse myocardial fibrosis. Circulation. (2010) 122:138–44. doi: 10.1161/CIRCULATIONAHA.109.9 30636
(61) Gulati A, Ismail TF, Jabbour A, et al. Clinical utility and prognostic value of left atrial volume assessment by cardiovascular magnetic resonance in non-ischaemic dilated cardiomyopathy. Eur J Heart Fail 2013;15:660–70
(62) Gulati A, Ismail TF, Jabbour A, et al. The prevalence and prognostic significance of right ventricular systolic dysfunction in nonischemic dilated cardiomyopathy. Circulation. (2013) 128:1623–33. doi: 10.1161/CIRCULATIONAHA.113.002518
(63) Bourantas CV, Loh HP, Bragadeesh T, et al. Relationship between right ventricular volumes measured by cardiac magnetic resonance imaging and prognosis in patients with chronic heart failure. Eur J Heart Fail 2011;13:52–60
(64) Merlo M, Cannatá A, Vitagliano A, et al. Clinical management of dilated cardiomyopathy: current knowledge and future perspectives. Expert Rev Cardiovasc Ther. (2016) 14:137–40. doi: 10.1586/14779072.2016.1125292
(65) Axel L, Dougherty L. MR imaging of motion with spatial modulation of magnetization. Radiology. (1989) 171:841–5. doi: 10.1148/radiology.171.3.2717762
(66) Chalil S, Stegemann B, Muhyaldeen S, et al. Intraventricular dyssynchrony predicts mortality and morbidity after cardiac resynchronization therapy. J Am Coll Cardiol. (2007) 50:243–52. doi: 10.1016/j.jacc.2007.03.035
(67) Merlo M, Pyxaras SA, Pinamonti B, et al. Prevalence and prognostic significance of left ventricular reverse remodeling in dilated cardiomyopathy receiving tailored medical treatment. J Am Coll Cardiol. (2011) 57:1468–76. doi: 10.1016/j.jacc.2010.11.030
(68) Priori SG, Blomstrom-Lundqvist C, Mazzanti A, et al. 2015 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J 2015;36:2793–867
(69) Yancy CW, Jessup M, Bozkurt B, et al. 2013 ACCF/AHA guideline for the management of heart failure: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol 2013;62:e147–239
(70) Neilan TG, Coelho-Filho OR, Danik SB, et al. CMR quantification of myocardial scar provides additive prognostic information in nonischemic cardiomyopathy. JACC Cardiovasc Imaging. (2013) 6:944–54. doi: 10.1016/j.jcmg.2013.05.013
(71) Becker MAJ, Cornel JH, van de Ven PM, et al. The prognostic value of late gadolinium-enhanced cardiac magnetic resonance imaging in nonischemic dilated cardiomyopathy. JACC Cardiovasc Imaging. (2018) 11:1274–84. doi: 10.1016/j.jcmg.2018.03.006
(72) Desai AS, Fang JC, Maisel WH, et al. Implantable defibrillators for the prevention of mortality in patients with nonischemic cardiomyopathy: a meta-analysis of randomized controlled trials. JAMA 2004;292:2874–9
(73) Mordi I, Jhund PS, Gardner RS, et al. LGE and NT-proBNP identify low risk of death or arrhythmic events in patients with primary prevention ICDs. J Am Coll Cardiol Img 2014;7:561–9
(74) Gutman SJ, Costello BT, Papapostolou S, Voskoboinik A, Iles L, Ja J, et al. Reduction in mortality from implantable cardioverterdefibrillators in non-ischaemic cardiomyopathy patients is dependent on the presence of left ventricular scar. Eur Heart J. (2019) 40:542–50. doi: 10.1093/eurheartj/ehy437
(75) Bloch Thomsen PE, Jons C, Raatikainen MJP, et al. Long-term recording of cardiac arrhythmias with an implantable cardiac monitor in patients with reduced ejection fraction after acute myocardial infarction: the Cardiac Arrhythmias and Risk Stratification After Acute Myocardial Infarction (CARISMA) study. Circulation. (2010) 122:1258–64. doi: 10.1161/CIRCULATIONAHA.109.902148
(76) Ruschitzka F, Abraham WT, Singh JP, et al., for the EchoCRT Study Group. Cardiac-resynchronization therapy in heart failure with a narrow QRS complex. N Engl J Med 2013;369:1395–405
(77) Wong JA, Yee R, Stirrat J, et al. Influence of pacing site characteristics on response to cardiac resynchronization therapy. Circ Cardiovasc Imaging. (2013) 6:542–50. doi: 10.1161/CIRCIMAGING.111.000146
(78) Leyva F, Foley PWX, Chalil S, et al. Cardiac resynchronization therapy guided by late gadolinium-enhancement cardiovascular magnetic resonance. J Cardiovasc Magn Reson. (2011) 13:29. doi: 10.1186/1532-429X-13-29
DOI: http://dx.doi.org/10.36162/hjr.v7i3.501
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