European Heart Journal Advance Access originally published online on May 15, 2008
European Heart Journal 2008 29(12):1591-1592; doi:10.1093/eurheartj/ehn187
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The new European definition of cardiomyopathies: which space for muscle dystrophies?
Division of Cardioangiology with CCU
Department of Clinical and Experimental Medicine
Federico II University Hospital
Via S. Pansini 5
80131 Naples
Italy
Tel: +39 081 7462145
Fax: +39 081 5466152
Email: mgalderi{at}unina.it
I read with interest the new classification of the cardiomyopathies of the European Society of Cardiology1 and the Editorial of Thiene et al.2 In relation with new knowledge of genetic alterations and with terrific progress in imaging techniques of the recent years, this issue is very controversial and the European classification differs deeply from similar classification formulated from the American Heart Association in 2006.3 The effort of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases shall be acknowledged with enthusiasm since cardiomyopathy subtypes are characterized and the concept of new, unclassified cardiomyopathies is introduced. The European Working Group defines cardiomyopathy as ‘a myocardial disorder in which heart muscle is structurally and functionally abnormal in the absence of coronary artery disease, hypertension, valvular disease, and congenital heart disease’. In this light, a space should be created for an appropriate identification of myocardial alterations occurring in muscle dystrophies, such as x-linxed Duchenne's and Becker's dystrophy, Steinert's myotonic dystrophy, and less-frequent Emery-Dreifuss dystrophy and associated disorders, limble-girdle muscular dystrophy, and facial–scapular–humeral muscular dystrophy. Diagnostic imaging techniques, mainly transthoracic Doppler echocardiography, have allowed the acquisition of important information on myocardial involvement of these diseases. In the Duchenne's muscular dystrophy, the most frequent inherited neuromuscular disorder (30 per 100 000 live male births), cardiac involvement is common (80% of patients) and death is due to cardiac dysfunction in about 10% of the cases.4 Children with Duchenne's dystrophy develop a significant decrease of ejection fraction as well as an enlarged left ventricle and left atrium and abnormalities of left ventricular (LV) diastolic function; even acquired regions of LV hypertrabeculation/non-compaction localized to LV apex and lateral wall have been reported in rare cases. Becker muscular dystrophy is less common (3 per 100 000 live male births), and the heart is affected in about 30% of patients in the early stages but in up to 90% in the advanced stages.4 Cardiac involvement leads to myocardial thickening, regional wall motion abnormalities, dilated cardiac chambers, secondary valve regurgitation and systolic and/or diastolic dysfunction. Up to one-third of the patients develop dilated cardiomyopathy and LV hypertrabeculation/non-compaction is more frequent than in Duchenne's dystrophy. Also patients with Steinert's myotonic dystrophy, a disease occurring in 13/100 000 births may develop a specific cardiomyopathy and symptoms of heart failure in the advanced stages.5 Subclinical biventricular involvement may be found in myotonic dystrophy6 and the greater predisposition of the right ventricle to early decompensation is consistent with the finding of the well-known altered electro-anatomic pattern in the right chambers of these patients.
In view of these findings, it is my opinion that cardiomyopathies related to muscular dystrophies should find a specific space in the classification of cardiomyopathies. The early identification of myocardial abnormalities may be crucial in the therapeutic management of these pathologies.
References
- Elliott P, Andersson B, Arbustini E, et al. Classification of the cardiomyopathies: a position statement from the European society of cardiology working group on myocardial and pericardial diseases. Eur Heart J (2008) 29:270–276.
[Abstract/Free Full Text] - Thiene G, Corrado D, Basso C. Revisiting definition and classification of cardiomyopathies in the era of molecular medicine. Eur Heart J (2008) 29:144–166.
[Free Full Text] - Maron BJ, Towbin JA, Thiene G, et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation (2006) 113:1807–1816.
[Abstract/Free Full Text] - Hoogerwaard EM, Bakker E, Ippel PF, et al. Signs and symptoms of Duchenne muscular dystrophy and Becker muscular dystrophy among carriers in The Netherlands: a cohort study. Lancet (1999) 353:2116–2119.[CrossRef][Web of Science][Medline]
- Pelargonio G, Dello Russo A, Sanna T, De Martino G, Bellocci F. Myotonic dystrophy and the heart. Heart (2002) 88:665–670.
[Free Full Text] - Parisi M, Galderisi M, Sidiropulos M, et al. Early detection of biventricular involvement in myotonic dystrophy by tissue Doppler. Int J Cardiol (2007) 118:227–232.[CrossRef][Web of Science][Medline]
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