Cardiomyopathy

Cardiomyopathy

Cardiomyopathy
Classification and external resources
Opened left ventricle of heart shows a thickened, dilated left ventricle with subendocardial fibrosis manifested as increased whiteness of endocardium.
ICD-10 I42.0
ICD-9 425.4
DiseasesDB 2137
MedlinePlus 001105
Patient UK Cardiomyopathy
MeSH D009202

Cardiomyopathy (literally "heart muscle disease") is the measurable deterioration for any reason of the ability of the myocardium (the heart muscle) to contract, usually leading to heart failure. Common symptoms include dyspnea (breathlessness) and peripheral edema (swelling of the legs). Those with cardiomyopathy are often at risk of dangerous forms of irregular heart rate and sudden cardiac death.[1]
The most common form of cardiomyopathy is dilated cardiomyopathy.[2][3]
Although the term "cardiomyopathy" could theoretically apply to almost any disease affecting the heart, it is usually reserved for "severe myocardial disease leading to heart failure".[4]

Contents

  • Classification 1
  • Types 2
  • Signs and symptoms 3
  • Images 4
  • Treatment 5
  • References 6
  • External links 7

Classification

The term 'cardiomyopathy' only came into use about 50 years ago. The definition has advanced as knowledge has increased and new diagnostic tests have been introduced. In 2008, the European Society of Cardiology defined it as a myocardial disorder in which the heart muscle was structurally abnormal and functioned abnormally.[5] [6] [7] Two years earlier, the American Heart Association had pointed out that cardiomyopathies were either confined to the heart or were part of a generalized disorder, both often leading to death or progressive heart failure. Both groups excluded heart disease due to coronary artery disease, hypertension, abnormalities of the heart valves, and heart disease present at birth from the definition. Earlier, simpler, categories such as intrinsic, (defined as weakness of the heart muscle without an identifiable external cause), and extrinsic, (where the primary pathology arose outside the myocardium itself), became more difficult to sustain. For example, as more external causes were recognized, the intrinsic category became smaller. Alcoholism, for example, has been identified as a cause of dilated cardiomyopathy, as has drug toxicity, and certain infections (including Hepatitis C). On the other hand, molecular biology and genetics have given rise to the recognition of various genetic causes, increasing the intrinsic category. For example, mutations in the cardiac desmosomal genes as well as in the DES gene may cause arrhythmogenic right ventricular cardiomyopathy (ARVC).[8][9]

At the same time, a more clinical categorization of cardiomyopathy as 'hypertrophied', 'dilated', or 'restrictive',[10] became difficult to maintain when it it became apparent that some of the conditions could fulfill more than one of those three categories at any particular stage of their development.

The current American Heart Association definition divides cardiomyopathies into primary, which affect the heart alone, and secondary, which are the result of illness affecting other parts of the body. These categories are further broken down into subgroups which incorporate new genetic and molecular biology knowledge.[11]

Types

Signs and symptoms

Symptoms and signs may mimic those of almost any form of heart disease. Chest pain is common. Mild myocarditis or cardiomyopathy is frequently asymptomatic; severe cases are associated with heart failure, arrhythmias, and systemic embolization. Manifestations of the underlying disease (e.g., Chagas' disease) may be prominent. Most patients with biopsy-proven myocarditis report a recent viral prodrome preceding cardiovascular symptoms.

ECG abnormalities are often present, although the changes are frequently nonspecific. A pattern characteristic of left ventricular hypertrophy may be present. Flat or inverted T waves are most common, often with low-voltage QRS complexes. Intraventricular conduction defects and bundle branch block, especially left bundle branch block, are also common. An echocardiogram is useful to detect wall motion abnormalities or a pericardial effusion. Chest radiographs can be normal or can show evidence of congestive heart failure with pulmonary edema or cardiomegaly.

Images

Treatment

Treatment depends on the type of cardiomyopathy and condition of disease, but may include medication (conservative treatment) or iatrogenic/implanted pacemakers for slow heart rates, defibrillators for those prone to fatal heart rhythms, ventricular assist devices (LVADs) for severe heart failure, or ablation for recurring dysrhythmias that cannot be eliminated by medication or cardioversion. The goal of treatment is often symptom relief, and some patients may eventually require a heart transplant. Treatment of cardiomyopathy (and other heart diseases) using alternative methods such as stem cell therapy is commercially available but is not supported by convincing evidence.

References

  1. ^ Kasper, Denis Lh. et al. (2005). Harrison's Principles of Internal Medicine, 16th edn. McGraw-Hill.  
  2. ^ Cardiopulmonary Pharmacology for Respiratory Care, Jahangir Moini, Ch.2; page 24
  3. ^ http://www.nhlbi.nih.gov/health/health-topics/topics/cm/types.html
  4. ^ Gabriel A. Adelmann; McKenna, W; Bristow, M; Maisch, B; Mautner, B; O'Connell, J; Olsen, E; Thiene, G et al. (1996). Cardiology Essentials in Clinical Practice. Circulation 93 (5). pp. 841–2.   (Full text)
  5. ^ "Report of the WHO/ISFC task force on the definition and classification of cardiomyopathies". Br Heart J. 44(6) (6): 672–3. Dec 1980.  
  6. ^ Abelmann WH (Sep–Oct 1984). "Classification and natural history of primary myocardial disease". Prog Cardiovasc Dis. 27(2) (2): 73–94.  
  7. ^ Richardson P, McKenna W, Bristow M, Maisch B, Mautner B, O'Connell J, Olsen E, Thiene G, Goodwin J, Gyarfas I, Martin I, Nordet P (Mar 1996). "Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies". Circulation. 93(5) (5): 841–2.  
  8. ^ Klauke B, Kossmann S, Gaertner A, Brand K, Stork I, Brodehl A, Dieding M, Walhorn V, Anselmetti D, Gerdes D, Bohms B, Schulz U, Zu Knyphausen E, Vorgerd M, Gummert J, Milting H (2010). "De novo desmin-mutation N116S is associated with arrhythmogenic right ventricular cardiomyopathy". Hum. Mol. Genet. 19 (23): 4595–607.  
  9. ^ Brodehl A, Hedde PN, Dieding M, Fatima A, Walhorn V, Gayda S, Šarić T, Klauke B, Gummert J, Anselmetti D, Heilemann M, Nienhaus GU, Milting H (2012). "Dual color photoactivation localization microscopy of cardiomyopathy-associated desmin mutants". J. Biol. Chem. 287 (19): 16047–57.  
  10. ^ Valentin Fuster; John Willis Hurst (2004). Hurst's the heart. McGraw-Hill Professional. pp. 1884–.  
  11. ^ McCartan C, Maso R, Jayasinghe SR, Griffiths LR (2012). "Cardiomyopathy Classification: Ongoing Debate in the Genomics Era". Biochem Res Int. 2012 2012: 796926.  
  12. ^ Improvement in Hypertrophic Cardiomyopathy; http://www.medscape.com/viewarticle/457562_4
  13. ^ Elliott, P.; Andersson, B.; Arbustini, E.; Bilinska, Z.; Cecchi, F.; Charron, P.; Dubourg, O.; Kühl, U.; Maisch, B.; McKenna, W. J.; Monserrat, L.; Pankuweit, S.; Rapezzi, C.; Seferovic, P.; Tavazzi, L.; Keren, A. (2007). "Classification of the cardiomyopathies: a position statement from the european society of cardiology working group on myocardial and pericardial diseases". European Heart Journal 29 (2): 270–276.  
  14. ^ Maron, B. J.; Towbin, J. A.; Thiene, G.; Antzelevitch, C.; Corrado, D.; Arnett, D.; Moss, A. J.; Seidman, C. E.; Young, J. B.; American Heart, A. (2006). "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 113 (14): 1807–1816.  

External links

  • Cardiomyopathy at DMOZ
  • Cardiomyopathy information for parents.
  • [1] The CMA Official Website, information on Cardiomyopathy for everyone.