Cardiomyopathy in children

Cardiomyopathy is a heterogeneous group of chronic, severe myocardial diseases leading to the development of myocardial dysfunction.

For the first time the term "cardiomyopathy" was proposed by W. Brigden (1957) for the definition of myocardial diseases of unknown origin. At present, this concept can not be considered clearly delineated, it is often invested in it with different meanings. In the modern nomenclature of diseases (WHO, 1996) idiopathic (unclear or unknown etiology), specific (for various diseases associated with the defeat of other organs, or with a known etiology), cardiomyopathies and unclassifiable myocardial diseases.

Classification

The following classification of cardiomyopathy was approved by WHO in 1996.

1. Idiopathic cardiomyopathy (of unknown origin).
2. Cardiomyopathy specific.
   1. Inflammatory cardiomyopathies:
      • infectious;
      • autoimmune.
   2. Ischemic cardiomyopathy - diffuse violations of myocardial contractility with pronounced changes in the coronary arteries.
   3. Hypertensive cardiomyopathy - with left ventricular hypertrophy due to arterial hypertension in combination with signs of dilated or restrictive cardiomyopathy.
   4. Metabolic cardiomyopathies:
      • endocrine (with acromegaly, hypokorticism, thyrotoxicosis, myxedema, obesity, diabetes, pheochromocytoma);
      • with infiltrative and granulomatous processes, accumulation diseases (amyloidosis, hemochromatosis, sarcoidosis, leukemia, mucopolysaccharidosis, glycogenosis - Pompe disease), lipidosis (Gaucher's disease, Fabry's disease);
      • with a deficiency of microelements (potassium, magnesium, selenium, etc.), vitamins and nutrients (beriberi, kwashiorkor), as well as in anemia.
   5. Cardiomyopathy in systemic connective tissue diseases (dermatomyositis, rheumatoid arthritis, systemic scleroderma, systemic lupus erythematosus).
   6. Cardiomyopathy with systemic neuromuscular diseases:
      • neuromuscular disorders (Friedreich's ataxia, Noonan's syndrome);
      • muscular dystrophies (Duchenne, Becker, myotonia).
   7. Cardiomyopathy under the influence of toxic and physical factors (alcohol, cobalt, lead, phosphorus, mercury, anthracycline antibiotics, cyclophosphamide, uremia, ionizing radiation).
3. Unclassified cardiomyopathies (fibroelastosis of endomyocardium, noncompact myocardium, mitochondrial diseases, systolic dysfunction with minimal dilatation).

At present, the clinical classification of cardiomyopathies (WHO, 1980, modified with WHO data, 1995), has been generally accepted, and has been constructed taking into account the leading pathophysiological mechanisms.

Pathophysiological classification of cardiomyopathies (WHO, 1995).

• Dilatation cardiomyopathy (DCMP). It is characterized by dilatation and violation of contractility of the myocardium of the left or both ventricles.
• Hypertrophic cardiomyopathy (HCMP). A typical symptom is myocardial hypertrophy of the left and / or right ventricle of the heart with preserved contractile and impaired diastolic function; distinguish two forms:
  • with obstruction;
  • without obstruction.
• Restrictive cardiomyopathy (RSCM). Dilation is characteristic. Atrial and diastolic filling of the ventricles; distinguish three forms:
  • primary myocardial;
  • endomyocardial with eosinophilia;
  • endomyocardial without eosinophilia.
• Arrhythmogenic cardiomyopathy of the right ventricle (ACHP). It is characterized by progressive replacement of the myocardium of the right and / or left ventricle with a fibrous-fat tissue, leading to severe arrhythmias and sudden death.

[1], [2], [3], [4], [5]