Cardiomyopathies: Temporal Review and Genetic Determination

Figures

• 

  Figure 1. John F. Goodwin, the father of the classification, definition and nomination of cardiomyopathies. He used the term cardiomyopathy for heart muscle diseases of unknown cause and specific heart muscle diseases associated with morbidities of other systems. Modified from [1].

• 

  Figure 2. The original classification of cardiomyopathies by John Goodwin [2] was as follows: (a) dilated, when the ventricular cavities appear increased in size; The arrows indicate the progressive enlargement of the ventricles in dilated cardiomyopathy; (b) hypertrophic, when the free wall or septum are thickened in an asymmetric way; (c) restrictive, when the ventricular cavities are reduced in size or even obliterated, with a thickened endocardium and thrombus. The problem is the reduced ventricular cavity, not the stiffness of the ventricular wall, hindering diastolic relaxation [1]. From [1] with permission.

• 

  Figure 3. Obliterative cardiomyopathy (a) due to endomyocardial eosinophilic inflammation (haematoxylin–eosin stain) (b), with thrombosis, known by the name of Loeffer cardiomyopathy in the setting of eosinophil leukemia or by the name of Davies disease when due to intestinal helminth infection with allergic eosinophil inflammation. From [3] with permission.

• 

  Figure 4. Example of a heart with dilated cardiomyopathy, gross view (a), and myocytolysis of cardiomyocytes on histology (b), haematoxylin–eosin stain. From [3] with permission.

• 

  Figure 5. Another type of dilated cardiomyopathy (a) with severe, non-ischemic myocardial fibrosis (b). The patient previously had viral myocarditis, which accounted for myocyte necrosis and scarring. Azan Mallory stain. From [3] with permission.

• 

  Figure 6. (a) Heart specimen with hypertrophic cardiomyopathy due to asymmetric ventricular septum thickness. Hypertrophy, long-axis view. (b) Disarray of cardiomyocytes on histology. Originally, the disarray was interpreted as a benign tumour (heart hamartoma). Azan Mallory stain. From [4] with permission.

• 

  Figure 7. Fiedler’s myocarditis on microscopy. A review of the original slides demonstrates two histology patterns: (a) lymphocytic inflammatory infiltrates, most probably due to a virus (molecular analysis was not available in that time); (b) immunological inflammation of giant cells. Haematoxylin–eosin stain. From [5] with permission.

• 

  Figure 8. Non-caseous granuloma, typical of sarcoid myocarditis. (a) Aneurysm of the left ventricle; (b) giant cells in the setting of non-caseous granuloma. Haematoxylin–eosin stain. From [3] with permission.

• 

  Figure 9. Hemochromatosis cardiomyopathy due to multiple blood infusions. (a) The left ventricle shows a brown colour; (b) intracellular iron storage. Iron histochemical staining (personal archive).

• 

  Figure 10. Glycogenosis: (a) concentric ventricular hypertrophy with a pale myocardium, cut in short-axis view; (b) cardiomyocytes appear empty because glycogen storage was mostly removed by technical procedures (personal archive).

• 

  Figure 11. Amyloid deposits in the interstitium of the ventricular myocardium: (a) The left cardiac chamber appears dilated and stiff, particularly the left atrium. The patient suffered from atrial fibrillation. (b) The amyloid deposits were extracellular within the interstitium and stained with Congo Red and (c) Thioflavin-T stain. From [3] with permission.

• 

  Figure 12. A case of an athlete who died suddenly from right ventricular arrhythmogenic cardiomyopathy during tennis play. (a) Four-chamber cut. Gross appearance of the right ventricle with transmural fibrofatty replacement and translucent free wall. (b) ECG recording ventricular tachyarrhythmia. (c) Compare the histology of the left and right ventricles. The former has an intact myocardium; the latter has transmural fibrofatty tissue. Also, the ventricular septum is intact (Azan Mallory stain). (d) Histology of the right ventricle at higher magnification, with fibrofatty replacement (Azan Mallory stain). From [4] with permission.