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Myocardial and Pericardial Disease: Difference between revisions
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Myocardial and Pericardial Disease (view source)
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==== Prognosis and outcome ==== | ==== Prognosis and outcome ==== | ||
DCM has a highly variable clinical course. Approximately half of DCM patients respond well to contemporary heart failure medication, and an minority of patients show a healing course. Conversely, a subgroup can be identified with a highly unfavourable clinical course, not responsive to heart failure medication and rapidly progressing to inotropy- or LVAD dependency. Overall, 5-year survival rates approximates 30%. | DCM has a highly variable clinical course. Approximately half of DCM patients respond well to contemporary heart failure medication, and an minority of patients show a healing course. Conversely, a subgroup can be identified with a highly unfavourable clinical course, not responsive to heart failure medication and rapidly progressing to inotropy- or LVAD dependency. Overall, 5-year survival rates approximates 30%. | ||
=== Restrictive and infiltrative cardiomyopathy === | |||
Restrictive cardiomyopathy is characterized by an increase in ventricular wall stiffness, impairing its diastolic function. Systolic function is usually preserved in early stages of the disease, but may deteriorate with progression of the disease. RCM is less frequent in the developed world than the previously described HCM and DCM, but is an important cause of death in Africa, India, South and Central America, and Asia due to the high incidence of endomyocardial fibrosis. The spectrum of restrictive cardiomyopathies can be classified as shown in [[table xx]], according to its cause. An important differentiation is that between RCM and constrictive pericarditis. Constrictive pericarditis is similarly characterized by impaired ventricular filling with preserved systolic function, but may be adequately treated by pericardiectomy. | |||
==== Restrictive (Non-infiltrative) ==== | |||
''Idiopathic cardiomyopathy'' | |||
''Familial cardiomyopathy'' | |||
==== Infiltrative ==== | |||
===== Amyloidosis ===== | |||
Amyloidosis is a disease that results from tissue deposition of fibrils that have a distinct secondary structure of a beta-pleated sheet configuration, leading to characteristic histological changes. Amyloid depositions can occur in almost any organ, but remains clinically undetected unless extensive depositions are present. | |||
'''''Types of amyloidosis''''' | |||
The most frequent types of amyloidosis are the AL (primary) and AA (secondary) types. AL amyloidosis is a plasma cell dyscrasia, which can occur solitarily or in association with multiple myeloma. AA amyloidosis can be considered a complication of chronic inflammatory disease states such as rheumatoid arthritis, in which the deposits consist of fragments of serum amyloid A, which is an acute phase reactant. | |||
Hereditary amyloidosis has been increasingly recognized in the last decade, and results from mutations in the gene for thransthyretin. Some mutations are clinically limited to the myocardium. Its incidence increases with increasing age, with a predilection for men, but its prognosis is better than that of the AL type. Senile systemic amyloidosis results from deposition of normal wild-type transthyretin. This form of amyloidosis is clinically predominated by an infiltrative cardiomyopathy, but progression is slow and prognosis is better than of other acquired forms. | |||
*Cardiac amyloidosis | |||