Myocardial and Pericardial Disease: Difference between revisions

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 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.

''Idiopathic cardiomyopathy''

''Familial cardiomyopathy''

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.

::: Early detetction of the disease is critical for its clinical course. Immunosuppression using corticosteroids to halt the progression of inflammation is the treatment of choice in sarcoidosis, to which myocardial dysfunction, conduction disturbances and arrhthmias may all respond. Most important is the differentiation of sarcoidosis from giant cell myocarditis, which is a more aggressive disorder requiring intensive medical and mechanical support and frequently necessitates heart transplantation. Pacemaker or ICD implantation is indicated in patients with conduction disorders or malignant arrhythmias, as medical treatment is usually ineffective.

'''''Hemochromatosis'''''

:: Repeated phlebotomy is the cornerstone of hemochromatosis treatment, although chelating agents such as deferoxamine may be considered. Early detection of the disease is critical, as depletion of iron overload may result in complete reversal of symptoms at this stage. Evidence was found that a threshold exists beyond which permanent damage is pertinent, at which stage iron depletion does not result in recovery of function. At end-stage disease, heart transplantation is a viable option with good survival rates. Importantly, screening of first degree relatives is pertinent to ensure early detection of hereditary forms of hemochromatosis.

Treatment of Fabry is safe and effective, and consist of enzyme-replacement therapy.

Gaucher disease is an inheritable deficiency of beta-glucosidase, resulting in accumulation of cerebrosides. Cardiac involvement results in impaired cardiac function due to reduced chamber compliance.

   

Treatment of Gaucher disease consists of enzyme replacement therapy, or hepatic transplantation as a last resort. Response to treatment with respect to recovery of symptoms may vary.

Glycogen storage disease may result in cardiac involvement in case of type II, III, IV and V, but survival until adulthood is rare except for type III disease. Cardiac involvement is characterized by left ventricular hypertrophy, electrographically and echocardiographically, but symptoms are frequently absent.