Myocardial and Pericardial Disease: Difference between revisions

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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.
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.


=== Endomyocardial ===
==== Endomyocardial ====
==== Endomyocardial fibrosis ====
===== Endomyocardial fibrosis =====
Endomyocardial fibrosis is an important cause of congestive heart failure in equatorial Africa. Studies have shown prevalence up to 20% of the population, mostly familial in children or young adults, and symptoms occurred in a minority of detected cases. The disease predominantly occurs in black individuals, but may rarely present in white subjects.
Endomyocardial fibrosis is an important cause of congestive heart failure in equatorial Africa. Studies have shown prevalence up to 20% of the population, mostly familial in children or young adults, and symptoms occurred in a minority of detected cases. The disease predominantly occurs in black individuals, but may rarely present in white subjects.
Fibrous lesions hamper cardiac function by impairing the inflow of the ventricles, affecting the left or both ventricles most frequently. Solitaire right-sided involvement is less frequent occurring in approximately 10% of cases. Symptoms are concordant with the ventricles involved, an atrial fibrillation and ascites are known factors associated with a poor prognosis.
Fibrous lesions hamper cardiac function by impairing the inflow of the ventricles, affecting the left or both ventricles most frequently. Solitaire right-sided involvement is less frequent occurring in approximately 10% of cases. Symptoms are concordant with the ventricles involved, an atrial fibrillation and ascites are known factors associated with a poor prognosis.
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Endomyocardial fibrosis is a relentless disease, half of patients not making it beyond 2 years after detection, depending on the extent of symptoms at presentation. Medical management is only effective in the early stages of the disease, and is aimed at relief of symptoms. Surgical correction of the results of the disease, i.e. valve replacement, improves hemodynamic characteristics, but mortality is high. Fibrosis may reoccur, but long-term survival can be good.
Endomyocardial fibrosis is a relentless disease, half of patients not making it beyond 2 years after detection, depending on the extent of symptoms at presentation. Medical management is only effective in the early stages of the disease, and is aimed at relief of symptoms. Surgical correction of the results of the disease, i.e. valve replacement, improves hemodynamic characteristics, but mortality is high. Fibrosis may reoccur, but long-term survival can be good.


==== Hypereosinophilic syndrome: Löffler Endocarditis ====  
===== Hypereosinophilic syndrome: Löffler Endocarditis =====  
The hypereosinophilic syndrome is a systemic disease, involving several organ systems. Cardiac involvement, Löffler endocarditis, is usually present when eosinophil counts are high for a longer period of time. The eosinophilia itself may occur from several different causes.
The hypereosinophilic syndrome is a systemic disease, involving several organ systems. Cardiac involvement, Löffler endocarditis, is usually present when eosinophil counts are high for a longer period of time. The eosinophilia itself may occur from several different causes.


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Routine care applies to these patients. Diuretics, and neurohumoral blockade are appropriate, as is anticoagulation. Corticosteroids and cytotoxic drugs increase survival in patients with Löffler endocarditis, and interferon may be used as a last option in refractory patients.  Surgical therapy may be considered as palliative treatment in the fibrotic fase of the disease.
Routine care applies to these patients. Diuretics, and neurohumoral blockade are appropriate, as is anticoagulation. Corticosteroids and cytotoxic drugs increase survival in patients with Löffler endocarditis, and interferon may be used as a last option in refractory patients.  Surgical therapy may be considered as palliative treatment in the fibrotic fase of the disease.


==== Arrythmic cardiomyopathy ''(See [http://www.textbookofcardiology.org/wiki/ARVC/D ARVC/D])''====
===== Arrythmic cardiomyopathy ''(See [http://www.textbookofcardiology.org/wiki/ARVC/D ARVC/D])''=====


==== Unclassified Cardiomyopathy ====
===== Unclassified Cardiomyopathy =====
Left ventricular non-compaction
Left ventricular non-compaction


==== Secondary cardiomyopathies ====
===== Secondary cardiomyopathies =====
Nine different subgroups of secondary myocardial disease exist, which are defined as diseases of the myocardium with a known cause.
Nine different subgroups of secondary myocardial disease exist, which are defined as diseases of the myocardium with a known cause.


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