Cardiac Sarcoidosis:Causes, Types, Diagnosis, Treatment

Cardiac Sarcoidosis: Causes
The exact cause of sarcoidosis is unknown. Granulomas appear to be the result of an immune system response to an unidentified trigger. Infections agents as well as environmental exposures are thought to be possible precipitants for this immune response; however, no clear triggers or causes have been identified. Genetic mutations in white blood cell proteins (called human leukocyte antigens, or HLA) as well as chemicals that control inflammation (called cytokines) have also been linked to sarcoidosis.

Cardiac Sarcoidosis: Types
Cardiac sarcoidosis (CS) can take many forms, some benign and others serious.

Heart Rhythm Disorders: Interference of electrical movement through the heart is the one of the  complications of CS and can include complete heart block which can be life threatening. Alternatively, fast heart rhythms such as atrial flutter, atrial fibrillation, supraventricular tachycardia, and ventricular tachycardia can be present. Ventricular tachycardia occurs in almost 25 percent of CS patients and is of particular concern since it can lead to sudden cardiac death. These possible complications may occur suddenly without warning.
Heart Failure: Heart failure is another common complication of CS. Sarcoidosis can cause the heart muscle to weaken and/or stiffen, leading to fluid retention in the lungs, abdomen, and lower extremities. In extreme cases, an aneurysm can form due to weakening of the heart wall. Granulomas can also infiltrate the heart valves, causing leaky valves also resulting in heart failure.
Coronary Disease: Although rare, CS can cause inflammatory disorder of the heart arteries called vasculitis. In severe forms, vasculitis can lead to coronary artery blockages, chest pain and, ultimately, heart attacks.
Pericardial Disease: Inflammation of the sack around the heart, called pericarditis, is another rare but important form of CS.

Diagnosing cardiac sarcoidosis (CS) can be very challenging. There are no widely accepted guidelines for either screening or diagnosing CS. Moreover, the current available diagnostic tests are variable in their ability to detect CS. Because of its devastating nature, most patients with other forms sarcoidosis are screened for CS.

Initial cardiac evaluation may include an EKG, a signal averaged EKG, an echocardiogram and a Holter monitor (extended EKG). Additional imaging tests may include single positron emission computed tomography (SPECT), positron emission tomography (PET) and cardiac MRI. A positive heart biopsy confirms CS, but a heart biopsy may more often be negative or normal even when there is sarcoid in the heart, especially if heart function is normal.

Cardiac Sarcoidosis: Treatment

Controversy exists as to the best treatment for cardiac sarcoidosis (CS). However, treatment is generally directed at minimizing the inflammation associated with CS and protecting against the life-threatening complications.

Because of their anti-inflammatory properties, corticosteroids (cortisone, prednisone and methylprednisolone) are the first-line therapy. When patients cannot take steroids, or when combination therapy is necessary, medications such as methotrexate, azathioprine, mycophenolate and antimalarials are used.

Additional therapies for specific CS-related heart disorders may be necessary. For example, heart rhythm disorders such as complete heart block typically require placement of a permanent pacemaker, whereas ventricular tachycardia generally requires internal cardiac defibrillator (ICD) placement.

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