Noted cardiologist Dr.Leslie Cooper joins CSSA Medical Advisory Board. Dr. Cooper writes about cardiac involvement in CSS

Dr. Leslie T. Cooper joined the CSS Association Medical Advisory Board this Fall. Dr. Cooper is a consultant in cardiovascular diseases and internal medicine at the Mayo Clinic in Rochester, MN. He is co-director of the Multidisciplinary Vasculitis Clinic at Mayo and has particular interest in inflammation of the heart (myocarditis) and aorta. His time is mostly spent in the heart failure and vascular disease clinics. He is also an Associate Professor of Medicine with NIH and FDA funded research projects in myocarditis. Dr. Cooper is editor of the textbook: Myocarditis: From Bench to Bedside, and cardiovascular section editor of the NORD Guide to Rare Disorders.

Following is an article that Dr. Cooper kindly wrote for us entitled:

Cardiac Involvement in Churg Strauss Syndrome

Churg Strauss Syndrome (CSS) is a rare systemic cause of blood vessel inflammation also known as allergic angiitis and granulomatosis. Cardiac involvement in CSS is a major cause of morbidity and mortality. The American College of Rheumatology diagnostic criteria require the presence of 4 of the following 6 criteria: (1) asthma, (2) peripheral eosinophilia, (3) neuropathy, (4) paranasal sinus abnormality, (5) nonfixed pulmonary infiltrates, and (6) biopsy-proven extravascular eosinophils. Cardiac manifestations are also common and include pericarditis (inflammation of the sac surrounding the heart), heart failure from eosinophilic myocarditis (heart muscle inflammation), cardiac thrombi (clots in the heart), valve leakage, and sudden death.

Cardiac involvement in CSS should be suspected in patients with CSS who develop chest pain, shortness of breath, leg swelling, feel rapid heart beats or have episodes with loss of consciousness. Patients with CSS who develop these symptoms should see their physician promptly. A careful medical history and cardiac physical examination are indicated to determine the explanation for these symptoms. Screening tests that can help establish the diagnosis of cardiac involvement in CSS include an electrocardiogram, chest x-ray, and blood tests such as brain naturetic peptide (BNP) and cardiac troponins.

Additional tests may be needed depending on the initial evaluation. These tests may include an echocardiogram, exercise stress test, 24 hour electrocardiogram (Holter monitor), or cardiac catheterization with coronary angiography. Sometimes a heart biopsy is also needed to diagnose the cause of heart failure in patients with CSS.

Because CSS can affect the heart in many ways, individual treatment will depend on the patient’s presenting symptoms, heart function, heart rhythm and degree of disease activity. Patients who develop blood clots in the heart usually require anticoagulation. Sometimes heart surgery is required to fix badly damaged heart valves, remove large clots, or remove constricting pericardium (lining surrounding the heart) if these affect heart function. Prednisone and other immunosuppressive medications may be required for active CSS.

Patients with CSS should be aware of the possibility of heart involvement and discuss questions about their health with their primary medical provider. Because CSS is rare the cause unknown, more clinical research is needed to determine the long term risks of heart disease in CSS patients. More research is also needed to determine the best diagnostic and treatment strategies in CSS patients with suspected heart disease. Doctors at Mayo Clinic have a longstanding and focused interest in heart disease in patients with CSS and other systemic inflammatory disorders.

Dr. Cooper’s publications include:

Cooper LT, Berry GJ, Shabetai R. Idiopathic Giant Cell Myocarditis- Natural History and Treatment. N Engl J Med 1997; 336:1860-66.
Menghini VV, Savcenko V, Olson LJ, Tazelaar HD., Dec GW, Kao A, Cooper LT. Combined Immunosuppression for the Treatment of Idiopathic Giant Cell Myocarditis. Mayo Clin Proceedings 1999;74:1221-1226.
Okura Y, Dec, GW, Hare JM, Berry GR, Tazelaar HD, Cooper LT. A Clinical and Histopathologic Comparison of Cardiac Sarcoidosis and Idiopathic Giant Cell Myocarditis. J Am Coll Cardiol 2003: 41.