Rheumatic Heart Disease

Pharmacist showing heart display on computer
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Rheumatic heart disease is the most dreaded complication of rheumatic fever. The term "rheumatic heart disease" refers to the chronic heart valve damage that can occur after a person has had an episode of acute rheumatic fever. This valve damage can eventually lead to heart failure.

Acute rheumatic fever often produces inflammation of the heart (carditis).

This carditis affects virtually all parts of the heart - the pericardial, or exterior, surface of the heart (pericarditis); the heart muscle itself (myocarditis); and the endocardial, or interior, surface of the heart (endocarditis).

The endocarditis seen in acute rheumatic fever may set off a more chronic process that can eventually produce heart valve damage. In other words, it can produce rheumatic heart disease. Once rheumatic valvular disease begins, it tends to continually worsen over time. Repeated episodes of rheumatic fever can accelerate the deterioration of the heart valves.

(It is worth noting that the endocarditis seen in rheumatic fever is different from "infectious endocarditis," since in rheumatic fever the endocarditis is not caused by a direct bacterial infection of the heart. Rather, the endocarditis in rheumatic fever is caused by an autoimmune process that affects many parts of the body in addition to the heart, and is triggered by a reaction to the streptococcal bacteria in strep throat.)

Rheumatic heart disease ends up affecting about half the people who have rheumatic fever with carditis. Most of the time, rheumatic heart disease is diagnosed 10 to 20 years after being "triggered" by acute rheumatic fever.

What Cardiac Problems Are Seen With Rheumatic Heart Disease?

Various cardiac complications can result from rheumatic heart disease.

These include:

Mitral valve disease: The most common cardiac problem seen in rheumatic heart disease,  the mitral valve becomes laden with heavy deposits of calcium, which disrupt the normal function of the valve. Because of these heavy calcium deposits, the valve often fails to open completely (a condition called mitral stenosis). The same calcium deposits can also prevent the valve from closing completely, leading to mitral regurgitation (a "leaky" valve). So, people with rheumatic mitral valves often have both mitral stenosis and mitral regurgitation.

Aortic valve disease: Common in rheumatic heart disease, aortic valve damage is also caused by calcium deposits that disrupt normal valve function. And as with rheumatic mitral valves, rheumatic aortic valves can develop either stenosis or regurgitation, or both.

The mechanical valve problems (both stenosis and regurgitation) caused by rheumatic heart disease can tremendously increase the workload on the heart muscle, and as a result heart failure frequently develops, often after a period of many years.

Atrial fibrillation: This complication is very commonly seen in rheumatic heart disease. Blood clots (which can lead to stroke, and which are always a risk in patients with atrial fibrillation) are a particular risk in people who have both atrial fibrillation and rheumatic mitral disease. So, virtually all patients with rheumatic mitral disease and atrial fibrillation should be on chronic blood thinning (anticoagulation) therapy.

How Is Rheumatic Heart Disease Treated?

The best way to deal with rheumatic heart disease, obviously, is to prevent it. Aggressive treatment of strep throat (with antibiotics) and of rheumatic fever (should it occur) can help to limit rheumatic heart disease. Read here about the treatment of rheumatic fever.

Once a person has had rheumatic fever, especially if it has caused carditis, it is critically important to prevent any more episodes of rheumatic fever. So anyone who has had rheumatic fever should be on preventative, or prophylactic, therapy with antibiotics to prevent a recurrence. Here are the American Heart Association's recommendations on prophylaxis for rheumatic fever.

Anyone who has had acute rheumatic fever should have a physical examination annually to see if any change has occurred in the heart. A new heart murmur or a change in a previous heart murmur might indicate that heart valve damage has begun. An echocardiogram would confirm the presence or absence of heart valve damage.

Once you have been diagnosed with rheumatic heart disease, it is critically important to have regular monitoring of the condition of your heart valves and your heart muscle, usually with periodic physical exams and echocardiograms. Since rheumatic heart disease is usually progressive, the heart valve problems tend to worsen over time -- and at some point, valve replacement surgery is likely to be required.

The proper timing of this surgery is important and tricky. It is critical to replace the valves before permanent heart muscle damage occurs, but on the other hand, it is generally not a good idea to replace the valves too early (since artificial valves themselves may deteriorate over a few decades, and additional surgery may become necessary). For this reason, people with rheumatic heart disease should generally be under the watchful eye of an experienced cardiologist.

Sources:

Albert, DA, Harel, L, Kurrison, T. The treatment of rheumatic carditis. A review and metaanalysis. Medicine 1995; 74:1.

Meira, ZM, Goulart, EM, Colosimo, EA, Mota, CC. Long term follow up of rheumatic fever and predictors of severe rheumatic valvar disease in Brazilian children and adolescents. Heart 2005; 91:1019.

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