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 develop after rheumatic fever, often many years later. This valve damage can eventually lead to heart failure if it is untreated.

Acute rheumatic fever frequently produces inflammation of the heart, a condition called “carditis.”

The carditis caused by rheumatic fever often 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 and occult inflammatory process that can eventually produce heart valve damage. This late heart valve damage is what we call 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 eventually affects 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 is mitral valve 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: Aortic valve disease is also 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 aortic stenosisaortic 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.

Tricuspid regurgitation: Tricuspid regurgitation (leaky tricuspid valve) often accompanies rheumatic aortic and/or mitral valve disease.

 

Atrial fibrillation: Atrial fibrillation 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.

 

Once a person has had rheumatic fever, especially if it is accompanied by 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 recommendationson prophylaxis for rheumatic fever.

People who have 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 may 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. Read more about heart valve disease.

A Word From Verywell

Rheumatic heart disease is disease of the heart valves that can follow an episode of rheumatic fever. The best treatment is prevention, with rapid treatment of strep throat, and of rheumatic fever, should it occur. 

Those with rheumatic heart disease need to be under the continual care of a cardiologist.

Sources:

Rothenbühler M, O'Sullivan CJ, Stortecky S, et al. Active Surveillance for Rheumatic Heart Disease in Endemic Regions: a Systematic Review and Meta-Analysis of Prevalence Among Children and Adolescents. Lancet Glob Health 2014; 2:e717.

Watkins DA, Johnson CO, Colquhoun SM, et al. Global, Regional, and National Burden of Rheumatic Heart Disease, 1990-2015. N Engl J Med 2017; 377:713.

Zühlke L, Engel ME, Karthikeyan G, et al. Characteristics, Complications, and Gaps in Evidence-based Interventions in Rheumatic Heart Disease: the Global Rheumatic Heart Disease Registry (the REMEDY study). Eur Heart J 2015; 36:1115.

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