Rheumatic Heart Disease

How a Case of Strep Throat Can Cause Heart Valve Damage

echocardiogram and ECG
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Rheumatic heart disease is a serious complication of rheumatic fever. It refers to the chronic heart valve damage that can develop years after a bout of rheumatic fever, often leading to heart failure if left untreated.

Rheumatic fever is an inflammatory autoimmune disease that can develop as a result of inadequately treated strep throat or scarlet fever.

When this happens, the immune system responds abnormally with chronic inflammation and the gradual deposit of calcium crystals in and around the valves of the heart.

Rheumatic fever is most common in children and adolescents. Although strep throat is common in the United States, rheumatic fever is considered rare is most developed countries.

Cause of Rheumatic Heart Disease

Acute rheumatic fever can trigger inflammation of the heart, either on the surface of the heart (pericarditis), within the heart (endocarditis), or involving the heart muscle itself (myocarditis).

In persons experiencing endocarditis, the inflammatory response can cause persistent damage to one or more of the ​four heart valves. Once this type of damage occurs, it typically worsens over time.

Rheumatic heart disease affects around half of all people who have had an acute rheumatic fever. Most cases are diagnosed 10 to 20 years after the rheumatic event.

Those who have had multiple bouts are at greatest risk.

Manifestations of Rheumatic Heart Disease

Rheumatic heart disease can manifest with different complications depending on which valve is affected and in which way the valve has been damaged.

Among the most common forms of rheumatic heart disease:

  • Mitral valve disease refers to the excessive deposit of calcium on the mitral valve. The mitral valve is the valve that lets blood flow from the left atrium to the left ventricle. The accumulation of calcium can prevent the valve from opening completely (mitral stenosis) or opening at all. This ultimately leads to a leaky valve, commonly referred to as mitral regurgitation.
  • Aortic valve disease is one in which excessive calcium deposits affect the aortic valve separating the left ventricle from the aorta. These deposits can lead to the development of aortic stenosis, aortic regurgitation, or both.
  • Tricuspid regurgitation involves the tricuspid valve separating the right atrium from the left ventricle. The condition commonly accompanies aortic and/or mitral valve disease.
  • Atrial fibrillation, the irregular and often rapid heart rate caused by decreased blood flow, is most often associated with mitral stenosis.

Treating Rheumatic Heart Disease

Clearly, the best way to deal with a condition like rheumatic heart disease is to prevent it. This requires the aggressive use of antibiotics to treat strep throat or scarlet fever, both of which are caused by group A streptococcal bacteria.

Once a person has had rheumatic fever, it is important to prevent future episodes. For persons with evidence of rheumatic heart disease, continuous antibiotic treatment may be used as a form of prophylactic (preventive) therapy. To reduce inflammation, aspirin, steroids, or non-steroidal inflammatory drugs (NSAIDs) may be prescribed.

People who have experienced acute rheumatic fever should undergo an annual physical exam to check for a possible heart murmur or any other heart abnormality.

If diagnosed with rheumatic heart disease, it is important to have your condition monitored regularly with an echocardiogram and other diagnostic tools. As heart valve problems tend to worsen over time, these routine exams can help determine if and when valve replacement surgery is needed.

The timing of a replacement surgery is critical in that you want to want to perform the operation before the person's quality of life is seriously diminished but not so early as to risk the person living longer the lifespan of the artificial valve itself.

Sources:

Rothenbühler, M.; O'Sullivan, C.; 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. DOI: 10.1016/S2214-109X(14)70310-9.

Watkins, D.; Johnson, C.; Colquhoun, S. et al. "Global, Regional, and National Burden of Rheumatic Heart Disease, 1990-2015." N Engl J Med. 2017; 377:713. DOI: 10.1056/NEJMoa1603693.

Zühlke, L.; Engel, M.; 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. DOI: 10.1093/eurheartj/ehu449.