A Guide to Rheumatic Heart Disease

Medically Reviewed By Uzochukwu Ibe, MD, MPH
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Rheumatic heart disease involves long-term damage to the heart valves due to rheumatic fever, an autoimmune response to a strep infection such as strep throat.   “Strep” refers to group A Streptococcus bacteria, specifically S. pyogenes. These bacteria cause a variety of infections that doctors treat with antibiotics.

Without treatment from medications and possible heart valve surgery, rheumatic heart disease may lead to serious cardiac complications, including heart failure. 

This article discusses the causes, symptoms, diagnosis, treatment, and prevention of rheumatic heart disease, as well as the potential complications. 

What is rheumatic heart disease?

illustration of steps leading to rheumatic heart disease, including strep infections and rheumatic fever
All about rheumatic heart disease. Illustration by Jason Hoffman.

Rheumatic heart disease is a disease that arises after exposure to group A strep. Rheumatic heart disease can have long lasting complications involving multiple organs if a person does not receive effective treatment. 

Rheumatism is an umbrella term for several conditions in which there is inflammation in the connective tissues of the body, such as the joints, cartilage, and muscles. Common rheumatic diseases include rheumatoid arthritis, gout, and fibromyalgia.

Exposure to group A strep can cause rheumatic fever, in which there is widespread inflammation throughout the connective tissues, including the heart valves. Sometimes, there is scarring and permanent damage. 

Heart valves

The heart contains four valves that open and close with every heartbeat:

  • aortic valve
  • tricuspid valve
  • mitral valve
  • pulmonary valve

The valves keep blood flowing in the right direction. Problems with any of the valves can cause the blood to flow backward. This is regurgitation. Another possible problem is stenosis, which is when the valve opening is narrower than usual.

In low and middle income countries, rheumatic heart disease accounts for 15–20% of cases of acquired heart disease, which is any heart disease that develops after birth.

What causes rheumatic heart disease? 

Rheumatic heart disease may occur after rheumatic fever. Rheumatic fever may develop if someone does not receive adequate treatment with antibiotics for a group A strep infection. Group A strep infections include strep throat and skin infections. Rheumatic fever can develop from asymptomatic group A strep infection, too.

The Centers for Disease Control and Prevention (CDC) note that it usually takes 1–5 weeks for rheumatic fever to develop after a strep infection.

Rheumatic fever is generalized inflammation. The body’s immune response to the previous strep infection causes it.

Exactly how group A strep causes the inflammatory response is not clear. However, experts believe that the antibodies and inflammatory molecules that the immune system makes in response to the bacteria also damage heart valve tissue. Some people may be more prone than others to rheumatic fever, possible due to genetic factors.

Over time, inflammation in the heart may cause irreversible damage, such as the valves stiffening, changing shape, or shortening. The mitral valve is the most commonly affected valve.

Risk factors

Risk factors include:

  • being ages 5–14 years
  • exposure to S. pyogenes infection
  • repeated strep infections
  • living in crowded conditions
  • living in under-resourced communities
  • having limited access to medical care
  • low socioeconomic status and inequalities in healthcare
  • malnutrition

Children living in low or middle income countries without access to antibiotic treatments are more likely than children in resource-rich countries to develop rheumatic heart disease.

What are the symptoms of rheumatic heart disease? 

Rheumatic heart disease produces several symptoms relating to heart valve damage. In time, heart valve damage can lead to heart failure and other cardiovascular conditions. The signs and symptoms can reflect heart failure, as well as the disruption of blood flow through the valve.

These symptoms may include:

  • swelling in the lower legs, ankles, or feet
  • shortness of breath at rest and with activity
  • abdominal distension, which is when the belly is swollen
  • the inability to lie flat due to feeling short of breath, known as orthopnea
  • chest pain

Clinical signs of rheumatic heart disease include unusual heart sounds such as a heart murmur and an irregular heartbeat and rhythm.

These signs and symptoms can be due to other conditions besides rheumatic heart disease.

What are the tests to diagnose rheumatic heart disease?

Doctors will begin the diagnosis by reviewing the person’s medical history and performing a physical exam. They may ask whether the person has had a recent strep infection or symptoms of rheumatic fever.

During the physical exam, the medical professional may hear unusual heart sounds and possibly an irregular heartbeat and rhythm. 

Tests may include:

  • EKG: This test involves looking at the electrical activity of the heart.
  • Chest X-ray: An X-ray of the chest may show signs of an enlarged heart or fluid buildup in the heart and lungs.
  • Echocardiogram: This is an ultrasound of the heart. It may show how well the valves are opening and closing during each heartbeat. It can also reveal how blood flows through the heart. An echocardiogram is helpful because it can show changes in the valves that are specific to rheumatic disease.

Learn about heart valve disorders.

What are the treatments for rheumatic heart disease?

Currently, there is no cure for rheumatic heart disease. Instead, doctors focus on two aspects: long-term management and prevention. The severity of the disease will determine the treatment plan.

Disease management

The goals of long-term management include:

  • Treating existing rheumatic fever: This involves medications that suppress the body’s inflammatory response, which helps prevent heart damage. Doctors also prescribe antibiotics to treat any strep infection.
  • Relieving the symptoms: Medications can help relieve the symptoms of heart failure due to rheumatic heart disease. These include:
    • angiotensin converting enzyme inhibitors
    • diuretics
    • beta-blockers
    • anticoagulants, commonly known as blood thinners
  • Repairing the valve or valves: If heart valve damage is severe, surgery may be necessary to repair or replace the valve.

Prevention

The prevention of rheumatic heart disease starts with the diagnosis of strep infections and appropriate antibiotic treatment.

Sometimes, doctors prescribe antibiotics to prevent strep infections and rheumatic fever. This is called prophylactic antibiotic therapy. According to the CDC, it may involve penicillin injections every few weeks or oral medications to prevent recurrent infection. This may continue for many years.

What are the complications?

Some people do not know that they have rheumatic heart disease until late adulthood, when complications of valve disease arise. 

Without treatment, rheumatic heart disease may lead to various complications, such as:

What is the outlook for people with rheumatic heart disease?

Life expectancy with rheumatic heart disease depends on disease severity, treatment, and socioeconomic factors, as well as the person’s age.

Worldwide, at least 200,000–250,000 premature deaths occur each year due to rheumatic heart disease. The majority of these are in countries where children and young adults do not receive adequate medical attention due to inequalities in healthcare. 

In a long-term study involving 2,960 people aged 15–52 years with rheumatic heart disease from low and middle income countries, 500 died within 2 years. The median age at death was 28.7 years.

In the United States, people are living longer with rheumatic disease as a result of valve surgery and treatment of heart failure. 

Frequently asked questions

Below are the answers to some common questions about rheumatic heart disease. Uzochukwu Ibe, MD, MPH, has reviewed them.

Is rheumatic heart disease fatal?

Rheumatic heart disease may be fatal without treatment, as serious cardiac complications can arise.

What is the most common complication of rheumatic heart disease?

The most common complications of rheumatic heart disease include heart failure, pulmonary hypertension, and atrial fibrillation.

Summary

Rheumatic heart disease is the result of long-term damage to the heart valves due to rheumatic fever. This preventable disease is of international importance. It occurs predominantly in low income and middle income countries, causing cardiac complications and disability. 

Rheumatic fever sometimes occurs after an untreated strep infection. Children in countries lacking resources are most at risk. Without monitoring and treatment, rheumatic heart disease can lead to serious cardiac complications, including heart failure. In some cases, it can be fatal. 

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Medical Reviewer: Uzochukwu Ibe, MD, MPH
Last Review Date: 2022 Sep 26
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