Rheumatic heart disease

RHEUMATIC HEART DISEASE

Rheumatic heart disease is damage to the heart and its valves caused by rheumatic fever an inflammatory disease that can develop as a complication of inadequately treated strep throat or scarlet fever. This damage may affect the aortic valve, the mitral valve or both causing the valves to leak or become narrowed over time.

Rheumatic heart disease (RHD) is a serious disease of the heart involving damage to one or more of the four small heart valves. The valve damage remains after an illness called acute rheumatic fever (ARF). During ARF the heart valve tissue, and sometimes other parts of the heart (the heart lining or muscle) can become swollen, and this is called carditis. Following carditis, the heart valves can remain damaged then become scarred, and the result is an interruption to normal blood flow through the damaged valves. Some blood may flow backward through a leaky valve that does not close properly, or blood may be blocked because a tight, scarred valve does not open properly. When the heart is damaged in this way, the heart valve is not able to function adequately, and heart surgery may be required to repair or replace the damaged valve. Regular antibiotics are prescribed for people with RHD to prevent recurrent ARF and subsequent worsening of the valve damage

What is rheumatic fever

Rheumatic fever is an inflammatory disease that can be triggered by a streptococcal bacterial infection. It usually starts out as a strep throat infection or scarlet fever that hasn't been treated with antibiotics. Rheumatic fever can cause inflammation of connective tissues throughout the body including the heart, joints, brain or skin.

Rheumatic fever can also affect the heart muscle, a condition known as myocarditis, or the outer covering (membrane) of the heart, a condition known as pericarditis.

Symptoms of rheumatic heart disease can appear 10 to 20 years after the original illness. Underlying rheumatic heart disease can be a cause of cardiac complications during pregnancy.

TYPES OF RHEUMATIC HEART DISEASE

Every part of the heart may be damaged by inflammation caused by rheumatic fever. That includes the outer sac (pericardium), the inner lining (endocardium) and the valves.

Some heart problems linked to rheumatic fever are:

• Valvular heart disease
• Pericarditis
• Endocarditis
• Heart block

The most common form of rheumatic heart disease affects the heart valves. It may take several years after an episode of rheumatic fever for valve damage to develop or symptoms to appear.

Although rheumatic fever can affect any heart valve, it most commonly affects the mitral valve which lies between the two chambers of the left side of the heart. The damage can cause valve stenosis, valve regurgitation and/or damage to the heart muscle.

• Valve stenosis occurs when there is narrowing of a valve, which restricts blood flow.
• Valve regurgitation is when blood leaks backward through a valve, instead of following its usual direction.
• The inflammation of rheumatic fever can damage the heart muscle itself. The damage can affect the heart's ability to pump blood effectively.

CAUSES RHEUMATIC HEART DISEASE

Rheumatic heart disease is caused by rheumatic fever, an inflammatory disease that can affect many connective tissues, especially in the heart, joints, skin, or brain. The heart valves can be inflamed and become scarred over time. This can result in narrowing or leaking of the heart valve making it harder for the heart to function normally. This may take years to develop and can result in heart failure.

SYMPTOMS OF RHEUMATIC HEART DISEASE

A recent history of strep infection or rheumatic fever is key to the diagnosis of rheumatic heart disease. Symptoms of rheumatic fever vary and typically begin 1 to 6 weeks after a bout of strep throat. In some cases, the infection may have been too mild to have been recognized, or it may be gone by the time the person sees a doctor.

These are the most common symptoms of rheumatic fever:

• Fever
• Swollen, tender, red and extremely painful joints - particularly the knees and ankles
• Nodules (lumps under the skin)
• Red, raised, lattice-like rash, usually on the chest, back, and abdomen
• Shortness of breath and chest discomfort
• Uncontrolled movements of arms, legs, or facial muscles
• Weakness

Symptoms of rheumatic heart disease depend on the degree of valve damage and may include:

• Shortness of breath (especially with activity or when lying down)
• Chest pain
• Swelling

WHO IS AT RISK

Rheumatic fever is rare in developed countries, but one Canadian study showed that people living in certain Indigenous communities have a higher risk of contracting rheumatic fever. There is also a higher risk for people who have emigrated from countries where rheumatic fever is more common. People who have had repeated strep infections are also at a higher risk of developing rheumatic heart disease.

People of any age can experience a short bout of rheumatic fever, but it is most common between the ages of 5 and 15. Any child with a persistent sore throat should have a throat culture to check for strep infection.

About 60% of people with rheumatic fever go on to develop rheumatic heart disease. Antibiotics can prevent streptococcal infection from developing into rheumatic fever.

COMPLICATIONS

Some complications of rheumatic heart disease include:

Heart failure. This can occur from either a severely narrowed or leaking heart valve.

Bacterial endocarditis. This is an infection of the inner lining of the heart, and may occur when rheumatic fever has damaged the heart valves.

Complications of pregnancy and delivery due to heart damage. Women with rheumatic heart disease should discuss their condition with their healthcare provider before getting pregnant.

Ruptured heart valve. This is a medical emergency that must be treated with surgery to replace or repair the heart valve.

WHEN TO SEE A DOCTOR

Have your child see a doctor for signs or symptoms of strep throat, which include:

Sore throat that comes on suddenly

Pain when swallowing

Fever

Headache

Stomach pain, nausea and vomiting

DIAGNOSIS

People with rheumatic heart disease will have or recently had a strep infection. A throat culture or blood test may be used to check for strep.

They may have a murmur or rub that may be heard during a routine physical exam. The murmur is caused by the blood leaking around the damages valve. The rub is caused when the inflamed heart tissues move or rub against each other.

Along with a complete medical history and physical exam, tests used to diagnose rheumatic heart disease may include:

Echocardiogram (echo). This test uses sound waves to check the heart's chambers and valves. The echo sound waves create a picture on a screen as an ultrasound transducer is passed over the skin overlying the heart. Echo can show damage to the valve flaps, backflow of blood through a leaky valve, fluid around the heart, and heart enlargement. It's the most useful test for diagnosing heart valve problems.

Electrocardiogram (ECG). This test records the strength and timing of the electrical activity of the heart. It shows abnormal rhythms (arrhythmias or dysrhythmias) and can sometimes detect heart muscle damage. Small sensors are taped to your skin to pick up the electrical activity.

Chest X-ray. An X-ray may be done to check your lungs and see if your heart is enlarged.

Cardiac MRI. This is an imaging test that takes detailed pictures of the heart. It may be used to get a more precise look at the heart valves and heart muscle.

Blood tests. Certain blood tests may be used to look for infection and inflammation..

TREATMENT

Specific treatment for rheumatic heart disease will be determined by your physician based on:

• your overall health and medical history
• extent of the disease
• your tolerance for specific medications, procedures, or therapies
• expectations for the course of the disease
• your opinion or preference

Since rheumatic fever is the cause of rheumatic heart disease, the best treatment is to prevent rheumatic fever from occurring. Penicillin and other antibiotics can usually treat strep throat (a streptococcus a bacterial infection) and stop acute rheumatic fever from developing.

Persons who have previously contracted rheumatic fever are often given continuous (daily or monthly) antibiotic treatments, possibly for life, to prevent future attacks of rheumatic fever and lower the risk of heart damage.

Antibiotic therapy has sharply reduced the incidence and mortality rate of rheumatic fever/rheumatic heart disease. To reduce inflammation, aspirin, steroids, or non-steroidal medications may be given. Surgery may be necessary to repair or replace the damaged valve.

KEY POINTS

• Rheumatic heart disease is a condition in which the heart valves have been permanently damaged by rheumatic fever.
• Rheumatic fever is an inflammatory disease that can affect many connective tissues, especially in the heart.
• Untreated or under-treated strep infections put a person at increased risk. Children who get repeated strep throat infections are at the most risk for rheumatic fever and rheumatic heart disease.
• A recent history of strep infection or rheumatic fever is key to the diagnosis of rheumatic heart disease. Symptoms of rheumatic fever vary and typically begin 1 to 6 weeks after a bout of strep throat.
• People with rheumatic heart disease may have a murmur or rub that may be heard during a routine physical exam.
• Treatment depends on how much damage has been done to the heart valves. It may even include surgery to replace or repair a badly damaged valve.
• Since rheumatic fever is the cause of rheumatic heart disease, the best treatment is to prevent rheumatic fever by using antibiotics to treat strep infections.

RHD IN CHILDREN

Causes of rheumatic heart disease in a child.

Rheumatic heart disease is caused by rheumatic fever. Rheumatic fever is a complication of an untreated or under-treated strep infection.

Children at risk for rheumatic heart disease

Rheumatic heart disease is uncommon in the U.S., because rheumatic fever is also not common. Rheumatic fever occurs more often in children between ages 5 and 15. This is especially true if they have had frequent cases of strep throat. Poor access to medical care is a risk factor for rheumatic heart disease as strep infections are more likely to be missed and go untreated.

Symptoms of rheumatic heart disease in a child

• Your child may have signs and symptoms that affect the heart, including:
• Trouble breathing
• Chest pain
• Swelling (edema) of the feet and ankles
• Heart murmur

The child may also have other signs and symptoms of rheumatic fever. These include:

• Joint pain and swelling
• Rash
• Small, hard, round bumps under the skin (nodules)
• Irregular or jerky movements
• Belly (abdominal) pain
• Bloody nose
• Fever

The symptoms of rheumatic heart disease can be like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.

When I should call the child's doctor

Call your child's healthcare provider if your child has any of the following:

• Fever
• Trouble breathing
• Chest pain
• Swelling (edema) of the feet or ankles
• Another sore throat.

Next steps

Before you agree to the test or the procedure for your child make sure you know:

• The name of the test or procedure
• The reason your child is having the test or procedure
• What results to expect and what they mean
• The risks and benefits of the test or procedure
• When and where your child is to have the test or procedure
• Who will do the procedure and what that person's qualifications are
• What would happen if your child did not have the test or procedure
• Any alternative tests or procedures to think about
• When and how will you get the results
• Who to call after the test or procedure if you have questions or your child has problems
• How much will you have to pay for the test or procedure

How is rheumatic heart disease diagnosed in a child

The healthcare provider will ask about your child's symptoms and health history, including having rheumatic fever or strep infections. He or she will give your child a physical exam. Your child may also have tests, such as:

• Throat culture
• Electrocardiography. A test to measure the electrical activity of the heart.
• Echocardiography. An imaging test that uses sound waves (ultrasound) to make detailed pictures of the heart.
• Blood tests

Treating rheumatic heart disease in a child

Treatment will depend on your child's symptoms, age, and general health. It will also depend on how severe the condition is. Your child's healthcare provider will likely refer you to a pediatric cardiologist. This is a doctor with special training to treat heart problems in children. Your child may also see other specialists, depending on his or her symptoms.

Children with rheumatic heart disease will need to rest until their symptoms get better.

The child's doctor may prescribe one or more of these medicines:

• Antibiotics to treat the acute strep infection
• Long-term antibiotics to prevent recurrent strep infection
• Steroids or nonsteroidal anti-inflammatory medicines to ease inflammation in the heart and in other parts of the body
• Water pills (diuretics) if heart failure develops
• Anti-inflammatory medicine for the management of fever and arthritis symptoms
• Your child may also need other medicines. Some children need surgery to fix or replace damaged heart valves.

Possible complications of rheumatic heart disease in children;

Complications of rheumatic heart disease in children include:

• Permanent heart damage
• Acute or chronic heart valve disease
• Heart failure
• Infection in the heart (endocarditis)

WOMEN GIRLS & RHD IN PREGNANCY

During pregnancy, the work of the heart is increased by up to 50%. For women who have RHD there are several considerations:

• Added stress on the heart from pregnancy can result in the development of RHD symptoms, where previously there were no symptoms, or the existence of RHD was unknown. This may result in unexpected complications for mother and baby. If a pregnant woman develops heart failure symptoms, experiences unexplained shortness of breath, or needs to sleep with two pillows to assist breathing, the possibility of RHD should be considered and investigated. The earlier RHD is diagnosed and managed, the less likelihood of complications during pregnancy.

• Added stress on the heart from pregnancy can result in the worsening of existing RHD symptoms. Women living with RHD need to be monitored carefully before and during pregnancy by a multi-disciplinary team which includes obstetric and cardiology specialists.
• Added stress on the heart can place extra pressure on repaired or mechanical heart valves.

Timing of heart valve surgery and future pregnancies should be part of a comprehensive early management plan for all girls and young women with RHD.

• Anticoagulation therapy poses a risk to mother and baby, and needs to be carefully monitored and regulated throughout pregnancy and delivery. Anticoagulation is required for all girls and women with mechanical prosthetic valves, and may be prescribed if there is atrial fibrillation. Options for anticoagulation during pregnancy requires careful balancing of risk following individual assessment.2

Transitional care

First episodes of ARF are most common among children aged 5 to 14 years, and diagnosis of RHD occurs from the age of 5, and into the 45-50-year age group.4 Therefore, child to adult transitional care that addresses the standards for quality healthcare in adolescents.5 is critical for the continued care of young women who desire future pregnancies. Planning for adulthood should include reproductive health and preconception care, as well as the transition to adult cardiovascular care.

Preconception counselling is important for all young women who have had acute rheumatic fever (ARF) and RHD. It provides an opportunity for young women and their families to talk with midwives and other health professionals about risks during pregnancy, how to minimise complications, contraception options, and being able to make informed choices for birthing options. Obstetric and related planning and care for young Aboriginal and Torres Strait Islander women should be provided within a cultural safety framework and include members of the Aboriginal and Torres Strait Islander health workforce.

Antibiotic prophylaxis during pregnancy

Secondary prophylaxis in the form of regular penicillin injections helps prevent recurrent ARF. Penicillins do cross the placenta in low concentrations, however, there is no evidence that penicillins have any teratogenic effects on the fetus. Developing ARF while pregnant poses a very high risk of additional cardiac complications; therefore, women who have had ARF, and women who have RHD should continue penicillin injections during pregnancy and while breastfeeding, as indicated