What is rheumatic heart disease?

One of the complications rheumatic fever causes is rheumatic heart disease (RHD) in young adults and children. In fact, it is the most critical acquired heart disease that results from throat (pharynx) infection by beta-hemolytic streptococcal bacteria. In other words, rheumatic heart disease is a systemic immune condition accounting for 250,000 young patients’ death annually. Actually, RHD is prevalent in endemic countries where it constructs 15-20% of heart failure cases.

What causes rheumatic heart disease?

Streptococcus pyogenes is a streptococcal group A pathogen that causes acute rheumatic fever. Consequently, rheumatic fever on its own is responsible for valvular damage caused by the unusual immune response against the pathogen. Besides, one or recurrent rheumatic fever episodes are behind the disease and its twice common in women. To clarify, rheumatic fever deforms the cusps of the valves and makes them rigid, fuses the commissures together, or shortens and fuses the chordae tendineae together.

The valves involved with rheumatic heart disease are:

• In case it’s chronic, 50-60% of cases have their mitral valve affected.
• 20% of cases have lesions targeting both the mitral and the aortic valves.
• Along with mitral or aortic disease, 10% of cases involve the tricuspid valve.
• It is rare for the pulmonary valve to be infected.

Who is at high risk for the disease?

• People with poor health care and unabated exposure to group A streptococci are the biggest proportion of RHD patients. For instance, Sub-Saharan African children are 6-7% of the globe rheumatic heart disease
• The risk increase with age (the 5 years old are at a lower risk than the 16 years old)
• Several infections by group A streptococcus cause rheumatic fever which leads to rheumatic heart disease.

How long does it take for rheumatic heart disease to occur after infection?

RHD usually develops about 3 weeks after group A streptococci causes pharyngitis. Not only it can affect the heart, but it also have an effect on the brain, joints and skin. As a result of recurrent rheumatic fever episodes, the heart valves may suffer from progressive fibrosis that can eventually cause rheumatoid valvular heart disease. Finally, if not treated, this ultimately leads to heart failure and death.

Symptoms of rheumatic heart disease:

• Swelling in the knees and ankles.
• Pain in the chest or tightness.
• Breath shortness or dyspnea where the patient feels suffocation.

Pathophysiological Characteristics of RHD:

• Aschoff nodules containing aschoff bodies due to heart muscle inflammation
• MacCallum plaques on the valves and the left atrium sub-endocardium

Diagnosis of rheumatic heart disease:

Just like any other throat bacterial infection, you may test for group A streptococcus via bacteria culture or a blood test. Moreover, valvular heart disease, congestive heart failure, myocarditis, and arrhythmias such as atrial fibrillation are among the clinical exhibitions of RHD. Therefore, heart examination are generally as follows:

• Electrocardiogram (EKG) is used to display first, second and third-degree of atrial valve (AV) blockage. Thus, this examination confirms myocarditis.
• Chest x-ray is necessary to assess congestive heart failure by examining for pulmonary vascular congestion or cardiomegaly.
• Transthoracic echocardiogram for physical examination is more trustworthy than auscultation. Notably, subclinical rheumatic heart disease is a RHD detected on the echocardiogram without there being a murmur or auscultation.
• MRI (magnetic resonance imaging) for observation of the heart.

Patients with rheumatic fever:

A close management of the patient heart condition is what prevents rheumatic heart disease. Certainly, check with a cardiologist if you start having any of the following conditions:

• Heart failure symptoms
• A murmur discovered newly
• Arrhythmia
• Valvular disease

Treatment of rheumatic heart disease:

Without doubt, to treat is to prevent and manage the disease.

-To prevent: it is vital to recognize group A streptococcal pharyngitis and stop it once a for all through treatment before it develops into rheumatic fever. The treatment here is done by intramuscular benzathine penicillin G.

-To manage: once you confirm acute rheumatic fever, the major direction of treatment is towards repressing the heart and joints inflammation. Among the cost-effective measures to avoid RHD progression, the World Federation suggests secondary prophylaxis every 3-4 weeks by administering penicillin injections.

-To treat rheumatic mitral stenosis when no regurgitation, arrhythmias, or left atrial thrombus are present, percutaneous mitral balloon valvuloplasty is a major approach.

-In case of severe valvular disease, valve replacement or repair through surgery is the standard approach.

-If the valve disease leads to heart failure, angiotensin-converting enzyme (ACE) inhibitors, beta-blockers and diuretics are given to the patient.

Rheumatic heart disease complications:

In general, latent Rheumatic heart disease does not show symptoms until it progresses into cardiac complications as late adulthood is reached.

The common complications are:

• Heart failure as the heart muscles fails to pump blood.
• Infective endocarditis in which the endocardial surfaces of the heart are infected.
• Pulmonary hypertension where high blood pressure exerts an effect on lungs’ arteries and the right part of the heart.
• Atrial fibrillation that comes from severe and ignored valvular disease.

When to see a doctor:

If you suffer from rheumatic RHD due to rheumatic fever, you must take antibiotic prophylaxis as prescribed by your doctor. However, even if you feel better, do not ignore the medication without consulting your doctor. Nonetheless, this serious disease must always be followed-up with a specialist. As a matter of fact, a doctor will check the patient’s compliance with the antibiotics and search for solutions to help the patients not having further complications.

Pregnancy and rheumatic heart disease:

Take into consideration that RHD is twice in women. To illustrate, RHD is the leading cause of maternal death in endemic regions, so it may be difficult to meet the Sustainable Development Goal 3 target of reducing maternal mortality by 2030 if RHD for women and girls is not a more priority.

Summary:

• Rheumatic heart disease is the outcome of rheumatic fever.
• Indeed, rheumatic fever is the result of throat (pharynx) infection by group A streptococcus.
• RHD is a systemic immune disease accounting for 250,000 children and adolescents mortality each year.
• People living in extreme poverty has low or no chance to avoid the infection and then the disease. Therefore, they are at a high risk.
• Without doubt, multiple infections poses a great risk to acquiring the disease.
• Mitral valve, aortic valve and the tricuspid valve are all involved with the disease but not at the same level.
• It takes about 3 weeks for the disease to develop after infection.
• Electrocardiogram (EKG), chest x-ray, blood test, echocardiogram and throat culture are the mainstay of diagnosis.
• Limit exposure to group A streptococcus, and check with a doctor if you have doubts.
• Prevention of the disease is by antibiotics usage such as intramuscular benzathine penicillin G.
• Surgery or valve replacement treats valvular disease.
• Complications of the disease are heart failure, pulmonary hypertension, infective endocarditis, and atrial fibrillation.
• Always follow up with a doctor to avoid dangerous outcomes, and take your antibiotics as prescribed.

References:

-Dass C, Kanmanthareddy A. Rheumatic Heart Disease. [Updated 2021 Jul 31]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-.

-Vaughan G, Dawson A, Peek M, Sliwa K, Carapetis J, Wade V, Sullivan E. Rheumatic Heart Disease in Pregnancy: New Strategies for an Old Disease? Glob Heart. 2021 Dec 20;16(1):84. doi: 10.5334/gh.1079. PMID: 35141125; PMCID: PMC8698226.