Rheumatic heart disease

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Dr N. T. Munyandu, Specialist Physician
Definition
Rheumatic heart disease is cardiac inflammation and scarring triggered by an autoimmune reaction to infection by group A streptococci. In the early stage, this condition involves inflammation of the three layers of the heart; myocardium, endocardium, and epicardium. Chronic disease is manifested by valvular fibrosis, (scarring) resulting in stenosis and/or insufficiency.
Stages of rheumatic heart disease
1. Acute rheumatic fever (ARF).
2. Chronic rheumatic heart disease (RHD).
Acute rheumatic fever
Rheumatic fever is a late inflammatory, non suppurative complication of pharyngitis (sore throat) that is caused by group A streptococci.
Rheumatic fever results from immune responses occurring 1-3 weeks after the onset of streptococcal pharyngitis .
It is rare before the age of 5 years and after the age of 25 years. Most commonly it occurs between 5 and 15 years.
It is more common in over crowded and poor living conditions and also occurs more commonly if the pharyngitis is not treated properly with an appropriate antibiotic.
The symptoms of acute rheumatic fever include fever, joint swelling and pain, skin rash, skin nodules, breathlessness, tiredness and rarely abnormal movements (chorea). The diagnosis is based on proof of the recent throat infection as well as a set number of clinical and laboratory criteria.
That episode of rheumatic fever marks the beginning of damage to heart valves and if there are recurrent untreated sore throats rheumatic fever recurs causing further damage to heart valves.
Chronic rheumatic heart disease
This is the result of either a severe acute rheumatic fever episode or many recurrent episodes of acute rheumatic fever.
Chronic rheumatic heart disease is estimated to occur in 5-30 million children and young adults worldwide; 90 000 individuals die from this disease each year.
It is the commonest cause of valvular heart disease in developing countries like Zimbabwe.
The interventions to correct valve damage are quite costly. There are potential serious complications and these occur largely in young potentially productive people.
The most commonly affected valve is the mitral valve. There is either stenosis (narrowing which causes restricted blood flow) or regurgitation which is when blood leaks and flows backwards.
The other heart valves can also be affected ie the aortic, tricuspid and pulmonary valves. When there is valvular stenosis or regurgitation the heart has to work extra hard to provide enough blood supply to the tissues.
It may adapt and cope for some years but because of worsening scarring, progress of the disease and extra demands like during pregnancy the heart eventually fails to maintain an adequate output. The patient then develops symptoms of heart failure which include leg swelling, breathlessness, cough, tiredness.
Complications of rheumatic heart disease
1. Heart failure; this is when the heart fails to maintain an adequate blood supply to all tissues. The symptoms include breathlessness, leg swelling, abdominal swelling, cough, tiredness and can lead to death.
2. Infective endocarditis; the damaged valve can get infections easily. The patient will feel tired, have fever and may go into heart failure
3. Stroke this is when there is a sudden onset of neurorologic deficit. It can be leg and or arm weakness, facial weakness, blindness in one eye, loss of consciousness or even death.
4. Irregular heart rhythmn; palpitations,breathlessness, stroke or it may be picked up on electrocardiogram.
Investigations
The tests that are performed on patients suspected to have acute rheumatic fever or chronic rheumatic heart disease include;
1. Throat swab to detect the throat infection in ARF/blood tests to check for group A streptococcal infection.
2. Chest x-ray to determine the size of the heart.
3. Electrocardiogram (ECG) to check heart rhythm.
4. Echocardiogram to determine the structure and function of the valves and heart chambers.
Treatment
Acute rheumatic fever should be treated with antibiotic and pain relief for the joint pains. Once the diagnosis is made the patient should be followed up and be given a regular monthly penicillin injection to prevent any further sore throat by the same infection and avert further heart damage. In case of penicillin allergy an oral alternative antibiotic is used.
Chronic rheumatic heart disease should be identified. If in heart failure treatment will be given for the heart failure. This includes tablets such as frusemide, enalapril, digoxin as well as measures to reduce fluid and salt intake.
If there are no symptoms patients need to be followed up regularly so that timing for surgical valve interventions is appropriate. The definitive treatment for a damaged valve is surgical but this has to be done at the appropriate time which is determined by presence of symptoms and/or findings on tests.
Prevention
Rheumatic heart disease is a preventable disease. There are several levels of prevention.
1. Improvement in socioeconomic status of the population and ensuring that housing is spacious and there is access to primary health care so that streptococcal sore throat can be identified.
2. Primary prevention; Treatment of group A streptococcal infection with an appropriate antibiotic will reduce the incidence of acute rheumatic fever significantly.
3. Secondary prevention; identifying patients with acute rheumatic fever and following them up, administering regular prophylaxis for any further episodes of acute rheumatic fever will prevent serious valvular damage which results from recurrent acute rheumatic fever.
Rheumatic heart disease can potentially be eradicated as what has happened in some developed countries.
In Africa there is an initiative to try and eradicate rheumatic heart disease.
This is summarised in the communiqué below.
The Addis Ababa Communique
Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) remain major causes of heart failure, stroke and death among African women and children, despite being preventable and imminently treatable.
From 21 to 22 February 2015, the Social Cluster of the Africa Union Commission (AUC) hosted a consultation with RHD experts convened by the Pan-African Society of Cardiology (PASCAR) in Addis Ababa, Ethiopia, to develop a “roadmap” of key actions that need to be taken by governments to eliminate ARF and eradicate RHD in Africa.
Seven priority areas for action were adopted:
(1) Create prospective disease registers at sentinel sites in affected countries to measure disease burden and track progress towards the reduction of mortality by 25 percent by the year 2025.
(2) Ensure an adequate supply of high-quality benzathine penicillin for the primary and secondary prevention of ARF/RHD.
(3) Improve access to reproductive health services for women with RHD and other non-communicable diseases (NCD).
(4) Decentralise technical expertise and technology for diagnosing and managing ARF and RHD (including ultrasound of the heart).
(5) Establish national and regional centres of excellence for essential cardiac surgery for the treatment of affected patients and training of cardiovascular practitioners of the future.
(6) Initiate national multi-sectoral RHD programmes within NCD control programmes of affected countries, and
(7) Foster international partnerships with multinational organisations for resource mobilisation, monitoring and evaluation of the programme to end RHD in Africa.
This Addis Ababa communiqué has since been endorsed by African Union heads of state, and plans are underway to implement the roadmap in order to end ARF and RHD in Africa in our lifetime.

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