Diagnosis and Management of Acute Rheumatic Fever and Rheumatic Heart Disease

1. Diagnosis and Management of Acute Rheumatic Fever and Rheumatic Heart Disease

2. This presentation is intended to support the Curriculum for training health workers and others involved in the diagnosis and management of acute rheumatic fever and rheumatic heart disease. It has been made possible thanks to the support of the Vodafone Group Foundation and the International Solidarity, State of Geneva, and the ongoing support of Menzies School of Health Research, Caritas Australia, Fiji Water Foundation, Cure Kids and Accor Hospitality.

3. IntroductionAcute Rheumatic Fever and Rheumatic Heart Disease

4. Introduction Rheumatic Heart Disease is the most common cause of heart disease in children and young adults • Approximately 15.6 million people affected worldwide • Almost 500,000 new cases each year • Approx 350,000 deaths each year • Most disease occurs in developing countries. Rheumatic Heart Disease is a disease of poverty Rheumatic Heart Disease can be prevented.

5. Definitions Group A beta-haemolytic streptococci(GAS) • Humans are exposed to GAS in the environment • Throat and skin are common sites of GAS infection • GAS infections usually resolve without treatment • Untreated GAS infections can lead to acute rheumatic fever in some people.

6. Definitions Acute Rheumatic Fever (ARF) • A delayed auto-immune response following untreated GAS infection • Develops after the GAS infection has resolved • Commonly affects the joints, heart, central nervous system and skin • Most common between the ages of 5 and 15 years • Can recur following further untreated GAS infections Rheumatic Heart Disease (RHD) • Residual damage to heart valves following recurrent ARF • Valves become scarred, stiff, thickened • Blood leaks (blood flows backwards through valves which do not close properly) • Blood is blocked (blood can not flow through valves which do not open properly)

7. Risk factors ARF include Poverty Poor housing, overcrowded housing Lack of adequate health care Untreated GAS infections Risk factor for RHD Recurrent ARF Prevention The first episode of ARF can be prevented by treating GAS infections with penicillin(primary prophylaxis) If the first ARF episode is not prevented, recurrent ARF can be prevented with long-term penicillin (secondary prophylaxis) Risk Factors

8. Disease Progression

9. ARF and RHD can be prevented by sustainable control strategies including Trained health staff who diagnose and management disease effectively Secondary prophylaxis to prevent further ARF and the development or worsening of RHD. Community education and awareness Screening for unknown RHD in the community. Control strategies should focus on Prompt identification and treatment of GAS infections Identifying people who have had ARF once and preventing further ARF and the development of RHD. Control of Disease

10. Acute Rheumatic FeverDiagnosis and Management

11. Revised Jones Criteria ARF can be confirmed if certain signs and symptoms are present. The Revised Jones Criteria (below) can help guide the diagnosis. MAJOR Criteria - signs and symptoms more often associated with ARF MINORCriteria- signs and symptoms that help support the diagnosis Evidence of recent GAS Infection is required

12. Revised Jones Criteria The World Health Organisation set the international standard for diagnosis of ARF. First episode or recurrent episode of ARF (no RHD): • 2MAJORmanifestations or1MAJORand2MINORmanifestations and • Evidence of preceding Group A streptococcal infection … (within 3 weeks before ARF symptoms) ARF (with existing RHD): • 2MINORmanifestations and • Evidence of preceding Group A streptococcal infection … (within 3 weeks before ARF symptoms) ** Individual country guidelines also exist **

13. Signs and Symptoms MAJOR Manifestations Arthritis • Painful, swollen joints (usually knees, ankles, wrists, elbows) • Very common with ARF, often the first symptom • Usually ‘migratory’- disappears from one joint as it starts in another (poly-arthritis), however may just be present in one joint (mono-arthritis). • Carditis • May present as a heart ‘murmur’ • Chest pain and/or difficulty breathing may be present in more severe cases

14. Signs and Symptoms Sydenham’s chorea • Twitchy, jerking movements and muscle weakness (most obvious in the face, hands and feet) • May occur on both sides or only one side of body • More common in teenagers and females (rare after age 20) • May be associated with irritability and or depression • May begin up to 3-4 months after the streptococcal throat infection, and often occurs without other symptoms • Usually resolves within 6 weeks (may last 6 months or more) • May recur in females during pregnancy

15. Signs and Symptoms Subcutaneous nodules • Painless lumps on the outside surfaces of elbows, wrists, knees, ankles in groups of 3-4 (up to 12) • The skin is not red or inflamed • Last 1-2 weeks (rarely more than 1 month) • Nodules are more common when Carditis is also present. Erythema marginatum • Painless, flat pink patches on the skin that spread outward in a circular pattern • Usually occurs early, may last months, rarely lasts years • Usually on the back or front of body, almost never on the face • Hard to see in dark-skinned people.

16. Signs and Symptoms MINOR Manifestations Fever • Occurs in the majority of cases, usually with the onset of symptoms • Usually ranging from 38.4 – 40.0º C (101-104º F) Arthralgia • Usually involves large joints • May be mild or severe Group A streptococcal infection • Group A beta-haemolytic streptococci may not be seen on a throat swab since the infection may be resolved at the time of onset of ARF symptoms. • ASOT – serum reaches a peak level around 3-6 weeks after infection and starts to fall at 6-8 weeks • Anti DNase B – reaches a peak level up to 6-8 weeks after infection and starts to fall at around 3 months after the infection. ** Normal antibody titre ranges vary with age and geography **

17. Difficulties with ARF Diagnosis A combination of signs and symptoms is required to confirm ARF People with ARF do not always present to the health system with symptoms because • Symptoms may not be considered serious • Transport to the health facility may be difficult Health staff may not recognise the signs and symptoms of ARF ARF may be confused with other illnesses, for example • Sore joints may be confused with a sports injury or ‘growing pains’

18. Treatment for ARF Treat the acute illness • Benzathine penicillin G injection or • Oral Penicillin for 10 days Relieve symptoms • Bed rest • Relief of arthritis, pain and fever (Paracetamol or Aspirin) • Treat chorea (use Carbamazepine or Valproic acid if severe) • Anti-heart failure medication (e.g. Diuretics, ACEi, Digoxinif required)

19. ARF Management Plan First dose of Benzathine penicillin G (start secondary prophylaxis) Baseline echocardiogram (if available) ARF alert on medical notes & computer systems (if applicable) Education for person and family Refer to local doctor / health facility Dental examination Long-term secondary prophylaxis plan

20. Management of Probable ARF • Treat the symptoms } • Dose of Benzathine penicillin G } as for ARF • Echocardiogram(if available) } Medical officer review after one month, and Repeat echocardiogram(if available) • If NOT ARF…cease Benzathine but monitor for ARF symptoms • If ARF… continue Benzathine and manage as for ARF

21. Summary The Jones Criteria is used to guide the diagnosis of ARF with a combination of MAJOR Manifestations, MINOR Manifestations and evidence of recent GAS Infection A long-term Management Plan should be established to prevent recurrence of ARF and development or worsening of RHD Probable ARF cases should also be monitored

22. Secondary Prophylaxisto prevent recurrent ARF

23. Secondary Prophylaxis Secondary prophylaxis is the terms used to describe regular delivery of antibiotics to prevent recurrence of GAS infection and subsequent development of ARF. Secondary prophylaxis is recommended for people who have had ARF, or who have RHD to • Prevent further Group A Streptococcal infections • Prevent recurrence ARF • Prevent the development or worsening of RHD • Reduce the severity of RHD • Help reduce the risk of death from severe RHD.

24. Standard Treatment Benzathine penicillin G 1,200,000 units for ALL people ≥30kg 600,000 units for children <30kg Every 3 or 4 weeks (by intramuscular injection) Penicillin V Given if needles cannot be given due to excessive bleeding 250mg twice daily (by mouth) Erythromycin Given if Penicillin allergy has been confirmed by a Medical Officer 250mg twice daily (by mouth)

25. Considerations When should secondary prophylaxis be considered? • ARF confirmed by the Revised Jones Criteria • RHD confirmed on echocardiogram • ARF or RHD not confirmed by the Revised Jones Criteria, but considered highly ‘probable’ Precautions • Do not give Benzathine Penicillin G or Penicillin V if there is a documented Penicillin allergy • Drug reactions are rare Continue secondary prophylaxis during pregnancy Continue secondary prophylaxis during anticoagulation (e.g. with Warfarin)

26. Guidelines for Secondary Prophylaxis Length of time for secondary prophylaxis depends on a number of factors including • Age at first diagnosis of ARF (or RHD) • Severity of disease • If carditis was present with first ARF • Time (years) since last ARF illness • Ongoing risk factors (e.g. level of poverty) • If medication is received regularly World Health Organisation guidelines for secondary prophylaxis duration: ** Secondary prophylaxis guidelines may vary **

27. Ceasing Secondary Prophylaxis Secondary Prophylaxis should only be ceased following: No ARF signs/symptoms for at least 5 years, and Medical Specialist review (Paediatrician / Physician / Cardiologist) and Echocardiogram to establish presence & severity of RHD (if available)

28. Benzathine Penicillin injection delivery Assessment and Preparation • Confirm person’s identity • Review known drug allergies • Discuss and record any recent ARF or RHD symptoms (refer to medical officer if required) • Obtain consent for injection

29. Benzathine Penicillin injection delivery Check medication name, dose and expiry date Prepare medication according to the product information • Administer 1,200,000 units for all persons ≥ 30kg • Administer 600,000 units for small children <30kg Administer with a size 23-gauge needle Dispose of used needles and syringes in a puncture-proof container. Use a new needle and syringe for each injection Administer medication immediately after preparation

30. Documentation Record in the Benzathine penicillin injection book and/or medical notes • Dose and batch number • Date given and date next due • Signature (of person giving injection) Record next date due on a reminder card(if applicable)

31. Calculating Injection Delivery • Record the number of injections PRESCRIBED for the full year • 13 injections should be given each year if prescribed every 4 weeks • 17 injections should be given each year if prescribed every 3 weeks • Count the number of injections GIVEN in the full year 3. Calculate the number of injections GIVEN (10) divided by the number PRESCRIBED (13) and multiply by 100. EXAMPLE: If 13 injections are PRESCRIBED, and 10 were GIVEN: (10 ÷ 13) x 100 = 77%RECEIVED In this example, 77% of injections were RECEIVED for the individual for the year.

32. Notes on Injection Delivery Receiving less than 80% of injections places an individual at higher risk of recurrent ARF • Follow-up may be required If injections were PRESCRIBED for the full year but none were GIVEN, record 0%. Receiving less than 50% of injections places an individual at extreme risk of recurrent ARF and progression of RHD • Immediate intervention is required for this individual.

33. Factors affecting Injection Delivery Relationship between the person with ARF/RHD & the health system Education of the person, family & health workers Person / family refusing treatment Person forgetting treatment Difficulty traveling to health facility Pain and fear of injections Health staff workloads and priorities Alternative therapy use / distrust of health service

34. Strategies to improve Injection Delivery Appoint a dedicated staff member at each clinic to oversee secondary prophylaxis coordination Identify people who need secondary prophylaxis Identify local health facility for each person Develop systems for follow-up Provide ongoing education for people who require injections and their families Communicate with local RHD programme and other health service providers Reduce injection pain Discuss alternative therapy issues

35. Penicillin Allergy Symptoms • Skin rash • Itchy eyes Treatment Antihistamine (oral or injection)

36. Anaphylaxis Symptoms • Wheezing • Hives • Itching • Swelling of the face and lips • Difficulty breathing • Vomiting • Falling Blood pressure • Loss of consciousness • Cardiac arrest Treatment Adrenaline (subcutaneous injection)

37. Summary Antibiotics need to be present in the body at all times to help prevent GAS infections and prevent recurrent ARF Benzathine penicillin injections should be given unless there are contraindications to injections or documented penicillin allergy Medical Specialist review is required before ceasing secondary prophylaxis Strategies to improve secondary prophylaxis delivery: • Good relationships between community and health staff • Education for the community and health staff • Systems for follow-up • Communication between health services • Reduce injection pain DocumentBenzathine Penicillin injections and monitor injection delivery

38. Rheumatic Heart DiseaseDiagnosis and Management

39. Rheumatic heart disease is the result of damage to the heart valves which occur after repeated episodes of ARF Early diagnosis and treatment of RHD are important to prevent progression of disease Signs and symptoms may not develop for many years The aim of RHD management is to prevent or delay heart valve surgery RHD can be prevented if ARF is diagnosed and managed early. 50% of people with RHD donot remember having ARF Introduction

40. Definitions Valve Regurgitationsuggests that heart valves • Are thickened and sticky against the walls of the heart • Do not meet in the middle • Leak (the blood flows backwards over the valve) Valve Stenosissuggests that heart valves • Become stuck to each other • Do not allow blood to flow through easily (restricted forward flow)

41. Signs and Symptoms of RHD Symptoms of RHD may not develop for many years • A murmur but no symptoms usually suggests mild-moderate disease • Symptoms usually suggest moderate-severe disease Symptoms depend upon the type and severity of disease, and may include • Breathlessness with exertion or when lying down flat • Waking at night feeling breathless • Feeling tired • General weakness • Peripheral oedema

42. Heart valve involvement Mitralvalve is affected in over 90% of cases of RHD • Mitral regurgitation most commonly found in children & adolescents • Mitral stenosis represents longer term chronic disease,commonly inadults • Most common complication of mitral stenosis is atrial fibrillation Aorticvalve next most commonly affected • Generally associated with disease of the mitral valve. • Tends to develop as a long term complication of aortic regurgitation Tricuspidand pulmonaryvalves are much less commonly affected • Usually affected in very severe RHD when all valves are affected

43. Clinical Examination Mitral regurgitation A pansystolic murmur heard loudest at the apex and radiating laterally to the axilla Mitral stenosis A low-pitched, diastolic rumble heard best at the apex with the bell of the stethoscope and with the person lying in the left lateral position. Aortic regurgitation A diastolic blowing decrescendo murmur best heard at the left sternal border with the person sitting up and leaning forward in full expiration. Aortic stenosis A loud, low pitched mid-systolic ejection murmur best heard in the aortic area, radiating to the neck.

44. Investigations Electrocardiogram (ECG) • To determine sinus rhythm Chest X-ray(CXR) • To determine size and placement of heart • To identify cardiac failure (pulmonary congestion) Echocardiography • To identify heart valve damage • To estimate severity of disease • Useful to compare results with future echocardiogram results

45. Key element in RHD Management Secondary prophylaxis Functions of secondary prophylaxis with established RHD • Prevent Group A Streptococcal infections • Prevent the repeated development of ARF • Prevent the development of RHD • Reduce the severity of RHD • Help reduce the risk of death from severe RHD.

46. Elements in RHD Management Effective baseline assessment, education and referral Initial management • heart failure (treatment with diuretics and ACEi) • atrial fibrillation (Digoxin and anti-coagulation) Routine review and structured care planning • Regular secondary prophylaxis • Regular clinical assessment and follow-up echocardiography (if available) • Dental care and Infective endocarditis prophylaxis plan • Family planning referral (for women) • Vaccination (if available) Appropriate surgical intervention Special consideration in particular circumstances(e.g. pregnancy)

47. RHD and Pregnancy The cardiovascular changes which occur during pregnancy may threaten the health of the woman and the foetus. Changes include • increased heart rate and blood volume • reduction in systemic and pulmonary resistance • increased cardiac output. RHD may be identified for the first time during pregnancy. Highest risk of complications immediately after delivery

48. Management of RHD in Pregnancy Management generally includes • restricting physical activity and salt intake • administering secondary prophylaxis (Benzathine penicillin can be continued during pregnancy) • avoiding community-acquired infectious diseases • education about monitoring own signs and symptoms and seeking care if shortness of breath • close monitoring of heart function (specifically in woman who have symptoms of RHD). Special attention should be given to women with high risk RHD including women with • mitral and/or aortic stenosis • atrial fibrillation • prosthetic heart valves • those receiving anticoagulant therapy with warfarin.

49. Infective Endocarditis Infective Endocarditis is a serious complication of RHD Endocarditis is caused by bacteria in the bloodstream. In RHD, endocarditis most commonly occurs in the mitral or aortic valves Uncommonly occurs during dental or surgical procedures but often the source of the infection is not clear May occur after heart valve surgery Antibiotics prior to dental and surgical procedures are given to help prevent endocarditis. All people with ARF and RHD should have regular dental care to prevent dental decay and the potential risk of endocarditis.

50. Procedures that increase risk of Endocarditis