©2007 World Heart Federation … Updated October 2008 Diagnosis and Management of Acute Rheumatic Fever and Rheumatic Heart Disease

©2007 World Heart Federation … Updated October 2008 Rheumatic Heart Disease Diagnosis and Management

©2007 World Heart Federation … Updated October 2008 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.

©2007 World Heart Federation … Updated October 2008 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 do not remember having ARF Introduction

©2007 World Heart Federation … Updated October 2008 Definitions Regurgitation Valve Regurgitation suggests 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) Stenosis Valve Stenosis suggests that heart valves –Become stuck to each other –Do not allow blood to flow through easily (restricted forward flow)

©2007 World Heart Federation … Updated October 2008 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

©2007 World Heart Federation … Updated October 2008 Heart valve involvement Mitral Mitral valve 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 in adults –Most common complication of mitral stenosis is atrial fibrillation Aortic Aortic valve next most commonly affected –Generally associated with disease of the mitral valve. –Tends to develop as a long term complication of aortic regurgitation Tricuspidpulmonary Tricuspid and pulmonary valves are much less commonly affected –Usually affected in very severe RHD when all valves are affected

©2007 World Heart Federation … Updated October 2008 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. Clinical Examination

©2007 World Heart Federation … Updated October 2008 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

©2007 World Heart Federation … Updated October 2008 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.

©2007 World Heart Federation … Updated October 2008 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) Elements in RHD Management

©2007 World Heart Federation … Updated October 2008 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 RHD and Pregnancy

©2007 World Heart Federation … Updated October 2008 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. Management of RHD in Pregnancy

©2007 World Heart Federation … Updated October 2008 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. Infective Endocarditis

©2007 World Heart Federation … Updated October 2008 Procedures that increase risk of Endocarditis DENTAL PROCEDURESOTHER PROCEDURES Dental extractionsTonsillectomy/adenoidectomy Periodontal proceduresBronchoscopy with a rigid bronchoscope Dental implant placementSurgery involving the bronchial mucosa Gingival surgerySclerotherapy of oesophageal varices Initial placement of orthodontic appliancesDilatation of oesophageal stricture Surgical drainage of dental abscessSurgery of the intestinal mucosa or biliary tract Maxillary or mandibular osteotomiesEndoscopic retrograde cholangiography Surgical repair or fixation of a fractured jawProstate surgery Endodontic surgery and instrumentationCystoscopy and urethral dilatation Intra-ligamentary local anaesthetic injectionsVaginal delivery in the presence of infection, prolonged labour or prolonged rupture of membranes Dental cleaning where bleeding is expectedSurgical procedures of the genitourinary tract in the presence of infection Placement of orthodontic bands

©2007 World Heart Federation … Updated October 2008 Surgery for RHD The need for surgery depends on –Severity of symptoms –Evidence that the heart valves are severely damaged –Left ventricular chamber size and function –Availability of long-term management after surgery (i.e. anticoagulation) Heart valves can be repaired or replaced Assessment before surgery includes –Echocardiogram to assess severity of heart valve damage –Complete dental assessment and treatment (if required) –Review and management of other health problems (e.g. kidney, vascular and chronic respiratory disease, cancers and obesity)

©2007 World Heart Federation … Updated October 2008 Heart valve REPAIR Heart valve REPLACEMENT Anticoagulation required Longer time before re-operation RHD No Anticoagulation Shorter time before re-operation Surgery Outcomes

©2007 World Heart Federation … Updated October 2008 Mild Guidelines for managing Mild RHD Definition - RHD with any trivial to mild valve lesion. Secondary ProphylaxisLong-term prevention of recurrent ARF Primary care managementBy local Medical Officer Specialist medical review for children aged to 18 yearsEvery 12 months Earlier if clinical deterioration Echocardiogram (if available)Every 2 years for children Every 5 years for adults Specialist medical reviewBefore ceasing secondary prophylaxis Dental review following diagnosisWith appropriate endocarditis prevention

©2007 World Heart Federation … Updated October 2008 Moderate Guidelines for managing Moderate RHD Definition - Any moderate valve lesion, no symptoms, and normal LV function with stable metallic prosthetic valves, or children (to 18 years old) with a history of chorea including those with no valve damage Secondary ProphylaxisLong-term prevention of recurrent ARF Primary care managementBy local Medical Officer Specialist medical reviewEvery 12 months Earlier if clinical deterioration Echocardiogram (if available)Every 1 years for children Every 2 years for adults Specialist medical reviewBefore ceasing secondary prophylaxis Dental review following diagnosisWith appropriate endocarditis prevention

©2007 World Heart Federation … Updated October 2008 Severe Guidelines for managing Severe RHD Definition - Any moderate-severe valve lesion with shortness of breath, tiredness, oedema, angina or syncope and impaired or increased left ventricular function or a history of valve surgery including mitral valvotomy, any valve repair and bio-prosthetic valves (porcine and homograph) Secondary ProphylaxisLong-term prevention of recurrent ARF Primary care managementBy local Medical Officer Specialist medical reviewEvery 6 months Refer to Heart SpecialistManagement Plan

©2007 World Heart Federation … Updated October 2008 Summary RHD presents as damage to the heart valves The mitral valve is most commonly affected, followed by Aortic, Pulmonary and Tricuspid RHD can be mild, moderate or severe RHD may be asymptomatic Management of RHD includes –Treatment of cardiac and other symptoms –Long-term secondary prophylaxis (to prevent recurrent ARF) –Regular medical and cardiology review –Management of existing pregnancy –Dental assessment, family planning referral