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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
Introduction
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
©2007 World Heart Federation … Updated October 2008
Definitions
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)
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 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 valve next most commonly affected
– Generally associated with disease of the mitral valve.
– Tends to develop as a long term complication of aortic regurgitation
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
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.
©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
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)
©2007 World Heart Federation … Updated October 2008
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
©2007 World Heart Federation … Updated October 2008
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.
©2007 World Heart Federation … Updated October 2008
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.
©2007 World Heart Federation … Updated October 2008
Procedures that increase risk of Endocarditis
DENTAL PROCEDURES
OTHER PROCEDURES
Dental extractions
Tonsillectomy/adenoidectomy
Periodontal procedures
Bronchoscopy with a rigid bronchoscope
Dental implant placement
Surgery involving the bronchial mucosa
Gingival surgery
Sclerotherapy of oesophageal varices
Initial placement of orthodontic appliances
Dilatation of oesophageal stricture
Surgical drainage of dental abscess
Surgery of the intestinal mucosa or biliary tract
Maxillary or mandibular osteotomies
Endoscopic retrograde cholangiography
Surgical repair or fixation of a fractured jaw
Prostate surgery
Endodontic surgery and instrumentation
Cystoscopy and urethral dilatation
Intra-ligamentary local anaesthetic injections
Vaginal delivery in the presence of infection,
prolonged labour or prolonged rupture of membranes
Dental cleaning where bleeding is expected
Surgical 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
Surgery Outcomes
Heart valve
REPLACEMENT
Heart valve
REPAIR
Anticoagulation required
No Anticoagulation
Longer time before re-operation
Shorter time before re-operation
RHD
©2007 World Heart Federation … Updated October 2008
Guidelines for managing Mild RHD
Definition - RHD with any trivial to mild valve lesion.
Secondary Prophylaxis
Long-term prevention of recurrent ARF
Primary care management
By local Medical Officer
Specialist medical review for children aged to 18 years
Every 12 months
Earlier if clinical deterioration
Echocardiogram (if available)
Every 2 years for children
Every 5 years for adults
Specialist medical review
Before ceasing secondary prophylaxis
Dental review following diagnosis
With appropriate endocarditis prevention
©2007 World Heart Federation … Updated October 2008
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 Prophylaxis
Long-term prevention of recurrent ARF
Primary care management
By local Medical Officer
Specialist medical review
Every 12 months
Earlier if clinical deterioration
Echocardiogram (if available)
Every 1 years for children
Every 2 years for adults
Specialist medical review
Before ceasing secondary prophylaxis
Dental review following diagnosis
With appropriate endocarditis prevention
©2007 World Heart Federation … Updated October 2008
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 Prophylaxis
Long-term prevention of recurrent ARF
Primary care management
By local Medical Officer
Specialist medical review
Every 6 months
Refer to Heart Specialist
Management 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
©2007 World Heart Federation … Updated October 2008