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