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
Dental extractions
Periodontal procedures
Dental implant placement
Gingival surgery
Initial placement of orthodontic appliances
Surgical drainage of dental abscess
Maxillary or mandibular osteotomies
Surgical repair or fixation of a fractured jaw
Endodontic surgery and instrumentation
Intra-ligamentary local anaesthetic injections
Dental cleaning where bleeding is expected
Placement of orthodontic bands
2007 World Heart Federation Updated October 2008
OTHER PROCEDURES
Tonsillectomy/adenoidectomy
Bronchoscopy with a rigid bronchoscope
Surgery involving the bronchial mucosa
Sclerotherapy of oesophageal varices
Dilatation of oesophageal stricture
Surgery of the intestinal mucosa or biliary tract
Endoscopic retrograde cholangiography
Prostate surgery
Cystoscopy and urethral dilatation
Vaginal delivery in the presence of infection,
prolonged labour or prolonged rupture of membranes
Surgical procedures of the genitourinary tract in the
presence of infection
 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 Heart valve

REPLACEMENT 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