Diagnosis and Management of Acute Rheumatic Fever and Rheumatic Heart Disease

©2007 World Heart Federation … Updated October 2008

Diagnosis and Management of Acute Rheumatic Fever

and

Rheumatic Heart Disease


Rheumatic Heart Disease Diagnosis and Management


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.


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 50% of people with RHD dodo not remembernot remember having ARF having ARF

Introduction


Definitions

Valve RegurgitationRegurgitation 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 StenosisStenosis suggests that heart valves – Become stuck to each other

– Do not allow blood to flow through easily (restricted forward flow)


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


Heart valve involvement

MitralMitral 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

AorticAortic valve next most commonly affected

– Generally associated with disease of the mitral valve.

– Tends to develop as a long term complication of aortic regurgitation

TricuspidTricuspid and pulmonarypulmonary valves are much less commonly affected– Usually affected in very severe RHD when all valves are affected


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


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


Key element in RHD Management

Secondary prophylaxisSecondary prophylaxisFunctions 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.


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


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 deliveryHighest risk of complications immediately after delivery

RHD and 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.

Management of RHD in Pregnancy


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


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


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)


Heart valve Heart valve REPAIRREPAIR

Heart valve Heart valve REPLACEMENTREPLACEMENT

Anticoagulation required

Longer time before re-operation

RHD

No Anticoagulation

Shorter time before re-operation

Surgery Outcomes


Guidelines for managing MildMild 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


Guidelines for managing ModerateModerate 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



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


Guidelines for managing SevereSevere 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)



Specialist medical review Every 6 months

Refer to Heart Specialist Management Plan


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

Diagnosis and Management of Acute Rheumatic Fever and Rheumatic Heart Disease

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