The Prevention of

Rheumatic Fever and

Rheumatic Heart
Disease
Dr Liesl Zühlke
Paediatric Cardiologist: Red Cross War
Memorial Childrens Hospital
Cape Town South Africa
Liesl.zuhlke@uct.ac.za
• I graduated from University of Cape Town medical School in 1991 and
qualified as a pediatrician in 1999 and as a paediatric cardiologist in 2007. I
am currently a doctoral fellow involved in full-time research related to
rheumatic fever and rheumatic heart disease working within the framework
of the A.S.A.P programme.
• Sub-Saharan Africa remains a hotspot for rheumatic fever and rheumatic
heart disease. It is the only truly preventable chronic cardiac condition, yet
still reigns rampant in poor countries years after virtual eradication in
developed nations.
• Rheumatic heart disease is a major killer of children, adolescents and
young adults. Health practioners practising in resource-poor settings such
as Africa, need to work together to raise awareness of this condition,
survey incidence, prevalence and temporal trends , be advocates for
patients whose lives are affected by rheumatic heart disease and institute
prevention strategies.
• Rheumatic heart disease is a neglected disease of poverty – we need to
turn the tide and address the fundamental issues surrounding this condition
that still remain.
• As a paediatric cardiologist seeing patients with rheumatic heart disease on
a regular basis, I feel passionate about being part of the solution and
working towards the eradication of rheumatic heart disease in our lifetime-
a very achievable goal.
Learning Objectives:

• To understand the pathogenesis of acute rheumatic fever

and rheumatic heart disease
• To appreciate the burden of disease
• To recognize the features of a streptococcal sore throat
• To know the treatment regimens of a streptococcal sore
throat
• To be aware of secondary prevention measures
• To understand the role of a register-based programme
Performance Objectives:

• Examine the burden of disease within own communities

• Timely recognition of a streptococcal sore throat with
correct treatment
• Institute secondary prevention programme
• Institute the above measure within a register-based
programme
• Join the global community fighting Rheumatic fever and
rheumatic heart disease
 What is the
pathogenesis of
acute rheumatic
fever?
 ACUTE RHEUMATIC FEVER

• Autoimmune consequence of
infection with Group A streptococcal
infection

• Results in a generalised
inflammatory response affecting
brains, joints, skin, subcutaneous
tissues and the heart.
 ACUTE RHEUMATIC FEVER

• The clinical presentation can be

vague and difficult to diagnose.

• Currently the modified Duckett-

Jones criteria form the basis of
the diagnosis of the condition.
Carapetis. Lancet 2005;366:155
 RHEUMATIC HEART
DISEASE
• Rheumatic Heart Disease is the
permanent heart valve damage
resulting from one or more
attacks of ARF.
• It is thought that 40-60% of
patients with ARF will go on to
developing RHD.
 RHEUMATIC HEART
DISEASE

• The commonest valves affecting

are the mitral and aortic, in that
order. However all four valves
can be affected.
 RHEUMATIC HEART
DISEASE
• Sadly, RHD can go undetected
with the result that patients
present with debilitating heart
failure.
• At this stage surgery is the only
possible treatment option.
 RHEUMATIC HEART
DISEASE
• Patients living in poor countries
have limited or no access to
expensive heart surgery.
• Prosthetic valves themselves
are costly and associated with a
not insignificant morbidity and
mortality.
 What is the
incidence of acute
rheumatic fever and
rheumatic heart
disease?
• In the Pacific Islander population of
New Zealand the incidence rate of
ARF is 80-100 per 100 000 compared
to non-indigenous new Zealanders <10
per 100 000.
• In a recent systematic review of the
incidence of first attack of rheumatic
fever, a Maori community in New
Zealand has a disturbingly high
incidence of >80/100,000 per year.
 Incidence of ARF:
Population-based Studies
Figure 5: Trend in Incidence of First Attack of Acute
Rheumatic Fever Over Time
40
USA (all ages)
35

Incidence/100,000 population
Martinique (<20yrs)
New Zealand (<30yrs)
Kuwait (5-14yrs) 30
Iran (all ages
25

20

15

10

5
1 2 0
3 4
5 6
7 8
Time (years) 9 10 11
 Incidence of newly
diagnosed RHD
• A prospective , clinical registry captured data
from new presentation of structural and
functional valvular heart disease presenting to
the department of cardiology in 2006/7.
• Of the 4005 de novo cases, 344 (8.6%) were
diagnosed as having RHD. A significant
proportion presented with complications and
22% subsequently underwent surgery.
 What is the
prevalence of
rheumatic
heart disease?
In some developing countries , however,
remarkable progress has been made in terms
of decreasing incidence of ARF.
In 1986 a comprehensive 10-year prevention
programme was conducted in a Cuban
province.

This programme relied on comprehensive

primary and secondary prevention of RF/RHD
as well as awareness and education
programmes.
 RHEUMATIC FEVER IS
PREVENTABLE

Costa Rica

Cuba
The main content of the activities focused
around early detection and treatment of
sore throats and streptococcal pharyngitis.

The project also included primary and

secondary prevention of RF/RHD, training
of personnel, health education,
dissemination of information, community
involvement and epidemiological
surveillance.
There was a progressive decline in the
occurrence and severity of acute RF and RHD,
with a marked decrease in the prevalence of
RHD in school children.
A marked and progressive decline was also seen
in the incidence and severity of ARF.

There was an even more marked reduction in

recurrent attacks of RF as well as in the number
and severity of patients requiring hospitalisation
and surgical care.
What are the
clinical
features of
strep sore
throat?
 Hallmarks of STREP
sore throat
• Tender lymph nodes
• Close contact with infected person
• Scarlet fever rash
• Excoriated nares( crusted lesions) in infants
• Tonsillar exudates in older children
• Abdominal pain
• GOLD STANDARD: POSITIVE THROAT
CULTURE
 Hallmarks of VIRAL
sore throat
• Coryza: runny nose or mouth ulcers
• Other family with COLD symptoms
• Evidence of another viral infection
• Itchy watery eyes
• Hoarseness and cough: non-specific
• Fever: not specific
• Red Throat: not specific
What are the
treatment
regimens of
streptococcal
sore throat?
 Primary Prevention of
Rheumatic Fever by
treating sore throat
Antibiotic Administration Dose
Benzathine Single IM injection 1.2 MU > 30kg
benzyl penicillin 600 000 U < 30 kg
Phenoxymethyl PO for 10 days 250-500mg qds for 10 days
penicillin 125mg qds X 10 if <30 kg
(Pen VK)
Erythromycin PO for 10 days Use same dose as above.
ethylsuccinate

Oral penicillin is less efficacious than Penicillin IMI

Anaphylaxis is extremely unusual
 Is it cost-effective to
administer penicillin for all
cases of suspected strep
sore throat?
• An overall protective effect for the use of
penicillin against acute rheumatic fever of
80% with an NNT of 60 children per year to
prevent 1 episode of rheumatic fever.

• Mild hypertension: have to treat 800 people

per year to prevent 1 episode of stroke
 Is it cost-effective to
administer penicillin for all
cases of suspected strep sore
throat?
• The estimated cost of preventing one case of
rheumatic fever by a single intramuscular
injection of penicillin is US$46
• Valve replacement surgery for 1 case of RHD
is at least US$15, 000
• Cardiac surgery only available in S Africa,
Ghana and Egypt
 Rheumatic Heart Disease:
SECONDARY PREVENTION

PICTURE TAKEN
OUT FOR SPACE
ISSUES
THIS IS TOO
LATE
 Secondary Prevention
Stops sore throat, prevents recurrences of ARF and
aids in regression of RHD

Antibiotic Administration Dose

Benzathine Single IM injection 1.2 MU > 30kg
benzyl penicillin monthly 600 000 U < 30 kg
Phenoxymethyl BD PO daily 250-500mg bd
penicillin
(Pen VK)
Erythromycin BD po daily Use same dose as above.
ethylsuccinate

Oral penicillin has been shown to be less effective than

Penicillin IMI
Anaphylaxis is extremely unusual
Review: Penicillin for secondary prevention of rheumatic fever
Comparison: 02 Two-weekly versus 4-weekly penicillin injections
Outcome: 02 Streptococcal throat infections

Study 2-weekly injections 4-weekly injections RR (fixed) Weight RR (fixed)

or sub-category n/N n/N 95% CI % 95% CI

Kassem 1996 38 / 190 57 / 170 100.00 0.60 [0.42, 0.85]

0.1 0.2 0.5 1 2 5 10

Favours treatment Favours control

Review: Penicillin for secondary prevention of rheumatic fever

Comparison: 03 Three-weekly versus 4-weekly intramuscular penicillin
Outcome: 02 Streptococcal throat infections

Study 3-weekly injections 4-weekly injections RR (fixed) Weight RR (fixed)

or sub-category n/N n/N 95% CI % 95% CI

Lue 1996 39 / 124 59 / 125 100.00 0.67 [0.48, 0.92]

0.1 0.2 0.5 1 2 5 10

Favours 3-weekly Favours 4-weekly
 During an episode of
ARF, valve changes can
be minor and are still able
to regress.

After recurrent episodes of

ARF, thickening of
subvalvar apparatus,
chordal thickening and
shortening and
progression to permanent
valve damage is evident.
 Secondary
prevention: Duration
CATEGORY DURATION OF PROPHYLAXIS
All persons with MINIMUM 10 years after most recent
ARF with no or mild
carditis episode or age 21
All persons with MINIMUM 10 years after most recent
ARF and moderate
carditis episode or age 35

All persons with MINIMUM 10 years after most recent

ARF and severe
carditis episode or age 35 and then specialist
review for need to continue. Post surgical
cases definitely lifelong.
Awareness ♦ Surveillance ♦ Advocacy ♦ Prevention
Secondary prevention:
specifics
PENCILLIN

Secondary prophylaxis also reduces the

severity of RHD.
It is associated with regression of heart
disease in approximately 50-70% of those
with good adherence over a decade and
reduces mortality.
Route:
BPG is most effective when given as a
deep intramuscular injection.
 Secondary prevention:
Adherence
How can we reduce the pain associated with
IM Penicillin?

• Use a 23-gauge needle- deeper is better

• Local pressure to area for 10 secs
• Warm syringe to room temperature
• First allow alcohol to dry or use ethylchloride
spray
.
 Secondary prevention:
Adherence

• Deliver injection very slowly(over 2-3mins)

• Distraction techniques
• Good rapport with the case, is a significant aid to
injection comfort, compliance and understanding.
• Use 0.5-1ml of 1% lignocaine. Reduces pain
significantly and excellent for younger patients.
Ensuring that patients
understand their disease,
are informed regarding
their future and receive
secondary prophylaxis

EDUCATION
Health education is critical at all levels
Lack of parental awareness of the causes and
consequences of ARF/RHD is a key contributor to
poor adherence amongst children on long-term
prophylaxis.
 “Kenyan-Heart Talking Walls”: Dr. Aggrey Primary
School

Elizabeth Gatumia, Kenyan Heart Foundation/ Danish Heart

Foundation
 Rheumatic Fever Week
South Africa, 7-13 August 2006
What is the role of
a register-based
programme?
In 1972, the WHO launched a register-based
programme to combat RF.RHD.

By 1990, registers had been established in 16

countries with over a million school-going
children involved. However in 2001, the WHO
ceased its funding to this global programme.

Experience elsewhere however provides

conclusive evidence of registers realising
notable successes in reducing RF recurrence.
The purpose of a register:

Collect data on demographic profiles

Highlight deficiencies in service delivery
Priority-based guidelines to evaluate and
manage patients

Most importantly:

A register of cases of RF and RHD can be

used to improve treatment adherence in
order to prevent recurrent RF and the
development of RHD, necessitating surgery.
 A.S.A.P. Programme for the
Control of RHD in Africa:
Focus areas for action
• Awareness raising: public, healthcare
workers
• Surveillance: incidence, prevalence,
temporal trends
• Advocacy: appropriate funding of the
treatment and prevention programmes
• Prevention: application of existing
knowledge in primary & secondary
prevention
 Summary
• Rheumatic heart disease is the only
truly preventable chronic heart condition
• Primary prevention:
– Penicillin for suspected strep sore
throat
• Secondary prevention
– Penicillin prophylaxis
• It is a legal requirement to notify
cases of acute rheumatic fever to
the local authority in South Africa
 Summary
The A.S.A.P. Programme for the
Eradication of
Rheumatic Fever
in Africa:
An achievable goal