CARDIO-NOTES, Part III: Management of chronic cardiac cases

Chapter (10)
Rheumatic fever and Rheumatic Heart Disease

CARDIO-NOTES
Part III: Management of Chronic Cardiac Cases
(An ESC and ACC/AHA guidelines based approach)

Ahmed Abdelmageed Alfarra, MSc

National Heart Institute

Cairo, Jan 2024

This chapter will be published in the upcoming part III of CARDIO-NOTES series
Part I: Cardiac Emergencies (ESC and ACC guidelines based step-by-step approach),
published, 2023
Part II: CVS Drugs (the clinical use of CV drugs), published, 2023.
Part III: Management of Chronic Cardiac Cases (an ESC and ACC/AHA guidelines
based approach), still under final revision for publishing

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CARDIO-NOTES, Part III: Management of chronic cardiac cases

Rheumatic fever and Rheumatic Heart Disease

Introduction
Rheumatic fever is a multisystem autoimmune inflammatory disease that is triggered by
infection with group A streptococcus (GAS). It is a disease of overcrowding and bad immune
response.

 Rheumatic fever (RF) may present in acute stage (acute rheumatic fever) or in chronic
stage (chronic rheumatic heart disease), acute rheumatic fever (ARF) may resolve
completely or progress to chronic stage.
 Repeated or severe episode of ARF leads to chronic rheumatic heart disease (RHD),
more than half of those with ARF progress to RHD within 10 years of their initial episode
and more than one-third of those people develop severe RHD, for this reason we need
to give secondary prophylaxis (to decrease ARF recurrence → decrease progression).
 Peak prevalence of ARF is between 5-14 years and peak prevalence for RHD between
35-44 years.
 About 3% of untreated acute streptococcal sore throat were followed by acute
rheumatic fever and about 10-40% (30%) of sore throat and all cases of tonsillitis are
caused by GAS. Skin strept infection (impetigo) is also associated with development of
ARF.
 Appropriate antibiotic therapy for GAS infection will prevent ARF in most cases (not all
cases); in up to two-thirds.
 Asymptomatic GAS infection can trigger ARF and recurrent ARF can occur in the setting
of adequate GAS treatment.
 About 1/3 of ARF cases are not preceded with clinical GAS Prevalence of RHD in
infection, and about 50-70 of RHD are not preceded by ARF Egypt is about 1-3%
(only about 30-50% of RHD are preceded by ARF). (10-30/1000) of
 Chronic RHD evolves over years after the first episode of school aged children
ARF (borderline echocardiographic findings suggestive of
RHD >>> subclinical RHD {no murmur} >>> clinical RHD {with murmur} >>> symptomatic
RHD).

Criteria for diagnosis of acute rheumatic fever, Revised Jones Criteria 2015
High risk population (as Egypt)* Low risk population**
Definite ARF 2 major criteria + evidence of GAS infection.
(initial) 1 major + 2 minor criteria + evidence of GAS infection.
Definite ARF 2 major criteria + evidence of GAS infection.
(recurrent, after 1 major + 2 minor criteria + evidence of GAS infection.
> 90 days) 3 minors criteria + evidence of GAS infection
Likely ARF (the Patient has 1 major & 1 minor criteria, or patient who has no evidence of
most likely GAS infection. Then those are further categorized according to level of
diagnosis) confidence in diagnosis into probable (highly suspected) or possible
(uncertain/just a clinical suspicion). In another words probable: 1 major +
1 minor or 2 minors with inclusion of evidence of preceding GAS infection
as a minor criteria, possible: doesn’t met definite or probable criteria

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CARDIO-NOTES, Part III: Management of chronic cardiac cases

Major criteria  Carditis including subclinical carditis  Carditis (including

(chronic carditis may be the only subclinical carditis)
manifestation of RF at time of its  Polyarthritis only
presentation)  Sydenham chorea
 Polyarthritis or monoarthritis or  Erythema marginatum
polyarthralgia (migratory,  Subcutaneous nodules
asymmetrical, large joints).
 Sydenham chorea (may be the only Polyarthralgia or
manifestation of ARF at time of its monoarthritis are
presentation) majors in high risk
 Erythema marginatum (almost never and minors in low
occur as the sole major manifestation) risk population
 Subcutaneous nodules (almost never
occur as the sole major manifestation)
Minor criteria  Fever ≥ 38  Fever ≥ 38 .5
 Monoarthralgia  Polyarthralgia or
 ESR ≥ 30 mm/h monoarthritis
 CRP ≥ 3 mg /dl  ESR ≥ 60 mm/h
 Prolonged PR interval on ECG  CRP ≥ 3 mg /dl
 Prolonged PR interval
* Living in community with low rate of ARF (> 2/100,000 per year in 5-14 year-old) or RHD > 1/1000 per year at any age.
** Living in community with low rate of ARF (≤ 2/100,000 per year in 5-14 year-old) or RHD ≤ 1/1000 per year at any age.
 Evidence of GAS infection = throat culture or ASOT or RADT.
 If polyarthritis is considered a major criterion, don’t count arthralgia or monoarthritis as
an additional minor criteria.
 If carditis is present, don’t consider prolonged P-R interval as additional minor criterion.
 If there is established RHD, recurrent attacks diagnosed by 2 minor criteria + evidence of
GAS infection (WHO).
 Indolent carditis: sub-acute illness develops over several weeks or months with severe
cardiac involvement and little or no joint symptoms, usually have modest elevation of
inflammatory marker and evidence of gas infection isn’t required.
 Some patients present with arthritis not typical for ARF but with evidence of GAS
infection and are said to have poststreptococcal reactive arthritis (in these cases arthritis
may affect small joints and less likely to respond to anti-inflammatory and patient are
considered not at risk of carditis and need no 2ry prophylaxis) but this diagnosis should
rarely, if ever, be made in high risk population and antibiotic prophylaxis should be
considered for at least 5 years as some patients developed later episodes of ARF
indicating that initial diagnosis was atypical ARF.

 Echo with Doppler is recommended for all suspected or confirmed cases of ARF (class 1).
 Consider serial echo in any patient with suspected or confirmed ARF even in absence of
carditis (class 2a).
 You may repeat echo within one month if the initial echo wasn’t clear, or showed severe
carditis or pericardial effusion.

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CARDIO-NOTES, Part III: Management of chronic cardiac cases

 Patient presenting with monoarthritis should be considered to have septic arthritis until
prove otherwise, and patient presenting with polyarthritis or polyarthralgia should be
investigated for alternative diagnoses.
 ASOT usually rises within 1-2 weeks of infection and reach peak within 3-6 weeks, then
start to decrease within 6-8 weeks and may remain elevated
In ARF: you may
for months after GAS infection and reach to pre-infection
measure inflammatory
level within 6-12 months. Single titer is usually misleading, markers (ESR, CRP)
and ideally sequential samples are more accurately define once weekly until
occurrence and time of infection, take one sample in acute become normal for one
phase then in the convalescent phase (2-4 weeks later) with month.
positive test defined as rising of twofold or more, but if only
a single sample is available, a titer exceeding the upper limit of normal is considered an
evidence of preceding GAS infection.
 The antibody titer varies with age and geographic location, ideally should be determined
for each geographic location. The upper limit of normal ASOT in normal Egyptian
children is high; up to 400 IU/ml. ASOT upper limit of normal in New Zealand used for
diagnosis of ARF is ≥ 480 IU/ml for children < 15 years. If the test is below upper limit of
normal, it should be repeated 10-14 days (four-fold rise or fall is diagnostic). The
recommended levels in Australia are as the following:

Age (year) 1-4 5-14 15-24 25-34 ≥ 35

ASOT (IU/ml) 170 276 238 177 127

Primary prevention of RHD

The aim is to promptly diagnose and treat GAS infection for prevention of initial attacks of
ARF/RHD.

 Use of penicillin prevents primary attacks of RF even when started as long as 9 days
after onset of acute illness.
 Full course of an antibiotic is recommended even if the patient becomes asymptomatic
few days after starting therapy.
 Nearly all GAS are susceptible to penicillin and beta lactams.
 The following are the drugs and doses that can be used in 1ry or 2ry prevention:

Drug Dose for 1ry prevention Dose for 2ry prevention

Phenoxymethyl- 250 mg (15 mg/kg) 2-3 times 250 mg twice daily orally.
penicillin daily if BW < 27 kg and 500 mg
(penicillin V) 2-3 times daily if BW > 27 kg for
10 days.
Amoxicillin 50 mg/kg/d once daily or in 3 -
doses, maximum 1 g/d for 10
days.
Benzathine 600,000 IU if < 20* kg and 600,000 IU if < 20* kg and 1,200,000
benzylpenicillin G 1,200,000 IU if BW ≥ 20 kg, IU if BW ≥ 20 kg every 2-4 weeks, IM.
(BPG) single IM dose in acute infection

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CARDIO-NOTES, Part III: Management of chronic cardiac cases

Cephalexin 500 mg (25 mg/kg) up 1000 mg -

twice daily for 10 days.
Azithromycin 12 mg/kg/d maximum 500 mg 250 mg once daily orally.
once daily for 5 days.
Erythromycin 40 mg /kg/d or 500 mg twice 250 mg twice daily orally
daily for 10 days.
Cotrimoxazole 4 mg/kg/dose for trimethoprim -
(Septin) for skin component (40 mg/5 ml syrup
sores preparation), for adult 1 tab
Spetrin DS twice daily for 3
days.
< 20 kg in Australian, < 30 kg in WHO and New Zeeland and < 27 kg in American guidelines.

Treatment of acute rheumatic fever:

Anti-inflammatory/analgesic:
 Ibuprofen: 200-400 mg 3 times daily up to 2400 mg daily (5-10 mg/kg 3 times daily).
 Aspirin: 50-60 mg /kg/d in 4-5 doses (maximum 100 mg/kg/d, 4-8 g/d).

Treatment of carditis (valvulitis):

 Treatment of HF symptoms (anti-failure medications and valve surgery if indicated).
 Prednisolone (1-2 mg/kg/d, maximum: 80 mg/d) in patient with severe carditis and
acute HF, and stop aspirin or other NSAID until discontinuation of steroid which stopped
(gradually) after improvement of inflammatory markers and HF symptoms.

Treatment of chorea (by neurologist):

 Carbamazepine: 3.5-10 mg/kg orally twice daily.
 Sodium valproate: 7.5-10 mg/kg orally twice daily.
 Prednisolone: 1-2 mg/kg/d, is used only in severe chorea.
 Other advanced therapies include IVIG, and plasmapheresis.

Secondary prevention of RF/RHD

The aim is to prevent recurrent ARF and so progression of RHD

 Risk of recurrence of RF is low after age of 25 and is extremely low after age of 40 years.
 Risk of recurrence of ARF decreases once patient received 40% of
Using of SAP may
BPG doses and after that there is 17% more reduction in risk for
reduce attacks of
every 10% increase in adherence.
RF following GAS
 BPG is more effective than oral penicillins.
infection by
 Deep intramuscular (IM) BPG is given every 4 weeks but in high risk
about 80%
group or patient with recurrent ARF despite 4-weeks regimen it is
preferred to be given every 3 weeks or 2 weeks (2-weeks regimen is
more effective than 3-weeks regimen which is more effective than 4-weeks regimen).

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CARDIO-NOTES, Part III: Management of chronic cardiac cases

 BPG can be reconstructed with 1-2% lidocaine to relief injection site pain with no loss of
efficacy. BPG preparation for
 In case of uncertainty about diagnosis (possible RF), give BPG injection: dilute the vial in
for 12 months then reevaluate the patient with clinical 3 ml sterile water or 1-2%
assessment and Echocardiography (class 2a). lidocaine and shake
 If the patient who is on 2ry prophylaxis showed recurrent vigorously.
symptoms (e.g; joint pain) but lack evidence of infection and
normal echo, you may consider discontinuation of prophylaxis (class 2a).
 If the patient who is on 2ry prophylaxis showed sore Penicillin sensitivity test: Draw out
throat or skin sore and BPG dose was given > 7 days 0.1 ml of diluted drug then further
ago, give additional antibiotic dose as for active dilute with 1 ml sterile water then
infection (BPG level decreases to prophylactic level inject 0.1 ml ( ‫ شرطات في سرنجة‬01
after 7 days). )011‫ االنسولين ال‬subcutaneously in the
 Perform penicillin sensitivity test before forearm then circle this area and wait
administration, every time. for 15 min to look for itching,
 Benzathine Penicillin severe reactions are very rare, swelling or any unusual symptoms. In
allergenic reaction is about 3.2% but severe case of doubt, repeat on the other
anaphylaxis is about 0.2% and fatal reactions are arm with double strength test dose
exceptionally rare. If a rheumatic patient
experienced true penicillin allergic reaction then he should be referred to immunologist
to verify the type and severity of reactions and determine if there is absolute
contraindication to penicillin.

Immediate True reactions (within 15 min- 2 hr) Delayed true reactions (within 5-15 days)
Felling of fainting, itching, rashes, swelling Rash, fever, joint pain.
and respiratory distress.
Management: for mild reactions: reassurance, hydrocortisone (100 mg Solu-Cortef),
antihistamine (avil). In case of anaphylactic shock/ severe reactions consider
adrenaline (IM), fluids and oxygen ± CPR.

Duration of secondary antibiotic prophylaxis (SAP)

Duration according to Australian guidelines (2022)
Diagnosis Definition Minimum duration of Features to stop Timing of
prophylaxis ECHO after
cessation
Possible Incomplete 12 months (then No S/S of ARF, At 1 year
ARF features of ARF reassess). For 5 years in Normal echo or
(uncertain), with normal NZ guidelines with still uncertain (if
no cardiac ECHO & ECG regular review probable or
involvement definite >>
continue therapy)
Probable Highly 5 years or until age of 21 No S/S of ARF At 1, 3, 5
ARF suspected ARF (whichever is longer), within the years
previous 5 years,

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CARDIO-NOTES, Part III: Management of chronic cardiac cases

with normal count from the most Normal echo

ECHO recent episode
Definite ARF ARF with 5 years or until age of 21 No S/S of ARF At 1, 3, 5
(with no normal ECHO (whichever is longer) within the years
cardiac and ECG ‫ سنة يبقى لغاية‬01 ‫لو أقل من‬ previous 5 years,
involvement) throughout the 01 ‫ سنة ولو أكبر من‬10 ‫عمر‬ Normal echo
episode ‫ سنين‬5 ‫سنة يبقى لمدة‬
Definite ARF ARF with Most cases of RHD lack a history of past ARF.
(with cardiac carditis or RHD
involvement) on ECHO or AV
conduction Further classified as the following:
abnormalities
on ECG.
Borderline Borderline RHD Minimum for 2 years No ARF in the After 1-2
RHD (≤ 20 on ECHO then reassess, if still previous 2 years. years of
years) without history borderline, continue for Normalization of diagnosis
of ARF further 2 years and echo after 2 and -2
Stage A reassess years of follow up years of
stopping
prophylaxis
Mild RHD Mild stenosis or 10 years or until age of Stable echo At 1, 3, 5
regurgitation of 21 years in case of features for 2 years
a single valve or documented history of years, no
Stage B AV conduction ARF (whichever is evidence of
abnormalities longer) or 5 years or progression of
on ECG during until age of 21 years if no RHD or no ARF in
ARF. history of ARF the previous 10
(whichever is longer) years
‫ سنة يبقى لغاية‬00 ‫لو أقل من‬
00 ‫ سنة ولو أكبر من‬10 ‫عمر‬
‫ سنين‬01 ‫سنة يبقى لمدة‬
Moderate Moderate 10 years or until age of Stable echo Every 12
RHD regurgitation or 35 years in case of features for 2 months
stenosis of a documented history of years, no
single valve or ARF (whichever is probable or
Stage C or D combined mild longer) or 5 years or definite ARF
regurgitation until age of 35 years if no within the
and/or stenosis history of ARF previous 10 years
of one or more (whichever is longer).
valve. For 10 years or till age of
30 in NZ guidelines then
review risk of GAS
exposure; if high
continue prophylaxis.
Severe RHD Severe stenosis 10 years or until age of Stable echo Every 6
or regurgitation 40 years in case of features for 3 months
of a single documented history of years,

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CARDIO-NOTES, Part III: Management of chronic cardiac cases

Stage C or D valve, or ARF (whichever is Patient or family

combined longer) or 5 years or preference to
moderate until age of 40 years if no cease.
regurgitation history of ARF
and/or stenosis (whichever is longer), or
of one or more lifelong if surgery
valve, or indicated or causing HF
impending or
previous valve
intervention
Duration according to American guidelines (2009)
RF without carditis For 5 years or until age of 21 years whichever is longer.
RF with carditis but For 10 years or until age of 21 years whichever is longer.
no residual lesion
RF with carditis with For 10 years or until age of 40 years whichever is longer.
residual lesion
Duration according to New Zealand (NZ) guidelines (2014)
No or mild RHD For 10 years or until age of 21 years whichever is longer.
Moderate RHD Until age of 30 then reassess.
Severe RHD Until age of 40 then reassess but review at age of 30 years.
Duration according to WHO guidelines (2004) and Indian guidelines (2015)
RF without carditis For 5 years or until age of 18 years whichever is longer.
RF carditis For 10 years or until age of 25 years whichever is longer.
Severe RHD For life or up to 40 years of age.
 Echo may be more frequent based on clinical status.
 Normal ECG = no AV conduction abnormalities.
 Normal echo = see below.
 No prophylaxis is needed for patient older than 35 years diagnosed with mild or
moderate RHD without evidence of ARF (older than 40 years with severe RHD,
prophylaxis determined by specialist).
 Despite ARF recurrence is rare in older than 40 years, lifelong prophylaxis is
recommended if patient has had or likely to need valve surgery.
 Some cases, rarely, may improve on echo, if moderate or severe cases improved,
follow recommendations of the new status. Mild RHD may resolve over 10 years with
appropriate prophylaxis.
 Endocarditis prophylaxis before dental procedure is recommended in patient with
RHD in Australian and NZ guidelines but not in American guidelines.
 Dental review is recommended in all patients with RHD every 6-12 mo (also
recommended by ESC 2023 IE guidelines).

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CARDIO-NOTES, Part III: Management of chronic cardiac cases

Follow up of patients on SAP and monitoring of medication adherence

We measure BPG adherence by measuring the percentage delivered annually.

Percentage delivered (%) = number of doses administered divided by number of doses

recommended multiplied in 100. Increased percentage delivered means increased adherence.

This method is simple and easy but accurate as it doesn’t take in account the dose timing;
patient may show 100% delivery percentage but some doses may be delivered at shorter
intervals and other at longer intervals with substantial breaks in between.

So the second method is more accurate and predictive of ARF recurrence which will estimate
the days-at-risk of recurrent exposure, the days that should be covered by BPG but actually
missed, it is calculated from first day of the next BPG dose that is not delivered and this is
determined according to the selected regimen, for example if you decide to give BPG every 3
weeks, and it is given on day 1 then the next BPG dose is due on day 22 and if this next dose is
given later than day 22, then the first day at risk is day 22 and all subsequent days before
administration. We calculate this days through calculation of the proportion of days covered
(PDC) by dividing the days actually covered by BPG by the total numbers of days from the first
dose, the ideal result should be 1 but if 0.8 or more then it is accepted.

Measures to decrease injection site pain with BPG:

 Firm pressure at site of injection for 10 seconds immediately before injection.
 Refrigeration of the needle before injection.
 Ice pack applied to the site before injection.
 Use any distracting stimuli to the skin.
 Use lidocaine with BPG (for dilution, 1ml of 1% preparation).
 Use anesthetic spray before injection (not so effective).
 Deliver injection very slowly (over 2-3 min).
 Use analgesic (paracetamol) if needed after injection.
 Use sedative if needed during injection procedure.

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CARDIO-NOTES, Part III: Management of chronic cardiac cases

Echo features of RHD according to 2023 World Heart Federation (WHF) guidelines

Screening criteria
Consider RHD Echo active case
It is designed to be used by non-expert for detection of finding (screening) in children
suspected cases of RHD in settings of high provenance aged 5-20 years living in endemic
and limited resources, this is applicable for individuals regions (class 2A) and in first
aged 20 years or less, for that you may use hand-held degree relatives of index cases
machine (doesn’t rely on spectral Doppler measurments (class 2A), and pregnant women
for simplification). Positive screening (presence of any or young adults aged 21-39 years
defined MR, AR, and MS) is followed by confirmatory (class 2B) by confirmatory criteria
not screening criteria.
echocardiography.

MR on screening (all criteria met) AR on screening (all criteria met)

1. Seen in at least 1 view. 1. Seen in at least 1 view.
2. Seen for at least 2 consecutive frames. 2. Seen for at least 2 consecutive frames.
3. Minimum Jet length ≥ 2 cm if BW ≥ 30 kg 3. Any AR.
or ≥ 10 years or ≥ 1.5 cm if BW < 30 kg).
MS: restricted leaflet motion with reduced screening opening

Confirmatory criteria:
Designed for experts to confirm a diagnosis of RHD with standard ECHO machine

Pathological MR (all 4 criteria met) Pathological AR (all 4 criteria met)

Doppler features
4. Seen in at least 2 views. 1. Seen in at least 2 views.
5. Jet length ≥ 2 cm in at least 1 view (≥ 1.5 2. Jet length ≥ 1 cm in at least 1 view.
cm if BW < 30 kg). 3. Peak velocity > 3 m/s.
6. Peak velocity > 3 m/s. 4. Pan-diastolic in at least 1 envelope.
7. Pan-systolic in at least 1 envelope.
Morphological features
 Excessive leaflet motion (tip of AML) Focal or irregular leaflets thickening
during systole with abnormal coaptation Coaptation defect
(chordal elongation). Leaflets prolapse
 Restricted leaflets motion (subchordal Restricted leaflets mobility.
thickening) Differential diagnosis: physiological
 Leaflets thickening/nodularity or beading (≥ (trivial) regurgitation (not fulfill above
3 mm if < 20 years and ≥ 5 mm if > 40 years
and ≥ 4 if 20-40 years of age); during diastole criteria), myxomatous mitral valve,
at full excursion at the thickest portion. mitral valve prolapse, Barlow
 Chordal thickening and fusion. syndrome, congenital anomalies
In advanced cases: (parachute mitral valve, cleft MV,
Leaflets calcification, diastolic doming on AML double orifice MV & fibroelastoma).
 Some morphological features of RHD take time to develop but may be present in ARF as acute on
chronic presentation.
 On occasion, valves may appear normal morphologically on echo while Doppler shows
regurgitation.

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CARDIO-NOTES, Part III: Management of chronic cardiac cases

2012 WHF criteria for echocardiographic diagnosis of RHD

These criteria are widely accepted as the reference standard for diagnosis of RHD in absence of
history of acute rheumatic fever.

Definite RHD (one of the following):

1. Pathological MR and at least two morphological features
of RHD of the MV.
These mentioned terms
2. Pathological AR and at least two morphological features
(definite, borderline and
of RHD of the AV (< 35 year of age).
latent) are no longer
3. MS mean gradient ≥ 4 mmHg (exclude other causes of
recommended by the latest
MS).
2023 WHF guidelines and
4. Borderline disease of both the AV & MV (combined
replaced by a new
borderline valves).
classification system that
5. If age> 20 year, pathological AR and at least two
include ECHO criteria and
morphological features of RHD of the MR.
care recommendations to
Borderline RHD (one of the following), applicable only for identify the risk of
individuals aged < 20 years: progression (in this system,
nearly but not precisely the
1. At least two morphological features of RHD of the MV term “borderline” is
without MR or MS. replaced by the term “stage
2. Pathological MR. A RHD”).
3. Pathological AR.

Stage A RHD as in 2023 WFH guidelines requires echo features of mild MR or mild AR without
morphological features

Latent RHD: Valvular changes consistent with RHD in individual with no history of ARF.
Normal echocardiographic findings (all of the following):
1. Physiological MR (doesn’t met pathological MR). Trivial or physiological
2. Physiological AR (doesn’t met pathological AR). valve regurgitation or
3. Isolated morphological feature of RHD of the MV (for isolated morphological
example: valvular thickening). changes should be
4. Isolated morphological feature of RHD of the AV (for considered normal or
example: valvular thickening). physiological.

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CARDIO-NOTES, Part III: Management of chronic cardiac cases

RHD stages according to WHF 2023 guidelines

Stage Echo features Risk of progression Care
Stage A (applicable Mild MR or mild AR Might be at risk of SAP for 1-2 years
only for children without VHD then re-view
aged ≤20 years) morphological
features
Stage B Mild MR or mild AR At moderate or high SAP and other
(mild RHD) plus 2 morphological risk of progression to therapies according
features (if > 20 VHD and at risk of to local guidelines
years) or 1 feature (if developing
< 20 years) or both symptoms
mild MR and AR

Stage C Moderate or severe At high risk of SAP and other

(advanced RHD at valve regurgitation, developing therapies according
risk) any MS or AS or PH complications to local guidelines
or decreased LVEF.
Stage D Moderate or severe Established clinical SAP and other
(advanced RHD with valve regurgitation, complications therapies according
complications) any MS or AS or PH to local guidelines
or decreased LVEF.

Finally, we have two problems still unresolved!

 Problem of diagnosis: we don’t have a diagnostic tool, research of a diagnostic

biomarker is ongoing.
 Problem of prevention: the ideal tool is the development of a vaccine for GAS which is
under investigation.

References

 2022 Australian guidelines for ARF and RHD (V 3.2).

 2014 New Zealand guidelines for rheumatic fever; diagnosis, management and
secondary prevention of ARF and RHD
 Prevention of RF, diagnosis and treatment of acute strept pharyngitis (a scientific
statement from the AHA)
 Contemporary diagnosis and management of RHD (AHA 2020).
 Revision of the Jones Criteria for diagnosis of ARF in the era of Doppler echo a scientific
statement form AHA 2015.
 World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart
disease- an evidence based guideline, 2012.
 World Heart Federation guidelines for the echocardiographic diagnosis of rheumatic
heart disease, 2023.
 Handbook on prevention and control of rheumatic fever and rheumatic heart disease,
ministry of health and family Welfare, government of India, 2015.
 Uptodate.com; management of RHD.

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