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Acute Rheumatic Fever &

Rheumatic Heart Diseases
The Unrecognized Killers among Young Adults

dr. Faqrizal Ria Qhabibi

BRIEF EPIDEMIOLOGY
OF ARF/RHD
BRIEF EPIDEMIOLOGY
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OF ARF/RHD 2

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This Photo by Unknown Author is licensed under CC BY-SA-NC

• ARF which is a sequela of GAS infection primarily manifests in children and

adolescents. The peak incidence of ARF is between 5 to 15 years, and it is exceedingly
rare around 30 years of age.
• Global incidence: approximately 500,000 new cases of ARF are reported worldwide
• Non-endemic regions: 10 cases per 100,000 in industrialized countries
• Endemic regions: 374 cases per 100,000 in Pacific countries
• Worst Case globally: Around 60% of individuals residing in endemic populations
Image courtesy of R. Seth, Telethon Kids Institute, Perth, Australia
who have ARF will ultimately develop RHD.
Carapetis, J. R. et al. (2015) Acute rheumatic fever and rheumatic heart disease
Nat. Rev. Dis. Primers doi:10.1038/nrdp.2015.84 3 4

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• Global prevalence: 40.5 million cases of RHD (GBD 2019 study)

• Global incidence: 15.6 million cases
• Non-endemic regions: 3.4 cases per 100,000
• RHD has been declared eliminated in developed nations, however migration has an influence.
Screening in Italy found subclinical RHD in 2.1% of refugees.
• Endemic regions: >1,000 cases per 100,000
• Mortality rate: 6% to 12% in endemic areas (Ethiopia, Pakistan)
• Situation in Indonesia:
Case
• No integrated national data on prevalence and incidence of RHD in Indonesia
• Jakarta à 471 RHD cases in 2011-2016; and 2880 of 7112 (40.5%) valvular cases during 2016– 2019
were RHD cases
• Bandung à 108 of 4,682 (2.3%) RHD cases
• Annual case-fatality rate globally: 1.5% per year
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Identitas:
Riwayat Penyakit Dahulu:
Nama : Tn. YEL
Pasien mengaku pernah dirawat di rumah sakit saat remaja dengan keluhan serupa.
Usia : 20 tahun
Riwayat Obat: -
Jenis kelamin : Laki-laki Riwayat Keluarga:
Pemeriksaan : 2 Maret 2025 Anggota keluarga dengan keluhan serupa disangkal, riwayat penyakit jantung maupun metabolik juga
No. RM : 23.05.84 disangkal.
Pemeriksaan Fisik
Keluhan Utama: Nyeri dada
Keadaan umum : Lemas
Riwayat Penyakit Sekarang:
GCS : E4V5M6
Pasien datang dengan keluhan nyeri dada sebelah kiri terlokalisir nyut-nyutan/seperti ditindih sejak 1
jam yang lalu atau jam 17.00 WITA. Nyeri hilang timbul tanpa pencetus dirasakan sampai sekarang. HR : 86 x/menit reguler kuat angkat
Keluhan nyeri tidak menjalar ke leher maupun bahu dan punggung. Nyeri juga tidak dipengaruhi oleh
RR : 20 x/menit
pergerakan tubuh maupun pernafasan. Saat nyeri dada muncul, disaat yang bersamaan pasien juga
mengeluhkan kedua distal/pergelangan tangan dan kaki tiba tiba ada gerakan involunter. Pasien juga Suhu : 38,4 C
mengeluhkan agak demam. Saat ini dikeluhkan lemas, mual dan muntah disangkal, ruam kemerahan SpO2 : 95% RA
dengan batas tegas disangkal, benjolan disangkal, dan nyeri di sendi yg berpindah-pindah disangkal
oleh pasien. BB : 73 kg

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Foto Klinis:

Status Generalis:
Kepala/ Leher: a/i/c/d -/-/-/-

Thorax:
Cor: S1S2 tunggal, murmur + di apex, gallop -/-

Pulmo: ves/ves, rhonki -/+, wheezing -/-

Abdomen: Soefl, bising usus (+) normal

Ekstremitas: Akral hangat, kering, merah, CRT <2 detik, oedema -/-, didapatkan gerakan involunter pada
distal manus dan pedis.

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EKG: Darah Lengkap:

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Penegakan Diagnosa:
Mayor:
Tatalaksana Sesuai Instruksi Sp.JP:
- Nyeri dada tidak khas tanpa dipengaruhi aktivitas, pergerakan, maupun pernafasan susp. Carditis (perlu
konfirmasi Echocardiography karena suara ditemukan suara murmur). Non-farmakologi:

- Sydenham chorea 1. Oksigenasi 3 lpm menggunakan nasal canule

Minor:
2. Pro-rawat ICU dan rujuk ke RSUP
- Febris/ demam.
- Leukositosis (peningkatan WBC). Farmakologis:

1. Aspilet 320 mg PO loading

Syarat Tegak Diagnosa Secara Klinis:
2. Pantoprazole 8 mg IV
2 Mayor atau 1 Mayor + 2 Minor.
3. Diazepam 1x2 mg IV
Assessment: 4. Haloperidol 1x0,5 mg
Acute rheumatic fever

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RHD is a residual heart valvular dam age as a result of carditis sequelae due
to acute rheum atic fever (ARF). W hile peak prevalence of RHD occurs
between 25 and 45 years of age, reflecting the cum ulative effects of
recurrent episodes of ARF.

ARF it self is a post-infectious syndrom e involving the heart,

joints, subcutaneous tissues, and brain, while alm ost all
sym ptom s resolve over weeks to m onths without any sequelae.

KNOWINGG MORE G roup A

The m ain driver of ARF is frequent untreated GAS infection. M

ABOUT ARF/RHD
Stre ptococcus
protein encoded by the em m gene, is essential for GAS
virulence. Furtherm ore, were im plicated strongly and
repetitively in outbreaks of ARF.

D o u g h e rty S , O k e llo E , M w a n g i J , K u m a r R K . R h e u m a tic h e a rt d is e a s e : J A C C fo c u s

s e m in a r 2 /4 . J o u rn a l o f th e A m e ric a n C o lle g e o f C a rd io lo g y . 2 0 2 3 J a n 3 ;8 1(1):8 1-9 4 .
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PATHOGENESIS OF ARF PATHOGENESIS OF ARF

1. 2. Neo-
Molecula antigen
r Mimicry Theory

D ougherty S, Okello E, M wangi J, Kum ar RK. Rheum atic heart

disease: JACC focus sem inar 2/4. Journal of the Am erican 17 Longo DL, Jameson JL, Kaspe D. Harrison's Principles of Internal 18
College of Cardiology. 2023 Jan 3;81(1):81-94. Medicine: Volume 2. Macgraw-Hill; 2011.

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PATHOGENESIS OF ARF
DIAGNOSIS

The diagnosis of ARF rem ains dependent on clinical

criteria first described by Dr. T. Duckett Jones in 1944

Dougherty S, Okello E, M wangi J, Kum ar RK. Rheum atic heart

19 disease: JACC focus sem inar 2/4. Journal of the Am erican 20
College of Cardiology. 2023 Jan 3;81(1):81-94.

19 20

DIAGNOSIS DIAGNOSIS

The criteria have undergone M ore recently, in 2015, the

num erous revisions to m aintain Dr. Am erican Heart Association has
Jones’s original intention of supported a fundam ental overhaul
m aintaining high specificity for of the Jones criteria to account for
ARF in low-risk populations. the sharp contrast in disease
burden between low-risk and
m edium / high-risk areas.

Gewitz MH, Baltimore RS, Tani LY, Sable CA, Shulman ST, Carapetis
J, Remenyi B, Taubert KA, Bolger AF, Beerman L, Mayosi BM.
Revision of the Jones Criteria for the diagnosis of acute rheumatic
Dougherty S, Okello E, M wangi J, Kum ar RK. Rheum atic heart fever in the era of Doppler echocardiography: a scientific statement
21 22
disease: JACC focus sem inar 2/4. Journal of the Am erican from the American Heart Association. Circulation. 2015 May
College of Cardiology. 2023 Jan 3;81(1):81-94. 19;131(20):1806-18.

21 22

D ougherty S, Okello E, M wangi J, Kum ar RK. Rheum atic heart

disease: JACC focus sem inar 2/4. Journal of the Am erican

DIAGNOSIS
College of Cardiology. 2023 Jan 3;81(1):81-94.
PREVENTION AND CONTROL

In the guidelines has been the In the guideli

addition of echocardiography for addition of ec
the diagnosis of carditis. Detection the diagnosis o
of pathologic m itral and/or aortic of pathologic m
regurgitation by regurgitation
echocardiography, regardless of echocardiograp
the presence or absence of a the presence
m urm ur, is now also considered m urm ur, is no
diagnostic of carditis diagnostic of ca

D ougherty S, Okello E, M wangi J, Kum ar

RK. Rheum atic heart disease: JACC focus
23 sem inar 2/4. Journal of the Am erican 24
College of Cardiology. 2023 Jan 3;81(1):81-
94.

23 24

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PREVENTION AND CONTROL PREVENTION AND CONTROL Primordial Prevention

• Requires
M EDICAL M ANAGEM ENT improvement in M EDICAL M ANAGE
living conditions and
an effective vaccine.

Secondary Prevention
Secondar
• Involves preventing
progression of latent • Involve
or clinical RHD. progre
• A large single-center or cl
Primary Prevention trial in Uganda • A large
Primordial Prevention trial
assessed the impact Primary Preventi
• Targeted at early of 4-weekly assesse
• Requires
detection and intramuscular • Targeted at ear of
improvement in treatment of GAS
living conditions and benzathine penicillin detectionintr
and
pharyngitis and G (BPG) injections on treatment of G
benzat
an effective vaccine. impetigo. G (BPG
the progression of pharyngitis an
latent RHD over 2 impetigo.
the pr
years showed that a latent
strong protective years s
effect of BPG. stron
• Recommend effe
penicillin prophylaxis • Rec
for latent RHD penicill
for l

Dougherty S, Okello E, M wangi J, Kum ar Dougherty S, Okello E, M wangi J, Kum ar

RK. Rheum atic heart disease: JACC focus RK. Rheum atic heart disease: JACC focus
sem inar 2/4. Journal of25the Am erican sem inar 2/4. Journal of26the Am erican
College of Cardiology. 2023 Jan 3;81(1):81- College of Cardiology. 2023 Jan 3;81(1):81-
94. 94.

25 26

MANAGEMENT OF ARF MANAGEMENT OF CLINICAL RHD

M EDICAL M ANAGEM ENT M EDICAL M ANAGEM ENT

M edical m anagem ent is often all that can be offered initially, M edical m anagem ent is often all that can be offered initially, and it is
Secondary Prevention
and it is targeted at consequences of valvular regurgitation targeted at consequences of valvular regurgitation or stenosis,
or stenosis, including heart failure and atrial fibrillation (AF). • Involves preventing including heart failure and atrial fibrillation (AF).
progression of latent
or clinical RHD.
• A large single-center
Primary Prevention trial in Uganda
Primordial Prevention
assessed the impact Isolated m itral stenosis benefits significantly from reductions in heart
• Targeted at earlyof 4-weekly
• Requires
detection andintramuscular rate, which im proves diastolic filling and reduces the transvalvular
improvement in treatment ofbenzathine
GAS
living conditions and penicillin gradient à Beta-blockers or digoxin for those w ith AF. Ivabradine
pharyngitisGand
(BPG) injections on
the progression of is an additional option for patient w ith sinus rhytm .
an effective vaccine. impetigo.
latent RHD over 2
years showed that a
strong protective
effect of BPG.
Severe regurgitation of the m itral or aortic valves à ACEi or ARB can
• Recommend
reduce
penicillin the afterload thus partially m itigating the physiological
prophylaxis
for latent RHD
consequences. However, there is no evidence showing long-term
benefits.
Dougherty S, Okello E, M wangi J, Kum ar
RK. Rheum atic heart disease: JACC focus Dougherty S, Okello E, M wangi J, Kum ar
sem inar 2/4. Journal of the Am erican RK. Rheum atic heart disease: JACC focus
College of Cardiology. 2023 Jan 3;81(1):81-
27 sem inar 2/4. Journal of the Am erican 28
94. College of Cardiology. 2023 Jan 3;81(1):81-
94.

27 28

MANAGEMENT OF CLINICAL RHD

M EDICAL M ANAGEM ENT

• Angiotensin II contributes to inflam m atory process. ACEIs suppress the release of Th1 and
Beta-blockers have also been used in regurgitant lesions. Th17 cytokines and induces regulatory T-cells.

• Continuous inflam m ation prom oted by sST2 and m ediated by NF-κB causes valvular
dam age in RHD.
• Cardio-protection effect of ACEIs are m ediated by:
System ic vena congestion due to tricuspid valve involvem ent à
• ACEIs inhibit the production of Angiotensin II
diuretic agents.
• ACEIs decrease IL-33 binding to sST2
• ACEIs inhibit TGF-β/MAPK/Smad signaling

Patients with clinical RHD who have had surgery m ay need

lifelong penicillin prophylaxis, or at least until age 40 years.
Three-to-four weekly BPG is the first-line choice for secondary
prophylaxis of ARF and RHD.

D ougherty S, Okello E, M wangi J, Kum ar Ambari, A.M. et al. (2020) Front. Cardiovasc. Med.
RK. Rheum atic heart disease: JACC focus
sem inar 2/4. Journal of the Am erican 29 30
College of Cardiology. 2023 Jan 3;81(1):81-
94.

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MANAGEMENT OF CLINICAL RHD

M EDICAL M ANAGEM ENT CATHETER-BASED THERAPY

Beta-blockers have also been used in regurgitant lesions.

Patients with clinical RHD who have had

surgery m ay need lifelong penicillin System ic vena congestion due to tricuspid valve involvem ent à
prophylaxis, or at least until age 40 years. diuretic agents.
Three-to-four weekly BPG is the first-line
choice for secondary prophylaxis of ARF
and RHD.

Dougherty S, Okello E, M wangi J, Kum ar

RK. Rheum atic heart disease: JACC focus
sem inar 2/4. Journal of the
31 Am erican 32
College of Cardiology. 2023 Jan 3;81(1):81-
94.

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MANAGEMENT OF CLINICAL RHD

CATHETER-BASED THERAPY SURGICAL INTE
CATHETER-BASED THERAPY

• Good adherence to penicillin administration significantly reduced the odds of Patients with severe sym ptom atic m itral stenosis and favorable valve
ARF recurrence and RHD progression by up to 63% compared to the poor Beta-blockers
m orphology,have also been used
percutaneous in regurgitant
m itral lesions.
balloon com m issurotom y (PM BC) is the
adherence (pooled OR 0.37 [0.24-0.57]; I²=0% [p=0.94]; Z=4.54 [p<0.00001]) treatm ent of choice

System ic vena
The best congestion
candidates due to BC
for PM tricuspid
are: valve involvem ent à
diuretic agents.
• Isolated m itral stenosis and pliable
• Noncalcified valves that are predom inantly fused at
the com m issures with lim ited subvalvular
pathology
• No left atrial throm bus.

Forest plot on good adherence to penicillin as secondary prevention of ARF recurrence Isolated aortic stenosis generally not suited for ballon
and RHD progression dilatation à Trans-catheter aortic valve im plantation

Ambari, A.M. et al. (2023) Dougherty S, Okello E, M wangi J, Kum ar

RK. Rheum atic heart disease: JACC focus
33 sem inar 2/4. Journal of the Am erican 34
College of Cardiology. 2023 Jan 3;81(1):81-
94.

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MANAGEMENT OF CLINICAL RHD

SURGICAL INTERVENTION CATHETER-BASED THERAPY

D ougherty S, Okello E, M wangi J, Kum ar D ougherty S, Okello E, M wangi J, Kum ar

RK. Rheum atic heart disease: JACC focus RK. Rheum atic heart disease: JACC focus
sem inar 2/4. Journal of the Am erican 35 sem inar 2/4. Journal of the Am erican 36
College of Cardiology. 2023 Jan 3;81(1):81- College of Cardiology. 2023 Jan 3;81(1):81-
94. 94.

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Challenges in Indonesia
Suggestion for RHD
management
• No RHD screening program à undetected ARF • Enable screening of ARF and RHD in prim ary
leading to progression to RHD care
• Lack of disease surveillance and insufficient • Creation of a sustainable supply chain of
data penicillin
• High cost of diagnostic technology and skilled • Procurem ent of sufficient diagnostic facilities
staff for RHD and training of staff

• Lack of awareness am ong health care • Establishing policies to m anage RHD cases
professionals
• Building a system atic registry and database
• Unavailability of penicillin in the prim ary health
care

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• Collaboration between prim ary health care centers (Puskesm as) and referral hospitals

• Patients with a high index of clinical suspicion for RHD should be referred to secondary hospitals.
Patients will be referred back to Puskesm as for secondary prevention to avoid recurrent ARF.

TERIMA
KASIH

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