Rheumatic fever and

Rheumatic Heart Disease (RHD)

2017
 Anatomy and physiology of Heart
 Heart.
• A hollow and muscular organ, the base above and the
apex below. The apex inclines towards the left side.

 Position of the heart.

• Lies in the thoracic cavity in the mediastrinum
between the lungs and behind the sternum.
 Cont…
 Structure of the heart.
• Four chamber (rt.&lt.) artrium and (rt.&lt.)
Ventricles.
• Four valves they are tricuspid (three flaps or
cusps), mitral (Two flaps), aortic, and pulmonary.
• Composed of three layers of specialized cardiac
muscles
o Pericardium,
o Myocardium, and
o Endocardium.
Anatomy and physiology of Heart
 Introduction
• Rheumatic fever is a complication of
untreated streptococcus throat infection
caused by bacteria called group (group A
beta‑hemolytic streptococcus)
• Acute rheumatic fever primarily affects the
heart, joints and central nervous system
and it is estimated that approximately 60
percent of patients with ARF will develop
RHD 
 Introduction
• Rheumatic fever can cause permanent
damage to the heart known as rheumatic
heart disease.

• Rheumatic fever causes RHD and is the

most common cause of cardiac mortality
in children and adults aged less than 40
years.
 Introduction cont…
• Rheumatic fever (RF) and rheumatic heart disease
(RHD) continue to be a major health hazard in most
developing countries as well as in developed countries

• Rheumatic fever is most common in 5- to 15-year-old

children, though it can develop in younger children and
adults
• Socio economic and environmental factors play a
greater role in contributing to magnitude and severity
of Rheumatic fever
 Global scenario
• 31 % of global death are due to
CVDS(WHO, 2015)
• Globally, about 2% of deaths from
cardiovascular diseases is related to
rheumatic heart disease (WHO, 2015)
• A minimum of 15 million people
worldwide have the disease, resulting in
300,000 deaths each year (WHO, 2015)
 Global scenario
• World wide the incidence is estimated to be
19/100,000 ( Bharati and Bharati, 2014)

• At least 15.6 million people are estimated to

be currently affected by RHD with significant
number of them requiring
hospitalization(World Heart federation, 2012)
Global scenario of RHD in world
 South east Asia scenario
• In developing countries of southeast Asia
rheumatic fever is leading cause of death
in children with 5 lakhs of patients
annualy (Bharati & Bharati, 2014)

• South east Asia prevalence of RHD is

48/1000 population.
 South east Asia scenario
•  In India, 47 - 59% of all cardiac admission to
hospitals in major urban centres are said to be
attributed to Rheumatic Heart Disease which is
higher than rest of the world
• China prevalence of RHD is 1.86/1000 in
the adult population.
 National Scenario

Rheumatic heart disease morbidity(national level)

Year Case

• 2010 899
• 2011 843
• 2012 718
• 2013 664
• 2014 1629

Source:(AHB.2015).
Cont…

• Rheumatic heart disease mortality (national level)

Year Cases
• 2010 17
• 2011 11
• 2012 14
• 2013 05
• 2014 11
 Definition
AS per WHO fact sheet, 2015
Rheumatic fever
Rheumatic fever is an inflammatory
disease that occurs following a
streptococcus pathogenesis infection such
as streptococcal pharyngitis that may
affect many connective tissue of the body.
 Rheumatic Heart diseases

Rheumatic heart disease refers to the

chronic heart valve damage that
occurs after a person has an episode
of acute rheumatic fever
 Causes of Rheumatic heart disease

• Group A beta‑hemolytic streptococcus.

• Repeated Rheumatic fever causes RHD

resulting from autoimmune response
 
 Risk factors of Rheumatic fever
• 5-15 years old children
• Family history- Some people may carry a gene or
genes that make them more likely to develop rheumatic
fever.
• Type of strep bacteria- Certain strains of strep
bacteria are more likely to contribute to rheumatic
fever than are other strains.
• Environmental factors- A greater risk of rheumatic
fever is associated with overcrowding, poor sanitation
and other conditions that may easily result in the rapid
transmission or multiple exposures to strep bacteria.
 Risk factors of RHD

• Chronic Phryngitis
Cont…

• Chronic Tonsilities
 Types of Rheumatic Heart disease

• Acute rheumatic heart disease

• Chronic rheumatic heart disease
 Pathogenesis

• There is a well –defined association

between group-A ß haemolytic
streptococcal pharyngitis and rheumatic
fever.
• After pharyngeal infection with group-A
ß haemolytic streptococci, specific
antibody is produced against cell was
constituents (N- acetyl-glycosamine) of
the bacteria.
 .
Condt..

• As heart valve or cardiac tissue glycoprotein

shares the same antigenic properties of (N-
acetyl-glycosamine), so the antibody produce
against bacterial cell wall cross-react with heart
valve
 Pathophysiology
Group A beta hemolytic
Streptococcus[antigen]

Pharyngitis[Produce
N-acetyl glucosamine, antibody]

Cross Reactivity

Heart[Same antigenic property]

Mitral Valve[90%]

Brain Skin Joints

Pathophysiology
 History
• A diagnosis of rheumatic heart disease is made
after confirming antecedent rheumatic fever.

• The modified Jones criteria (revised in 1992)

provide guidelines for the diagnosis of
rheumatic fever.
 The Jones criteria

• Major Criteria: • Minor Criteria:

• Carditis • Fever
• Arthralgia
• Arthritis
• Elevated acute-phase
• Sydenham’s chorea
• reactants: ESR, CRP
• Erythema marginatum • Prolongation of PR
• Subcutaneous nodules • interval on ECG
 Clinical Diagnosis for Rheumatic fever

It require the presence of:

 Two major or One major criteria
and
 Two minor criteria for the diagnosis
of rheumatic fever.
Manifestations of
Rheumatic fever

• Major criteria
 Arthritis

• Most frequent major

manifestation 60-85% of
patients have arthritis
during their first attack.

• Often presents as
migratory polyarthritis,
most often in the larger
joints.
 Arthritis:
• Joint pain with hot,
red, tender and
swollen and sever
pain (large joint
Such as Knee, ankle
and wrist joint).

Arthritis usually
subsides with in 3
week
Complication of Polyarthritis-
• Polyarthritis-sharp sudden pain starts from sternum
and radiates to neck, shoulder, back and arm
Carditis (heart problems ):

Central Chest pain,

breathlessness, palpitation
and murmur may present .

It is very dangerous as it
causes permanent damage to
the heart.
 Complication
• Of those with
valvular problems:
• Mitral
regurgitation is the
most common
• Aortic regurgiation

Mitral regurgitation
 Mitral
regurgitation
Chest Xary
 Physical Cardiac finding
•  A cardiac murmur of almost any kind (systolic or
• diastolic) is almost universal

•  A friction rub, distant heart sounds, or chest pain with

ECHO showing a pericardial effusion

•  Cardiomegaly

•  Unexplained CHF, almost always associated with

valvular involvement
Erythema nodosum
• Subcutaneous nodules
(erythema nodosum )
painful red/ blue
raised lumps over the
front of the lower legs
and the back
of the forearms.
Erythema nodosum
• 2-10% affected
• Painless nodules
over extensor
surfaces of large
joints, the occiput,
and/or vertebral
processes
cont…

• Subcutnous nodules-a firm movable non

tender and about 3mm in diameter
Erythema marginatum (Skin lesions)
• It starts as transient
pink or pale red
macula with fade
centre appears and
disappears within a
period hours.
 Sydenham’s chorea
• It is neurological
manifestation of
acute rheumatic
fever .
• 15% of patients
are affected
• Abnormal
behavior and/or
involuntary,
purposeless
 Other physical finding
Musculoskeletal
• Pain, limited motion, erythema, warmth of 2 or more
• large joints

Neurologic
• Abnormal movements

Dermatologic
• Pink rash with pale central region
• Firm painless nodules
 Investigations
 Blood CBC : (Leucocytosis ( W.B.C count increased ) and high
ESR )

 Anti-streptolysis O anitibody (ASO titres) : Rising titre > 200

units ( adult) and > 300 unit for children.

 Chest X-ray : Cardiomegaly ( Enlargement of heart).

 Throat swab culture : Group A beta haemolytic streptococci .

 ECG

 Echocardiography : Cardiac dilatation and valve abnormalities .

ECHO left side of the heart
 Primary Treatment

Antibiotics
• Full course of penicillin to eradicate the active
Strep

For infection
• Normally Penicillin V ( 250 mg) qids for 10 days
• Anti-inflammatory
• High dose aspirin is standard
• Aspirin 60-100 mg/kg/day
 Primary treatment

• Reduced when fever and acute phase

reactants have normalized for 6-8
weeks
• Steroids may work for severe carditis
but arecontroversial
 Cardiac support

• Aggressive support of cardiac function

systemic after load reduction for severe disease
• Surgical valve replacement
• In Bhutan requires a referral to India
• Bed rest is controversial
• But recommended for severe carditis
 Secondary Prevention

• Administration of Benzathine Penicillin

G ( 6-12 lakh units) I/M every 3weeks
(every 3 weeks is recommended in high-
risk situations)
 Treatment for children

• Child under 30 kg: 600 000 IU and

children
• Adult over 30 kg: 1.2 million I/M in
buttock, on diagnosis, once weekly
for 3-4 weeks then monthly for 1 year
to 5 years.
• Follow up by ECG and
Echocardiography, yearly.
Duration of antibiotic is based on clinical presentation
• ARF without cardiac involvement: 5
years or until age
• 18, whichever is longer
• ARF with mild or resolved carditis: 10
years or until age
• 25, whichever is longer
• ARF with severe carditis or cardiac
surgery: lifelong
Refer the patient as soon as possible;
– All new patients – for diagnosis by a
doctor
– Heart murmur with developing of
cardiac symptoms and signs
– Worsening of clinical signs of heart
disease
– Any other newly developing medical
condition; fever
 Prophylactive treatment

• Tablet penicillin V (250mg ) 1 tablet

BD for Life long
 Surgical
• Vulvular
replacement
 Follow up check up:

By yearly such as;

ECG
Echocardiography
Blood for ASO titre and CBC, ESR.
 Prognosis
• Acute Rheumatic Fever recurrence rate is as high as
36% without prophylaxis

• Chorea may last weeks to months and has a similarly

high recurrence rate

• Carditis may resolve spontaneously (70-80%) or progress.

• Severity of the initial carditis is a major determinant of

progression.
• Complications of Rheumatic fever
• Heart failure
• Valvular diseases
• Percarditis
• Percardial effusion
 Long-term Complications
• Mitral stenosis
• Mitral regurgitation
• Aortic stenosis
• Aortic regurgitation
• Chronic Heart Failure
 Health education

• Prevent from getting sore throat especially children

• Take early treatment if there is throat pain and cough
• Take antibiotic regularly and in full course
• Drink lots of hot water and do warm saline gargle
• Prevent from frequent infection from sore throat
• Keep your neck warm and cover with cloth
• Get regular check up by doctors if there is frequent
infection
 Primary prevention
• Good dental and oral hygiene

• Get vaccination of influenza and pneumococcal vaccinations

• Good prenatal care, and care during pregnancy

• Avoid from dust and maintain good sanitation

• Early diagnosis of streptococcus infection

 Secondary prevention

• Early prevention, diagnosis and, antibiotic treatment

• Regular preventative antibiotic with full course

• Treat streptococcus throat infections

• Regular check-ups with a cardiologist

• Prevent spread of streptococcus infection to others

 Tertiary prevention
• Hospital admission of chronic rheumatic fever

• Prevent from complication

• Heart valve surgery to repair or replace

• Rehabilitative services to chronic patients

 References
WHO fact sheet. (2015). Cardiovascular diseases.
Retrieved from
http://www.who.int/mediacentre/factsheets/fs317/en/
Sudeep, D. D., Sredhar, K. (2015). The Descriptive
Epidemiology of Acute Rheumatic Fever and
Rheumatic Heart Disease in Low and Middle-Income
Countries. American journal of epidemiology and
infectious disease. currrent Issue: Volume 3, Number
3, 2015
 References cont…
Walker, B. R., Colledge, N. R., Ralston, s. H., & Penman,
I. D. (2014). (22nd EDS). Davidson’s principles &
practice of medicine.London: Elsevier limited.
MINISTRY of Health. (2013). Package of essential
NCD(PEN): Protocol for BHU’s. Phuntsholing, Bhutan:
Thinley pelbar publishers and printers.
Bharati, S., & Bharati, s. (2011). Etiopathogenesis
and pathology of carditis in rheumatic fever.
Retrieved on 3/8/2015 from
https://books.google.bt/books?id=VAM8gg_m
xz0C&pg=PR15&lpg=PR15&dq=Bharti+rhe
umatic+heart+diseases&source=bl&ots