This document discusses rheumatic fever and rheumatic heart disease. Some key points: - Rheumatic fever is an autoimmune disease that can develop after a streptococcal throat infection, causing inflammation in the heart, joints, brain, and skin. It is characterized by Jones criteria and symptoms include carditis, arthritis, chorea, and subcutaneous nodules. - Rheumatic heart disease is scarring of the heart valves caused by repeated rheumatic fever infections. It can lead to stenosis or regurgitation of the valves. Symptoms depend on which valves are affected and include heart failure. - Treatment involves antibiotics to prevent recurrent infections, anti-inflammatories, management

1. DR.MAGDI AWAD SASI
CARDIOLOGY 7TH OCTOPER
LMB

2.  Definition: is an acute, immunologically mediated, multi-
system inflammatory disease that follows, after a few weeks
of an episode of group A-beta hemolytic streptococcal
infection with cardiac and extra cardiac manifestations.
 It is characterized by inflammatory reaction involving heart
60% of patients affected by RF, joints in 75% , central nervous
system in 10 % and skin in 2%.

4. For rheumatic fever to occur:
 Pharyngeal infection with group A streptocooci
 Certain rheumatogenic strains of GAS with M proteins
 Throat infection of sufficient duration- persistence of GAS
 Throat infection may or may not be symptomatic
 Throat infection is a must, not with pyoderma( skin
infection)
 Infection of sufficient duration to produce antibody
 Brisk and sufficient antibody response to the infection
 Genetic predisposition

5.  Jones criteria for initial attack of rheumatic
fever
 Evidence of preceding streptococcal infection
 + 2 major manifestations or one major
manifestation and 2 minor manifestations
indicates a high probability of acute rheumatic
fever.

6.  Carditis
 Polyarthritis
 Chorea
 Subcutaneous nodules
 Erythema marginatum

7.  Clinical findings-
A. Arthralgia (joint pain without swelling )
B. Fever
 Laboratory findings-
C. Elevated acute phase reactants
 Raised ESR
 Raised CRP
D. Prolonged P-R interval

8.  Supporting evidence for antecedent Group A
streptococcal infection
1. Positive throat culture (in 25% of patients &
75% will be –ve)
2. Rapid streptococcal antigen test
3. Elevated or rising streptococcal antibody titer
– ASO [anti-streptolysin]
4. ( others- Anti DNAseB, AH [anti-hyoluronic
acid] )
 If these antibodies ( >300 in children >200 in
adults) suggest previous infection.

9.  Occurs 10 days to 6 weeks after pharyngitis caused by
strept infection so anti streptolysin O (ASO) titer will be
high.
 Peak incidence: in children 5-15 years.
 Acute carditis: pericardial friction rubs, weak heart sounds,
tachycardia and arrhythmias.
 Extracardiac: fever, migratory polyarthritis of large joints,
arthralgia, skin lesions, chorea

10. vegetations Aschoff body pericarditis
Strep throat
Antibody
production
Antibody cross-reaction
with heart

11.  Affect large joints as knee,ankle which show:
 Redness, swollen,hot.
 Fleeting , migratory.
 No residual deformity, rapid response to aspirin
 given,( 24to48hrs joint pain will disappear) ;thus used as
 diagnostic test)
 Inflamed joints , self limited
 Become normal within 1-3 days even without
treatments so no chronic deformities.

12.  5-10% of cases
 Mainly in girls of 1-15 yrs age
 Clinically manifest as-clumsiness, deterioration of
handwriting,emotional lability or grimacing of face
 A characteristic series of rapid movements of the face
and arms. This occurs late in the disease

13.  Occur in <5%.
 Unique,transient,serpiginous-looking lesions of
1-2 inches in size
 Pale center with red irregular margin
 More on trunks & limbs & non-itchy
 Worsens with application of heat
 Often associated with chronic carditis

15.  Occur in 10%
 Painless,pea-sized,palpable nodules
 Painless, hard nodules beneath skin, over bony
prominence,
 tendons and joints
 Always associated with severe carditis

18.  It is a heart disease caused by rheumatic fever.
 Rheumatic heart disease can be acute or chronic.
 The incidence and mortality of rheumatic fever has
declined over the past 30 years due to improved
socioeconomic condition and rapid diagnosis and
treatment of group A beta hemolytic streptococcus
infection of the Pharynx or skin.

20.  Manifest as pancarditis
 40-50% of cases
 Carditis leaves a sequlae + permanent damage to the organ
 Valvulitis occur in acute phase
 Chronic phase- fibrosis, calcification & stenosis of heart
valves( fishmouth valve)

21. It affects all the 3 layers of the heart;
 Affect the heart during its acute phase  acute rheumatic
carditis/ pancarditis (inflammation of endocardium,
myocardium and pericardium)
1- Endocarditis — vegetations due to edema, and fibrin
deposits on valve leaflets along lines of closure. Mostly mitral
and aortic valve.
.

22.  2 - Myocarditis- presents with heart failure symptoms.
 Left ventricular failure = respiratory symptoms
Dyspnea ,paroxysmal nocturnal dyspnea ,orthopnea ,cough
,sputum –watery ,wheezing ,chest pain
 right ventricular failure = systemic swelling /symptoms
Leg swelling ,abdominal swelling ,right hypochondrial pain,
Nausea ,vomiting , change of bowel habit ,constipation.

23.  New or changing murmur
 Tachycardia
 Signs of heart failure
 Auscultary findings depends upon the valve involved

25. 3- Pericarditis — chest pain on laying on the back
 Acute changes may resolve completely or progress to
scarring and development of chronic valvular
deformities many years after the acute disease.

26. CBC, ESR
CRP
RFT ,K ,NA
THROAT SWAP
ASO TITRE
 ASO titre >200 Todd units.(Peak value attained at 3 weeks,
then comes down to normal by 6 weeks)
 Throat culture-GABH streptococci but negative when RHD
appear
ECG
CXR

27. •Rapid antigen detection test
 specificity >95%
 sensitivity 60-90%
Extracellular- ASO
Anti DNAse B
Antihyluronidase
Cellular-Antiteichoic acid
Anti M PROTEIN Ab

28.  Rheumatic fever is mainly a clinical diagnosis
 No single diagnostic sign or specific laboratory
test available for diagnosis
 Diagnosis based on MODIFIED JONES CRITERIA

30.  Step I - primary prevention
(eradication of streptococci)
 Step II - anti inflammatory treatment
(aspirin,steroids)
 Step III- supportive Tx & management of complications
 Step IV- secondary prevention
(prevention of recurrent attacks)

31.  Bed rest 2-6 weeks (till inflammation subsided)
 Supportive therapy - treatment of heart failure
 Anti-streptococcal therapy - Benzathine penicillin( long
acting) 1.2 million units once (IM injection) or oral
penicillin 10 days, if allergic to penicillin erythromycin 10
days
 (antibiotic is given even if throat culture is negative)
 Anti-inflammatory agents -
 Aspirin 100 mg/kg per day for arthritis and in the absence
of carditis- for 4-6 weeks to be tapered off
 Corticosteroids in presence of carditis – 1-2 mg/kg per day
– for 4-6 weeks to be tapered off

32.  Clarithromycin (in patients allergic to penicillin)
7.5 mg/kg PO bid for 10 days
 Azithromycin (in patients allergic to penicillin)
12 mg/kg (not to exceed 500 mg) PO OD for 5 days

33.  Aspirin indicated
100 mg/kg/day q.i.d po x 3-5 days
Then,
75 mg/kg/day q.i.d po x for 4 wks

34.  Prednisolone
2-3 mg/kg/day x 2-3 weeks
Tapered by 5 mg/day every 3-5 days
 Aspirin
Added 75mg/kg Q.I.D for 6 wks

35.  Bed rest
 Treatment of congestive cardiac failure:
Restrict fluids
Restrict salt
Diuretics therapy
Inotropic support
After load reduction
Digoxin

36.  Treatment of chorea: -
diazepam or haloperidol
 Rest to joints & supportive splinting

37.  Secondary prevention – prevention of recurrent attacks
 Benzathine penicillin G 1.2 million units IM every 4 weeks
Or Penicillin V 250 mg twice daily orally
Or Sulfadiazine 1 g daily orally
 If allergic to both – Erythromycin 250 mg twice daily orally

38.  Rheumatic fever + carditis + persistent valve disease-
10 years since last episode or until 40 years of age,
sometimes life long.
 Rheumatic fever + carditis + no valvar disease –
10 years or well into adulthood whichever is longer
Rheumatic fever without carditis-

39.  Valvular Endocarditis heals by progressive fibrosis
leading to Irreversible deformity in the form of:
a- stenosis (Reduction of diameter): fish mouth (button
hole) stenosis
b- regurgitation (improper closure) : if fibrosis
occurred in chordae tendonae so leaflets are retracted.
 Affection of the cardiac valves can also lead to
cardiac failure secondary to ventricular hypertrophy
then dilatation, thromboembolism and infective
endocarditis,pulmonary congestion and hypertension.

41. Left side valves –mitral and aortic wether
stenosis or regurgitation are presented with left
ventricular failure symptoms .
 LVF = CHEST SYMPTOMS
Right side valves –pulmonary and tricuspid
whether stenosis or regurgitation are presented
with right ventricular failure symptoms
 RVF= ABDOMENAL SYMPTOMS –LEG SWELLING
 Arrhythmia, thromboembolism and infective endocarditis.
 Treatment may require valve surgery.

42.  Etiology
 Physical Examination
 Assessing Severity
 Natural History
 Prognosis
 Timing of Surgery

43.  Causes •
1) Rheumatic fever
2) Congenital abnormality, calcification,
 Natural history
 •RF age 12
 •Murmur 1st heard 20 yrs later
 • Symptoms in 3-4th decade

44.  Severity Sypmptoms
 Mild Asymptomatic or mild DOE-dyspnea on exertion
 Moderate Mild mod - DOE; orthopnea PND, hemoptysis
 Severe Dyspnea at rest; possible pulmonary edema
 Very Severe Severe PHT; RV failure, marked dyspnea at
rest; severe fatigue; cyanosis

45.  Inspection :
 Malar flush ,Peripheral cyanosis (severe MS) Jugular venous
distension (right ventricular failure)
 Palpation:
 Parasternal right ventricular impulse Palpable pulmonary arterial
impulse Palpable S1, P2, and occasionally, the diastolic rumble
 Auscultation :
 Increased intensity of the first heart sound ,Low-pitched diastolic
rumbling murmur

47.  Medical :
 Diuretic - pulmonary congestion
 Prevent embolism - cause of 19% deaths,
↑with ↑LA size and LA size and ↑age
anticoagulate all with PAF/AF, SR in older age
 Control atrial fibrillation

48.  • Balloon Mitral Valvuloplasty
 Open mitral valvotomy
 •Mitral valve replacement

49.  - Aetiology:
 Primary
 Annulus annular calcification
 Leaflet
1) Myxomatous degeneration
2) Rheumatic deformity
 Chordae
1. Sppontaneous rupture
2. Rheumatic shortening
3. Infectious destruction
 Papillary infarction ischemic lengthening
 LV dilatation and PM displacement

50.  Acute dyspnoea, orthopnoea
 no cardiomegaly, short murmur, S3
 Chronic variable sypmtoms
cardiomegaly, murmur, P2 loud, S3
Quantification:
Echocardiography, angiography
Serial studies, LV function

51.  Symptomatic severe - survival 33% at 5 years
mortality ~5% per year
 LV dysfunction most important factor

52.  Acute
•Diuretics ↓LV filling P, ↓ p oedema
•Vasodilators ↑forward SV
•IABP
Chronic
No known effective therapy
Vasodilators - theoretical risks
Treat complications
ACEI--- if hypertensive
AF requires rate control, anticoagulation and 1 attempt at
restoration of SR

53.  Mitral valve repair
 Mitral valve replacement with preservation
of subvalvular apparatus
 Mitral valve replacement with excision of
subvalvular apparatus
 MVR with CABG (in ischemic MR)

54.  Normal aortic valve area is 3.0 - 4.0 cm2
 Circulation affected when valve area is
reduced by ~ 75% (i.e. 0.75 - 1.0 cm2)
valve area (cm sq) mean
gradient (mm Hg)*
Mild > 1.5 < 25
Moderate 1.0 - 1.5 25 - 50
Severe < 0.75 > 50
* assumes normal cardiac output

56.  Congenital 1st Congenital 1st -3rd decade
 Valve degeneration and calcification
 Rheumatic - 4th decade
 Bicuspid valve; 1%, males
males >females, 5 -6th decades

57.  •None
 •DOE, dizziness
 •HF, syncope, angina
Examination
•Pulse - ↓amplitude, delay
•Sustained apex
•S2- soft and single → paradoxical splitting
•ESM - loud → late peak → soft

60. Medical: medications and careful
follow-up
Surgical: Valve replacement is
the best approach in most cases

61. © Continuing Medical Implementation
…...bridging the care gap
 Palpitation
 Dyspnea
 Orthopnea
 PND
 Chest pain.
 Nocturnal angina >> exertional angina
 With extreme reductions in diastolic
pressures (e.g. < 40) may see angina

62. © Continuing Medical Implementation
…...bridging the care gap
 Quincke’s sign:
capillary pulsation
 Corrigan’s sign: water
hammer pulse
 De Musset’s sign:
systolic head bobbing
 Durosier’s sign:
femoral retrograde
bruits
 Traube’s sign: pistol
shot femorals
 Hill’s sign:BP Lower
extremity >BP Upper
extremity by

63. © Continuing Medical Implementation …...bridging the care gap
 Widened pulse pressure
 Systolic – diastolic =pulse pressure
 High pitched, blowing, decrescendo
diastolic murmur at LSB
 Best heard at end-expiration & leaning
forward

64. © Continuing Medical Implementation
…...bridging the care gap
 Apex:
 Enlarged
 Displaced
 Hyper-dynamic
 Palpable S3
 Austin-Flint
murmur
 Aortic diastolic
murmur
 length correlates with
severity (chronic AR)
 in acute AR murmur
shortens as
Aortic DP=LVEDP
 in acute AR - mitral
pre-closure

66.  Medical
 Afterload reduction: ACEI, nifedipine,
hydralazine
 Use BB cautiously, if at all, given prolonged
diastole and therefore  regurg volume
 Surgical
 AVR – 4% mortality alone, 6.8% with CABG
 LV dysfunction often irreversible, despite AVR

67.  Congenital
 Leads to RVH with decrease blood flow to the
pulmonary circulation
 Clinically—ESM in PA
 DX---ECHO
 TREATMENT--surgery

68.  Seen in pulmonary HTN
 IT is called GRAMM STEEL M.
 Can lead to dyspnea
 Rare primary

69.  Congenital
 2ry to left sided failure
 Symptoms of right heart failure
 Clinically ---raised JVP with leg swelling and
abdomenal distention with hepatomegally
 Valve replacement is the treatment of choice
in sever TR