DR.MAGDI AWAD SASI

CARDIOLOGY 7TH OCTOPER

LMB

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%.

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

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.

Carditis

Polyarthritis

Chorea

Subcutaneous nodules

Erythema marginatum

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

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.

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

vegetations Aschoff body pericarditis

Strep throat

Antibody

production

Antibody cross-reaction

with heart

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.

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

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

Occur in 10%

Painless,pea-sized,palpable nodules

Painless, hard nodules beneath skin, over bony

prominence,

tendons and joints

Always associated with severe carditis

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.

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)

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.

.

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.

New or changing murmur

Tachycardia

Signs of heart failure

Auscultary findings depends upon the valve involved

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.

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

•Rapid antigen detection test

specificity >95%

sensitivity 60-90%

Extracellular- ASO

Anti DNAse B

Antihyluronidase

Cellular-Antiteichoic acid

Anti M PROTEIN Ab

Rheumatic fever is mainly a clinical diagnosis

No single diagnostic sign or specific laboratory

test available for diagnosis

Diagnosis based on MODIFIED JONES CRITERIA

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)

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

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

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

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

Bed rest

Treatment of congestive cardiac failure:

Restrict fluids

Restrict salt

Diuretics therapy

Inotropic support

After load reduction

Digoxin

Treatment of chorea: -

diazepam or haloperidol

Rest to joints & supportive splinting

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

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-

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.

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.

Etiology

Physical Examination

Assessing Severity

Natural History

Prognosis

Timing of Surgery

Causes •

1) Rheumatic fever

2) Congenital abnormality, calcification,

Natural history

•RF age 12

•Murmur 1st heard 20 yrs later

• Symptoms in 3-4th decade

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

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

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

• Balloon Mitral Valvuloplasty

Open mitral valvotomy

•Mitral valve replacement

- 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

Acute dyspnoea, orthopnoea

no cardiomegaly, short murmur, S3

Chronic variable sypmtoms

cardiomegaly, murmur, P2 loud, S3

Quantification:

Echocardiography, angiography

Serial studies, LV function

Symptomatic severe - survival 33% at 5 years

mortality ~5% per year

LV dysfunction most important factor

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

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)

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

Congenital 1st Congenital 1st -3rd decade

Valve degeneration and calcification

Rheumatic - 4th decade

Bicuspid valve; 1%, males

males >females, 5 -6th decades

•None

•DOE, dizziness

•HF, syncope, angina

Examination

•Pulse - ↓amplitude, delay

•Sustained apex

•S2- soft and single → paradoxical splitting

•ESM - loud → late peak → soft

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

© 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

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…...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

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Widened pulse pressure

Systolic – diastolic =pulse pressure

High pitched, blowing, decrescendo

diastolic murmur at LSB

Best heard at end-expiration & leaning

forward

© 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

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

Congenital

Leads to RVH with decrease blood flow to the

pulmonary circulation

Clinically—ESM in PA

DX---ECHO

TREATMENT--surgery

Seen in pulmonary HTN

IT is called GRAMM STEEL M.

Can lead to dyspnea

Rare primary

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