rheumat

Rheumatic fever and valvular vices

Aiyub medicine

Formation of the immune complexes

Proliferative arterial vasculities

Mucoide Intumescences

Fibrinoid intumescences

Fibrinoid necrosis of the connective tissue

Inflammatory reaction stage

Rheumatic fever

Acute rheumatic fever inflammatory disease with devastating sequelae Link to pharyngeal infection with group A beta hemolytic streptocci Continues to be a problem worldwide - sporadic outbreaks in developed countries - frequent occurrences in developing countries Still gaining understanding of etiology - link between genetic predisposition and clinical

manifestations Best prevention still correct use of antibiotics

Etiology

Angina

Immune response

Lymphatic node

Lymphocyte -BAntistreptococal antibody

Blood vesselHeart involvement

vegetation Aschoff Bodies Fibrinous pericarditis

Pathogenesis

1048708 Group A strep pharyngeal infection precedes clinical manifestations of ARF by 2 - 6 weeks1048708 Antibodies made against group A strep cross-react with human tissue1048708 heart valve and brain share common antigenic

sequences with GAS bacteria1048708 theory of molecular mimicry1048708 Host immune responses may play a role in determining who gets ARF following infection1048708 Virulent strains rheumatogenic serotypes

CLINICAL FEATURES

Migratory Polyarthritis

MyocarditisSubcutaneous

nodulesErythema

marginatumSydenham chorea

Arthritis

1048708 Most common feature present in 80 ofpatients1048708 Painful migratory short duration excellentresponse of salicylates1048708 Usually gt5 joints affected and large jointspreferred1048708 Knees ankles wrists elbows shoulders1048708 Small joints and cervical spine less

commonly involved

Subcutaneous Nodules

Usually 05 - 2 cm long Firm non-tender isolated or in clusters Most common along extensor surfaces of

joint Knees elbows wrists Also on bony prominences tendons dorsi

of feet occiput or cervical spine Last a few days only Occur in 9 - 20 of cases Often associated with carditis

Erythema Marginatum

Present in 7 of patients Highly specific to ARF Cutaneous lesion Reddish pink border Pale center Round or irregular shape Often on trunk abdomen inner arms or

thighs Highly suggestive of carditis

Sydenhamrsquos Chorea

1048708 Extrapyramidal disorder1048708 Fast clonic involuntary movements

(especially face andlimbs)1048708 Muscular hypotonus1048708 Emotional lability1048708 First sign difficulty walking talking writing1048708 Usually a late manifestation months after

infection1048708 Often the only manifestation of ARF

Carditis

Most serious manifestation May lead to death in acute phase or at later stage Any cardiac tissue may be affected Valvular lesion most common mitral and aortic Seldom see isolated pericarditis or myocarditis Mitral and aortic regurgitation most common Apical systolic and basal diastolic murmurs Pericarditis usually asymptomatic Occasionally causes chest pain friction rubs or distant heart sounds

ACUTE-Inflammation

-Aschoff bodies

-Anitschkow cells

-Pancarditis

-Vegetations on chordae tendinae at leaflet junction

CHRONICTHICKENED VALVES

COMMISURAL FUSION

THICK SHORT CHORDAE TENDINAE

Acute Rheumatic vegetations

Fish mouth Mitral stenosis

Rheumatic endocarditis

Diffuse endocarditis

valvulitis Verucous acute

endocarditis Fibroplastic

endocarditis Recurrent

verucous endocarditis

Diffuse endocarditis

Recurrent verucous endocarditis

McCallum plaques in the left atrium

McCallum plaques

Aortic valve calcification

Left atrium dilation and left ventricle hypertrophy

Fish mouth mithral opening

Shortening of the tendineum cords

Granulomatous stage of RF Aschoff nodulesşi and Anitschkow cells

Interstitial granulomatous myocarditis

Exudative diffuse interstitial myocarditis

Focal exudative interstitial myocarditis

Rheumatic myocarditis

Interstitial granulomatous myocarditis

Interstitial granulomatous myocarditis(H-E)

Exudative diffuse interstitial myocarditis

Rheumatic pericarditis

EXUDATE SEROUS FIBRINOuS MIXED

Fibrinous pericaditis

Diagnosis Jones Criteria

Major criteriabull Arthritisbull Carditisbull Sydenhamrsquos choreabull Erythema

marginatumbull Subcutaneous

nodues

Minor criteriabullFeverbullArthralgiabullElevated c-reactive protein orbullErythrocyte sedimentation ratebullProlonged PR interval on EKG

CONGENITAL HEARTDEFECTS

Faulty embryogenesis (week 3-8)Usually MONO-morphic (ie SINGLE

lesion) (ASD VSD hypo-RV hypo-LV)May not be evident until adult life

(Coarctation ASD)Overall incidence 1 of USA birthsINCREASED simple early detection via

non invasive methods eg US MRI CT etc

Tricuspid atresia

1emsp120emspTotal anomalous pulmonary venous connection

1emsp136emspTruncus arteriosus

4emsp388emspTransposition of great arteries

4emsp388emspAortic stenosis

4emsp396emspAtrioventricular septal defect

5emsp492emspCoarctation of aorta

5emsp577emspTetralogy of Fallot

7emsp781emspPatent ductus arteriosus

8emsp836emspPulmonary stenosis

101043Atrial septal defect

424482Ventricular septal defect

Incidence per Million Live

BirthsMalformation

CONGENITAL HEARTDEFECTS

LR SHUNTS all ldquoDrsquosrdquo in their names NO cyanosis Pulmonary hypertension SIGNIFICANT pulmonary hypertension is

IRREVERSIBLERL SHUNTS all ldquoTrsquosrdquo in their names

CYANOSIS VENOUS EMBOLI become SYSTEMIC

OBSTRUCTIONS

LRASDVSDASVDPDA

NON CYANOTIC

IRREVERSIBLE PULMONARY HYPERTENSION IS THE MOST FEARED CONSEQUENCE

ASDNOT patent foramen ovaleUsually asymptomatic until

adulthoodSECUNDUM (90) Defective fossa

ovalisPRIMUM (5) Next to AV valves

mitral cleftSINUS VENOSUS (5) Next to

SVC with anomalous pulmonary veins draining to SVC or RA

VSDBy far most common CHD defectOnly 30 are isolatedOften with TETRALOGY of FALLOT90 involve the membranous septum If muscular septum is involved likely to

have multiple holesSMALL ones often close spontaneouslyLARGE ones progress to pulmonary

hypertension

PDA90 isolatedHARSH machinery-like murmurLR possibly RL as pulmonary

hypertension approaches systemic pressure

Closing the defect may be life saving

Keeping it open may be life saving (Prostaglandin E) Why

AVSDAssociated with defective inadequate AV valves

Can be partial or COMPLETE (ALL 4 CHAMBERS FREELY COMMUNICATE)

RLTetralogy of FallotTransposition of great arteries

Truncus arteriosus

Total anomalous pulmonary venous connection

Tricuspid atresia

RL SHUNTSTETRALOGY of FALLOT most COMMON

1) VSD large 2) OBSTRUCTION to RV flow 3) Aorta OVERRIDES the VSD 4) RVH SURVIVAL DEPENDS on SEVERITY of

SUBPULMONIC STENOSIS Can be a ldquoPINKrdquo tetrology if pulmonic obstruction

is small but the greater the obstruction the greater is the RL shunt

TGA (TRANSPOSITION of GREAT ARTERIES)

NEEDS a SHUNT for survivalPDA or PFO (65)

ldquounstablerdquo shuntVSD (35) ldquostablerdquo

shuntRVgtLV in thicknessFatal in first few monthsSurgical ldquoswitchingrdquo

TRUNCUS ARTERIOSIS

TRICUSPID ATRESIA

Hypoplastic RVNeeds a shunt ASD VSD or

PDAHigh mortality

Total Anomalous Pulmonary Venous Connection (TAPVC)

PULMONARY VEINS do NOT go into LA but into L innominate v or coronary sinus

Needs a PFO or a VSDHYPOPLASTIC LA