1. Assessment of CVS & Murmurs
Rheumatic Fever
Assessment of CVS & Murmurs

2. Rheumatic Fever Definition:
Multisystem, Autoimmune, Inflammatory disorder of Skin, joint &heart.
Following group A, ß-hemolytic streptococcal Pharyngitis *
Acute: Fever, Inflammed Skin, joint &heart
Chronic: scarring of heart valves (mitral) dysfunction.
Rheumatoid Arthritis ?
Pharyngitis only ?
Other infections ?
Why life long therapy ? 2

3. Introduction:
“The most important consequence of rheumatic fever is recurrent autoimmune inflammation of heart valves due to ‘GABH Strep’ causing scarring of valves leading to severe cardiac dysfunction decades later”
RHD – Mitral Stenosis. 3

4. Epidemiology Incidence more during - winter & early spring
Ages 5-18 yrs are most susceptible
Rare <3 yrs
M:F equally except Sydenham’s chorea which is more common in girls
Common in 3rd world countries
Environmental factors-- over crowding, poor sanitation, poverty,
Incidence more during - winter & early spring

5. Etiology
Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A beta hemolytic streptococcal infection
It is a delayed non-suppurative sequelae to URTI with GABH streptococci.
It is a diffuse inflammatory disease of connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue and CNS

6. Etiology: Genetic Susceptibility – HLA DR 2 & 3
Environmental factor – GABH strep.
Autoimmunity – Autoantibodies ? .
Auto Ab to cardiac & brain stroma.
Infl. mediators correlate with activity…
But, level of Autoantibody does not correlate with clinical severity. ? Other Ab, other cause? 6

7. Etiology of Autoimmune disorders:7

8. Pathogenesis
Delayed immune response to infection with group.A beta hemolytic streptococci.
After a latent period of 1-3 weeks, antibody induced immunological damage occur to heart valves,joints, subcutaneous tissue & basal ganglia of brain

9. Group A Beta Hemolytic Streptococcus
Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24
Different serotypes from strains causing impetigo or glomerulonephritis
Pharyngitis- produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonepritis
Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditis as skin lipid cholesterol inhibit antigenicity

10. Pathologic Lesions
Fibrinoid degeneration of connective tissue, inflammatory edema, inflammatory cell infiltration & proliferation of specific cells resulting in formation of Ashcoff nodules , resulting in-
-Pancarditis in the heart
-Arthritis in the joints
-Ashcoff nodules in the subcutaneous tissue
-Basal gangliar lesions resulting in chorea

11. Summary: RHD Pathogenesis.
Acute Rheumatic Fever

12. Clinical Features: Children 5-15 years common
Fever 2-3 weeks following pharyngitis
Migratory polyarthritis of large joints
Pericardial friction rub Weak heart sounds
Tachycardia, arrhythmias
Increased vulnerability to reactivation with subsequent pharyngitis.
Cumulative cardiac damage over decades 12

13. 1.Arthritis
Flitting & fleeting migratory polyarthritis, involving major joints
Commonly involved joints-knee,ankle,elbow & wrist
Occur in 80%,involved joints are exquisitely tender
In children below 5 yrs arthritis usually mild but carditis more prominent
Arthritis do not progress to chronic disease

14. 2.Carditis
Manifest as pancarditis(endocarditis, myocarditis and pericarditis),occur in % of cases
Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ
Valvulitis occur in acute phase
Chronic phase- fibrosis,calcification & stenosis of heart valves (fishmouth valves)

15. 3.Sydenham Chorea Occur in 5-10% of cases
Mainly in girls of 1-15 yrs age
May appear even 6/12 after the attack of rheumatic fever
Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face
Clinical signs- pronator sign, jack in the box sign , milking sign of hands

16. 4.Erythema Marginatum 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

17. 5.Subcutaneous nodules Occur in 10%
Painless,pea-sized,palpable nodules
Mainly over extensor surfaces of joints,spine,scapulae & scalp
Associated with strong seropositivity
Always associated with severe carditis

18. Rheumatic Fever: Clinical Features
Polyarthritis – w/ low grade fever, large joints, ( > 75%)
migratory - affects 1 at a time, no permanent dysfx.
Carditis - pericarditis, cardiomeagly, or valvulitis ( ~ 50%)
(valvulitis is the most serious manifestation.)
Chorea – late occurrence, months after ( ~ 10%)
infection, self-limiting, resolves in 1- 3 months.
Erythema Marginatum – “classic” truncal rash, ( ~ 10%)
migratory - appears & disappears within hours.
(pink rash – irregular red edges – clear center)
Subcutaneous Nodules – occurs late ( months (1 - 2%)
after infection), painless small nodules over bony
prominences - elbows, knees, spine.

22. Other features (Minor features)
Fever-(upto 38.3 degree centigrade)
Arthralgia
Pallor
Anorexia
Loss of weight

23. Jones clinical Criteria of Diagnosis:
Major Criteria
Migratory Polyarthritis
Carditis
Subcutaneous nodules
Erythema marginatum
Sydenham Chorea
Minor Criteria
Nonspecific symptoms
Fever
Arthralgia
High ESR / CRP
Prolonged PR Interval
Positive: 2 Major or 1 major + 2 minor
Following Group-A strep. pharyngitis. 23

24. Heart Disease
Rheumatic Heart Disease – usually occurs years after initial attack. Mitral valve is more commonly involved than aortic valve. Classically, pts have mitral stenosis as a result of calcification.

25. Laboratory Investigations:
No specific laboratory investigations*
Throat culture-GABH streptococci - Cultures are usually negative.
High ESR
Anemia, leucocytosis
Elevated C-reactive protien
ASO titre >200 Todd units. (Peak value attained at 3 weeks,then comes down to normal by 6 weeks)
High Anti-DNAse B titres
High Acute phase reactants –
CRP, SAP, Complements, Coagulation Proteins. 25

26. Laboratory Findings (Contd)
ECG- prolonged PR interval, 2nd or 3rd degree blocks,ST depression, T inversion
2D Echo cardiography- valve edema,mitral regurgitation, LA & LV dilatation,pericardial effusion,decreased contractility

27. Diagnosis Evidence of recent streptococcal infection can include:
Increased antistreptolysin O or other streptococcal antibodies (anti-DNAse B)
Positive throat culture for Group A beta-hemolytic streptococci
Positive rapid direct Group A strep test
Recent scarlet fever
Rheumatic fever is mainly a clinical diagnosis
No single diagnostic sign or specific laboratory test available for diagnosis
Diagnosis based on MODIFIED JONES CRITERIA

29. Differential Diagnosis
Juvenile rheumatiod arthritis
Septic arthritis
Sickle-cell arthropathy
Kawasaki disease
Myocarditis
Scarlet fever
Leukemia

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

31. Primary prophylaxis
Timely diagnosis of GAS pharyngitis and appropriate treatment. Treatment of choice is still Penicillin as all GAS is susceptible.
Treatment administered within 10 days of onset of illness has been shown to prevent ARF.
Alternatives – amoxicillin, erythromycin, 1st generation cephalosporin

32. STEP I: Primary Prevention of Rheumatic Fever
STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis)
Agent Dose Mode Duration
Benzathine penicillin G U for patients Intramuscular Once kg (60 lb) U for patients >27 kg or
Penicillin V Children: 250 mg 2-3 times daily Oral d (phenoxymethyl penicillin) Adolescents and adults: mg 2-3 times daily
For individuals allergic to penicillin
Erythromycin: mg/kg/d 2-4 times daily Oral 10 d Estolate (maximum 1 g/d) or
Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral d (maximum 1 g/d)

33. Step II: Anti inflammatory treatment
Clinical condition Drugs

34. 3.Step III: Supportive management & management of complications
Bed rest
Treatment of congestive cardiac failure: -digitalis,diuretics
Treatment of chorea: diazepam or haloperidol
Rest to joints & supportive splinting

35. Secondary prophylaxis
Pts diagnosed with ARF need to undergo secondary prophylaxis to prevent relapses.
Prophylaxis regimens include oral Pen VK BID, Pen G IM qmonth, oral sulfisoxazole qday, or oral erythromycin BID.

36. STEP IV : Secondary Prevention of Rheumatic Fever
STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks)
Agent Dose Mode
Benzathine penicillin G U every 4 weeks* Intramuscular or
Penicillin V 250 mg twice daily Oral
Sulfadiazine g once daily for patients 27 kg Oral g once daily for patients >27 kg
For individuals allergic to penicillin and sulfadiazine
Erythromycin 250 mg twice daily Oral
*In high-risk situations, administration every 3 weeks is justified and recommended

37. Duration of Secondary Rheumatic Fever Prophylaxis
Category Duration
Rheumatic fever with carditis and At least 10 y since last residual heart disease episode and at least until (persistent valvar disease*) age 40 y, sometimes lifelong prophylaxis Rheumatic fever with carditis 10 y or well into adulthood, but no residual heart disease whichever is longer (no valvar disease*)
Rheumatic fever without carditis 5 y or until age 21 y, whichever is longer
*Clinical or echocardiographic evidence.

38. Duration of treatment Rheumatic Fever without carditis
5yrs or until 21yo – whichever is longer
Rheumatic Fever with carditis but no valvular disease
10yrs or “well into adulthood” – whichever is longer
Rheumatic Fever with carditis and persistent valvular disease
At least 10yrs since last episode and at least until 40yo; sometimes lifelong

39. Prognosis
Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection,if not on prophylactic medicines
Good prognosis for older age group & if no carditis during the initial attack
Bad prognosis for younger children & those with carditis with valvar lesions

40. Assessment of
CVS & Murmurs

41. General Appearance Marfan Syndrome Acromegaly Turner Syndrome
Tall, long extremities
Associated with: aortic root dilitation, MV prolapse
Acromegaly
Large stature, coarse facial features, “spade” hands
Associated with: Cardiac hypertrophy
Turner Syndrome
Web neck, hypertelorism, short stature
Associated with: Aortic coarctation, pulmonary stenosis
Pickwickian Syndrome
Severe obesity, somnolence
Associated with: Pulmonary hypertension
Fredreich ataxia
Lurching gait, hammertoe, pes cavus
Associated with: hypertrophic cardiomyopathy
Duchenne muscular dystrophy
Pseudohypertrophy of the calves
Cardiomyopathy
Ankylosing spondylitis
Straight back syndrome, stiff (“poker”) spine
Associated with: AI, CHB (rare)
Lentigines (LEOPARD syndrome)
Brown skin macules that do not increase with sunlight
Associated with: HOCM, PS

42. General Appearance- 2 Sarcoidosis Tuberous Sclerosis Myxedema
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu)
Small capillary hemangiomas on the face or mouth
Associated with: Pulmonary arteriovenous fistula
Lupus
Butterfly rash on face, Raynaud phenomenon- hands, Livedo reticularis
Associated with: Verrucous endocarditis, Myocarditis, Pericarditis
Pheochromocytoma
Pale diaphoretic skin, neurofibromatosis- café-au-lait spots
Associated with: Catecholamine-induced secondary dilated CM
Sarcoidosis
Cutaneous nodules, erythema nodosum
Associated with: Secondary cardiomyopathy, heart block
Tuberous Sclerosis
Angiofibromas (face; adenoma sebaceum)
Associated with: Rhabdomyoma
Myxedema
Coarse, dry skin, thinning of lateral eyebrows, hoarseness of voice
Associated with: Pericardial effusion, LV dysfunction

43. Pathophysiology Stenosis- Regurgitation (insufficiency)
narrowed valve, sloews forward blood flow
increases afterload, dec. CO
Regurgitation (insufficiency)
increases preload
heart pumps same blood again
blood volume and pressures reduced in front of affected valve; increased behind affected valve
results in heart failure

44. All valvular diseases have characteristic murmurs
Damaged valve disrupts blood flow=turbulence & sound!
Caused by
Rheumatic Heart Disease
Acute conditions (infective endocarditis)
Acute MI
Congenital Heart Defects
Aging, etc

45. Auscultation Use the diaphragm for high pitched sounds and murmurs
Use the bell for low pitched sounds and murmurs
Sequence of auscultation
upper right sternal border (URSB)
upper left sternal border (ULSB)
lower left sternal border (LLSB)
apex
apex - left lateral decubitus position
lower left sternal border (LLSB)- sitting, leaning forward, held expiration

46. Grading the Intensity of Murmurs
Grade 1
Murmur heard with stethoscope, but not at first
Grade 2
Faint murmur heard with stethoscope on chest wall
Grade 3
Murmur hears with stethoscope on chest wall, louder than grade 2 but without a thrill
Grade 4
Murmur associated with a thrill
Grade 5
Murmur heard with just the rim held against the chest
Grade 6
Murmur heard with the stethoscope held away and in from the chest wall

47. Cardiac Murmurs
Most mid systolic murmurs of grade 2/6 intensity or less are benign
Associated with physiologic increases in blood velocity:
Pregnancy
Elderly
In contrast, the following murmurs are usually pathologic:
Systolic murmurs grade 3/6 or greater in intensity
Continuous murmurs
Any diastolic murmur

48. Maneuver Hemodynamic Effect Murmur Effect Normal respiration
Transient ↑ in venous filling during inspiration
↑ right-sided murmurs
Passive leg elevation
↑ venous return (transient ↑ in LV size and preload)
↑ right-sided murmurs, ↓murmur of HOCM and MVP
Stand to squat
↑ right-sided murmurs,
↓murmur of HOCM and MVP
Squat to stand
↓ venous return (transient ↓ in LV size and preload)
↑ murmur of HOCM, moves midsystolic click of MVP closer to S1 and ↑ MVP murmur, ↓ AS murmur
Valsalva
↓ venous return (transient ↓ in LV size, preload, and relative systemic hypotension)
↑ murmur of HOCM, moves midsystolic click of MVP closer to S1, and ↓ murmur of MVP
Isometric handgrip exercise
↑ afterload
↑ murmur of MR and VSD, ↓the murmur of HOCM, ↓AS murmur
Inhaled amyl nitrate
↓ afterload
↓ murmur of MR and VSD, no change in AS murmur

49. Diagnostic Testing ECHOCARDIOGRAM Exercise testing
To assess the clinical severity of valvular heart disease
Those with inconsistent resting hemodynamics
Equivocal history of symptoms
Exercise testing in AS patients
Should be ended promptly if:
Cardiac symptoms provoked
Decrease or minimal increase (<20 mmHg) in blood pressure
Prior history of angina, congestive heart failure, or exertional syncope absolute contraindications to exercise testing
Cardiac catheterization
Usually not needed for primary evaluation

50. Innocent Murmurs Common in asymptomatic adults
Characterized by
Grade I – LSB
Systolic ejection pattern - no  with Valsalva
Normal precordium, apex, S1
Normal intensity & splitting of second sound (S2)
No other abnormal sounds or murmurs
No evidence of LVH
S S2

51. Characteristic of the NOT Innocent Murmur
Diastolic murmur
Loud murmur - grade IV or above
Regurgitant murmur
Murmurs associated with a click
Murmurs associated with other signs or symptoms e.g. cyanosis
Abnormal 2nd heart sound – fixed split, paradoxical split or single

52. Common Murmurs and Timing
Systolic Murmurs
Aortic stenosis
Mitral insufficiency
Mitral valve prolapse
Tricuspid insufficiency
Diastolic Murmurs
Aortic insufficiency
Mitral stenosis
S S S1

53. Rheumatic Fever Prophylaxis
Primary prophylaxis
If living in an endemic area, with pharyngitis and a +test for group A strep or positive throat culture
Given once, may be repeated as needed:
PCN G 1.2 million U IM or PCN V 500 mg TID x 10d
Azithromycin 500 mg on day 1, 250 mg daily for 4d
Secondary prophylaxis
PCN G 1.2 million units IM every 4 weeks or PCN V 250 mg PO BID or erythromycin 250 mg BID
RHD without carditis- At least 5 years or until >21 y of age
RHD with carditis, no valvular HD- At least 10 y or well into adulthood
RHD with carditis and valvular HD- At least 10 years from last episode or until patient is older than 40 years