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  • N Z Med J. 2014 May 2;127(1393):26-37.

    Retrospective epidemiology of acute rheumatic fever: a 10-year review in the Waikato District Health Board area of New Zealand.

    Pennock V1, Bell A, Moxon TA, Reed P, Maxwell F, Lennon D.

    Author information



    Acute rheumatic fever (ARF) is a preventable disease which remains a prominent burden of health in New Zealand, with an annual incidence comparable to that of developing countries.


    The aim of this study was to describe the epidemiology of ARF and recurrent ARF cases in the Waikato District Health Board (DHB) area of New Zealand from 1 January 2002 to 31 December 2011.


    A total of 106 cases of ARF and four cases of recurrent ARF were identified through the Public Health Database - EpiSurv and the Hospital coding system, ICD-10.


    The overall Waikato DHB annual incidence of ARF was 3.1 per 100,000 population with Maori children aged 5-14 years experiencing higher rates of 46.1 per 100,000 population. Eighty-five percent of the cases were of Maori ethnicity, and 10% Pacific. Almost three-quarters of all cases lived in areas of the three most deprived deciles as described by the New Zealand Deprivation Index 2006.


    The rates of ARF seen in the Waikato DHB are comparable to that seen previously locally and nationally. High risk groups have been identified as children aged 5-14 years, Maori and Pacific ethnicity, and those living in lower socioeconomic areas which could be targeted by the Rheumatic Fever Prevention Programme (RFPP) with the intention to reduce the incidence of ARF nationally to 0.4 cases per 100,000 population by 2017.
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  1. 1. AAccuuttee RRhheeuummaattiicc FFeevveerr An entirely preventable disease
  2. 2. bu Leaks developed butt bbiitteess ddeevveellooppiinngg
  3. 3. The theory ooff mmoolleeccuullaarr mmiimmiiccrryy GAS pharyngitis triggers an autoimmune response to epitopes in the organism that cross-react with similar epitopes in the heart, brain, joints, and skin, and repeated episodes of rheumatic fever lead to RHD Cunningham MW: Streptococcus and rheumatic fever. Curr Opin Rheumatol 24:408, 2012.
  4. 4. French physician EErrnnsstt--CChhaarrlleess LLaassèègguuee -- 11888844 “Pathologists have long known that rheumatic fever licks at the joints, but bites at the heart.”
  5. 5. EEppiiddeemmiioollooggyy TTrriiaadd 1. Agent: virulence 2. Host: Genetic susceptibility[3-5%] 3. Environment: Challenged socioeconomic
  6. 6. Hot spot  Kyrgyzstan Highest incidence of RF/RHD  543/100,000 population per year
  7. 7. (Modified from Parry E, Godfrey R, Mabey DD,, GGiillll GG [[eeddss]]:: PPrriinncciipplleess ooff MMeeddiicciinnee iinn AAffrriiccaa.. 33rrdd eedd.. CCaammbbrriiddggee,, CCaammbbrriiddggee UUnniivveerrssiittyy PPrreessss,, 22000044,, pp 886611..))  4 patterns RF in 150 years. ◦ A- Preantibiotic fall in the incidence of ARF of industrialized countries ◦ B-Persistent high incidence RF [Africa and south Asia]. ◦ C-Postantibiotic fall in the incidence of rheumatic fever in countries that instituted comprehensive programs for primary and secondary prevention of rheumatic fever, such as Cuba, Costa Rica, Martinique, and Guadeloupe. ◦ D-Fall and rise in the incidence of rheumatic fever in the formerly Soviet Republics of Central Asia.
  8. 8. AAggeenntt Group A beta-haemolytic streptococcus A poisonous “GAS”
  9. 9. PPaatthhooggeenneessiiss
  10. 10. Hit -1:cross reaction Hit-2:T lymphocyte invasion Epitopes on the cell wall of Streptococcus forms cross reacting antibodies to host antigens The antigen and antibody complex at the target site invites T lymphocytes to come out of vessel and stimulates local epitheloid cell to become Anitkoff’s cell around the central Fibrinoid degeneration forming together called “Aschoff- Geipel bodies” 22 HHiitt hhyyppootthheessiiss
  11. 11. Intracellular Extracellular Cardiac myosin Brain tubulin Laminin on the endothelial surface of the valve Lysoganglioside and dopamine receptors in the brain TTaarrggeettss ooff mmoolleeccuullaarr mmiimmiiccrryy
  12. 12. SSuusscceeppttiibbiilliittyy ooff hhoosstt  3-6% without primary Rx  X5 time if family Hx positive  Poor fellow  No hygiene  Lives in tight pack  X6 time in monozygotic  X3 times in children if one parent +  The heritability of rheumatic fever is 60% Family history is must in Rheumatic heart disease
  13. 13. PPhhoottoommiiccrrooggrraapphh  Aschoff nodule of acute rheumatic fever. The nodule is composed of Anitschkow cells; these have clear nuclei with a central bar of chromatin, said to resemble a caterpillar. There is a central area of fibrin. This central necrosis is further surrounded by a mononuclear cell infiltrate. Myocardial fibres adjacent to the Aschoff body are undergoing Fibrinoid necrosis. (Sebire NJ, Ashworth M, Malone M, Jacques TS [eds]: Diagnostic Pediatric Surgical Pathology. Churchill Livingstone, United Kingdom, 2010.)
  14. 14. PPootteennttiiaall bbaarrrriieerr ttoo RRxx RRFF/RRHHDD Streptococcal pharyngitis- 2 to 3 Wk-no lab test + except throat culture Rheumatic fever ◦ 30% -asymptomatic GAS pharyngitis ◦ 50% -asymptomatic GAS pharyngitis in epidemic time ◦ Age :4-15 yrs ◦ Juvenile(3-5 yrs) -India Think of vaccine
  15. 15. AArrtthhrriittiiss Almost 100% Severe in young adults than in teenagers (82%) and children (66%) Migratory A few days to a week 2/3rd -polyarthritis resolves completely If joint swelling persists after 4 weeks, it is necessary to consider other conditions
  16. 16. PPoossttssttrreeppttooccooccccaall rreeaaccttiivvee aarrtthhrriittiiss Not typical of rheumatic fever Recent streptococcal infection shorter latent period  responds less well to NSAID renal manifestations No carditis Rx 2ndary prophylaxis with pencillin
  17. 17. CCaarrddiittiiss  most serious  CRHD  Accidental detection with chorea  The incidence of carditis during the initial attack of RF ◦ 40%-No echo ◦ 91%-with echo  Varies with the age ◦ 90% to 92% of children <3 years ◦ 50% of children 3 to 6 years of age ◦ 32% of teenagers aged 14 to 17 years ◦ 15% of adults Myocarditis in the absence of valvulitis is unlikely to be rheumatic in origin
  18. 18. CCoonnttdd CHF - 5% to 10% during initial attack and increases with repeated carditis Transient apical mid-diastolic murmur (Carey-Coombs) may occur in association with the murmur of mitral regurgitation
  19. 19. WHF:Minimum Echocardiographic CCrriitteerriiaa ffoorr tthhee DDiiaaggnnoossiiss ooff PPaatthhoollooggiicc VVaallvvuullaarr RReegguurrggiittaattiioonn SSeeccoonnddaarryy ttoo RRhheeuummaattiicc CCaarrddiittiiss PATHOLOGIC MITRAL REGURGITATION (ALL FOUR DOPPLER CRITERIA MUST BE MET) PATHOLOGIC AORTIC REGURGITATION (ALL FOUR DOPPLER CRITERIA MUST BE MET) 1. Seen on 2 views 1. Seen on 2 views 2. On at least 1 view jet length is ≥2 cm* 2. On at least 1 view jet length is ≥1 cm* 3. Peak velocity ≥3 meters/sec 3. Peak velocity ≥3 meters/sec 4. Pansystolic jet in at least 1 envelope 4. Pandiastolic jet in at least 1 envelope
  20. 20. SSyyddeennhhaamm CChhoorreeaa  may be the only initial manifestation F>M after puberty-more 6 to 8 weeks from pharyngitis Chorea-involuntary, purposeless, jerky movements of the hands, arms, shoulders, feet, legs, face, and trunk along with hypotonia and weakness,interfere voluntary activity and disappear during sleep
  21. 21. Hemichorea- completely unilateral  jack-in-the-box tongue  “the milking sign”  Emotional lability last for a week to 2 years but generally persists for 8 to 15 weeks Serological markers may be normal because of long latency
  22. 22. PPAANNDDAASS subgroup of children with tic or obsessive-compulsive disorders that are triggered by GAS infection with no associated cardiac valve damage  if ever, make a diagnosis of PANDAS and should rather err on the side of diagnosis of rheumatic fever and implement secondary prophylaxis
  23. 23. SSuubbccuuttaanneeoouuss NNoodduulleess Detected over the occiput, elbows, knees, ankles, and Achilles tendons Over olecranon Firm, painless, and freely movable over the subcutaneous tissue. The nodules vary in size from 0.5 to 2 cm 1.5% In crops-carditis
  24. 24. EErryytthheemmaa MMaarrggiinnaattuumm less common upper part of the arms or trunk but not on the face not pathognomonic The rash Evanescent, pink, and nonpruritic. It extends centrifugally whereas the skin at the center returns to normal—hence the name “erythema marginatum.” It has an irregular serpiginous border. The rash may also become more prominent after a hot shower. Erythema marginatum generally occurs only in patients with carditis and may develop early or later in the course of the disease.
  25. 25. 11997700--11999900
  26. 26. 1991-2011
  27. 27. In India, rheumatic fever is endemic and remains one of the major causes of cardiovascular disease, accounting for nearly 25-45% of the acquired heart disease. ROUTRAY SN2003 PRIMARY ATTACK RATE OF RF FOLLOWING STREPTOCOCCAL PHARYNGITIS ◦ EPIDEMICS: 3% ◦ SPORADIC:0.3%
  28. 28. RF is a delayed autoimmune response to Group A streptococcal pharyngitis, and the clinical manifestation of the response and its severity in an individual is determined by host genetic susceptibility, the virulence of the infecting organism, and a conducive environment
  29. 29. AAGGEENNTT  Beta-haemolytic streptococci can be divided into a number of serological groups on the basis of their cell-wall polysaccharide antigen  Serological group A (streptococcus pyogenes) can be further subdivided into more than 130 distinct M types.  The available evidence does not link streptococci in Non-group A types with the pathogenesis of rf and rhd
  30. 30. Group A streptococci are the most common bacterial cause of pharyngitis, with a peak incidence in children 5–15 years of age. 15–20% of sore throats are caused by group A streptococci. A patient with a true infection is at risk of developing RF and of spreading the organism to close contacts, while this is not thought to be the case with carriers Positive throat culture rate for Gr A streptococci are around 13.5% in Northern India in sore throat cases.
  31. 31. RRHHEEUUMMAATTOOGGEENNIICC SSTTRRAAIINNSS Very rich in M-protein Heavily encapsulated produce striking "mucoid" colonies on blood agar plates Tropic primarily for the throat M 1, 3, 5, 6, 18, 19 and 24 The site of infection must be pharyngeal GAS virulence ◦ (Extractable and heterotypic antigen, the M protein) ◦ Capsule of hyaluronic acid("mucoid" appearance of GAS colonies) ◦ M protein and capsule, are primarily responsible for the striking resistance of virulent strains of GAS to phagocytosis
  32. 32. MM pprrootteeiinn aanndd aannttiiggeennss
  33. 33. MM pprrootteeiinn  The streptococcal M-protein extends from the surface of the streptococcal cell as an alpha–helical coiled dimer,  Shares structural homology with cardiac myosin and other alpha-helical coiled molecules, such as Tropomyosin, keratin and laminin(lines valve structure and is a target for poly reactive antibody)
  34. 34. Nonsuppurative sequel, such as RF and RHD, are seen only after group A streptococcal infection of the upper respiratory tract. Bramhanathan et al 2006 Exception: skin infection leading to RF described in some aborginal tribes of australia Chronic streptococcal “carrier” states do not trigger the development of RF. The role of group A streptococcus infection is complex and repeated infection is necessary to prime the immune response, quantitatively and qualitatively ,before the first episode of ARF occurs
  35. 35. HOST FACTORS An inherited susceptibility to ARF and RHD is supported by twin studies that have found a significantly increased concordance in monozygotic twins compared with dizygotic twins. 2 % OF ARF INFECTIONS HAVE BEEN FOUND TO BE FAMILIAL Padmavathi 1962 GAS pharyngitis is primarily a disease of children 5 to 15 years of age
  36. 36. HHOOSSTT FFAACCTTOORRSS ARF is a rare disease in the very young; Only 5% of first episodes arise in children younger than age 5 years and the disease is almost unheard of in those younger than 2 years.
  37. 37. HHOOSSTT FFAACCTTOORRSS  First episodes of ARF are most common just before adolescence, wane by the end of the second decade, and are rare in adults older than age 35 years.  Recurrent episodes are especially frequent in adolescence and early adulthood, and occasional cases are seen in people older than age 45 years
  38. 38. HHOOSSTT FFAACCTTOORRSS In many populations, ARF and RHD are more common in females than males ◦ ?Innate susceptibility, ◦ ? Increased exposure to group a streptococcus because of greater involvement of women in child rearing, ◦ ?Or reduced access to preventive medical care for girls and women. In populations exposed to rheumatogenic group A streptococci, the lifetime cumulative incidence of ARF is 3% to 6%.
  41. 41. Direct and indirect results of environmental and health-system determinants on rheumatic fever and rheumatic heart disease
  43. 43. THE IMMUNE RESPONSE  Myosin is not present in cardiac valves, so how can an immune response against myosin induce valvulitis?  The initial damage to the valve might be due to the presence of laminin, another alpha-helical coiled-coil molecule present in the valvular basement membrane and around endothelium, and which is recognised by T cells  There is also evidence that antibodies to cardiac valve tissues cross-react with N-acetyl glucosamine in group A carbohydrate.  An exaggerated antibody response to group A carbohydrate was noted in patients with ARF, and titres remained raised in individuals with residual mitral valve disease, providing further support for the notion that these antibodies cause valve damage
  44. 44. Immune complexes may produce nondestructive synovitis of the joints in patients with ARF and nondestructive reactions in the basal ganglia observed in Sydenham's chorea, whereas cell mediated autoimmune cytotoxic reactions may destroy heart valves.
  45. 45. Are spheroidal or fusiform distinct tiny structures or granulomas, 1-2 mm in size, occurring in the interstitium of the heart in RF. Especially found in the vicinity of small blood vessels in the myocardium and endocardium and occasionally in the pericardium. Lesions similar to the aschoff nodules may be found in the extracardiac tissues .
  48. 48. Every revision increased the specificity but decreased the sensitivity of the criteria,
  49. 49. 2002–2003 WHO criteria for the diagnosis of rheumatic fever and rheumatic heart disease (based on the revised Jones criteria) These revised WHO criteria facilitate the diagnosis of: — A primary episode of RF — Recurrent attacks of RF in patients without RHD — Recurrent attacks of RF in patients with RHD — Rheumatic chorea — Insidious onset rheumatic carditis — Chronic RHD.
  50. 50. DEFINITIONS Recurrence: A new episode of rheumatic fever following another GABHS infection; occurring after 8 week following stopping treatment Rebound: Manifestations of rheumatic fever occurring within 4-6 wk of stopping treatment or while tapering drugs. Relapse: Worsening of rheumatic fever while under treatment and often with carditis. Sub clinical carditis: When clinical examination is normal but echocardiogram is abnormal. Around 30 percent of patients having chorea present as subclinical carditis. Indolent carditis: It is a common entity in our country. Patient presents with persistent features of CHF, murmur and cardiomegaly.
  52. 52.  75%subside within 6 weeks  90% subside within 12 weeks  <5% active after 6 months  MORTALITY FROM ARF ◦ GROVER: 7% ◦ SHARMA:1.2% PROGRESSION TO RHD: India 5-20yrs West 15-40yrs.
  53. 53. CARDITIS  Most important manifestation  Most often causes no symptoms of its own and is most often diagnosed in the course of examination of a patient with arthritis or chorea.  In 93% carditis develops with in 3 months  Rare to hear murmur after 6 months after the onset of ARF
  54. 54. CARDITIS 1. SLEEPING HR > 100 2. NEW ONSET MURMURS 3. CHF 4. CARDIOMEGALY 5. PERICARDIAL RUB 6. S3 Incidence ◦ 33 to 55%( India) ◦ 40-50% west) Murmurs manifest in 85%by 2nd or 3 rd week. In an RHD patient CCF should be suspected as a reccurence of carditis
  55. 55. MMyyooccaarrddiittiiss Due to an acute hemodynamic overload on the left ventricle from acute/ subacute mitral and/or aortic regurgitation. Myocarditis (alone) in the absence of valvulitis is unlikely to be of rheumatic origin. It should always be associated with an apical systolic or basal diastolic murmur.
  56. 56. PPEERRIICCAARRDDIITTIISS  Rheumatic pericarditis is relatively less common clinically and is present in up to 15% patients.  Since pericarditis neither results in tamponade nor constriction and clears up without leaving a residue, its limited clinical significance lies in the fact that it provides clear cut evidence for the presence of active carditis as well as active RF.  Pericarditis does not occur in the absence of clinical findings indicative of valvulitis.  Simultaneous demonstration of valvular involvement generally considered essential.
  57. 57. CONGESTIVE HEART FAILURE Least common but most serious manifestation. Occurs in5 to 10% of first attacks of carditis. More common in children <6yrs of age.
  58. 58. MMaalliiggnnaanntt rrhheeuummaattiicc ffeevveerr Severe disease with multi valvular lesions, gross cardiac enlargement, and congestive failure can occur in young patients, and such children show more symptoms of congestive failure than of rheumatic disease. This severe disease may be due in large measure to a lack of rest during the initial carditis
  59. 59. The wide difference in the reported prevalence of carditis in the first attack could thus be related to clinically undiagnosed carditis in the first attack which becomes apparent after recurrences of acute RF
  60. 60. AArrtthhrriittiiss aanndd aarrtthhrraallggiiaa  Most common and least specific  75% of pts with 1st attack of ARF.  Occurs early in the course of the disease, as the presenting complaint  Incidence increases with age.(Often the only major manifestation in adolescents, as well as in adults, where carditis and chorea become less common in older age groups.)
  61. 61.  Inflamed joints are characteristically warm, red and swollen, and an aspirated sample of synovial fluid may reveal a high average leukocyte count  Important to differentiate from arthalgia( less specific)  Usually large joint  Almost any joint can be affected
  62. 62. Tenderness in rheumatic arthritis may be out of proportion to the objective findings and severe enough to result in excruciating pain on touch. “MIGRATORY” reflects the sequential involvement of joints, with each completing a cycle of inflammation and resolution, so that some joint inflammation may be resolving while others are beginning.
  63. 63.  If untreated as many as 16 joints can be involved and atleast 6 in half of the patients  Resolves spontneously with in 3 weeks without sequelae( except jaccoud’s)  Inverse relation with carditis severity Total no number % carditis 1 Red hot/ swollen 179 47 26 2 tender 30 12 40 3 Joint pains 25 24 96 4 No joint symptoms 29 29 100 Feinstein AR, Sterno EK, Spagnuolo M. The prognosis of acuterheumatic fever. Am Heart J 1964; 68: 817–834
  64. 64. JOCCOUD CHRONIC POSTRHEUMATIC ARTHRITIS Periarticular fibrosis of the metacarpophalangeal joints. It usually occurs in patients with severe RHD,but is not associated with evidence of RF
  65. 65. POST STREPTOCOCCAL REACTIVE ARTHRITIS (PSRA) • Does not fulfill jones criteria • Latent period is shorter (1 week). • Arthritis is additive rather than migratory • Poor response to salicylates • Arthiritis persists for a mean period of two months. • Evidence of recent GABS infection is Mandatory • 6% develop mitral heart disease. Not associated with other major manifestations of RF
  66. 66. MMiiggrraattoorryy aarrtthhrriittiiss  RF , Gonococcemia Meningococcemia Viral arthritis Systemic lupus erythematosus Acute leukemia Whipple's disease
  67. 67. SYDENHAM’S CHOREA Occurs primarily in children Rare after the age of 20 Occurs primarily in females Less commonin postpubertal males. Prevalence of chorea in RF patients varied from 5–36%
  68. 68. CHOREA Concomitant subclinical carditis detected by echocardiography appears to be as high as 70% Chorea is a uniquely delayed manifestation of RF, with a wide range in reported incidence between 5% and 35%, latency of 1 to 7 months, and choreiform manifestations that may last for months and occasionally years
  69. 69. CHOREA There is a substantial risk of subsequent RHD in these patients. Neurologic deficits typically resolve within 2 years, but residual psychiatric disturbances occur in a small but significant number of patients in the subsequent decades
  70. 70. CHOREA A syndrome of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS), in a fashion similar to poststreptococcal reactive arthritis, has a temporal relationship to GABHS infection but is not associated with other features of RF
  71. 71. SSuubb ccuuttaanneeoouuss nnoodduulleess  Firm round painless.  0.5 to 2cms  Overlying skin freely mobile  Occurs in crops  Located over bony prominences  Lasting for 1 to 2 weeks  Incidence:  sanyal et al India: 2.3%combined with erythema marginatum  Subcutaneous nodules are almost always associated with cardiac involvement and are found more commonly in patients with severe carditis
  72. 72. SSuubbccuuttaanneeoouuss nnoodduulleess They may also be found over the scalp, especially theocciput, and the spinous processes of the vertebrae. The number of nodules varies from one to a few dozen, but usually three or four. They persist from days to 1–2 weeks to, rarely, more than a month
  73. 73. EErryytthheemmaa mmaarrggiinnaattuumm  Erythema marginatum occurs in up to 15% of RF patients  In view of the evanescent nature may be easily missed.  Appear first as a bright pink macule or papule that spreads outward in a circular or seripiginous pattern.  The lesions are multiple, appearing on the trunk or proximal extremities, rarely on the distal extremities, and never on the face.  They are nonpruritic and nonpainful, blanch under pressure
  74. 74. Erythema marginatum usually occurs early in the course of a rheumatic attack. It may, however, persist or recur for months or even years, continuing after other manifestations of the disease have subsided, and it is not influenced by anti-inflammatory therapy. Nodules and erythema marginatum tend to occur together
  75. 75.  The latent period between streptococcal infection and onset of RF is shortest in arthritis and erythema marginatum and longest in chorea with carditis and subcutaneous nodules in between.  Atleast 1/3 rd of cases of acute rheumatic fever may present with inapparent streptococcal infections Arthralgia and fever are termed “minor” clinical manifestations of RF in the jones diagnostic criteria, because they lack diagnostic specificity
  76. 76. Elevated or rising streptococcal antibody titers. It is recommended that acute serum be collected at the onset of illness, and that the antibody titer be compared to a convalescent serum collected 2-4 weeks later, to detect a rise in titer
  77. 77. 1. The mitral valve is most often involved 2. Mitral regurgitation is the most common finding on color flow imaging. 3. Mitral regurgitation in rheumatic carditis is related to ventricular dilatation and/or restriction of leaflet mobility. 4. Rheumatic carditis does not result in congestive heart failure in the absence of hemodynamically significant valve lesions. 5. In a quarter of patients with rheumatic carditis, valve nodules were present that may represent echocardiographic equivalents of rheumatic verrucae
  79. 79. EEcchhooccaarrddiiooggrraapphhiicc eevviiddeennccee ooff ddeeffiinniittee RRHHDD  ANY OF: a) A mitral regurgitant jet at least 2 cm from the coaptation point of the valve leaflets, seen in two planes and persisting throughout systole plus thickened mitral valve leaflets and/or elbow or dog leg deformity of the anterior mitral valve leaflet. b) An aortic regurgitant jet at least 1 cm from the coaptation point of the valve leaflets, seen in two planes plus thickened mitral valve leaflets and/or elbow or dog leg deformity of the anterior mitral valve leaflet. c) Any significant mitral stenosis (defined as flow acceleration across the mitral valve with a mean pressure gradient greater than 4mmHg
  80. 80.  Echocardiographic demonstration of valvular regurgitation is not a prerequisite for the diagnosis of rheumatic carditis and should not be considered a limitation where the facilities are not available.  Currently, data do not allow subclinical valvular regurgitation detected by echocardiography to be included in the Jones criteria, as evidenceof a major manifestation of carditis.
  81. 81. CARDIAC ENZYMES Markers of myocardial damage in the form of troponin I, myoglobin and CPK-MB were evaluated in patients with acute rheumatic carditis with and without cardiomegaly or congestive cardiac failure. The markers of myocardial damage remained normal inspite of clinically active carditis.  Gupta M, Kaplan EL,. Serum cardiac troponin I in acute rheumatic fever. Am J Cardiol 2002
  82. 82. NNAATTUURRAALL HHIISSTTOORRYY OOFF MMSS In India, critical MS may be found in children as young as 6 to 12 years old. ( UP TO 20%) In the asymptomatic or minimally symptomatic patient, survival is greater than 80% at 10 years,  with 60% of patients having no progression of symptoms. once significant limiting symptoms occur, there is a dismal 0% to 15% 10-year survival rate Once there is severe pulmonary hypertension, mean survival drops to less than 3 years.
  83. 83. 30 to 40% of patients with MS develop atrial fibrillation (AF).  Atrial fibrillation occurs more commonly in older patients and is associated with a poorer prognosis, with a 10-year survival rate of 25% compared with 46% in patients who remain in sinus rhythm.
  84. 84. The mortality of untreated patients with MS is due to 1.Progressive pulmonary and systemic congestion in 60% to 70%, 2.Systemic embolism in 20% to 30%, 3.Pulmonary embolism in 10%, 4. Infection in 1% to 5%. Serial hemodynamic and Doppler-echocardiographic studies have reported annual loss of MV area ranging from 0.09 to 0.32 cm2.
  85. 85. Mitral regurgitation can be alone or with other lesions As high as 70% of MR in initial attack can disappear over a period of time. If AS is present with MV involvement it is likely to be rheumatic
  86. 86. AAOORRTTIICC RREEGGUURRGGIITTAATTIIOONN Asymptomatic patients with normal LV systolic function ◦ Progression to symptoms &/or LV dysfn: 6% ◦ Progression to asymptomatic LV dysfunction < than 3.5% per year Asymptomatic patients with LV dysfunction ◦ Progression to symptoms: more than 25% per year
  89. 89. HOSPITAL BASED SURVEYS AUTHOR YEAR REGION TOTAL CARDIAC CASES % RF/RHD KUTUMBAIAH 1932-38 VIZAG 1155 39.5 RAMAN 1935-41 VIZAG 2076 35.6 SANJEEV 1941 MADRAS 616 46.8 VAKIL 1941-45 BOMBAY 1860 24.7 PADMAVATHI 1951-55 DELHI 2360 39.1 BENARJEE 1936-43 CALCUTTA 717 44.6 VAKIL 1946-55 BOMBAY 6825 29.7 MALHOTRA, GUPTA 1949-59 PUNJAB 5378 27.6 SEPAHA ET AL 1952-62 INDORE 61.38 13.5 JOSHI ETAL 1957-62 GUJARAT 1216 35.6 BHARGAVA 1945-1964 RAJASTHAN 3722 33.39 AGARWAL 1966-73 ALLAHABAD 2843 40.6 K S MATHUR 1947-61 AGRA 3309 35.1
  90. 90. AUTHOR YEAR CARDITIS% ARTHRITIS % ARTHRALGI A% CHOREA% SC MMaanniiffeessttaattiioonnss ooff RRFF NODULES% ERYTHEMA MARG% ROY 1960 46 32 94 4 3 0 PADMAVATHI 1962 30.9 60.1 8.3 1.5 0 MAHAJAN 1972 77.1 33.9 45.7 77 18 0.3 SANYAL ET AL 1974 33.3 66.6 20 1.9 1.9 ARORA 42 30 42 2.6 6 0.2 GROVER 1ST 1988-1991 37.5 75 8.3 4 2 RECCURENCE 41 50 8.3 4 2
  92. 92. KKiinnaarree eett aall RHEUMATIC HEART PATHOLOGY IN THE YOUNG: AUTOPSY SERIES 1. 144 autopsy cases below the age of 18 years were included. Mitral Aortic Tricuspid Pulmonary vasculature 100% 63.89% 54.86% 75% 2. Mitral stenosis was present in 80.23% cases. Pure mitral valve incompetence was noted in 12.79%. 3. Tricuspid lesions were minor in most of the cases, only in 7.50% had significant stenosis. 4. Multivalvular disease was noted in 75.69%, 5. Pulmonary vasculature was affected in 75% cases. 6. Calcification of valve was uncommon and was present in 6% of mitral valve lesions and 2% of aortic valve lesions
  93. 93. IMPORTANT FEATURES OF B N DATTA AUTOPSY SERIES Mural thrombi: 13% Active pericarditis: 30% Aschoff bodies: 26% Bacterial endocarditis: 9% Organic TV disease: 34.2% When compared to the west: young age of death and high rate of TV disease.
  95. 95. Study Patients ARF RECURRENCE RATE/ PATIENT YEAR PREVALANCE OF RHD % UK-US 324 0.026 31.2 Wood 156 0.004 NA Miller 47 0 NA Tompkins 115 0.001 26.1 Thomas 73 0.013 42.5 SANYAL 65 0.006 35.4
  96. 96. SSuujjooyy rrooyy  Clinical and physiopathological findings in 108 patients with mitral stenosis who were below the age of 20 years.  History of at least one attack of rheumatic fever was obtained in 71 (66%), and of more than one attack in 30(28%) patients.  Chorea and subcutaneous nodules appeared infrequently (3%), and erythema marginatum was conspicuously absent.  High prevalence of congestive heart-failure (45%)  Low prevalence of atrial fibrillation (6%)  The estimated mitral-valve area was less than 1 sq. Cm. In most of the patients  Isolated mitral stenosis in patients below the age of 20 with rheumatic heart-disease is common in india.  Boys are affected oftener than girls
  97. 97. SSuujjooyy rrooyy  The frequency of atrial fibrillation was found to increase with each decade, reaching 40% in patients over the age of 40.  Angina(12%) is due to functional impairment of the coronary flow caused by limitation of the cardiac output.  Absence of calcification in the mitral valve and of thrombi could be due to the youth of the patients.  Severe pulmonary hypertension with gross pulmonary vascular obstruction, fairly normal cardiac output
  98. 98. MS IN YOUNG(( IINNDDIIAANN SSCCEENNAARRIIOO)) In developing countries, mitral stenosis is severe enough to require commissurotomy before the age of 20 or even 15 years.  In1408 patients with rheumatic heart disease seen at the G B Pant Hospital, New Delhi, between 1967 and-1973  713 (51 %) had mitral stenosis  140 patients below age 20 <10 10-15 15-20 4 (2.8%) 55 (39.4%) 81 (57.8%)
  99. 99. ECHOCARDIOGRAPHY 2010 High prevalence of rheumatic heart disease detected by echo in school children. PANWAR et al 1059 school children aged 6-15 years Careful cardiac auscultation and echo. The prevalence of lesions suggestive of rheumatic heart disease by echo was 51 per 1,000
  100. 100. AAIIIIMMSS 22000088--22001100 BALLABHGARH CLINICAL RHD 0.8/1000 SUBCLINICAL RHD 20/1000 Heart 2011;97:201
  101. 101. 2012
  102. 102. MANAGEMENT ASPECTS PPRRIIMMAARRYY PPRREEVVEENNTTIIOONN OOFF AARRFF Treatment of GAS pharyngitis with a single intramuscular injection of 1.2 million units of benzathine penicillin G is the most reliable way to prevent primary attacks of ARF
  103. 103. SSeeccoonnddaarryy pprroopphhyyllaaxxiiss  Defined as the continuous administration of specific antibiotics to patients with a previous attack of rheumatic fever, or documented RHD  Purpose is to prevent colonization or infection of the upper respiratory tract with group A beta-hemolytic streptococci and the development of recurrent attacks of rheumatic fever  After surgery or intervention secondary prophylaxis should be continued  IMPORTANCE of secondary prophylaxis 1. Prevents reccurences 2. Reduces new cardiac damage, 3. Facilitate resolution of previous damage 4. Reduces mortality due to RHD. 5. The risk of reccurence is highest in first year after an index attack of RF
  104. 104. WHO GUIDELINES 2004
  105. 105. WHO GUIDELINES 2004
  106. 106. SSeeccoonnddaarryy pprroopphhyyllaaxxiiss Because of the high infection rate in India, it has been suggested that penicillin should be given once every 3 rather than 4 weeks to maintain adequate blood levels during reinfection, and this has certainly resulted in a fall in the infection rate.
  107. 107. RREECCUURRRREENNCCEE OONN PPRROOPPHHYYLLAAXXIISS Sanyal 0.6/100 pt years Padmavathi 0.1/100 pt years With out prophylaxis recurrence rate around 11.6/100 pt years
  110. 110. RHDAustralia (ARF/RHD writing group), National Heart Foundation ooff AAuussttrraalliiaa aanndd tthhee CCaarrddiiaacc SSoocciieettyy ooff AAuussttrraalliiaa aanndd NNeeww ZZeeaallaanndd:: AAuussttrraalliiaann GGuuiiddeelliinnee ffoorr PPrreevveennttiioonn,, DDiiaaggnnoossiiss aanndd MMaannaaggeemmeenntt ooff AAccuuttee RRhheeuummaattiicc FFeevveerr aanndd RRhheeuummaattiicc HHeeaarrtt DDiisseeaassee.. 22nndd eedd.. DDaarrwwiinn,, AAuussttrraalliiaa,, MMeennzziieess SScchhooooll ooff HHeeaalltthh RReesseeaarrcchh,, 22001122  Recommended for All Cases White blood cell count ESR or CRP Throat swab before giving antibiotics for GAS culture Blood culture if febrile Antistreptococcal serology: both antistreptolysin O and anti-DNase B titers (repeated after 10-14 days if the first test is not confirmatory) Electrocardiogram Chest radiograph Echocardiogram  Tests for Alternative Diagnoses, Depending on Clinical Features Repeated blood cultures with temperature spikes if infective endocarditis is suspected Joint aspiration for possible septic arthritis (microscopy and culture) Copper, ceruloplasmin, antinuclear antibody, and drug screen for choreiform movements Serology and autoimmune markers for arboviral, autoimmune, or reactive arthritis Peripheral blood smear for sickle cell disease
  111. 111. PPrriimmaarryy pprroopphhyyllaaxxiiss Antiobiotic Route doses Benzathine benzylpenicillin Single IM injection 1.2 million units; 50% if <30 kg Phenoxymethylpenicillin (penicillin VK) PO for 10 days 250-500 mg tid for 10 days Erythromycin ethylsuccinate PO for 10 days Varies with the formulation
  112. 112. WHO Technical Report Series No. 923. Rheumatic FFeevveerr aanndd RRhheeuummaattiicc HHeeaarrtt DDiisseeaassee:: RReeppoorrtt ooff aa WWHHOO EExxppeerrtt PPaanneell,, GGeenneevvaa 2299 OOccttoobbeerr--11 NNoovveemmbbeerr 22000011.. GGeenneevvaa,, WWHHOO,, 22000044.. Medication Route Doses Benzathine benzylpenicillin Single intramuscular injection every 3-4 weeks For adults and children ≥30 kg in weight: 1,200,000 units For children <30 kg in weight: 600,000 units Penicillin V Oral 250 mg twice daily Sulfonamide (e.g., Oral For adults and sulfadiazine, children ≥30 kg in sulfadoxine, weight: 1 g daily sulfisoxazole)
  113. 113. WHO Technical Report Series No. 923. Rheumatic FFeevveerr aanndd RRhheeuummaattiicc HHeeaarrtt DDiisseeaassee:: RReeppoorrtt ooff aa WWHHOO EExxppeerrtt PPaanneell,, GGeenneevvaa 2299 OOccttoobbeerr--11 NNoovveemmbbeerr 22000011.. GGeenneevvaa,, WWHHOO,, 22000044.. No carditis: 5 years after the last attack or until 18 years of age (whichever is longer) Mild carditis (mild mitral regurgitation or healed carditis):10 years after the last attack or at least until 25 years of age (whichever is longer) Severe valvular disease: Life-long After valve surgery: Life-long
  114. 114. IN INDIA  Endemicity of carditis  Erythema marginatum almost nonexistent  Chorea and subcutaneous nodules infrequent  Polyarthralgia >polyarthritis  Young >Older  Short interval - ARF to RHD  Start at Young  Rapid progression  More PAH/CCF  Rheumatic fever in < 50%  High incidence of organic tricuspid valve disease
  115. 115. FFUUTTUURREE PPEERRSSPPEECCTTIIVVEESS Overcoming barrier to transmission ◦ Socioeconomic/Political/awareness Special task force in highly endemicity Identification of genetic susceptibility(3-5%) Primary and 2ndary prophylaxis reinforcement Very long acting penicillin(>3 months) Vaccine Understanding molecular genetic
  117. 117. Socioecomical progress does not mean tthhee eexxttiinncctt ooff nnaattuurree