RHD presentation.pptx......................

Rhd

1. RHEUMATIC HEART DISEASE MODERATOR -- DR. SANJEEVA.G.N PRESENTER – DR.SANDEEP.R

2. RHEUMATIC FEVER – [ Rheumatic – Joints] Rheumatic fever is an acute, immunologically mediated multisystemic inflammatory disease that follows group A beta haemolytic streptococcus infection of the throat. “ Licks the JOINT, bites the HEART ” - Lasegue

3. Rheumatic fever emerged from the potpourri of “ Rheumatism " in the seventeenth century Guillaume de Baillou in France described it under the name of "acute articular rheumatism“ Deformities of the heart valves, first described anatomically by Morgagni in Italy in 1761 In 1886 Cheadle had described the full rheumatic fever syndrome: carditis , polyarthritis , and chorea , as well as subcutaneous nodules and erythema marginatum . Brief history of rheumatic fever Ref- MILTON MARKOWITZ Book of Rheumatic fever

4. EPIDEMIOLOGY - Incidence - Incidence of acute rheumatic fever varies with geographical location and population which ranges from 3 to 61 per 1 lakh school children . In India incidence of rheumatic fever by eco studies is 0.5 to 11 per thousand Predisposing factors Low socioeconomic status Overcrowding Poor medical care Ref- Piyush Guptha PG Text book PEDIATRICS

5. Etiology Agent - It is caused by the Group A beta- hemolytic streptococci and more specifically by group A betahemolytic streptococcal infections of the throat . Group A streptococcal infections of the throat always precede the development of rheumatic fever, whether first attacks or recurrences To initiate a rheumatic fever attack, group A streptococci must cause an infection of the pharynx, not just a superficial colonization.

6. Group a beta hemolytic streptococci Gram-positive coccoid shaped bacteria grows in chains. Broadly classified by their hemolytic activity Beta hemolytic - SEQUELA M TYPE ACUTE RHEUMATIC FEVER 1, 3, 5, 6, 18, 29 [ pharyngeal serotypes] ACUTE GLOMERULONEPHRITIS Throat 1, 4, 12 Skin 49, 55, 57, 60. Ref- Nelson 21 ST Edition.

7. Climate - Temperate Countries – WINTER Tropical Countries- RAINY SEASON Host factors- Age- It is peak between 5 and 15 years Sex – both sexes equally effected Patients who have already had one attack of the disease tend to have recurrences Ref- Nelson 21 ST Edition.

8. Pathogenesis and pathology Non suppurative sequel of streptoccal infection Post streptococcal WHY? only streptococcal throat infection leads to rheumatic fever?

9. Continue..... To cause an infection the streptococci must first of all attach to the epithelial cells in the pharynx of the host Virulent streptococci have fimbriae , avirulent ones don't streptococci get attached to epithelial cells by means of lipoteichoic acid on the streptococcal side, and of a receptor for lipoteichoic acid on the epithelial cell side The M proteins , which are also located on the fimbriae , make it difficult for phagocytes to ingest the strep, thus further increasing virulence

10. Continue.... A hyaluronic acid capsule , which further hinders phagocytosis and thus enhances virulence, is often found on rheumatogenic strains, especially in epidemic or near-epidemic streptococcal outbreaks As the streptococcal products diffuse out of the pharyngeal epithelium they encounter, of course, lymphoid cells and stimulate antibody responses. Several streptococcal antigens happen to cross-react immunologically with human tissue antigens

11. Virulent strains Fimbriae - M protein Capsule- hyaluronic acid Non virulent strains Colonization f/b infection of the throat Products diffuse out and there occurs cross reaction b/w streptoccal antigens and human tissue antigens Lipoteichoic acid Attachment of GAS to the epithelial cells of pharynx

12. Laboratory manifestations Evidence of a recent streptococcal infection

13. Evidence of systemic inflammation— ESR and CRP reflect the magnitude of the inflammatory process ("rheumatic activity") are useful to find out if the inflammatory process is still going on after the symptoms and signs have subsided If these tests are abnormal, such patients should be reexamined at close intervals for other evidence of disease.

14. Laboratory evidence of heart involvement Enlargement of the heart, pericardial effusion, and valve deformities are all to be looked for Radiography of the heart, echocardiography, and electrocardiography are the methods most often used to detect heart involvement Prolongation of the P-R interval occurs in 20-40% of the patients; Doppler ultrasonography has proven to be useful to identify evidence of valvular involvement

15. diagnosis Jones criteria- Ref- WHO Guidelines Rheumatic fever- 2004

16. MODIFIED JONES CRITERIA Ref- AHA Journals- 2015

17. Sub clinical carditis It is an echocardiographic evidence of mitral or aortic valvulitis in the abscnce of ascultatory findings Current version of Jones criteria focused on this concept Ref- Nelson 21 ST Edition.

18. Duration of the rheumatic attack Duration as a whole varies according to the criterion used to determine it, and to the clinical manifestations present It is the shortest in attacks characterized by arthritis alone; it is longer in the presence of chorea and longest in the presence of carditis . The duration of an initial attack of rheumatic fever ranges from less than 6 weeks (in one-third of cases) to 3 months In patients with severe carditis , the active rheumatic process may continue for 6 months or more. These patients have "chronic" rheumatic fever

19. ACTIVE RHEUMATIC FEVER??? Arthritis New origanic murmur Enlarged heart size Sleeping tachicardia Congestive heart failure in the absence of long standing sever valvular disease Subcutaneous nodules. Persistence of an elevated sedimentation rate (ESR) for more than 6 months should not be considered a sign of rheumatic activity if no clinical signs are present Ref- MILTON MARKOWITZ Book of Rheumatic fever

20. ARTHRITIS 60-75% CARDITIS 50-60% 3. CHOREA 10-15% 4. ERYTHEMA MARGINATUM 1-2% 5. SUB CUTANEOUS NODULES <1% INCIDENCE OF 5 MAJOR CRITERIA Ref- Nelson 21 ST Edition.

21. RHEUMATIC HEART DISEASE “It is an non suppuartive immune mediated complication of group A streptococcal infection characterised by inflammation of all layers of the heart” It is the most common acquired heart disease in children It is the long term sequelae of acute rheumatic fever Most commonly its is manifested as valvular disease

22. EPIDIMIOLOGY- Industrialised nations Incidence -less than 0.5 per 1 lakh Prevalence -less than 0.05/1000 children Developing countries Prevalence – 0.4 -21/1000 children Ref- Piyush Guptha PG Text book PEDIATRICS

23. PATHOGENSIS Molecular Mimicry – antibodies against M proteins of certain streptococcal strains binds to protein in myocardial valves and causes injury by activation of complements . CD4 + T cells that recognises streptococcal peptide can also cross react with host antigen leading to cytokine mediated inflammatory response Latent period - 2-3 weeks

24. PATHOLOGICAL MORPHOLOGY Acute rheumatic fever is characterised by an discrete myocardial inflammatory lesion called ASCHOFF BODIES - pathognomic for rheumatic fever Aschoff bodies are collection of lymphocytes, scattered plasma cells and anitschkow cells ( activated macrophages) occasionally punctuating zone of fibrinoid necrosis Aschoff bodies can be found in any of the 3 layers of the heart hence rheumatic fever said to cause PANCARDITIS

25. Pericardium – fibrinous exudates – generally resolves without squealae Myocardium – myocarditis – in the form of scattered aschoff bodies within the intertial connective tissue Endocardium - valvular lesions – in the form of verrucae ( fibrinoid necrosis and fibrin deposition along the lines of closure- 1-2 mm ) The most Important functional consequence of rheumatic heart disease is valvular lesions ( insufficiency/ stenosis ) PANCARDITIS FEATURES

26. Ref- WHO Guidelines Rheumatic fever- 2004

27. Order of valve involvement in RHD- Mitral valve 70% Combined mitral and aortic disease 25% Tricuspid – less frequently involved Pulmonary valves – almost always escapes injury Ref- MILTON MARKOWITZ Book of Rheumatic fever

28. MITRAL INSUFFICINECY Mitral insufficient is the result of structural changes that may include some loss of valvular substance and/ or changes to sub valvular apparatus in the form of elongation of chordae . In case of ARF with severe cardiac failure is secondary to mitral insufficiency and pan carditis .

29. PATHOPHYSIOLOGY OF ACUTE SEVERE MITRAL INSUFFIENENCY Valve or Sub valvular damage secondary to inflammatory process MITRAL INSUFFICINCY Increased volume load leading to left ventricular dilatation. Increased left atrial pressure resulting in pulmonary congestion LEFT HEART FAILURE

30. CHRONIC SEVERE INSUFFIENCY Leaflet and chordal thickening, chordal fussion and restricted leaflet motion secondary to fibrosis Persistent mitral insuffiency Increased Pulmonary artery pressure [PAH] Development of RA/RV dilatation RIGHT HEART FAILURE

31. CLINICAL MAINFESTATION Mild disease – NO sign of heart failure Precordium normal , apex normal Ascultation - Normal S2 Holosystolic Murmur (best heard at apex, radiating to axilla )

32. Severe Disease – Signs of Heart failure Apex beat – heaving apical impulse with cardiomegaly Ascultation – wide split S2 , P2 may be louder if Pulmonary hypertension develops , S3 present , CAREY COOMBS MUMRMUR

33. ELECTROCARDIOGRAM AND CHEST RADIOGRAPHS Both are normal in mild disease Severe disease – ECG – prominent ,long duration and often bifid ‘p’ waves . Chest radiography – LA/LV dilatation , prehilar congestion if PAH is developed.

34. 2D ECHO FINDINGS IN SEVERE MITRAL INSUFFIENCY Acute phase Mitral annnular dilatation , chordal elongation and at times evidence of chordal rupture – resulting in flail leaflet Chronic phase Leaflet and chordal thickening, chordal fussion and restricted leaflet motion leading to variable degree of regurgitation

35. COMPLICATION OF MITRAL INSSUFIENCY Cardiac failure Atrial fibrilation Arrythmias Infective endocarditis

36. MITRAL STENOSIS It results from firbrosis of mitral ring, commissural adhesions and contracture of the valve leaflets, chordae and papillary muscles. • Pure or predominant MS occurs in approximately 40% of all patients with rheumatic heart disease It is a chronic processes and often takes >10years for lesions to become fully establish • Two‐thirds of all patients with MS are female • Reduction of valvular size to 25% or less • Rarely it may occur in 6 months to 2 years (juvenile mitral stenosis)

37. • Normal valve area: 4‐6 cm 2 • Mild mitral stenosis: – MVA 1.5‐2.5 cm2 – Minimal symptoms • Mod mitral stenosis – MVA 1.0‐1.5 cm2 usually does not produce symptoms at rest • Severe mitral stenosis – MVA < 1.0 cm2

38. PATHOPHYSIOLOGY

39. CLINICAL MANIFESTATIONS- SYMPTOMS Palpitations Cough Orthopnea PND Respiratory infections Hepatic congestion Edema hemoptysis COMPLICATIONS Atrial fibrillations Systemic embolism Left heart failure Pulmonary hypertension Right heart failure

40. RECOGNIZING MITRAL STENOSIS Palpation: • Small volume pulse • Tapping apex‐palpable S1 • +/‐ palpable opening snap (OS) • RV lift • Palpable S2 ECG: • LAE, A Fib, RVH, RAD

41. PHYSICAL EXAMINATION • First heart sound (S1) is accentuated and snapping S1_____________S2__OS________________S1 • Opening snap (OS) after aortic valve closure • Low pitch diastolic rumble at the apex • Pre‐systolic accentuation (esp. if in sinus rhythm)

42. PROGRESSION- Progressive / life long disease, • Usually slow & stable in the early years. • Progressive acceleration in the later years • 20‐40 year latency from rheumatic fever to symptom onset.

43. AORTIC INSUFFIENCY It is the most common complication of rheumatic aortic valvitis . Isolated A.R is seen in 1/12 th Combined mitral and aortic insuffiency in acute ARF is more common presentation. Most common in men Patients with recurrent rheumatic episodes will have more rapid progression .

44. PATHOPHYSIOLOGY ACUTE – Poor coaptation of leaflet or leaflet prolapse Regurgitation of blood leading to LV volume overload Compensatory mechanisms- Increased chamber compliance and both concentric and eccentric hypertrophy of ventricles. degree of dilatation directly proportional to severity of A.R.

45. Chronic Aortic Regurgitation Increase in the ventricular dilatation over an period of time leading to increased diastolic compliance and permits ventricles to accommodate a large regurgitationt blood without increase in end diastolic pressure. Aortic lesion were associated with increased peripheral vasodilatation

46. Rapid dissipation of blood into the dilated perpheral vasculature resulting in low diastolic pressure . Reduced coronary flow Inadequate nutrition to peripheral tissue Peak systolic pressure increases as a compensation Leading to increase in MEAN ARTERIAL PRESSURE Signs of Aortic Regurgitation.

47. CLINICAL FEATURES Symptoms seen in severe in aortic insuffiency with mitral involvement or Myocardial dysfunction Palpitation is the earliest symptom in patient of AR • Wide pulse pressure • Exertional angina • Heaving apex • Blowing early diastolic murmur in aortic area‐ best heard in expiration • AUSTIN FLINT MURMUR – apical presystolic murmur due to large flow across mitral valve

48. Signs of Aortic Regurgitation.

49. INVESTIGATION Chest radiograph – Enlargement of left ventricle and aorta ECG – normal in majority of cases , in advance case it reveals signs of LV hypertrophy with a strain pattern and prominent ‘p’ waves 2D ECHO – dilatation of left ventricle and diastolic mitral valve flutter or oscillation caused by aortic regurgitant flow hitting the valve leaflet

50. TRICUSPID VALVE DISEASE Involvement is rare in both acute and chronic phases of rheumatic fever It is more common secondary to RV dilatation , resulting from significant left sided cardiac lesion

51. CLINICAL MANIFESTATIONS- Prominent pulsation of jugular veins Systolic pulsation of liver Blowing holosystolic murmur at lower left sternal border that increases in intensity during inspiration Concomitant signs of mitral or aortic valve disease with or without AF

52. PULMONARY VALVE DISEASE It is rare and occurring on functional basis secondary to pulmonary hypertension It is an late findings of mitral stenosis It is confirmed by 2D Echo and Doppler findings

53. MURMURS OF RHD

54. Cardiac conditions simulating rheumatic carditis and rheumatic heart disease Innocent murmurs – Always systolic Best heard in pulmonary / lower left sternal border ex- Pulmonic murmur Stills murmur

55. Infective Endocarditis - May be mistaken for a recurrent attacks of rheumatic fever in a patient with established rheumatic heart disease Confusion occurs because often IE affects the joints as well as heart Differentiating points- Joint involvement is monoarticular High grade fever Associated with extra cardiac manifestations

56. Mitral Valve Prolapse [floppy mitral valve, click syndrome, or Barlow's syndrome ] Benign disorder It can be distinguished from its rheumatic counterpart clinically on the basis of the characteristic systolic click and late systolic murmur. Echocardiography is the most reliable way to confirm the diagnosis.

57. Congenital heart disease- The systolic murmur of a ventricular septal defect due to a small left-to-right shunt can resemble the murmur of mild mitral regurgitation. Atrial septal defects associated with a deformed mitral valve can mimic rheumatic mitral regurgitation. Congenital bicuspid aortic valve- diastolic murmur of aortic regurgitation, which can occur in patients with ventricular septal defect and aortic insufficiency

58. Viral Carditis - A number of viral agents, especially Coxsackie B and Arboviruses , can cause myocarditis with cardiac enlargement, arrhythmias, and heart failure. The absence of significant heart murmur generally excludes rheumatic fever as the etiology

59. Arthritis- Most Common – 30 to 50% Early manifestation Pain > swelling Large joint involvement Pathogenesis - The articular and periarticular structures are swollen and edematous , but there is never erosion of the joint surfaces or pannus formation. The exudate is turbid but never purulent. Migratory Polyarthritis ????? No Permanent Sequelae Resolves in Six weeks

60. Arthritis often affects the legs first and then spreads to the arms. The knees are most frequently affected (75%) followed by the ankles (50%), elbows, wrists, hips, and small joints of the feet (each 12-15%), shoulders and small joints of the hand (7-8%). Sacroiliac, Temporo‐mandibular and Cervical joints involvement is rare . Ref- MILTON MARKOWITZ Book of Rheumatic fever

61. Jaccoud's arthritis Jaccoud's arthropathy appears after multiple, prolonged, and severe attacks of rheumatic fever Jaccoud's arthritis is also called, more descriptively, chronic post-rheumatic fever arthropathy . It is a rare, indolent, slowly progressive process that deforms the fingers and sometimes the toes. The deformity consists of ulnar deviation of the fingers, flexion of the metacarpophalangeal joints, and hyperextension of the proximal interphalangeal joints

62. JACCOUD'S ARTHRITIS

63. SYDENHAM’S CHOREA 10 – 15 % of patients Usually delayed/often sole manifestation of ARF Sydenham's chorea, chorea minor, or "St Vitus ' dance" is a neurological disorder consisting of involuntary movements , muscular weakness , and emotional disturbances . It disappear during sleep , but may occur at rest and may interfere with voluntary activity

64. In exceptional cases the psychological manifestations may be quite severe, and result in transient psychosis "chorea insaniens " The neurological examination does not reveal sensory losses or pyramidal tract involvement. Chorea is unusual after puberty, and does not occur in adults, with the exception ofrare cases during pregnancy "chorea gravidarum "

65. Milkmaid's grip – irregular contractions of the muscles of the hands while squeezing the examiner's fingers Spooning and pronation of the hands when the patient's arms are extended(St. Vitus Dance) Wormian movements of the tongue upon protrusion - Jack in the Box Handwriting to evaluate fine motor movements

66. St. Vitus Dance

67. Pronator sign

68. ERYTHEMA MARGINATUM Occurs 1-2% of Patients Macular non pruritic rash Serpiginous border, raised edges, central clearing Most common on trunk less common on face

69. ERYTHEMA MARGINATUM

70. SUBCUTANEOUS NODULES Seen in around <1% Small, pea sized, 0.5 to 2 cms in diameter , Firm, mobile, Painless Seen over the extensor surface of Wrist, Elbow, Spine Usually seen in individuals with long standing carditis

71. SUBCUTANEOUS NODULES

72. POINTS TO REMEMBERED... Rheumatic fever is an acute, immunologically mediated multisystemic inflammatory disease that follows group A beta haemolytic streptococcus { 1, 3, 5, 6, 18, 29 [ pharyngeal serotypes]} infection of the throat. In India incidence of rheumatic fever by eco studies is 0.5 to 11 per thousand, most common age group is 5-15 Diagnosis is based on MODIFIED JONES CRITERIA Exceptions for Jones criteria is Carditis , recurrence of Rheumatic fever and Choria .

73. ARTHRITIS 60-75%, CARDITIS 50-60%, CHOREA 10-15%, ERYTHEMA MARGINATUM 1-2%, SUB CUTANEOUS NODULES <1% RHD can lead to pancarditis in which ENDOCARDITIS is must. Pathgnomic of RHD is ASCHOFF BODIES Sub clinical carditis is an echocardiographic evidence of mitral or aortic valvulitis in the abscnce of ascultatory findings

74. Valvitis -Mitral valve 70%, Combined mitral and aortic disease 25%, Tricuspid – less frequently involved Pulmonary valves – almost always escapes injury . Clinical history and physical examination remains the mainstay for dianosing RF and RHD particularly in resource poor settings Infective endocarditis remains a major treat for individuals with rheumatic valvular disease.

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