Rheumatic Fever And RHD

Presentation on theme: "Rheumatic Fever And RHD"— Presentation transcript:

1 Rheumatic Fever And RHD
Dr. Abdulelah Mobeirek (FRCPC) Consultant Cardiologist KFCC

2 Lecture Outline What is ARF And RHD? Diagnosis
Jones Criteria & 2015 revision Differential Diagnosis Investigations, Management Rheumatic Valvular Heart Disease Prevention

3 Rhuematic Fever A multisystem disease
Follows group A beta hemolytic streptococcal throat infection It represents a delayed immune response to infection with manifestations appearing after a period of 2-4 weeks Age 5-15 yrs A multisystem disease RHD is a long term complication og ARF Major effect on health is due to damage to heart valves

4 Pathologic Lesions Ashcoff nodules
Fibrinoid degeneration of connective tissue, inflammatory cells

5 Global Burden of RHD A leading cause of CV morbidity & mortality in young people Total cases with RHD:20 Millions CHF:3Million,valve surgery required in 1Million Annual incidence of RF: 0.5 Million, nearly half develop carditis Estimated deaths from RHD: 250,000/YR Imposes a substantial burden on health care systems with limited budgets

6 Epidemiologic Background
The incidence of RF and the prevalence of RHD has declined substantially in Europe, North America and other developed nations this decline has ben attributed to improved hygiene, reduced household crowding, and improved medical care

7 Epidemiologic Background
The major burden is currently found in low and middle income countries (India, middle east), and in selected indigenous populations of certain developed countries (Australia and Newzealand). A disease of poverty and low socioeconomic status In underdeveloped countries RHD is the leading cause of CV death during the first five decades of life

8 Epidemiologic Background
The incidence in indigenous population of Australia: cases/ people/yr in yrs age group. In Saudi Arabia: incidence 30 cases/ people/yr and prevalence 310/ people in 6-15 yrs age group Low risk population ARF incidence < 2/100000/yr (5-14 yrs) or all age prevalence of RHD <1/1000 population/yr

9 Diagnosis of ARF No single test to diagnose ARF
The symptoms and signs are shared by many inflammatory and infectious diseases Accurate diagnosis is important Overdiagnosis will result in individuals receiving treatment unnecessarily Underdiagnosis may lead to recurrences of ARF causing further damage, the need for valve surgery, CHF and premature death

10 Diagnosis of ARF Diagnosis is primarily clinical and is based on a constellation of signs and symptoms, which were initially established as the Jones criteria In 1944 Dr. TD Jones published a set of guidelines for diagnosis of ARF “Jones Criteria” Subsequently Modified in 1965, 1984 and by AHA Revised recently by AHA

11 1992 Modified Jones Criteria

12 Carditis Occurs in 50-70% of cases
Only manifestation of ARF that leaves permanent damage May be subclinical Murmurs of MR or AR may occur in acute stage while mitral stenosis occurs in late stages Cardiomegaly and CHF may occur

13 Arthritis Common: present in 35-66% Earliest manifestation of ARF
Large joints: The knees and ankles, shoulders, elbows “Migrating”, “Fleeting” polyarthritis Duration short < 1 week Rapid improvement with salicylates Does not progress to chronic disease

14 Sydenham Chorea Also known as Saint Vitus’dance
Occur in 10-30%, extrapyramidal manifestation, female predominnce Abrupt Purposeless involuantry movements of muscles of face, neck, trunk, and limbs. Delayed manifestation of ARF -months Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face

15 Subcutaneous Nodules Occur in 10% Usually 0.5 – 2 cm long
Firm non-tender Occur over extensor surfaces of joints, on bony prominences, tendons, spine Short lived: last for few days Associated with severe carditis

16 Subcutaneous nodules

17 Subcutaneous Nodules

18 Erythema Marginatum Present in <6%
Less common, but highly specific manifestation of ARF Reddish border, pale center, round or irregular serpiginous borders, non- pruritic, transient rash Occurs on trunk, abdomen or proximal limbs Associated with carditis

19 Erythrma Marignatum

20 2015 Revised Jones Criteria

21 2015 Revision of Jones Criteria
In accordance with the degree of prevalence of ARF/RHD in the population: low risk populations have been defined as those with ARF incidence < 2: school-age children or all age prevalence of RHD of < 1: population per year Children not from low risk population have been considered to be at moderate or high risk

22 2015 Revision of Jones Criteria
2. Advocated the use of Echocardiography in all cases of confirmed or suspected ARF or RHD, to diagnose valvulitis( subclinical carditis) and has been included as a major criterion to diagnose carditis 3. Aseptic monoarthritis has been included as a major criteria in moderate or high risk population

23 2015 Revision of Jones Criteria
4. Polyarthritis has been recognized as a major manifestation for moderate or high risk population 5. Fever >38.5 c, ESR >60 and or CRP > 3mg/dl for low risk population, and fever >38 and ESR >30 and or CRP > 3mg/dl for moderate or high risk population

24 Revised Jones Criteria-2015

25 2015 Revised Jones Criteria
A firm diagnosis requires 2 Major manifestations or 1 Major and 2 Minor manifestations and 2 ) Evidence of a recent streptococcal infection.

26 2015 Revised Jones Criteria
Evidence of Preceding GAS Infection: Increased or rising ASO titer or Anti- Dnase B titer A positive throat culture

27 Rheumatic Fever Recurrences
Reliable past history of ARF: 2 major or 1 major and 2 minor or 3 minor manifestations sufficient for diagnosis Presence of antecedent streptococcal infection When minor manifestations only present exclude other causes.

28 DDX of ARF

29 Investigations

30 Investigations

31 Treatment of ARF Bed rest Salicylates : Aspirin
mg /kg/day given as 4 divided doses for weeks Attain a blood level mg/dl Penicillin: Procaine Penicillin 4 million units/day x10 days Prednisolone:2mg/kg/day taper over 6 weeks, Given when there is severe carditis Heart Failure Treatment: diuretics, ACEI

32 Chronic Rheumatic Heart Disease
Most commonly in Mitral-70% Frequently in Aortic-40% Less frequently Tricuspid-10% Rarely pulmonary valve-2% Mitral Stenosis is more common in females(3:1), while males have higher incidence of Aortic Regurgitation

33 Mitral Stenosis The normal MVA= 4-5 cm2 In severe ms <1.5 cm2
High LAP The rise in LAP causes a similar rise in pulmonary capillaries, veins and artery

34 Mitral Stenosis Mitral stenosis with diffuse fibrous thickening and distortion of the valve leaflets, commissural fusion (arrows), and thickening and shortening of the chordae tendineae. Marked dilation of the left atrium is noted in the left atrial view

35 Clinical Features Dyspnea Fatigue Palpitation Hemoptysis (10%)
Hoarseness ( Ortner’s syndrome) Dysphagia Storke or peripheral embolization

36 Clinical Features Cyanosis (Mitral facies,malar flush)
Tapping apex ( S1) Parasternal heave Diastolic thrill Accentuated S1 , accentuated S2 Opening snap Mid-diastolic rumble

37 Investigations CXR Straightening of the left heart border
Double density Kerley B lines , CA in MV ECG: LAE, P Mitrale ,RV dominance Echodoppler

38 Echo In Mitral Stenosis
Fig. 1.1 Two-dimensional echocardiography in mitral stenosis. (a) Parasternal long-axis view through a stenotic mitral valve, demonstrating typical diastolic doming of the anterior leaflet.

39 Management B-Blockers ,CCB Digoxin ( AF ) Warfarin
Balloon Valvuloplasty Mitral valve replacement

40 BMV Fig. 4.4 Inoue catheter for mitral balloon valvotomy. The balloon-tipped catheter is positioned across the stenotic valve and inflated. The narrowed orifice is subjected to the force from the transiently inflated balloon. When the procedure is successful, these forces break the scar tissue and allow greater opening of the mitral valve leaflets. This technique is a balloon commissurotomy that has evolved from the earlier extensive experience with closed and open surgical commissurotomy.

41 Mitral Regurgitation Asymptomatic Dyspnea , orthopnea, PND Displaced PMI, Thrill Soft S1, Pansystolic murmur Treatment is surgical

42 ECHO Fig. 1.5 Echocardiographic studies in mitral regurgitation. Parasternal long‑axis views show (a) prolapse of the posterior mitral leaflet (arrow) with corresponding flow directed along the anterior mitral leaflet towards the atrial septum. Apical four-chamber views show (c) the prolapse more clearly (arrow), and the extension of the regurgitation is more clearly visualized (d). The jet is ‘spoon shaped’, broad and thin along the wall, and it is therefore easily underestimated. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle.

43 Aortic Regurgitation Water-hammer / collapsing pulse
Wide pulse pressure Corrigan’s sign De Musset sign Muller sign Quincke’s pulse Hill’s sign

44 ECHO Fig Severe aortic regurgitation. (a) Apical view showing a wide jet reaching to the apex.

45 Aortic Stenosis Fig. 9.3 Gross pathology of degenerative aortic stenosis. The figure shows the masses of lipo-calcification on the aortic side of all three leaflets and the absence of commissural fusion.

46 Symptoms Angina Syncope Dyspnea

47 Signs Arterial Pulse wave form : Plateau Small (Parvus)
Slow rise (Tardus) Sustained not displaced PMI Systolic thrill S4

48 Signs Late peaking of murmur Single S2 : Soft or absent A2
Paradoxical splitting of S2

49 Aortic Valve Disease Treatment: Aortic valve Replacement
Transcathter Aortic Valve Replacement

50 For individuals allergic to penicillin and sulfadiazine
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 (60 lb Oral g once daily for patients >27 kg (60 lb) 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

51 Duration of Secondary Rheumatic Fever Prophylaxis
Category Duration Rheumatic fever with carditis and y since last episode residual heart disease or until age 40y ,(which (persistent valvar disease*) ever is longer), sometimes life long prophylaxis Rhumatic fever with carditis yrs or until age 21yrs But no residual VHD (whichever is longer) Rheumatic fever without carditis 5 y or until age 21 y, ( whichever is longer) *