1. RHEUMATIC HEART DISEASE
HA MWAKYOMA, MD

2. Introduction
Rheumatic heart disease is the most serious manifestation of acute rheumatic fever and is the end result of carditis, which affects 30% to 45% of patients with acute rheumatic fever.

3. Introduction—cont--
Damage to the cardiac valves may be chronic and progressive and can lead to congestive heart failure and death
Pockets of resurgence of rheumatic fever have occurred all over the world in recent years

4. Epidemiology
Acute rheumatic fever is a disease of the young, occurring most commonly in preadolescent children
It is much rarer in children younger than 5 years and adults older than 35 years

5. Epidemiology—cont--
Recurrent episodes occur through adolescence and into early adulthood, and it is thought that the cumulative effect of recurrent episodes of acute rheumatic fever leads to the development of rheumatic heart disease
Both acute rheumatic fever and rheumatic heart disease are more common in females

6. Pathogenesis Incompletely understood;
Although streptococci have not been found in the heart tissues of patients with acute rheumatic fever, there is strong circumstantial evidence that acute
rheumatic fever is the result of an exaggerrated immune response to pharyngeal infection with group A streptococcus.

7. Pathogenesis-cont--
Outbreaks of acute rheumatic fever closely follow epidemics of streptococcal pharyngitis or scarlet fever, and adequate treatment of documented pharyngeal streptococcal infection clearly decreases the incidence of subsequent rheumatic fever.

8. Pathogenesis-cont--
Appropriate prophylaxis with antibiotics can prevent recurrence in patients with prior episodes of acute rheumatic fever

9. Pathogenesis—cont--
Most patients with acute rheumatic fever also have elevated titers to one or more of the three antistreptococcal antibodies (streptolysin O, hyaluronidase, and streptokinase).
Group A streptococcal infection of the pharynx is a necessary element for the subsequent development of acute rheumatic fever

10. Valvular Heart Diseases
Heart -Pathology
Valvular Heart Diseases
Rheumatic Heart Disease (RHD)
Rheumatic Fever (RF)
Acute, immune-mediated multi-system disease occurring a few weeks after an episode of group A streptococcal pharyngitis
MC in children 5-15 yrs old
RF  RHD (deforming fibrotic valvular disease)
After first attack -  susceptibility to reactivation of RF with subsequent pharyngitis

11. Rheumatic Heart Diseases
Heart -Pathology
Rheumatic Heart Diseases
Etiopathogenesis
Hypersensitivity ( immunological) reaction to “M” protein of Group A streptococci
RF develops weeks after Streptococcal pharyngitis
Antibodies against “M” protein cross react with
Cardiolipins of Heart Rheumatic disease
GBM of Kidney  Post – streptococcal Glomerulonephritis (PIGN/PSGN/ Acute nephritic syndrome)

12. Morphology
Acute (RF) Aschoff bodies (Myxoid degerantion with surrounding inflammation), Pancarditis, Anitschkow cells (macrophages with caterpillar like nucleus)
Chronic (RHD) fibrosis of valve cusps, fusion of commisures,

13. Rheumatic Heart Diseases
Heart -Pathology
Rheumatic Heart Diseases

14. Clinical Features
Onset of acute rheumatic fever is typically characterized by an acute febrile illness 2 to 4 weeks after an episode of pharyngitis.
Diagnosis is primarily clinical and is based on a constellation of signs and symptoms, which were initially established as the Jones criteria in 1944:

15. Jones Criteria (1992 Revision) for Diagnosis of Acute Rheumatic Fever
Major manifestations
Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules

16. Jones Criteria (1992 Revision) for Diagnosis of Acute Rheumatic Fever– cont--
Minor manifestations
Fever
Arthralgias
Previous rheumatic fever or rheumatic heart disease
Increased C- reactive protein concentrations or ESR
Prolonged PR interval on ECG
Evidence of antecedent group A streptococcal infection
Positive throat culture or rapid antigen test positive for group A streptococcus
Increased or increasing streptococcal antibody titer

17. The Jones Criteria for Rheumatic Fever, Updated 1992
Major Criteria
Minor Criteria
Carditis
Clinical
Migratory polyarthritis
Fever
Sydenham's chorea
Arthralgia
Subcutaneous nodules
Laboratory
Erythema marginatum
Elevated acute phase reactants
Prolonged PR interval
plus
Supporting evidence of a recent group A streptococcal infection (e.g., positive throat culture or rapid antigen detection test; and/ or elevated or increasing streptococcal antibody test)

18. Jones Criteria (1992 Revision) for Diagnosis of Acute Rheumatic Fever
A firm diagnosis requires
2 major manifestations or 1 major and 2 minor manifestations
and
2 ) Evidence of a recent streptococcal infection.
However, when chorea or carditis is clearly present, evidence of an antecedent group A streptococcal infection is not necessary.

19. Rheumatic Heart Diseases
Heart -Pathology
Rheumatic Heart Diseases
Types
Feature
Rheumatic Fever (RF)
Rheumatic Heart Disease (RHD
Onset
Acute
Chronic
Age
Children ( 5 – 15 yrs)
Adults
Pathology
Carditis, arthritis, chorea
Valvular disease ( MS, MR)
Aschoff bodies
Pathognomonic
Not seen
Diagnosis
John’s criteria
Not applicable

20. Clinical Features
Although the clinical usefulness of the Jones criteria has been recently reaffirmed, the main features have been modified or updated several times in order to increase the specificity of the criteria.
The World Health Association (WHO) has more recently developed criteria that favor sensitivity over specificity; they may be the preferred guidelines in countries with populations at high risk for acute rheumatic fever

21. World Health Association for Diagnosis of Acute Rheumatic Fever
First episode- same as the Jones criteria
Recurrent episode in a patient without established RHD- same as for first episode
Recurrent episode in a patient with established RHD- requires 2 minor Jones criteria manifestations and evidence of an antecedent gropu A streptococcus infection

22. Clinical Features
Beyond fever, arthritis is typically the earliest manifestation of acute rheumatic fever. In untreated patients, the arthritis is classically described as “migrating” from joint to joint in quick succession.
The knees and ankles are often the first affected.
The duration of joint inflammation is short (≤ 1 week), and the synovial fluid is generally sterile when examined.

23. Chorea, also known as Sydenham chorea or St. Vitus dance,
Clinical Features
Chorea, also known as Sydenham chorea or St. Vitus dance,
is a neurologic movement disorder characterized by abrupt, purposeless involuntary movements of the muscles of the face, neck, trunk, and limbs.
Muscular weakness (hypotonia) and behavioral disturbances such as obsessive- compulsive behaviors are considered to be additional findings of chorea. .

24. Chorea-- Clinical Features—cont--
The course of the syndrome is variable. Symptoms tend to develop subtly, progressively worsen over 1 to 2 months, and spontaneously resolve gradually after 3 to 6 months. Residual waxing and waning of symptoms may occur for a year or more, and 20% of patients have recurrences within 2 years

25. Clinical Features
Two classic skin lesions with well-described identifying characteristics occur in acute rheumatic fever:
Subcutaneous nodules ranging from several millimeters to 2 cm occur for approximately 2 weeks over bony surfaces or near tendons.
The nodules are described as firm and painless, and the overlying skin is not inflamed. They are typically smaller and shorter lived than nodules of rheumatoid arthritis.

26. Subcutaneous nodule

27. Clinical Features—cont--
Erythema marginatum
is a classic skin rash that occurs early in the course of acute rheumatic fever.
The rash is evanescent, pink to red and nonpruritic.
It typically occurs on the trunk or proximal limbs.

28. Erythema marginatum
The rash appears as a ring that extends centrifugally, while the skin in the center of the ring returns to normal. The rash can persist or recur after other symptoms of acute rheumatic fever have passed.
Interestingly, erythema marginatum and the subcutaneous nodules usually occur only in patients with carditis.

29. Erytherma marginatum

30. Erytherma marginatum

31. Carditis and Rheumatic Heart Disease
The term carditis refers to diffuse inflammation of the pericardium, epicardium, myocardium, and endocardium.
Valvular involvement, with leaflet thickening, occurs as a rule; in addition, the valves frequently display small rows of vegetations called verrucae along their appointing surfaces.

32. Carditis and Rheumatic Heart Disease
Symptoms include tachycardia and mild or moderate chest discomfort that is commonly pleuritic in nature.

33. Rheumatic Heart Diseases
Heart -Pathology
Rheumatic Heart Diseases
Commissural fusion

34. Vagetations

35. RHD:-(thickened mitral valve, thickened chordae tendineae, hypertrophied left ventricular myocardium).

36. Carditis and Rheumatic Heart Disease
The cardiac physical examination often reveals the presence of pericardial friction rub and typically, new or changing murmurs.
In young patients, mitral valve regurgitation is the predominant cardiac lesion
A new apical systolic murmur is characteristic
Aortic regurgitation is less common but can develop

37. The pulmonary and tricuspid valves are rarely involved
Mitral stenosis becomes progressively more common in early to mid adulthood.
Heart block of all degrees is seen on ECGs
The most common radiologic finding is cardiomegaly.

38. Carditis and Rheumatic Heart Disease
Myocarditis is characterized by infiltration of
mononuclear cells,
vasculitis, and
degenerative changes of the insterstitial connective tissue.
The pathognomonic lesion is the Aschoff body in the proliferative stage, which is present in 30% to 40% of biopsy samples from patients with acute rheumatic fever.

39. Carditis and Rheumatic Heart Disease
The use of echocardiography has contributed much toward understanding the pathogenesis of valvular regurgitation in rheumatic carditis.
At least initially, the regurgitation appears to result from geometric changes and stresses affecting the left ventricle rather than from the rheumatic process directly involving the valve leaflets.

40. Carditis and Rheumatic Heart Disease
Acute rheumatic fever is also the predominant cause of mitral stenosis; approx. 25% of patients with rheumatic heart disease have pure mitral stenosis.
Mitral stenosis is characterized by progressive thickening, fibrosis and calcification of the leaflets and chordae tendineae.
The leaflets show fibrous obliteration and the mitral valve orifice becomes funnel- shaped, like a fish- mouth.
This feature and the classic hockey-stick appearance of the anterior mitral leaflet in diastole are well seen on echocardiograms

42. Rheumatic Heart Diseases
Heart -Pathology
Rheumatic Heart Diseases
? cells
pathognomonic