ARF AND RHD

Question 1

Rheumatic fever

Answer 1

Rheumatic fever is a diffuse inflammatory disease characterized by a delayed response to an infection by GAS in the tonsilopharyngeal area, affecting the heart, joint, CNS, skin and subcutaneous tissues

Question 2

risk factors

Answer 2

poverty, crowding , which facilitates spread of GAS infections
children 5 to 15 years of age
genetic predisposition

Question 3

pathogenesis

Answer 3

cytotoxicity theory
immune-mediated pathogenesis

Question 4

Clinical Manifestations and Diagnosis

Answer 4

Jones Criteria
5 major and 4 minor criteria

Question 5

2015 revision includes separate criteria for Low-Risk populations (defined as those with incidence ≤2 per 100,000 school-age children per year or all-age RHDprevalence of ≤1 per 1,000 population) and Moderate/High-Risk populations

Question 6

major criteria

Answer 6

migratory polyarthritis
carditis
subcutanous nodules
erythema marginatum
syndenhams chorea

Question 7

minor criteria

Answer 7

fever
acute phase reactant elecation
crp > 10
esr > 30 in high and > 60 in low prevalence
arthralgia
prolonged pr interval

Question 8

diagnosis

Answer 8

2 M with evidence
1 M and 2m with evidence
5 m with evidence

Question 9

evidence of preceding strep infection is by

Answer 9

anti streptolysin o
antistreptococcal antibody titer rise
postive throat culture

Question 10

Migratory Polyarthritis

Answer 10

75% of patients with acute rheumatic fever
typically involves larger joints, particularly the knees, ankles, wrists, and elbows.
classically hot, red, swollen, and exquisitely tender, with even the friction of bedclothes being uncomfortable
migratory in nature
a severely inflamed joint can become normal within 1-3 days without treatment
dramatic response to even low doses of salicylatesis
almost never deforming.

Question 11

Synovial fluid in acute RF

Answer 11

usually has 10,000-100,000 white blood cells/μL with a predominance of neutrophils
protein level of approximately 4 g/dL
normal glucose level
forms a good mucin clot.

Question 12

carditis

Answer 12

occurs in approximately 50–60%
the most serious manifestations of acute RF and account for essentially all the associated morbidity and mortality
characterized by pancarditis
varies in severity
Endocarditis (valvulitis) is a universal finding
Most rheumatic heart disease is isolated mitral valvular disease or combined aortic and mitral valvular disease. Isolated aortic or right-sided valvular involvement is quite uncommon

Question 13

A major change in the 2015 revision of the Jones Criteria is the acceptance of subclinical carditis which is

Answer 13

defined as without a murmur of valvulitis but with echocardiographic evidence of valvulitis) or clinical carditis (with a valvulitis murmur) as fulfilling the major criterion of carditis in all populations

Question 14

Sydenham chorea

Answer 14

occurs in approximately 10–15% of patients with acute RF
usually presents as an isolated, frequently subtle, movement disorder.
Emotional lability, incoordination, poor school performance, uncontrollable movements, and facial grimacing are characteristic, all exacerbated by stress and disappearing with sleep.
occasionally is unilateral (hemichorea).
latent period from acute GAS infection to chorea is usually substantially longer than for arthritis or carditis and can be months.
Onset can be insidious, with symptoms being present for several months before recognition.

Question 15

Clinical maneuvers to elicit features of chorea include

Answer 15

1) demonstration of milkmaid’s grip (irregular contractions and relaxations of the muscles of the fingers while squeezing the examiner’s fingers),
2) spooning and pronation of the hands when the patient’s arms are extended
3) wormian darting movements of the tongue on protrusion,
4) examination of handwriting to evaluate fine motor movements.

Question 16

Erythema Marginatum

Answer 16

Erythema Marginatum
approximately 1% of patients with acute RF
characteristic rash consists of erythematous, serpiginous, macular lesions with pale centers that are not pruritic
It occurs primarily on the trunk and extremities, but not on the face
can be accentuated by warming the skin.

Question 17

Subcutaneous Nodules

Answer 17

Subcutaneous nodules are a rare (≤1% of patients with ARF finding
consist of firm nodules approximately 0.5-1 cm in diameter along the extensor surfaces of tendons near bony prominences.
There is a correlation between the presence of these nodules and significant rheumatic heart disease

Question 18

syndenham chorea why

Answer 18

dysfunction of basal ganglia due anti neuronal antibody

Question 19

treatment

Answer 19

primordial prevention of rf

primary prevention - of strep infection

secondary prophylaxis - of RF recurrence

Medical and surgical managemnt of rhd

Question 20

tretament of acute carditis

Answer 20

general management

anti inflam. aspirin

steroids

Question 21

primary prophylaxis of strep infection

Answer 21

penicillin G

allergy
cephal
azithro
alinda

Question 22

secondary prophylaxis

Answer 22

penicillin G
sulfadiazine

allergy
macrolides

Question 23

chorea treatment

Answer 23

phenbarbitone sedative

Question 24

complication

Answer 24

Congestive heart failure:
Cardiomegaly
Chronic valve lesions
Rheumatic activity (Recurrences ):
Pulmonary hypertension:

Question 25

Differential diagnosis
other causes arthritis

Answer 25

rheumatoid arthritis
infection
hematologic
immunologic

Question 26

Rheumatoid arthritis:

Answer 26

• Chronic arthritis ;last at least for 6 weeks.
• Can involve small peripheral joints.
• Non migratory
  -No evidence of recent strept. infection.
  -No response to salcylates within 48 hours.
• Deformities are common

Question 27

. Other causes of chorea

Answer 27

• Wilson disease.
• Huntington chorea
• Cerebral palsy.

Question 28

2ry prevention:
* Prevent recurrence of Rheumatic fever by:
* Long acting penicillin (Benzathine penicillin)
- Dose : 1.2 million unit single injection, I.M every 3 - 4 weeks.
-For at least 5 years after last episode for cases without carditis
- For at least 10 years after last episode for cases with carditis without residuals
- For life or till age of 40 for cases with carditis with residuals
*Alternatives: daily oral penicillin V or erythromycin (250 mg twice daily)

Question 29

Mitral regurgitation

Answer 29

is the result of structural changes that may include some loss of valvular substance and/or changes to the subvalvular apparatus, including elongation of the chordae
In ARF with severe cardiac involvement, heart failure is caused by a combination of mitral insufficiency coupled with a pancarditis

Question 30

PATHOPHYSIOLOGY
of Mr

Answer 30

Increased volume load from the mitral insufficiency and the inflammatory process the LV dilates.
LA also enlarges to accommodate the regurgitant volume. Increased left atrial pressure results in pulmonary congestion and symptoms of left-sided heart failure
with severe chronic mitral insufficiency, PAP becomes elevated, the right ventricle and atrium become enlarged, right-sided heart failure

Question 31

On hx of MR

Answer 31

Patients are usually asymptomatic with mild MR.
Rarely, fatigue (caused by reduced forward cardiac output) and palpitation (caused by AF) develop.

Question 32

Physical Examination
MR

Answer 32

The S1 is normal or diminished. The S2 may split widely as a result of shortening of the LV ejection and early closure of the aortic valve. The S3 commonly is present and loud.
The hallmark of MR is a regurgitant systolic murmur starting with S1, grade 2 to 4 of 6, at the apex, with good transmission to the left axilla (best demonstrated in the left decubitus position).

Question 33

Complications

Answer 33

cardiac failure
recurrent episodes of ARF
atrial fibrillation (AF) or other arrhythmias
infective endocarditis

Question 35

Management

Answer 35

medical
surgical

Question 36

Medical

Answer 36

Prophylaxis against recurrence of rheumatic fever
Activity need not be restricted in most mild cases.
Afterload-reducing agents are particularly useful in maintaining the forward cardiac output.
Anticongestive therapy (with diuretics and digoxin) is provided if CHF develops.

Question 37

surgery

Answer 37

annuloplasty
valve replacement

Question 38

indication for surgery

Answer 38

symptoms
LV dysfunction
marked ventricular enlargement
new-onset atrial fibrillation
pulmonary hypertension (possible intervention for severe MR in asymptomatic patients with preserved LV function if valve repair likely in a center with expertise)

Question 39

Aortic Regurgitation

Answer 39

occurs due to leaflet thickening, fibrosis, and leaflet contracture

resulting in abnormal leaflet coaptation and a regurgitant orifice.
leads to both volume and pressure overload of the left ventricle
During a compensatory phase, ventricular dilation occurs to maintain forward stroke volume and cardiac output, and ejection fraction remains normal
may remain asymptomatic for years
Over time, decompensation may occur, resulting in decreased left ventricular function and/or symptoms,

Question 40

Clinical manifestation
Hx in AR

Answer 40

dyspnea on exertion
decreased exercise tolerance.

Answer 41

wide pulse pressure
bounding pulses

Precordial activity is increased, and the apical impulse is displaced laterally due to the dilated left ventricle.

diastolic murmur high-pitched, decrescendo, and heard best along the LSB with the patient leaning forward at end-expiration.

Question 41

In patients with moderate-to-severe aortic regurgitation, a low-pitched mid-to-late diastolic rumbling murmur may be audible at the apex in the absence of organic mitral stenosis
called

Answer 41

(“Austin Flint” murmur)

Question 42

Chest Radiography
AR

Answer 42

Cardiomegaly of varying degree involving the LV is present.
A dilated ascending aorta and a prominent aortic knob frequently are present.
Pulmonary venous congestion develops if LV failure supervenes.

Question 43

AR managment

Answer 43

Specific medical :
vasodilator therapy for patients with symptoms or LV dysfunction who are poor surgical candidates (cardiac or noncardiac reasons)
vasodilator therapy may also be used for short term to improve hemodynamics prior to aortic valve

surgery
Indications for surgery: symptoms, ventricular dysfunction, marked LV enlargement, and for patients undergoing other cardiac surgery (coronary, other valves, etc.)

Question 44

Mitral Stenosis
pathogenesis

Answer 44

results from fibrosis of the mitral ring, commissural adhesions, and contracture of the valve leaflets, chordae, and papillary muscles over time.
often takes ≥10 yr for the lesion to become fully.
Significant mitral stenosis results in increased LA pressure enlargement and hypertrophy of the LA, pulmonary venous hypertension,increased pulmonary vascular resistance, overt pulmonary hypertension.
RV hypertrophy and RA dilation ensue and are followed by RV dilation, TR, and clinical signs of right-sided heart failure.
results from fibrosis of the mitral ring, commissural adhesions, and contracture of the valve leaflets, chordae, and papillary muscles over time.
often takes ≥10 yr for the lesion to become fully.
Significant mitral stenosis results in increased LA pressure enlargement and hypertrophy of the LA, pulmonary venous hypertension,increased pulmonary vascular resistance, overt pulmonary hypertension.
RV hypertrophy and RA dilation ensue and are followed by RV dilation, TR, and clinical signs of right-sided heart failure.

Question 45

Physical Examination MS

Answer 45

An increased RV impulse is palpable along the LSB.
Neck veins are distended if right-sided heart failure supervenes.
loud S1 at the apex and a narrowly split S2 with accentuated P2 are audible if pulmonary hypertension is present.
An opening snap (a short snapping sound accompanying the opening of the mitral valve) may be audible. A low frequency mitral diastolic rumble is present at the apex
A crescendo presystolic murmur may be audible at the apex. Occasionally, a high-frequency diastolic murmur of PR (Graham Steell’s murmur) is present at the ULSB, but it is difficult to distinguish from AR

Question 46

Occasionally, a high-frequency diastolic murmur of PR called
MS

Answer 46

(Graham Steell’s murmur)