ARF AND RHD Flashcards
Rheumatic fever
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
risk factors
poverty, crowding , which facilitates spread of GAS infections
children 5 to 15 years of age
genetic predisposition
pathogenesis
cytotoxicity theory
immune-mediated pathogenesis
Clinical Manifestations and Diagnosis
Jones Criteria
5 major and 4 minor criteria
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
major criteria
migratory polyarthritis
carditis
subcutanous nodules
erythema marginatum
syndenhams chorea
minor criteria
fever
acute phase reactant elecation
crp > 10
esr > 30 in high and > 60 in low prevalence
arthralgia
prolonged pr interval
diagnosis
2 M with evidence
1 M and 2m with evidence
5 m with evidence
evidence of preceding strep infection is by
anti streptolysin o
antistreptococcal antibody titer rise
postive throat culture
Migratory Polyarthritis
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.
Synovial fluid in acute RF
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.
carditis
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
A major change in the 2015 revision of the Jones Criteria is the acceptance of subclinical carditis which is
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
Sydenham chorea
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.
Clinical maneuvers to elicit features of chorea include
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.
Erythema Marginatum
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.
Subcutaneous Nodules
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
syndenham chorea why
dysfunction of basal ganglia due anti neuronal antibody
treatment
primordial prevention of rf
primary prevention - of strep infection
secondary prophylaxis - of RF recurrence
Medical and surgical managemnt of rhd
tretament of acute carditis
general management
anti inflam. aspirin
steroids
primary prophylaxis of strep infection
penicillin G
allergy
cephal
azithro
alinda
secondary prophylaxis
penicillin G
sulfadiazine
allergy
macrolides
chorea treatment
phenbarbitone sedative
complication
Congestive heart failure:
Cardiomegaly
Chronic valve lesions
Rheumatic activity (Recurrences ):
Pulmonary hypertension:
Differential diagnosis
other causes arthritis
rheumatoid arthritis
infection
hematologic
immunologic
Rheumatoid arthritis:
- 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
. Other causes of chorea
- Wilson disease.
- Huntington chorea
- Cerebral palsy.
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)
Mitral regurgitation
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
PATHOPHYSIOLOGY
of Mr
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
On hx of MR
Patients are usually asymptomatic with mild MR.
Rarely, fatigue (caused by reduced forward cardiac output) and palpitation (caused by AF) develop.
Physical Examination
MR
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).
Complications
cardiac failure
recurrent episodes of ARF
atrial fibrillation (AF) or other arrhythmias
infective endocarditis
Management
medical
surgical
Medical
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.
surgery
annuloplasty
valve replacement
indication for surgery
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)
Aortic Regurgitation
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,
Clinical manifestation
Hx in AR
dyspnea on exertion
decreased exercise tolerance.
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.
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
(“Austin Flint” murmur)
Chest Radiography
AR
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.
AR managment
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.)
Mitral Stenosis
pathogenesis
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.
Physical Examination MS
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
Occasionally, a high-frequency diastolic murmur of PR called
MS
(Graham Steell’s murmur)
