Download Acute rheumatic fever

Acquired Heart Diseases
Dr. khalil Ibrahim
Acquired Heart Diseases
Acute rheumatic fever; cardiomyopathies;
cardiovascular infections, including
myocarditis and infective endocarditis;
and non cngenital valvular heart disease
are the most common acquired heart
diseases among the pediatric age group
patients.
Rheumatic Fever
DEFINITION
• Rheumatic fever is a delayed autoimmune
reaction
in
genetically
predisposed
individuals to group A, β-hemolytic,
streptococcal pharyngitis. It is a self-limited
disease that may involve joints, skin, brain,
serous surfaces, and the heart Despite the
dramatic nature of the acute episode, ARF
leaves no lasting damage to the brain, joints
or skin.
Epidemiology
• The incidence of acute rheumatic fever is
3 to 61 per 100,000 school children.
• ARF is predominantly a disease of
children aged 5-14 years and generally
does not affect children less than 3 years
old or adults.
• Acute rheumatic fever is most common
during winter and spring, a seasonal
variation similar to that of streptococcal
pharyngitis.
CLINICAL MANIFESTATIONS
•
Acute rheumatic fever is diagnosed using
the revised Jones criteria, which consist of
clinical and laboratory findings .
• The presence of either two major criteria or
one major and two minor criteria, along with
evidence of an antecedent streptococcal
infection, confirm a diagnosis of acute
rheumatic fever.
• The infection often precedes the
presentation of rheumatic fever by 2 to 6
weeks.
Supporting evidence of a
preceding streptococcal infection
1. Elevated or rising antistreptolysin-O
or other streptococcal antibody, or
2. Rapid antigen test for group A
streptococci, or
3. A positive throat culture, or
4.Recent scarlet fever.
Laboratory Findings
1.
2.
3.
4.
5.
6.
High ESR .
Anemia, leucocytosis .
Elevated C-reactive protein .
ASO titre >200 Todd units
Anti-DNAse B test .
Throat culture-GABH streptococci .
7. ECG- prolonged
PRinterval, 2nd or
3rd degree blocks ,
ST depression or Tinversion.
8. 2D Echo cardiography - valve
edema ,mitral regurgitation, LA & LV dilatation,
pericardial effusion,
decreased contractility.
Management
1-Benzathine penicillin G, 0.6 to 1.2
million units intramuscularly, is given
to eradicate streptococci. This serves
as the first dose of penicillin
prophylaxis as well .
In patients allergic to penicillin,
erythromycin, 40 mg/kg per day in two
to four doses for 10 days, may be
substituted for penicillin.
2-Bed rest of varying duration is
recommended. The duration depends
on the type and severity of the
manifestations and may range from a
week (for isolated arthritis) to several
weeks for severe carditis . Bed rest is
followed by a period of indoor
ambulation of varying duration before
the child is allowed to return to school.
3-Therapy with anti-inflammatory agents
should be started as soon as acute
rheumatic fever has been diagnosed as
follows.
Prednisone, 2 mg/kg/24 hours in 4 divided
doses.
Aspirin, 100 mg/kg/d in4-6 divided doses.
4-Treatment of CHF includes the following
a. Complete bed rest with O2.
b. Prednisone for severe carditis of recent onset
c. Digoxin, used with caution, beginning with
half the usual recommended dose, because
certain patients with rheumatic carditis are
supersensitive to digitalis.
d. Furosemide,1 mg/kg every 6 to 12 hour.
5-Management of Sydenham's chorea
a. Reduce physical and emotional stress.
b. For severe cases, any of the following
drugs may be used: phenobarbital ,
haloperidol ,valproic acid,
chlorpromazine (Thorazine), diazepam
(Valium), or steroids.
Prevention (prophylaxis)
A patient who has had acute rheumatic
fever is susceptible to recurrent
rheumatic fever for the rest of his life.
Recommended Duration of
Prophylaxis for Rheumatic Fever
Category
Rheumatic
fever without
carditis
Rheumatic fever
with carditis but
without residual
heart disease
(no valvular
disease)
Rheumatic fever
with carditis and
residual heart
disease
(persistent
valvular
disease)
Durartion
At least for 5 yr
or until age 21 yr,
whichever is
longer
At least for 10 yr
or well into
adulthood,
whichever is
longer
At least 10 yr
since last episode
and at least until
age 40 yr;
sometimes
lifelong
prophylaxis
Cardiomyopathies
Primary myocardial disease, or
cardiomyopathy, is a disease of the
heart muscle itself, not associated with
congenital, valvular, or coronary heart
disease or systemic disorders.
Cardiomyopathy has been classified
into three types based on anatomic and
functional features: hypertrophic,
dilated (or congestive), and restrictive
Feature
Features of different types
Hypertrophic
Etiology Inherited (AD in about
50%) Sporadic (new
mutation ±)
Hemodynamic Diastolic dysfunction
(rigid ventricular walls
dysfunction
impede ventricular
filling)
Dilated
Restrictive
Pluricausal (e.g., Myocardial fibrosis,
toxic, metabolic, hypertrophy, or
infectious, alcohol, infiltration (amyloid,
doxorubicin)
hemochromatosis)
Systolic
contractile
dysfunction (↓
cardiac output, ↓
stroke volume, ↑
LVEDP)
Diastolic dysfunction
(with normal systolic
function) (abnormally
stiff LV with impaired
ventricular filling)
Feature
Symptoms
Hypertrophic
Infants, frequently
present with signs
of CHF.
Older children
may be
asymptomatic,
with sudden death
as the initial
presentation.
Dyspnea, fatigue,
chest pain,
syncope or nearsyncope, and
palpitations may
be present.
Dilated
signs and
symptoms of
inadequate
cardiac output and
CHF.
Restrictive
Exercise
intolerance,
weakness and
dyspnea, or chest
pain.
Feature
On examination
Hypertrophic
A murmur is heard
in more than 50%
of children.
Dilated
Features of CHF.
Rales may be
audible on
pulmonary
auscultation. Heart
sounds may be
muffled, and S3 is
often present.
Concurrent
infectious illness
may result in
circulatory
collapse and
shock in children
with dilated
cardiomyopathies.
Restrictive
Jugular venous
distention, gallop
rhythm, and a
systolic murmur of
AV valve
regurgitation may
be present.
Feature
Hypertrophic
Dilated
Restrictive
Echocardiography
Thickened LV (and
occasionally RV)
wall
Biventricular
dilatation and
EF%
Biatrial
enlargement
Electrocardiography
(LVH), ST-T
changes, and
abnormally deep Q
waves (owing to
septal hypertrophy)
Sinus tachycardia,
LVH, and ST-T
changes are the
most common
findings.
Atrial
hypertrophy. It
may show
atrial
fibrillation and
paroxysms of
supraventricul
ar tachycardia.
Chest x ray
Mild left ventricular
enlargement with a
globular-shaped
heart.
Generalized
cardiomegaly is
usually present,
with or without
signs of
pulmonary venous
hypertension or
pulmonary edema.
cardiomegaly,
pulmonary
venous
congestion,
and
occasional
pleural
effusion.
Feature
Hypertrophic
Dilated
Restrictive
Treatment
-β-Adrenoreceptor
blockers
-Calcium antagonists
-(Digitalis/catechols
,Diuretics and nitrates
are contraindicated).
-Vasodilator therapy
-Digitalis plus
diuretics
-β-Adrenoceptor
blockers (±)
Antiarrhythmics (±)
Cardiac
transplantation (±)
-Diuretics
-Anticoagulants (±)
-Corticosteroids
(±)
-Permanent
pacemaker for
advanced heart
block (±)
-Cardiac
transplantation (±)