INTRODUCTION
RF and RHD remain significant
EPIDEMIOLOGY
Global burden (about 2.4 mil) caused by rheumatic
developing countries
A beta-hemolytic streptococcal
infection
completely
FACTORS
Host
Organism
Environment
HOST FACTORS
ARF is an inherited characteristic
not hereditary, but predisposition to acquire infection is
genetic
protection
receptors
ORGANISM
ETIOLOGY?
ETIOLOGY?
VIRULENCE
FACTOR
Streptolysin O
Streptolysin S
DESCRIPTION
VIRULENCE
FACTOR
Streptokinase
Hyaluronidase
DESCRIPTION
Enzymatically activates
plasminogen, a proteolytic
enzyme, into plasmin,
which in turn digests fibrin
and other proteins
facilitate the spread of the
bacteria through tissues by
breaking down hyaluronic
acid, an important
component of connective
tissue.
VIRULENCE FACTOR
Streptodornase
C5a Peptidase
DESCRIPTION
AKA DNases, which protect the
bacteria from being trapped in
neutrophil extracellular traps
(NETs) by digesting the NETs'
web of DNA
cleaves a potent neutrophil
chemotaxin called C5a, which
is produced by the
complement system which
minimizes the influx of
neutrophils early in infection as
the bacteria are attempting to
colonize the host's tissue.
VIRULENCE
FACTOR
Streptococcal
chemokine
protease
DESCRIPTION
Prevents the migration of
neutrophils to the
spreading infection. ScpC
degrades the chemokine
IL-8, which would otherwise
attract neutrophils to the
site of infection.
M PROTEIN (VIRULENCE)
M-protein is one of the best-
defined determinants of
bacterial virulence.
ENVIRONMENTAL FACTORS
Poor living conditions
Overcrowding
Poor access to health care
Seasonal variations
PATHOPHYSIOLOGY
develops following pharyngitis with group A beta-
PATHOPHYSIOLOGY
0.3-3% of cases, leads to rheumatic fever several weeks
PATHOPHYSIOLOGY
Precise mechanism is unknown
Two theories suggested
THEORIES
1. Cytotoxicity theory - GABHS produces several enzymes
DIAGNOSIS
No clinical or laboratory finding pathognomonic of RF
No definitive test
DIAGNOSIS
Evidence of preceeding GABHS +
2 major criteria or 1 major + 2minor manifestations
HEART INVOLVEMENT
Up to 60% of patients with ARF progress to RHD
Endocardium, pericardium, or myocardium may be
affected
(secondary)
HEART
SOUNDS
Mitral Stenosis
Mitral regurgitation
Aortic regurgitation
Softening of S1
Prolonged P-R interval
REMEMBER:
Carditis is the single most important prognostic factor in
ECHOCARDIOGRAPHY
is an imaging technique, currently it is a key component in the diagnosis of heart disease
technique includes transthoracic, transesophageal and intracardiac echocardiography
Three-dimensional and even four-dimensional echocardiography have also been
developed
MORPHOLOGY
During acute RF, focal inflammatory lesions are
VERRUCAE
Inflammation of the
Subendocardial lesions,
perhaps exacerbated by
regurgitant jets, can
induce irregular
thickenings called
MacCallum plaques,
usually in the left atrium.
JOINT INVOLVEMENT
Arthritis (75%)
Affects the large jointsknees, anklesand is
asymmetric.
salicylates)
CHOREA
536%
prolonged latent period
emotional lability
uncoordinated movements
muscular weakness
SIGNS
pronator (tendency to pronate the hands when extending
changes in handwriting
SKIN MANIFESTATION
Erythema Marginatum (<3%)
-rare
Subcutaneous nodules
painless, small (0.52 cm), mobile
lumps beneath the skin overlying
bony prominences (hands, feet,
elbows, occiput, and occasionally
the vertebrae.
delayed manifestation, appearing
23 weeks after the onset
last for just a few days up to 3 weeks
commonly associated with carditis.
REMEMBER
Subcutaneous nodules are almost always associated
MANAGEMENT
PRIMARY PREVENTION
defined as the adequate antibiotic therapy of group A
URT infection
therapy is intermittent
SECONDARY PREVENTION
defined as continuous administration of specific antibiotics
patients with a previous attack of RF, or a well-documented
circulating emm types in the United States, Canada and Europe as well
as developing countries.
volunteers.
RHEUMATIC HEART
DISEASE
Recurrences of ARF cause
rheumatic fever
MORPHOLOGY
PHYSICAL ASSESSMENT
INSPECTION: heaves
PERCUSSION
PALPATION: pulsation, thrill, lifts
AUSCULATION: S1, S2, murmur
AUSCULTATION
AUSCULTATION
ASSESSMENT OF MURMUR
Is there a murmur?
When is it heard?
Where is it located?
Does it radiate? Where?
What is the shape?
What is the pitch?
What is the intensity/grade?
TIMING
Systole
S1---------- S2---------------S1
2 components
Rapid Ejection
Slow Ejection
Diastole
S1---------- S2---------------S1
3 components
LOCATION/RADIATION
SHAPE
PITCH
HIGH - High Pressure Gradients (VSD)
LOW Large Volume across low pressure gradients
(Mitral Stenosis)
(Aortic Stenosis)
QUALITY
Subjective
Blowing?
Musical?
Rumbling?
Harsh?
Machine Like?
RESPONSE TO MANEUVERS
INTENSITY
MITRAL INSUFFICIENCY
Loss of valvular substance
shortening and thickening of the chordae tendineae
MITRAL INSUFFICIENCY
Spontaneous improvement usually occurs with time
Patient is asymptomatic (Quiet Precordium)
More than half of patients with acute mitral
MITRAL INSUFFICIENCY
High-pitched holosystolic murmur at
the apex that radiates to the axilla
Heart is enlarged, with a heaving apical
left ventricular impulse and often an
apical systolic thrill
2nd heart sound may be accentuated if
pulmonary hypertension is present
3rd heart sound is generally prominent
MITRAL INSUFFICIENCY
Short mid-diastolic rumbling murmur is
caused by increased blood flow across
the mitral valve as a result of insuff.
ECG: Prominent Bifid P waves ; signs
of LVH and RVH if hypertension is
present
MITRAL INSUFFICIENCY
Mild - prophylaxis against RF
Afterload-reducing agents (ACE inhibitors or
MITRAL STENOSIS
Results from fibrosis of the mitral ring,
fully established
MITRAL STENOSIS
Mild - asymptomatic
Severe - exercise intolerance and
dyspnea;
Critical - orthopnea, paroxysmal
nocturnal dyspnea, overt pulmonary
edema, atrial arrhythmias.
pulmonary hypertension/right
ventricular dilatation - functional
tricuspid insufficiency, hepatomegaly,
ascites, and edema
MITRAL STENOSIS
Hemoptysis caused by rupture of bronchial or
MITRAL STENOSIS
Parasternal right ventricular lift
Loud 1st heart sound, an opening snap of the mitral
MITRAL STENOSIS
A holosystolic murmur sec to tricuspid
insuff. may be audible
Pulmonic component of S2 is
accentuated due to presence of pul.
HPN
An early diastolic murmur may be
caused by associated aortic insuff. or
pulmonary valvular insuff sec. to pulm.
HPN
MITRAL STENOSIS
ECG
1. Severe: Notched P waves and Varying
degrees of RVH
2. Atrial Fibrillation is a common late
manifestation
MITRAL STENOSIS
Symptomatic
Surgical valvotomy or balloon catheter
mitral valvuloplasty
Valve replacement is avoided unless
absolutely necessary
Balloon valvuloplasty is indicated for
symptomatic, stenotic, pliable,
noncalcified valve of patients without
atrial arythmias or thrombi
AORTIC INSUFFICIENCY
Sclerosis of the aortic valve results in distortion
AORTIC INSUFFICIENCY
Symptoms are unsual except in severe
Palpitations, sweating and heat intolerance
Dyspnea on exertion
AORTIC INSUFFICIENCY
Wide pulse pressure, bounding peripheral pulses
Left ventricular apical heave and diastolic thrill
AORTIC INSUFFICIENCY
Typical murmur begins immediately with the 2nd
AORTIC
INSUFFICIENCY
Aortic
Systolic Ejection Murmur is
frequent because of Increased Stroke
Volume
An apical presystolic murmur (Austin
Flint Murmur) resembling that of
mitral stenosis is sometimes heard and is
a result of the large regurgitant aortic
flow in diastole preventing the mitral
valve from opening fully
Sign
Becker Sign
Description
Visible systolic pulsations of the retinal
arterioles
Corrigan pulse ("water- Abrupt distention and quick collapse on
hammer" pulse)
palpation of the peripheral arterial pulse
de Musset sign
Hill sign
Duroziez sign
Mller sign
Quincke sign
AORTIC
INSUFFICIENCY
ECG:
LVH
Strain with prominent P waves
AORTIC
INSUFFICIENCY
does
not regress; combined lesions
during the episode of acute rheumatic
fever may have only aortic involvement
1-2 yr later
afterload reducers and prophylaxis
against recurrence of acute rheumatic
fever
surgical intervention before
complications
cases.