Dr.
Musnidarti, SpJP, FIHA
Acute Rheumatic Fever
Definition
An acute, immunologically mediated, multi-system
inflammatory disease.
3% of pts with group A streptococcal pharyngitis.
Peak incidence: ages of 5-15 years.
Incidence : ↓ over the past 30 years
It affects the heart during its acute phase acute
rheumatic carditis after many years may cause
chronic valvular deformities
Acute Rheumatic Fever…
Etiology
Rheumatic fever follows usually a group A beta-
hemolytic streptococcal pharyngitis. The evidence
for this association include:
› Epidemiological studies and patient history : show that
streptococcal pharyngitis are followed by cases of
rheumatic fever.
› Serology: patients have elevated levels of antibodies to
streptococcal enzymes such as streptolysin O and
DNAse B.
Always remember blood cultures of patients with rheumatic
fever are sterile
Acute Rheumatic Fever…
Pathology
Inflammatory
infiltrates occur in a
wide range of tissues:
synovium, joints,
skin, and heart.
Focal fibrinoid
necrosis which
provokes
inflammatory
response
Fibrosis is common
especially in cardiac
tissues.
Acute Rheumatic Carditis (Pancarditis):
Pathology
Myocardium
• Scattered Aschoff bodies lie in close proximity to a small vessel.
• Diffuse interstitial inflammatory infiltrates (may lead to generalized
dilation of the cardiac chambers).
Endocardium
• Common and may affect any valve, mostly mitral and aortic valves.
• Valves are edematous and thickened with foci of fibrinoid necrosis.
(Aschoff nodules uncommon).
• Formation of small vegetations “fibrinous clots” along the lines of
valve closure (Verrucous Endocarditis).
Pericardial involvement
• Fibrinous pericarditis, sometime associated with serous or
serosanguinous pericardial effusion.
Acute Rheumatic Carditis (Pancarditis):
Clinical Manifestations
• Symptoms:
– Pericardial friction rubs,
– Weak heart sounds,
– Tachycardia (rapid beating) and
– Arrhythmias.
• In severe cases: myocarditis cardiac dilation
functional mitral valve insufficiency or even
congestive heart failure.
Acute Rheumatic Heart Disease
Pathogenesis and Key Morphologic Changes
Diagnosis
• Modified Jones criteria
– Two of the major criteria, or
– One major criteria plus two
minor criteria, are present along
with evidence of streptococcal
infection.
Major criteria
Migratory polyarthritis : a temporary migrating
inflammation of the large joints,
Carditis: inflammatory changes in all three layers of the
heart.
Subcutaneous nodules: painless, firm, appear on the back
of the wrist, the outside elbow, and the front of the knees.
Erytema marginatum : a long lasting rash that begins on
the trunk or arms as macula, never starts on the face and it
is made worse with heat.
Syndenham’s Chorea : rapid movements without purpose
of the face and arms.
Minor criteria
Fever
Arthralgia : Joint pain without swelling
Raised ESR or CRP
Leucocytosis
ECG showing features of heart block, such as a
prolonged PR interval
Supporting evidence of Streptococcal infection:
elevated or rising ASTO or DNAase.
Previous episode of rheumatic fever or inactive
heart disease
Acute Rheumatic Fever…
Prevention Strategies
• The best way to prevent RHD :
– immediate medical attention to a strep throat
and not let it progress to rheumatic fever.
• The Nurses also have a role in prevention,
primarily in screening school-aged children
for sore throats that may be caused by
Group A streptococci(especially Group A β
Hemolytic Streptococcus pyogenes).
• Persons who have previously contracted
rheumatic fever are often given continuous
(daily or monthly) antibiotic treatments,
possibly for life, to prevent future attacks of
rheumatic fever and lower the risk of heart
damage.
Prevention Strategies..
Primary Prevention.
› Effective eradication with antibiotics
› should be treated as early as possible in the
suppurative phase.
› Penicillin orally for a full 10-day course, or
benzathine penicillin is injected
Prevention Strategies..
Secondary Prevention
• Continuous administration of benzathine
penicillin every 4 weeks.
• Parenteral therapy is preferable, better
compliance
• Duration : the minimum recommendations
– 5 years or until age 18 in the absence of carditis,
– 10 years or until age 25 for pts with mild or apparently healed
carditis, and
– life-long for pts with moderate or severe carditis.
Antibiotics Therapy for Acute RF and
long-term prophilaxis
Modified from Rheumatic fever and
rheumatic heart disease. WHO Rep Ser
923:1, 2004
Acute Rheumatic Fever…
Complication
• As many as 39% of patients with acute
rheumatic fever may develop varying
degrees of pancarditis with associated
– valve insufficiency,
– heart failure,
– pericarditis,
– death.
Chronic Rheumatic Heart Disease
Characterized by irreversible deformity of
one or more cardiac valves.
Mitral valve 95%
Combined oartic & 25%
mitral valve disease
Aortic valve Rare
Pulmonary & Extrm rare
Tricuspid valves
Pathological changes
Chronic scarring and calcification of the valve
leaflets stiff and thickened structure
which may lead to:
› stenotic : valve fails to open completely, so
impairing forward blood flow.
› regurgitation : valve fails to close
completely, due to cusp abnormality or
disease of supporting structures, so allowing
reverse flow.
Shortening and fusion of the chordae
tendineae.
Valvular Heart Disease
MITRAL STENOSIS
Normal mitral valve
Stenotic mitral valve
Thickening of cusps
Fusion of chordae
Mitral Stenosis..
Etiology
• Primarily a result of rheumatic fever (~ 99%)
• Scarring & fusion of valve apparatus
• Pure or predominant MS occurs in
approximately 40% of all patients with
rheumatic heart disease
• Two-thirds of all patients with MS are female.
Mitral Stenosis…
Pathophysiology
• Normal valve area: 4-6 cm2
• Mild mitral stenosis:
– MVA 1.5-2.5 cm2
– Minimal symptoms
• Mod mitral stenosis
– MVA 1.0-1.5 cm2 usually
does not produce symptoms at
rest
• Severe mitral stenosis
– MVA < 1.0 cm2
Pathophysiology
Right Heart Failure: Pulmonary HTN
Hepatic Congestion Pulmonary Congestion
JVD LA Enlargement
Tricuspid Regurgitation Atrial Fib
RA Enlargement LA Thrombi
LA Pressure
RV Pressure Overload
RVH
RV Failure LV Filling
Mitral Stenosis..
Symptoms
• Fatigue • Afib
• Systemic embolism
• Palpitations
• Pulmonary infection
• Cough • Hemoptysis
• SOB • Right sided failure
– Hepatic Congestion
• Left sided failure – Edema
– Orthopnea • Worsened by conditions that
– PND cardiac output.
– Exertion,fever, anemia, tachycardia,
• Palpitation Afib, intercourse, pregnancy,
thyrotoxicosis
Mitral Stenosis..
DIAGNOSIS
1. Clinical examination
– Finds a murmur on auscultating (diastolic
murmur, opening snap).
2. Pulmonary x-ray
3. Electrocardiogram : LAE, RVH, RAD
AFIB,
4. Echocardiography
Mitral Stenosis..
X-Ray Findings
Mitral Heart
Configuration
•Small aorta
•Main pulm-artery
segment is prominent
•Convexity from
enlarged LAA
•Double density
•RVH
•Inverted comma sign
(+/-)
Mitral Stenosis..
Echocardiography
MS
AO
LV
LA
Mitral Stenosis..
Complications
• Atrial dysrrhythmias
• Systemic embolization (10-25%)
• Congestive heart failure
• Pulmonary infarcts (result of severe CHF)
• Hemoptysis
– Massive: ruptured bronchial veins (pulm HTN)
– Streaking/pink froth: pulmonary edema, or infection
• Endocarditis
• Pulmonary infections
Mitral Stenosis..
Therapy
• Medical
• Balloon valvuloplasty
– Effective long term improvement
• Surgical
– Mitral commissurotomy
– Mitral Valve Replacement
• Mechanical
• Bioprosthetic
Mitral valve, rheumatic mitral stenosis
The mitral valve demonstrates the typical "fish mouth" shape with
chronic rheumatic scarring.
Mitral stenosis with diffuse fibrous thickening and distortion of the
valve leaflets, commissural fusion (arrow)
MITRAL
REGURGITATION
Mitral Regurgitation..
Etiology
• Valvular-leaflets • Annulus
– Myxomatous MV – Calcification, IE (abcess)
Disease • Papillary Muscles
– Rheumatic – CAD (Ischemia,
– Endocarditis Infarction, Rupture)
– Congenital-clefts – HCM
• Chordae – Infiltrative disorders
– Fused/inflammatory • Functional regurgitation
– Torn/trauma • Trauma
– Degenerative
– IE
Mitral Regurgitation..
Etiology:Surgical series
• MVP(20-70%)
• Ischemia (13-40%)
• RHD (3-40%)
• Infectious endocarditis(10-12%)
Mitral Regurgitation..
Pathophysiology
• Chronic LV volume overload -»
compensatory LVE initially maintaining
cardiac output
• Decompensation (increased LV wall
tension) -»CHF
• LVE – » annulus dilation – » increased MR
• Backflow – » LAE, Afib, Pulmonary HTN
Mitral Regurgitation..
Symptoms
• Similar to MS
• Dyspnea, Orthopnea, PND
• Fatigue
• Pulmonary HTN, right sided failure
• Hemoptysis
• Systemic embolization in A Fib
Mitral Regurgitation..
DIAGNOSIS
1. Clinical examination
– Finds a murmur on auscultating (holosystolic
murmur).
2. Pulmonary x-ray
3. Electrocardiogram : LAE, Afib, LVH
(With severe MR), RVH, Combined
hypertrophy .
4. Echocardiography
Mitral Regurgitation..
X-Ray Findings
• In acute MR
– Pulmonary edema
– Heart is not enlarged
• In chronic MR
– LA and LV are markedly
enlarged
– Pulmonary vasculature is
usually normal
Mitral Valve Surgery
• Only effective treatment is valve
repair/replacement
• Optimal timing determined:
– Presence/absence of symptoms
– Functional state of ventricle
– Feasability of valve repair
– Presence of Afib/PHTN
– Preference/expectations of patient
AORTA STENOSIS
Aorta stenosis..
Etiology
Young patient Middle aged Old patient
congenital patient (4&5th
decades)
Bicuspid Bicuspid or Degenerative
2% population Rheumatic (6,7,8th
disease decades)
3:1 male:female
Co-existing
coarctation 6%
of patients
Aorta stenosis..
Etiology
• Congenital bicuspid valve is the most common
abnormality
• Rheumatic heart disease and degeneration with
calcification are found as well
Aorta stenosis..
Severity of Stenosis
• Normal aortic valve area 2.5-3.5 cm2
• Mild stenosis 1.5-2.5 cm2
• Moderate stenosis 1.0-1.5 cm2
• Severe stenosis < 1.0 cm2
Aorta stenosis..
Symptoms
• Cardinal Symptoms Other signs of LV
– Chest pain (angina) failure
• Reduced coronary flow
reserve •Diastolic & systolic
• Increased demand-high dysfunction
afterload
– Syncope/Dizziness
(exertional pre-syncope)
– Dyspnea on exertion & rest
– Impaired exercise tolerance
Aorta stenosis..
DIAGNOSIS
1. Clinical examination
– Finds a murmur on auscultating (mid -systolic
murmur)
2. Pulmonary x-ray
3. Electrocardiogram : LVH
4. Exercise test
5. Echocardiography
Aorta stenosis..
X-Ray Findings
• Depends on age patient/severity of disease
• In adults
– Normal heart size
• Until cardiac muscle decompensates
– Enlarged ascending aorta : post-stenotic
dilatation
– Normal pulmonary vasculature
Aortic Stenosis
Locations
• Supravalvular
• Valvular
• Subvalvular
Bicuspid Aortic Valve
© Continuing Medical
Implementation
…...bridging the care gap
Aorta stenosis..
Therapy
• Medical care
• Intervensional
– Percutaneous balloon valvuloplasty
– Surgical
Surgically removed specimen of rheumatic aortic stenosis
demonstrating thickening and distortion of the cusps with
commissural fusion (rigid triangular channel)
Prosthetic Valves
• MECHANICAL • BIO-PROSTHETIC
– Durable – Not durable
– Large orifice – Smaller
orifice/functional
– High stenosis
thromboembolic – Low thromboembolic
potential potential
– Best in Left Side – Consider in elderly
– Chronic warfarin – Best in tricuspid
therapy position
Disc Valve
Bio-prosthetic Valve
AORTA
REGURGITATION
Aorta regurgitation..
Etiology
• Rheumatic heart disease
• Marfan’s
• Luetic aortitis
• Ehlers-Danlos syndrome
• Endocarditis
• Aortic dissection
Aorta regurgitation..
RHD
• Thickened cusps
• May have commissural fusion
– In degenerative Ao regurg, no commissural
fusion
• Regurgitant jet is usually central
– In degenerative, usually not discrete jet
Peripheral physical signs of AR
• Related to the high pulse pressure and the rapid
decrease in BP during diastole
– large-volume, 'collapsing' pulse also known as:
• Watson's water hammer pulse
• Corrigan's pulse (rapid upstroke and collapse of the carotid
artery pulse)
– de Musset's sign (head nodding the heart beat)
– Quincke's sign (pulsation of the capillary bed in the nail)
– Traube's sign (syst &diast murmurs 'pistol shots’
– Duroziez's sign (a double sound heard over the femoral
artery when it is compressed distally)
Aorta regurgitation..
DIAGNOSIS
1. Clinical examination
– Finds a murmur on auscultating (diastolic
cressendo-decressendo murmur).
– Peripheral physical signs (Chronic )
2. Pulmonary x-ray
3. Electrocardiogram
4. Echocardiography
Aorta regurgitation..
X-ray Findings
• X-ray hallmarks
are
– LV enlargement
– Enlargement of
entire aorta
Care of Patients who have
undergone heart valve surgery
The outpatient treatment has three main aspects:
1. oral anticoagulation,
– all patients with a mechanical heart valve
prosthesis (artificial valve) must be orally
anticoagulated for life.
2. endocarditis prevention, for any dental
procedure for the rest of their lives, regardless
of whether a manifest infection is present.
3. echocardiographic follow-up.
Infective Endocarditis
Infective Endocarditis..
• Inflammation of the inner layer of the heart,
the endocardium.
• It usually involves the heart valves (native or
prosthetic valves); Congenital heart disease
• Endocarditis is characterized by a prototypic
lesion, the vegetation,
• Staphylococcus aureus most common
cause
Duke Criteria for IE
MAJOR CRITERIA
• Positive blood culture for IE
– Typical m.o consistent with IE from 2 separate blood
culture or
– M.o consistent with IE from persistently positive blood
cultures defined as:
• 2 positive cultures of blood samples drawn >12 hours apart,
or
• all of 3 or a majority of 4 separate cultures of blood (with
first and last sample drawn 1 hour apart)
• Evidence of endocardial involvement
– Positive echocardiogram for IE
Minor criteria
• Predisposition: intravenous drug use
• Fever: temperature > 38.0° C (100.4° F)
• Vascular phenomena: major arterial emboli, septic
pulmonary infarcts, mycotic aneurysm, intracranial hemorrhage,
conjunctival hemorrhages, and Janeway lesions
• Immunologic phenomena: glomerulonephritis,
Osler's nodes, and rheumatoid factor
• Microbiological evidence: blood culture (+) but
does not meet a major criterion as noted above
• Echocardiographic findings: consistent with IE
but do not meet a major criterion as noted above
Infective Endocarditis..
Clinical criteria for IE
• Two major criteria, or
• One major and three minor criteria,
or
• •Five minor criteria
Infective Endocarditis..
Treatment
• Medical Care
– The major goals of therapy for IE are to
eradicate the infectious agent from the
thrombus and to address the complications of
valvular infection. The latter includes both the
intracardiac and extracardiac consequences of
IE.
• Surgical Care.
Infective Endocarditis..
Complications
• Monitor patients for the development of the
following complications:
– Valvular dysfunction, usually insufficiency of the
mitral or aortic valves
– Myocardial or septal abscesses
– Congestive heart failure
– Metastatic infection
– Embolic phenomenon
– Organ dysfunction resulting from immunological
processes
Infective Endocarditis..
Prevention
• Approximately 15-25% of cases of IE are a
consequence of invasive procedures that
produce a significant bacteremia.
• Maintaining good oral hygiene is probably
more effective in the overall prevention of
valvular infection because gingivitis is the
most common source of spontaneous
bacteremias.
TEE
