RHEUMATIC

HEART
DISEASE
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DEFINITION

 Rheumatic fever is an acute inflammatory illness that occurs after

Group A Streptococcal (GAS) pharyngitis

 Rheumatic heart disease is the development of inflammatory

changes to cardiac valves and myocardium leading to pathological
changes of the cardiac valves, especially the mitral and aortic
valves leading initially to regurgitation and potentially in the
subsequent months or years to stenosis of affected valves

Heart Diseases in Children A Pediatrician’s Guide. Sawsan Mokhtar M. Awad and Daniel E. Felten. Springer, 2011.
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ETIOLOGY

 Group A beta-hemolytic Streptococcu

s

 A gram-positive coccus
 Group A streptococci elaborate the
cytolytic toxins streptolysins S and O
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PREVALENCE

Rheumatic heart disease: Not gone, but almost forgotten

http://www.theheart.org/article/746133
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PATHOPHYSIOLOGY

 Group A, beta-hemolytic streptococcal infections of the

pharynx stimulate T-cell and B-cell lymphocytes to produce
antibodies presumably against some antigenic component of the
bacteria that cross-react with an antigen on myocytes or cardiac
valve tissue
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PATHOPHYSIOLOGY

 Cross-reactive antibodies bind to cardiac tissue facilitating infiltration of

streptococcal-primed CD4+ T cells, which then trigger an autoimmune
reaction releasing inflammatory cytokines (including TNF-alpha and IFN-
gamma)
 Because few IL-4–producing cells are present in valvular tissue,
inflammation persists, leading to valvular lesions
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 8 HISTORY & CLINICAL MANIFESTATIONS

 After confirming antecedent rheumatic fever (the modified Jones

criteria)

 The presence of 2 major or 1 major and 2

minor criteria for the diagnosis of rheumatic
fever
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MAJOR CRITERIA
The major diagnostic criteria include
 Carditis (50%),
 Polyarthritis (70-75%),
 Chorea (10-30%),
 Subcutaneous nodules (0-8%), and
 Erythema marginatum (5-13%)
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MINOR CRITERIA

The minor diagnostic criteria include

 Fever,
 Arthralgia,
 Prolonged PR interval on ECG,
 Elevated acute phase reactants (increased erythrocyte
sedimentation rate [ESR]),
 Presence of C-reactive protein, and
 Leukocytosis
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 Additional evidence of previous group A streptococcal pharyngitis is

required to diagnose rheumatic fever.
 One of the following must be present:
 Positive throat culture or rapid streptococcal antigen test result
 Elevated or rising streptococcal antibody titer
 History of previous rheumatic fever or rheumatic heart disease

 These criteria are not absolute; the diagnosis of rheumatic fever can be
made in a patient with chorea alone if the patient has had documented
group A streptococcal pharyngitis.
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 M I T R A L VA LV E S T E N O S I S I S A VA LV U L A R
H E A RT D I S E A S E C H A R A C T E R I Z E D B Y T H E
13 N A R R O W I N G O F T H E O R I F I C E O F T H E M I T R A L VA LV E
O F T H E H E A RT ( 6 5 - 7 0 % O F PAT I E N T S )
 A O RT I C VA LV E S T E N O S I S ( A S ) I S A T Y P E O F
VA LV U L A R H E A RT D I S E A S E C H A R A C T E R I Z E D B Y A N
14 A B N O R M A L N A R R O W I N G O F T H E A O RT I C VA LV E
O P E N I N G ( 2 5 % O F PAT I E N T S )
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 Cardiac manifestations of acute rheumatic fever

 Pancarditis

 Cardiac manifestations of chronic rheumatic heart disease

 Valve deformities,
 Thromboembolism,
 Cardiac hemolytic anemia, and
 Atrial arrhythmias
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LABORATORIUM
EXAMINATION

 Throat culture
 Rapid antigen detection test
 Antistreptococcal antibodies
 Acute phase reactants
 Heart reactive antibodies
 Rapid detection test for D8/17
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CHEST
RADIOGRAPHY
 Cardiomegaly and increased broncho-vascular markings reflecting
pulmonary venous congestion may be noted
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DOPPLER-ECHOCARDIOGRAM

 From acute rheumatic valvulitis are annular dilatation, elongation

of the chordae to the anterior leaflet, and a posterolaterally
directed mitral regurgitation jet
 Chronic rheumatic heart disease, the leaflets of affected valves
become diffusely thickened, with fusion of the commissures and
chordae tendineae
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CARDIAC CATHETERIZATION

 Cardiac catheterization is seldom needed in the

diagnosis of cases of rheumatic heart disease
 To evaluate mitral and aortic valve disease and to
balloon stenotic mitral valves.
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HISTOLOGIC FINDINGS

 Aschoff bodies (perivascular foci of eosinophilic collagen

surrounded by lymphocytes, plasma cells, and macrophages) are
found in the pericardium, perivascular regions of the myocardium,
and endocardium
Group A Streptococcal Infections Microscopic appearance of an Aschoff body in rheumatic heart disease.
21

Red Book Online Visual Library, 2009. Image 124_34. Available at:
http://aapredbook.aappublications.org/visual.
Prevention and management strategies for acute rheumatic fever and rheumatic heart
disease as they relate to pathogenesis

Steer, A. C. and Carapetis, J. R. (2009) Prevention and treatment of rheumatic heart disease
in the developing world
Nat. Rev. Cardiol. doi:10.1038/nrcardio.2009.162
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 23 SURGICAL CARE

 Forty percent of patients with acute rheumatic heart disease

subsequently develop mitral stenosis as adults
 In patients with critical stenosis, mitral valvulotomy, percutaneous
balloon valvuloplasty, or mitral valve replacement may be
indicated
 Due to high rates of recurrent symptoms after annuloplasty or other
repair procedures, valve replacement appears to be the preferred
surgical option
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RHD & ORAL
SURGERY

 It is important to realize that transient bacteremia, which in healthy

patients is nonthreatening and which may develop after invasive
surgical procedures, is considered especially dangerous for patients
belonging to this category
 In this case, the endocardium generally presents great sensitivity to
bacterial infection, and, as a result, any invasive procedure in the
oral cavity without the use of antibiotics results in greater risk of
bacterial endocarditis
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The preventive measures that are recommended are:

 Premedication before the surgical procedure
 Avoidance of vasoconstrictors (or maximum concentration 1:100,000)
 Small amounts of vasoconstrictors in local anesthetic with particular
importance of aspiration

Oral Surgery. Fragiskos FD. Springer, 2007