RHEUMATIC FEVER AND

VALVULAR HEART DISEASE

Oleh :
RHEUMATIC FEVER
• Rheumatic fever (RF) is generally classified as
a connective tissue or collagen-vascular
disease
• It is an inflammatory reaction that causes
damage to collagen fibrils and to the ground
substance of connective tissue
• Involves multiple organs : primarily to the
heart, the joints, and the CNS
• Recurent attacks of RF may cause fibrosis of
heart valves, leading to chronic valvular heart
disease
 Epidemiology
• Peak incidence ages 5-15 years
• Rare before age 4 years and after age 40 years
• Incidence of RF and prevalence of rheumatic
heart disease (RHD) are variable
 Etiology and Pathogenesis
• Multiple factors
• Cross immune response between host and
streptococcal antigens

Streptococcal pharyngitis
↓
Abnormal reaction-autoimmunity disease
 Pathology
Pathological process :
• Exudative stage
• Proliferative stage : Aschoff nodule
(pathognomonic)
• Fibrosis and calcification (scar formation)
• Recurrent attacks of RF (rheumatic carditis,
valvulitis)  scar formation and deformity of
heart valves  chronic RHD

Valvular involvement :
• Mitral valve : 75%-80%
• Aortic valve : 30%
• Tricuspid & pulmonary valves : < 5%
VALVULAR HEART DISEASE
 MITRAL STENOSIS
LEFT ATRIAL OUTFLOW OBSTRUCTION
MITRAL OBSTRUCTION
 Mitral Stenosis
Rheumatic Valvular Heart Disease

• Rheumatic heart disease may causes mitral

stenosis
 Effect of Mitral Stenosis
On Heart
• Left atrium hypertrophies and dilates due to
↑pressure
• Atrial fibrillation and mural thrombosis
follow
• Left ventricles “protected” by stenotic mitral
valve
• LV usually normal in size and contour
 Effect of Mitral Stenosis
On Heart
• Pulmonary arterial pressure ↑
• Intimal and medial hypertrophy of pulmonary
arteries ↑pulmonary vascular resistance
• Right ventricle dilates from pressure overload
• Main pulmonary artery dilates  pulmonary valve
regurgitation
• Dilated RV Tricuspid regurgitation
• Volume overload  right atrium dilates
Right heart failure
 Effect of Mitral Stenosis
On Lung
• Pulmonary arterial hypertension develops
First passively
• Then muscular hypertrophy and hyperplasia
 increased pulmonary vascular resistance
• Chronic edema of alveolar walls  fibrosis
Pulmonary hemosiderin deposited in lungs
Pulmonary ossification may occur
 X-Ray Findings of MS
• Usually normal or slightly enlarged heart
• Double density of left atrial enlargement
• Enlarged atria do not produce cardiac
enlargement; only enlarged ventricles
• Straightening of left heart border or
convexity along left heart border  enlarged
atrial appendage
Only in rheumatic heart disease
 X-Ray Findings of MS
• Small aortic knob from decreased cardiac
output
• Rarely, right atrial enlargement from
tricuspid insufficiency
• Calcification of valve--not annulus--seen best
on lateral film and at angio
• Calcification of left atrial wall and pulmonary
arteries (rare)
MITRAL REGURGITATION
 Mitral Regurgitation
Causes
• Thickening of valve leaflets from rheumatic disease
• Rupture of the chordae
Posterior leaflet more often-Trauma, Marfan’s
• Papillary muscle rupture or dysfunction
Acute myocardial infarction
• LV enlargement  dilatation of mitral annulus
Any cause of LV enlargement
• LV aneurysm  valvular dysfunction
Acute myocardial infarction
 X-Ray Findings of MR
• In acute MR
Pulmonary edema
Heart is not enlarged
• In chronic MR
LA and LV are markedly enlarged
Volume overload
Pulmonary vasculature is usually normal
LA volume but not pressure is elevated
AORTIC STENOSIS
 Aortic Stenosis
Causes

• Most often as result of degeneration of

bicuspid aortic valve
• Degeneration of tricuspid aortic valve and
rheumatic heart disease in tricuspid valve
 Aortic Stenosis

• Congenital and Acquired

• Locations :
Supravalvular
Valvular
Subvalvular
 X-Ray Findings of Aortic Stenosis
• Depends on age patient/severity of disease
• In infants, AS  CHF/pulmonary edema
• In adults :
Normal heart size, until cardiac muscle
decompensates
Enlarged ascending aorta due to post-stenotic
dilatation due to turbulent flow
Normal pulmonary vasculature
• Thickening or calcification of a bicuspid / tricuspid
valve
AORTIC REGURGITATION
(AORTIC INSUFFICIENCY)
 Aortic Regurgitation
Causes
• Rheumatic heart disease
• Marfan’s
• Luetic aortitis
• Ehlers-Danlos syndrome
• Endocarditis
• Aortic dissection
 X-Ray Findings of
Aortic Regurgitation
• X-ray hallmarks are
Left ventricular enlargement
Enlargement of entire aorta
• Cine MRI (gradient refocused MRI)
“White blood” technique
Signal loss coming from Ao valve into LV
during diastole
• Color Doppler is also diagnostic
PULMONIC STENOSIS
Pulmonic Stenosis
Types

Subvalvular
Valvular
Supravalvular
 X-Ray Findings of
Pulmonic Stenosis

• Enlarged main pulmonary artery

• Enlarged left pulmonary artery (jet effect)
• Normal to decreased peripheral pulmonary
vasculature
• Rare calcification of pulmonary valve in older
adults
TERIMA KASIH