A 52-year-old female presented with fatigue, shortness of breath, and occasional palpitations over the past 6 months. She reported a serious childhood illness with fever, rash, joint pain, and difficulty controlling movements that resolved after 1 month. On examination, she had a loud S1, opening snap, and diastolic rumble. Chest X-ray and echocardiogram were ordered to evaluate for likely rheumatic heart disease given her history of childhood illness and current cardiac examination findings.
2. A 52-year-old female developed fatigue
and shortness of breath that have been
worsening over 6 months. She also
complained of occasional palpitations.
She described a serious illness she had
as a child, with fever, rash, joint pain, and
difficulty controlling her movements.
She recovered after a month.
Cardiac examination reveals a loud S1,
an opening snap, and a diastolic rumble.
What do you want to examine more?
What test do you want to order?
3. LEARNING OBJECTIVES
•Understanding the pathophysiology of
Rheumatic fever
•To know about the epidemiology of the
disease
•Describe the natural history and pathology of
Acute rheumatic fever and rheumatic heart
disease
•To know about the clinical features, minor
and major criteria for the diagnosis of
rheumatic fever.
•To know morphological features of the
disease.
•To know pathology of chronic rheumatic heart
disease and Rheumatic pericarditis.
•Enlist components of vegetation.
10. - It is a preventable immune mediated and multisystemic
inflammatory disease, triggered by group A B-hemolytic
streptococcus pharyngitis, in genetically predisposed
individuals.
- It occurs within 1-4 weeks of untreated infection.
- Common in F>M and in age between 5-15 years of life.
- It may spread to affect: heart, joint, connective tissue, and
brain.
11.
12. - The body immune system produce antibody against
Streptococcus bacteria that cross react with human
body tissue.
- It occurs due to antigenic similarity between bacteria
M-protein and body tissue.
- There is no direct invasion of the bacteria to the body
tissue.
Pathogenesis
13.
14.
15. - Most common complication of
Rheumatic heart disease is valvular
fibrosis and defects.
- VHD occurs secondary to
rheumatic pancarditis
- Mitral or aortic defect are more affected such as mitral
stenosis. Left ventricle is always not affected.
- Vulvar stenosis can complicate to arrhythmia and heart
failure.
16.
17. Rheumatic pancarditis
results in:
a- Chronic granulomatous
myocarditis
b- Verrucous endocarditis
c- Fibrinous pericarditis
and Serosanguinous
pericardial effusion
What is the clinical
presentation ?
18. What do you seen in Pathology of
myocardium in mitral vulvitis ?
24. - It is irreversible deformity of heart valves mainly affecting mitral valve
(90%)
- If the mitral valve affected, it causes stenosis more than regurgitation
- It occurs due to calcification and scarring
- It results in shortening and fusion of chordae tendineae
- Complications: arrythemia, CHF, LVH and dilatation,
thromboembolism and infective endocarditis.
25. Trick notes:
- Treating streptococcus pharyngiotonsilitis will prevent Rheumatic
disease.
- Child present with fever, rash and cardiac symptoms alert acute
rheumatic fever
- Young or middle age patient presents with vulvar disease alert VHD
secondary to chronic RHD
- Treating VHD prevents complications
29. - Valve abnormalities could be
congenital or acquired.
- The most common abnormalities
are acquired stenosis of the mitral
and aortic valves.
- Valve abnormalities produce
abnormal heart sounds called
murmurs
38. Causes:
Dysfunction of any mitral valve
leaflets components:
1- Mitral annulus due to:
a- Repeated MI
b- DCM
c- CRF
d- degenerative changes
2- Papillary muscles due to
ischemia and dilatation
3- Chordae tendineae due to
rupture from trauma or ischemia
4- Other affecting the leaflets such
as SLE, RHD and infective
endocarditis
49. -It occurs when two mitral valve
flaps fail to close smoothly which
bulge upward to the LA
-It is due to myxomatous
degeneration of mitral valve
-It occurs in adult and in range 3-
5% of population
-Risk factors : EDS, Marfan, PKD
-It is usually asymptomatic
-It is associated with mid or late
systolic murmur
-It can complicates to MR and
infective endocarditis.
50. Gross: ballooning of
leaflets into LA.
Elongated chordae
tendineae
Microscope: Excessive
edematous connective
tissue in spongiosa
52. A 38-year-old male who is
known as IV drug abuser
developed fatigue and fever
over 2 weeks. On physical
exam, he showed fingers
clubbing, mild splenomegaly
and lower leg rash.
Cardiac auscultation found
systolic murmur.
What is differential diagnosis
53. Toxic symptoms:
Fever, fatigue, rash
etc… + cardiac
symptoms
Infection
Bacteremia ?
Infection of
endocardium
to reach the
valve
Vegetation ?
56. Acute Subacute
Organism High virulant
staphylococcus
aureus 20%
Low virulant
hemolytic
streptococcus
Viridans 60%
Valve Normal and
deformed valves
Deformed valve
Progression Rapid Slow
Response Little local reaction,
lesion is
destructive
Local inflammation,
lesion is less
destructive
Resolution Death (50%) Recovery
(antibiotics)
62. NBTE: Non bacterial
thrombotic
endocarditis
- It is non bacterial, steriled,
thrombotic inflammatory
condition affecting valve
leaflets forming a mall
nodule less than 5 mm.
- Most common location
mitral valve
- It can be associated with
lung cancer and DVT
- Other example is Libman
sacks endocarditis that is
associated with SLE
63. Take Notes to Home:
- RHD is rare disease in developed countries.
- Rheumatic fever occurs secondary to
untreated group A streptococcus infection
associated carditis.
- CHD is long standing effect of rheumatic fever
that
can cause VHD, commonly mital stenosis
- VHD has many causes, most common RHD
- VHD, if uncontrolled or treated, leads to IE
64. Question
A 40 year-old west African female intravenous drug
abuser for the last 8 years presented with acute
confusion and high grade fever. Her physical
examination showed leg edema and tachypnea.
Cardiac examination revealed loud S1 and mild
pericardial rub. There was disturbed renal function
test with very high Creatinine level. Which one of
the following is most likely diagnosis:
•a. Chronic rheumatic pericarditis
•b. Mitral regurgitation and acute mitral stenosis
•c. Chronic renal injury due to infective endocarditis
•d. Acute rheumatic fever
•e. Acute myocardial infarction
65. Question
A 60 year-old Caucasian male has been diagnosed
with rheumatic heart disease since childhood. He
developed congestive heart failure 5 years ago. His
EF dropped to 10% and unfortunately he developed
complications and passed away in 5 days.
Postmortem examination of his heart showed thick
aortic valve cusps. Histological section examined
under microscope revealed Endomyocardial
plaques. The most likely underlying pathological
cause is:
•a. Aortic regurgitation
•b. Mitral stenosis
•c. Calcified aortic stenosis
•d. Combined mitral and aortic regurgitation
•e. Mitral valve prolapse
66. Thank you
Resources:
1- Robin Basic Pathology
2- Cardiovascular Pathology- Buja
2- Pathology-outline
3- Web-Pathology
4- PubMed
Editor's Notes
Examine: skin, chest, and neuro.
Tests: blood work, chest x-ray, and echocardiograph
Chest x-ray: LAD
Echo: Mitral stenosis and LAD
Mitral stenosis with left atrial enlargement (left ventricle is patent).
Rheumatic heart disease
Untreated group A streptococcus pharyngitis lead to bacteremia that reach heart causing Rheumatic. The systemic infection leads to endocarditis to cause mitral vulvitis with vegetation-
this may lead to complication such as arrhythmia and heart failure.
Infective endocarditis is infection of the endocardium and heart valve forming vegetation
Vegetation is thrombotic debri with microorganism
Stains to diagnose (gram stain and fungal stain)
Vegetation: chronic and acute inflammatory infiltrate with granulation tissue in the base. Vascularization is present.