GENERAL &

SYSTEMIC PATHOLOGY
2. What are the common causes of this patient’s condition in
F. 04 DISEASES OF THE HEART (Part 4) the elderly? In younger individuals?
Dr. Domantay (Reporters)

CASE 9
A 24-year-old man complains of easy fatigability, dyspnea on
mild exertion, and angina. He also admits to having occasional spells
of lightheadedness and fainting while playing basketball.

PE: Crescendo-decrescendo systolic ejection murmur to right

of sternum and radiating to neck; soft s2 with paradoxical splitting;
weak and delayed carotid pulses.

Labs: ECG: left ventricular hypertrophy

Imaging: CXR: calcification on valve leaflets and enlarged

cardiac shadow. Echo: presence of bicuspid aortic valve.

1. What valvular heart disease does this patient most likely

have?

CALCIFIC STENOSIS OF CONGENITALLY BICUSPID AORTIC

VALVE
3. What structural alterations are seen in this patient’s
condition?

BAV mostly occurs to male than female (2-4:1). Stenosis of a

bicuspid aortic valve is more likely to develop in persons older than 20
years and is caused by progressive sclerosis and calcification. This
coincides with the patient's age and sex, which is 24 y/o, male.

The three cardinal symptoms of Aortic stenosis: Exertional

dyspnea, angina and syncope

Nodular calcific masses in Calcific aortic stenosis of a

the sinus of Valsalva congenitally bicuspid valve

Mounded calcified In a congenitally

masses within the aortic cusps
that ultimately protrude
through the outflow surfaces
into the sinuses of Valsalva,
and prevent cuspal opening.
bicuspid aortic valve, there are
only two functional cusps,
usually of unequal size, with the
larger cusp having a midline
raphe, resulting from incomplete
commissural separation during
development

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2. What mechanisms are involved in the development of the

Mitral annular calcification disease?

Involvement of adjacent structures can actually lead to  Causes

impingement of the conduction system of the heart  Unknown
 Mutations in fibrillin-1 (FBN-1) – (inherited) cause of
Microscopically, the layered architecture of the valve is connective tissue disorder such as Marfan Syndrome
largely preserved. The calcific process begins in the -Fibrillin-1 defects alter cell-matrix interactions and
valvularfibrosa on the outflow surface of the valve, at the points dysregulate TGF-β signaling.
of maximal cusp flexion (near the margins of attachment).  Change:
Inflammation is variable, and metaplastic bone (and even bone  Characteristic structural laxity and myxomatous
marrow) may be seen. changes
 Consequences
 Disease entity
CASE 10
A 37-year-old white male complains of increasing fatigue 3. What structural alterations in the heart are seen in this
and shortness of breath during minimal physical exertion. He denies disease?
having any chest pain for having similar symptoms. A careful history
reveals rheumatic fever at the age of 7.  Anatomic changes:
 Interchordal ballooning of the mitral leaflets (often
PE-VS: jerky pulse; PE: High-pitched pansystolic murmur at enlarged, redundant, thick, and rubbery)
apex with radiation to axilla; S3  Associated tendinous cords may be elongated, thinned
or even ruptured
Labs: ECG: left axis deviation; left atrial and left ventricular  Annulus may be dilated
hypertophy

Imaging: CXR/Echo: enlargement of left atrium and  Histologic Changes

ventricle. Doppler: confirmatory
1. If the physician discovered a midsystolic click on
auscultation, what would be the most likely diagnosis?
Mitral Valve Prolapse (Myxomatous Degeneration of the
Mitral Valve)
 Myxomatous degeneration
 Marked thickening of the spongiosa layer with
deposition of mucoid (myxomatous material)
 Attenuation of the collagenous fibrosa layer of the
valve, on which the structural integrity of the
leaflet depends

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 GENERAL &
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 Secondary Changes - reflect the stresses and tissue injury

incident to the billowing leaflets:
(1) Fibrous thickening of the valve leaflets
(2) Linear fibrous thickening of left ventricular
endocardial surface
(3) Thickening of the mural endocardium of the left
ventricle or atrium
(4) Thrombi on the atrial surfaces of the leaflets or
the atrial walls
(5) Focal calcifications at the base of the posterior
mitral leaflet (C)
• Acute, immunologically mediated, multisystem inflammatory
disease
• Occurring after few weeks of Group A Streptococcal
pharyngitis
• Common manifestation: Acute rheumatic carditis
• May progress to chronic rheumatic heart disease (Valvular
abnormalities)
RHEUMATIC HEART DISEASE
• Deforming fibrotic valvular disease
• Only cause of mitral stenosis

4. What are the complications of this disease? 2. What mechanisms are involved in the development of this
patient’s disease?
Approximately 3% develop one of four serious complications:
(1) Infective endocarditis
(2) Mitral insufficiency (sometimes with chordal rupture)
(3) Stroke or other systemic infarct
(4) Arrhythmias, both ventricular and atrial

CASE 11

An 8-year-old female presents with pain and swelling of her

knee joints, elbows, and lower limbs, along with fever for the past two
weeks; she also complains of shortness of breath on exertion. The
patient had a sore throat two weeks ago.

PE-VS: fever; PE: blanching ring-shaped erythematous rash

over trunk and proximal extremities, subcutaneous nodules at occiput
and below extensor tendons in elbow; swelling with redness of both
knee joints and elbows; painfully restricted movement; pedal edema;
increased JVP; high frequency apical systolic murmur with radiation to
axillae; bilateral fine inspiratory basal crepitant rales; mild, tender
hepatomegaly.

Labs: CBC: leukocytosis; Streptococcus pyogenes on throat

swab; markedly elevated ASO titers, ESR, CRP; negative blood culture;
ECG: prolonged P-R interval

CXR: cardiomegaly; increased pulmonary vascular markings;

Echo: vegetations over mitral valve with regurgitation

1. What is the most likely diagnosis?

Acute rheumatic fever

with rheumatic heart
disease

RHEUMATIC FEVER

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 GENERAL &
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c. Verrucae

d. MacCallum Plaques
 Subendocardial lesions, perhaps exacerbated by regurgitant jets,
can induce irregular thickenings called MacCallum plaques,
3. Which heart valves are frequently involved in this usually in the left atrium.
patient’s disease?

• Mitral valve is the most commonly involved and can be seen

in roughly 2/3 of RHD
• Aortic valve in another 25%
• Tricuspid valve- infrequent
• Pulmonary valve- only rarely affected

4. What structural alterations are seen in the acute phase of

this patient’s disease? In the chronic phase?

ACUTE PHASE

a. Diffuse inflammation Irregular thickenings usually in the left atrium

• During acute RF, focal inflammatory lesions are
found in various tissues.
• Distinctive lesions occur in the heart, called
Aschoff bodies, consisting of foci of T
lymphocytes, occasional plasma cells, and plump
activated macrophages called Anitschkow cells
(pathognomonic for RF).
CHRONIC RHD
• Cardinal anatomic changes (mitral valve):
 Leaflet thickening
 Commissural fusion and shortening
 Fibrous thickening and Fusion of the tendinous cord

• Mitral valve is virtually always involved in chronic RHD

MICROSCOPIC CHANGES

 Valves show organization of the acute inflammation,

with post-inflammatory neovascularization;
 Transmural fibrosis
 Aschoff bodies are rarely seen

5. What is the Jones criteria? Explain this in relation to the

patient’s disease

• Guidelines decided on by the AHA to help doctors

clinically diagnose rheumatic fever.
b. Fibrinoid necrosis
• History of a Group A streptococcal infection
• Results from inflammation of the endocardium and
• Two (2) major criteria or one (1) major and two minor
the left-sided valves
• Within the cusps or tendinous cords.
MAJOR CRITERIA MINOR CRITERIA

• Migratory polyarthritis of • Fever

the large joints • Arthralgia
• Pancarditis – all layers of • Elevated ESR
the heart are inflammed • Elevated CRP
• Subcutaneous nodules • Prolonged P-R interval on
• Erythema marginatum of EKG
the skin – ring-shaped rash
• Sydenham chorea - a
neurologic disorder with

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involuntary rapid, composed of thrombotic debris and organisms, often associated
purposeless movements with destruction of the underlying cardiac tissues.

Clinical features of Acute Patient’s signs and symptoms

infective Endocarditis

 Flu like symptoms such as  High fever with chills

fever, chills, fatigue, aching
muscles and joints, night
sweats, headaches
 Cough or shortness of
breath
 New heart murmur or a
change in an existing heart
murmur
 malaise
 Productive cough
 Ejection systolic murmur,
increasing with inspiration,
heard in tricuspid area

 Skin changes

 Nausea

 Swelling in the feet, legs or

abdoment

Laboratory features of Acute Laboratory findings of the

Infective Endocarditis patient

Positive blood culture for a Blood culture yielded

For the patient: (+) Streptococcus Pyogenes on throat swab + more characteristic organism Staphylococcus aureus
than 2 positives for major criteria = Rheumatic Heart Disease
Anemia CBC: normochromic, normocytic
6. What are the complications of the patient’s disease?
anemia
Heart Changes secondary to MITRAL STENOSIS
Microscopic hematuria UA: Microscopic hematuria
• Left atrial hypertrophy and enlargement
• Atrial fibrillation (Arrhythmia) Presence of prototypic lesion of Echo: ejection systolic murmur,
• Thromboembolic Complications infective endocarditis upon increasing with inspiration, heard
• Pulmonary Edema and Congestion echocardiogram: vegetations in tricuspid area
• Right ventricular hypertrophy
• Congestive Heart Failure
• Increased risk for infective endocarditis
RISK FACTORS:
CASE 12
A 25-year-old IV drug user presents with a high fever with • Congenital heart defects
chills, malaise, a productive cough, haemoptysis, and right sided • Chronic rheumatic heart disease
pleuritic chest pain. He also reports multiple skin infections at injection • Degenerative valve disease
sites • Intracardiac devices (e.g.) artificial heart valves, implantable
cardiac defibrillator)
PE-VS: fever; PE: stigmata of intravenous drug abuse at • Illicit IV drug abuse
multiple injection sites; skin infections; thrombosed peripheral veins;
splenomegaly and pulsatile hepatomegaly; ejection systolic murmur, 2. What mechanisms are involved in the development of this
increasing with inspiration, heard in tricuspid area patient’s disease?

Labs: CBC: normochromic, normocytic anemia,
microscopic hematuria. Blood culture yields S. aureus.
Imaging: Echo: presence of vegetations on tricuspid valve
and tricuspid incompetence. CXR: consolidation
1. What is the most likely diagnosis? Specify.
UA; 2 factors needed to establish endocardial infection:
• Cardiac and vascular abnormalities (valves)
• Episode of bacteremia (Rheumatic heart disease with
valvular scarring has historically been the major
antecedent disorder)

ACUTE INFECTIVE ENDOCARDITIS – BACTERIAL

ENDOCARDITIS

Infective endocarditis (IE) is a microbial infection of the heart valves or

the mural endocardium that leads to the formation of vegetations

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Mechanisms in the Development of IE Vegetation usually erode into the underlying myocardium and produce
an abscess

4. What are the complications of this patient’s disease?

• Glomerulonephritis - glomerular antigen- antibody

complex deposition
• Microthromboemboli - manifest as splinter or subungual
hemorrhages
• Janeway lesions - erythematous or hemorrhagic
nontender lesions on the palms or soles
• Osler nodes - subcutaneous nodules in the pulp of the
digits
• Roth spots- retinal hemorrhages in the eyes

5. What is the Duke criteria? Explain this in relation to the

patient’s disease.

DUKE CRITERIA

• Facilitate evaluation of individuals with suspected IE that

takes into account predisposing factors, physical findings,
blood culture results, echocardiographic findings, and
laboratory information.
• Diagnosis by these guidelines, often requires:
• Either pathologic or clinical criteria
• If clinical criteria:
3. What structural alterations are seen in this patient’s  2 major
disease?  1 major + 3 minor
 5 minor criteria
(Refer to last page for picture)

Vegetations of the Heart

 Classic hallmark of IE
 Friable, bulky, potentially destructive lesions containing
fibrin, inflammatory cells, and bacteria or other organisms
 Aortic and mitral valves are the most common site of
infection
 IV drug abuser: valves of the right heart may also be
involved
 Prone to embolization  embolic fragments often
contain virulent organisms, abscesses frequently develop
where they lodge  sequelae such as septic infarcts or
mycotic aneurysms.

Infective endocarditis (IE) is characterized by large, irregular

masses on the valve cusps that can extend onto the chordae

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Checkpoint

Identification

1. Structural alterations in calcific stenosis of congenitally bicuspid aortic vave? (3)

• _________________________________
• _________________________________
• _________________________________
2. Heard upon auscultation in Mitral Valve Prolapse ____________________________________
3. Only cause of mitral stenosis ____________________________
4. Most commonly involved valve in RHD seen roughly 2/3__________________________________
5. In RHD, distinctive lesions occuring in the heart_______________________________________
6. Answer in number 5, consisting of foci of T lymphocytes, occasional plasma cells, and plump activated macrophages pathognomonic
for RF are called _______________________________
7. Guidelines decided on by the AHA to help doctors clinically diagnose rheumatic fever________________________
8. How many of the guidelines should be met for a diagnosis of RHD?____________________________________________________
9. Major antecedent disorder in Infective endocarditis___________________________________________
10. Classic hallmark of Infective Endocarditis (IE) ________________________________________
11. In IE, valve that may be involved in IV drug abusers_______________________________________
12. Complications of IE (5)
1. Nodular calcific masses in the sinus valsalva
Calcific aortic stenosis
Mitral annular calcification
2. Mid systolic click
3. RHD
4. Mitral Valve
5. Aschoff bodies
6. Anitschkow cells
7. Jones Criteria
8. Two (2) major criteria or one (1) major and two minor
9. RHD
10. vegetations of the heart
11. valves of the right heart (tricuspid, pulmonic)
12. Glomerulonephritis
Microthromboemboli
Janeway lesions
Osler nodes
Roth spots

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