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Pathology > CVS l > Flashcards

CVS l Flashcards

1
Q

Which chromosomal abnormalities are associated with Congenital heart disease

A

1) Turner syndrome (45X) associated with:
–> Coarctation of the aorta
2) Down syndrome associated with:
Endocardial cushion (atrioventricular cushion)
defects → Atrioventricular septal defects and
atrioventricular valve deformities
3) Foetal oxygen deprivation linked to patent ductus arteriosus

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2
Q

Conditions Characterised by Tetralogy of Fallot

A

1) Overriding aorta
2) Pulmonic stenosis
3) Ventricular septal defect
4) Right ventricular hyperthrophy

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3
Q

Causes of non-cyanotic heart diseases

A

1) Absence of a shunt
(e.g. aortic stenosis, coarctation of the aorta)
2) Presence of a left-to-right shunt
(e.g. patent ductus arteriosus, atrial or ventricular septal defect [atrial septal defects: both pressure and oxygen saturation becomes equal btw the twoatria])

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4
Q

Cause of Cyanotic congenital heart disease

A

1) Transposition of the great vessels
(survival depends on the presence of a shunt btw the left and right ventricles)
2) Malformations with a right-to-left shunt
(e.g. Tetralogy of Fallot)
3) A left-to-right shunt reverses to right-to-left shunt
(e.g. late cyanosis, tardive cyanosis)

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5
Q

Causes of Valvular heart disease

A

RF
–> could be congenital

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6
Q

Causes of Mitral valve stenosis

A

1) Most common: Post-rheumatic or postinflammatory disease (99% of cases)
2) Rare: Congenital valvular or supra-valvular
stenosis, SLE, Whipple endocarditis, and
extensive calcification of the mitral annulus

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7
Q

Macroscopic features:
* Swollen mitral valve leaflets
* Tiny flat vegetations, along the lines of closure

*Microscopic features:
* Oedema
* Platelet-fibrin thrombi
* “Aschoff” bodies (minority of cases)

Are features of which heart disorder?

A

Mitral valve stenosis cause by Rheumatic heart disease (in the Acute stage)

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8
Q

Macroscopic features:
* Fusion of the commissures
* Fibrous thickening, retraction, and
calcification of the leaflets
* Fusion and shortening of the chordae
* Orifice: Oval, narrow “fish mouth” opening

Microscopic features:
*Fibrosis, calcification with or without ossification
*Neovascularisation
* Variable chronic inflammatory cell infiltrate
(lymphocytes, monocytes, and mast cells)
* No “Aschoff” bodies
* Occurrence of: Superimposed sterile or infected vegetations and Papillary Fibroelastomas

are the features of what heart disease?

A

Mitral valve stenosis, caused by Rheumatic heart disesae (during the Fibrosing stage)

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9
Q

Epidemiology of Mitral valve prolapse

A

Common in women and Marfan syndrome

–> Myxoid degeneration of the valve’s ground
substance (Myxomatous valvulopathy)

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10
Q

Complications of Mitral valve prolapse

A
  • Usually, asymptomatic
  • Can lead to mitral valve insufficiency
  • Associated with arrhythmias
  • Predisposes to infective endocarditis
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11
Q

Treatment of Mitral valve prolapse

A

Surgical repair

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12
Q

Causes of Mitral valve insufficeiny

A
  • Mitral valve prolapse
  • Rheumatic heart disease
  • Infective endocarditis
  • Damage to a papillary muscle, after MI
  • Ruptured chordae tendinae
  • Annular calcification
  • Secondary to left ventricular dilatation
  • Drug induced (anorectic drug fen-phen and
    antimigraine drugs)
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13
Q

Pathogenesis of Mitral valve insufficiency

A

Improper closing of the mitral valve (during
systole) → Abnormal leaking of blood from left
ventricle backwards (through the mitral valve)
into the left atrium

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14
Q

Macroscopic features:
* Profound nodular calcifications in one or both
cusps
* Absence of commissural fusion
* Slit-like orifice

Are the features of which heart malformation?

A

Bicuspid Aortic valve stenosis

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15
Q

Histopathologic changes of Bicuspid aortic valve stenosis

A

Extensive calcific changes that begin in the
fibrosa layer and expand into the sinuses

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16
Q

Complications of Bicuspid aortic valve stenosis

A

Increased risk for ascending aortic aneurysm and dissection

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17
Q

Causes of senile (degenerative) Caclific tricuspid aortic stenosis

A

Calcification and other fibro-degeneration
in the valve cusps of an otherwise normal valve

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18
Q

Epidemiology of SENILE CALCIFIC
TRICUSPID AORTIC STENOSIS

A

Older age group than BAV >60
* NO association with aortic dilatation

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19
Q

Epidemiology of Bicuspid aortic valve stenosis

A

Men > Women
Occurrence in isolation
*Two cusps instead of three
*One cusp usually larger than the other, with the raphe present on the larger cusp

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20
Q

Macroscopic findings:
* Arrangement as nodular protrusions into the
sinuses, and distribution in all three leaflets
* Orifice: Triangular in shape
* Leaflets: Thickened, but the free edges of them are only minimally thickened

are the features of which cardiac malformation?

A

SENILE CALCIFIC TRICUSPID AORTIC STENOSIS

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21
Q

Macroscopic findings:
* Fusion of the commissures
* Post-inflammatory fibrous thickening of the free edge of the leaflets → Thickened, inflexible leaflets → Formation of a triangular orifice
* Calcification less severe than in either BAV or calcific tricuspid disease

are the features of which cardiac malformation?

A

POST-INFLAMMATORY/POST-RHEUMATIC AORTIC STENOSIS

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22
Q

complications of POST-INFLAMMATORY/POSTRHEUMATIC AORTIC STENOSIS

A

Infectious Endocarditis

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23
Q

causes of Aortic valve insufficieny

A

1) Aortic root dilatation
–> age-related aortic degeneration or coexisting disorders of the aorta in Marfan Syn. or Bicuspid Aortic Valve
2) Post-rheumatic and prior valvular intervention or septal myomectomy (less frequent; surgical
treatment for hypertrophic cardiomyopathy)
3) Syphilitic (luetic aortitis) → Dilatation of the aortic valve ring
4) Anorectic drug (fen-phen) → Insufficiency in leftand right-sided valves

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24
Q

Macro-/Microscopic findings:
* Age-related aortic root dilatation: Leaflets with
minimal or no degenerative features
* In root dilatation associated with Marfan
syndrome: Leaflets with a range of
myxomatous expansion of the spongiosa layer
* Aortic valve insufficiency, due to post-rheumatic lesions <>Limited amounts of calcific deposits and fibrosis

Are the features of which cardiac malformation?

A

Aortic valve insufficieny

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25
Q

Macroscopic findings:
* Thickened cusps with a glistening smooth
surface
* Nodular thickening in the central portion of the valve
Microscopic findings:
* Plaques composed of myofibroblastic cells
* Cells embedded in a myxoid stroma that are “plastered” onto the aortic side of the cusps

Are the features of which heart malformation?

A

Aortic valve insufficieny associated wiht Fen-phen drug

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26
Q

Causes of Tricuspid valve stenosis

A
  • Infective Endocarditis
  • Congenital tricuspid stenosis
  • Metabolic abnormalities (e.g. Fabry disease)
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27
Q

What syndrome is involved in Tricuspid valve disease?

A

Carcinoid Syndrome (Carcinoid Heart Disease) –> The right-sided valves affected, since these
are the first cardiac tissues bathed by the released mediators by GI carcinoid tumours

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28
Q

Macroscopic findings:
* Leaflets: Thickened, firm, and retracted
* Tips of papillary muscle insertions: “pearly white”
* Chordae: Thickened and fused

Microscopic findings:
* Deposits:
* On both surfaces of the leaflets and the chordae
* Composed of the same constituents as fen-phen valvulopathy
* Neovascularisation
* Chronic inflammation
* Mast cells

are the features of which heart disease?

A

Carcinoid valve disease

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29
Q

what characteristic feature is observed in microscopic findings of Carcinoid valve disease?

A
  • Tips of papillary muscle insertions: “pearly white”
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30
Q

Epidemiology of Pulmonary valve stenosis

A

Commonly affected by congenital
malformations (alone or together with other
congenital defects [e.g. Tetralogy of Fallot])

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31
Q

Epidemiology of Pulmonary valve regrugitation

A

Annular dilatation associated with pulmonary
arterial hypertension → Most common cause
of regurgitation

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32
Q

Clinical picture of Congestive heart failure

A

1) Dyspnoea
2) oedema

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33
Q

DD of congestive heart disease

A

1) Asthma,
2) Acute Coronary Syndrome
3) COPD,
4) Pulmonary Embolism

34
Q

Causes of Left-sided heart failure

A
  • Ischaemic Heart Disease (e.g. MI)
  • Hypertension
  • AV and MV disease
  • Myocardial diseases (Cardiomyopathies and
    Myocarditis)
35
Q

Clinical manifestaions of Left-sides heart disease

A

1) Dyspnoea and orthopnoea–> caused by
pulmonary congestion and oedema (common)
2) Pleural effusion with hydrothorax
3) Reduction in renal perfusion → Activation of
Renin-Angiotensin-Aldosterone system →
Retention of NaCl and H2O
4) Cerebral anoxia (less frequent)

36
Q

causes of right-sided heart failure

A
  • Left-sided heart failure (most common)
  • Left-sided lesions (e.g. Mitral Valve stenosis)
  • Pulmonary hypertension, caused by Cor pulmonale
  • Cardiomyopathy and diffuse Myocarditis
  • Tricuspid valve or pulmonary vascular disease
37
Q

clinical manifestations of Right-sided heart failure

A

1) Renal hypoxia → Fluid retention (pleural
effusion) and peripheral oedema
(“pitting” oedema)
2) Enlarged and congested liver and spleen
3) Distention of the neck veins

38
Q

causative agent of rheumatic fever

A

Group A β-haemolytic Strept.

39
Q

Pathogenesis of RF

A

Autoimmune disease that develops 1-4 weeks after an episode of pharygitis

40
Q

Histopatho:
“Aschoff nodule” = Focal interstitial myocardial inflammation consisted of:
* Fragmented collagen and fibrinoid material
* Large cells (“Anitschkow” myocytes)
* Multinucleated giant cells (“Aschoff” cells)

Are the characteristic features of which disease?

A

Rheumatic fever

41
Q

Describe the “granulomatous stage” of “Aschoff nodules” of Rheumatic fever

A

Arises 1-2 months after the onset of clinical
symptoms
* Develops within or near foci of fibrinoid necrosis
* Eventually, replaced by collagenous scar tissue

42
Q

complications of Rheumatic fever

A

1) Pericarditis → Pericardial, pleural effusions
2) Myocarditis → Cardiac failure → Death
3) Endocaridits –> valvular damage

43
Q

complications of endocarditis

A

1) Valvular damage
2) formation of MacCallum plaque at the points of valve closure of the left atrium

44
Q

Valves affected by endocarditis

A

1) mitral valve–> 50% cases
2) Aortic valve–> affected along with the MV
3) Tricuspid valve (TV): 5% of cases
4) Pulmonary valve (PV): Rarely involved

45
Q

macroscopic features:
1) Red and swollen valve leaflets
2) Tiny, warty, rubbery, but non-friable
vegetations (verrucae) along the lines of closure of the valve leaflets

A

early stages of endocarditis

46
Q

macroscopic features
- Fibrotic healing:
→ Thickened, fibrotic and deformed valves
→ Fusion of valve cusps
→ Thickening of chordae tendinae
→ Calcifications

A

later stages of Endocarditis (Caused by RHD)

47
Q

Non-cardiac manifestations of acute RF

A

1) Fever,
2) malaise and
3) ↑ Erythrocyte Sedimentation Rate (ESR)
4) Skin lesions–> Subcutaneous nodules and Erythema marginatum (skin rash)
5) CNS involvement–> Sydenham chorea (invlountary msucle movement)

48
Q

Epidemiology of infective endocarditis

A
  • Valvular endocarditis: Majority of patients with underlying congenital or acquired alteration
  • Left-sided valves more commonly affected than right-sided valves (aortic > mitral > tricuspid > pulmonic)
  • Valves with regurgitant alterations particularly
    at risk, as well as patients with prosthetic valves
    and pacemaker/intra-cardiac cardioverterdefibrillator wires, immunosuppressed patients,
    and intravenous drug abusers
49
Q

causes of infective endocarditis

A

Gram (+) & (-) bacterial, fungal, mycobacterial, rickettsial, and chlamydial organisms

50
Q

classification of infective endocarditis

A
  • Acute Endocarditis:
    –> Staphylococcus aureus(50% of cases)
    –> Secondary to infection elsewhere in the body
  • Subacute Endocarditis:
    –> Less virulent organisms (Streptococcus viridans; >50% of cases)
    –> Patients with congenital or valvular heart
    disease
51
Q

complications of infective endocarditis

A
  • Fragmentation of vegetations → Distal embolisation → Septic infarcts (brain or other organs)
  • Focal glomerulonephritis, caused by immune complex disease or septic emboli
52
Q
  • In the untreated, early phase of organisation:
    Acute fibrinous exudates with neutrophils and necrotic changes and tissue destruction in the valve
  • In the healing phases or in the setting of an
    insidious low-grade infection: neovascularisation, chronic inflammation, fibrosis, and calcification
    replace the damaged tissue

Are the microscopic features of which disease

A

Infective endocarditis

53
Q
  • Dependant on virulence of the organisms
  • Variety in size and shape
  • Large, soft, friable, easily detached vegetations
  • Virulent organisms can cause perforations of the leaflets or rupture of the chordae
  • Extension from the site of initiation at the cusp apposition line (atrial surface of atrio-ventricular valves and ventricular surface of semilunar valves) can proceed to the leaflets, chordae, and annular regions to form abscesses

Are the macroscopic features if what heart disease

A

Infective Endocarditis

54
Q

which valves are involved in infective endocarditis

A
  • Mitral and Aortic valve; 40% of cases
  • Tricuspid valve; 50% of drug users endocarditis (Staphylococcus infection)
55
Q
  • Small sterile platelet-fibrin vegetations (devoid of inflammatory cells or bacteria), arranged as continuous linear aggregates along the line of closure of left-sided valve leaflets
  • No tissue destruction of the underlying valve tissue

Are the pathological findings of which heart disease?

A

NON-BACTERIAL THROMBOTIC
(MARANTIC) ENDOCARDITIS

56
Q

complications of NON-BACTERIAL THROMBOTIC (MARANTIC) ENDOCARDITIS

A

Peripheral embolisation

57
Q
  • Small, flat vegetations on the atrial aspect of the posterior mitral valve leaflet and the ventricular aspect of the aortic valve
  • Vegetations may expand to cover both
    aspects of the valve or extend along the
    atrial or ventricular endocardium

Are the macroscopic features of which heart disease?

A

LIBMAN-SACKS ENDOCARD

58
Q

Libman-sacks endocarditis is ass. with?

A

Associated with SLE and antiphospholipid syndrome

59
Q
  • Microscopic findings:
    Libman-Sacks lesions can mimic ——————————- due to the presence of fibrin, cores of fibrinous necrosis of the valve, inflammatory cell infiltrates
A

Infective endocarditis

60
Q

Epidemiology of ENDOCARDITIS OF THE
CARCINOID SYNDROME

A

*Affects mainly right-sided valves (tricuspid and
pulmonic valve)
* Mitral and aortic valve are rarely involved

61
Q

laboratory of endocarditis of the carcinoid syndrome

A

Secretory products of carcinoid
tumours (VAPs [Vaso Active Peptides] and Amines)

62
Q

Macroscopic findings:
Thickened endocardial plaques (mural endocardium or valvular cusps)

are the features of which heart disease

A

Endocarditis of the carcinoid syndrome

63
Q

Epidemiology of Rhabdomyoma

A

–> Most common in infants and young adults
–> benign tumour

64
Q

disease ass. w/ Rhabdomyoma

A

Close association with Tuberous
Sclerosis Complex

65
Q

clinical signs and symptoms of Rhabdomyoma

A
  • depending on size, location, and number of masses:
  • Congestive heart failure
  • Conduction abnormalities
  • Obstruction of flow across a valve
66
Q

Macroscopic features:
* Solitary mass or multiple masses (always in the setting of Tuberous Sclerosis) in the ventricular wall or chamber
* Sharply circumscribed, non-encapsulated,
pale tan to yellow nodules that range in size
from 0.1 to 9.0 cm

Are the features of which heart carnimoa?

A

Rhabdomyoma

67
Q

Microscopic findings:
* Composed of large vacuolated cells with central round-to-oval nuclei and strands of eosinophilic cytoplasmic bands traversing the cells producing the “spider cells”
* The cells:
* Contain large amounts of glycogen
* Demonstrate immuno-reactivity for desmin, actin, myoglobin, and vimentin

Are the features of which heart carcinoma?

A

RHABDOMYOMA

68
Q

epidemiology of Fibroma

A
  • Second most common tumour in children
  • Rarely spontaneously regress, unlike
    rhabdomyomas
  • The majority are sporadic (infrequent) ; less than 5% are associated with Gorlin syndrome
69
Q

clinical symptoms of Fibroma

A

1) Congestive heart failure,
2) obstruction,
3) arrhythmias, or sudden death

70
Q

Macroscopic features:
* solitary lesions (up to 10 cm) within the interventricular septum or free wall of the RV or LV
* Resemblance to uterine leiomyomas (white whorled appearance)
* Sharply delineation from the adjacent myocardium
* May contain central foci of calcifications

Are the features of which heart carcinoma?

A

Fibroma

71
Q

Microscopic features:
* Bland fibroblastic proliferation resembling extraabdominal fibromatosis
* Variable cellularity with tumour cells inter-digitating into the adjacent myocytes
* Matrix, rich in collagen and elastin fibers

Are the features of which heart carcinoma?

A

Fibroma

72
Q

Epidemiology of cardiac Myaxoma

A
  • Most common primary neoplasm (75-80% of
    tumours)
  • part of the Carney complex or as a
    non-syndromic familial cardiac myxoma
73
Q

localisation of cardiac myxomas

A

1) mostly in the LA (70-80%)
2) less common in the Right Atrium (15-20%)
3) rarely Bi-atrial (2%), RV (2%), LV (2%) and Multifocal locations (3%)

74
Q

clinical features of cardiac myxoma

A

Symptoms at presentation depend on location (left vs. right atrium), size, and mobility:
1) Atrio-ventricular valve obstruction,
2) embolisation,
3) congestive heart failure

75
Q

Macroscopic features:
* Majority of cases: Solitary mass, on the endocardial surface of the left atrium, adjacent to foramen ovale
* Smooth and lobulated or gelatinous tumour
* Size: 2-8 cm
* Attachment (through a broad base or delicate
stalk) to the endocardium and protrusion into
the cardiac cavity
* Cut surface: Foci of haemorrhage, calcification, or cystic change

Are the features of which cardiac carcinoma?

A

Myxoma

76
Q

Microscopic features:
* Variable cellularity, including foci of high cellularity
* “lepidic” cells: Spindled or stellate cells with eosinophilic cytoplasm and uniform, round nuclei with inconspicuous nucleoli
* Toward the surface of the myxoma, there is
alignment of cells around vessels (in a more
complex ring-like pattern) and association with mononuclear inflammatory cells
* Abundant mucopolysaccharide matrix
* Calretinin immunopositivity in myxomas, but not in thrombi <> Useful marker in difficult cases

are the features of which cardiac carcinoma?

A

Myxoma

77
Q

Epidemiology/localization of cardiac Angiosarcoma

A

arise from the right atrium or pericardium

78
Q

clinical symptoms ofAngiosarcoma

A

spreads inside the thoracic

79
Q

Macroscopic features:
* Multi-lobulated mass, often with frond-like papillae
* Commonly, infiltration into the atrial chamber and/or extension into or through the pericardium
* Cut surface: Haemorrhagic with foci of necrosis

are the features of which cardiac carcinoma?

A

Angiosarcoma

80
Q

Microscopic findings:
* Range from well-differentiated sarcomas, with anastomosing channels lined by atypical, hyperchromatic endothelial cells with coarse chromatin and mitotic figures to anaplastic or epithelioid tumours

are the features of which cardiac carcinoma ?

A

Angiosarcoma

Pathology (11 decks)

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