CVS l Flashcards
Which chromosomal abnormalities are associated with Congenital heart disease
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
Conditions Characterised by Tetralogy of Fallot
1) Overriding aorta
2) Pulmonic stenosis
3) Ventricular septal defect
4) Right ventricular hyperthrophy
Causes of non-cyanotic heart diseases
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])
Cause of Cyanotic congenital heart disease
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)
Causes of Valvular heart disease
RF
–> could be congenital
Causes of Mitral valve stenosis
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
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?
Mitral valve stenosis cause by Rheumatic heart disease (in the Acute stage)
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?
Mitral valve stenosis, caused by Rheumatic heart disesae (during the Fibrosing stage)
Epidemiology of Mitral valve prolapse
Common in women and Marfan syndrome
–> Myxoid degeneration of the valve’s ground
substance (Myxomatous valvulopathy)
Complications of Mitral valve prolapse
- Usually, asymptomatic
- Can lead to mitral valve insufficiency
- Associated with arrhythmias
- Predisposes to infective endocarditis
Treatment of Mitral valve prolapse
Surgical repair
Causes of Mitral valve insufficeiny
- 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)
Pathogenesis of Mitral valve insufficiency
Improper closing of the mitral valve (during
systole) → Abnormal leaking of blood from left
ventricle backwards (through the mitral valve)
into the left atrium
Macroscopic features:
* Profound nodular calcifications in one or both
cusps
* Absence of commissural fusion
* Slit-like orifice
Are the features of which heart malformation?
Bicuspid Aortic valve stenosis
Histopathologic changes of Bicuspid aortic valve stenosis
Extensive calcific changes that begin in the
fibrosa layer and expand into the sinuses
Complications of Bicuspid aortic valve stenosis
Increased risk for ascending aortic aneurysm and dissection
Causes of senile (degenerative) Caclific tricuspid aortic stenosis
Calcification and other fibro-degeneration
in the valve cusps of an otherwise normal valve
Epidemiology of SENILE CALCIFIC
TRICUSPID AORTIC STENOSIS
Older age group than BAV >60
* NO association with aortic dilatation
Epidemiology of Bicuspid aortic valve stenosis
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
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?
SENILE CALCIFIC TRICUSPID AORTIC STENOSIS
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?
POST-INFLAMMATORY/POST-RHEUMATIC AORTIC STENOSIS
complications of POST-INFLAMMATORY/POSTRHEUMATIC AORTIC STENOSIS
Infectious Endocarditis
causes of Aortic valve insufficieny
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
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?
Aortic valve insufficieny
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?
Aortic valve insufficieny associated wiht Fen-phen drug
Causes of Tricuspid valve stenosis
- Infective Endocarditis
- Congenital tricuspid stenosis
- Metabolic abnormalities (e.g. Fabry disease)
What syndrome is involved in Tricuspid valve disease?
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
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?
Carcinoid valve disease
what characteristic feature is observed in microscopic findings of Carcinoid valve disease?
- Tips of papillary muscle insertions: “pearly white”
Epidemiology of Pulmonary valve stenosis
Commonly affected by congenital
malformations (alone or together with other
congenital defects [e.g. Tetralogy of Fallot])
Epidemiology of Pulmonary valve regrugitation
Annular dilatation associated with pulmonary
arterial hypertension → Most common cause
of regurgitation
Clinical picture of Congestive heart failure
1) Dyspnoea
2) oedema
DD of congestive heart disease
1) Asthma,
2) Acute Coronary Syndrome
3) COPD,
4) Pulmonary Embolism
Causes of Left-sided heart failure
- Ischaemic Heart Disease (e.g. MI)
- Hypertension
- AV and MV disease
- Myocardial diseases (Cardiomyopathies and
Myocarditis)
Clinical manifestaions of Left-sides heart disease
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)
causes of right-sided heart failure
- 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
clinical manifestations of Right-sided heart failure
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
causative agent of rheumatic fever
Group A β-haemolytic Strept.
Pathogenesis of RF
Autoimmune disease that develops 1-4 weeks after an episode of pharygitis
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?
Rheumatic fever
Describe the “granulomatous stage” of “Aschoff nodules” of Rheumatic fever
Arises 1-2 months after the onset of clinical
symptoms
* Develops within or near foci of fibrinoid necrosis
* Eventually, replaced by collagenous scar tissue
complications of Rheumatic fever
1) Pericarditis → Pericardial, pleural effusions
2) Myocarditis → Cardiac failure → Death
3) Endocaridits –> valvular damage
complications of endocarditis
1) Valvular damage
2) formation of MacCallum plaque at the points of valve closure of the left atrium
Valves affected by endocarditis
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
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
early stages of endocarditis
macroscopic features
- Fibrotic healing:
→ Thickened, fibrotic and deformed valves
→ Fusion of valve cusps
→ Thickening of chordae tendinae
→ Calcifications
later stages of Endocarditis (Caused by RHD)
Non-cardiac manifestations of acute RF
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)
Epidemiology of infective endocarditis
- 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
causes of infective endocarditis
Gram (+) & (-) bacterial, fungal, mycobacterial, rickettsial, and chlamydial organisms
classification of infective endocarditis
-
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
complications of infective endocarditis
- Fragmentation of vegetations → Distal embolisation → Septic infarcts (brain or other organs)
- Focal glomerulonephritis, caused by immune complex disease or septic emboli
- 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
Infective endocarditis
- 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
Infective Endocarditis
which valves are involved in infective endocarditis
- Mitral and Aortic valve; 40% of cases
- Tricuspid valve; 50% of drug users endocarditis (Staphylococcus infection)
- 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?
NON-BACTERIAL THROMBOTIC
(MARANTIC) ENDOCARDITIS
complications of NON-BACTERIAL THROMBOTIC (MARANTIC) ENDOCARDITIS
Peripheral embolisation
- 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?
LIBMAN-SACKS ENDOCARD
Libman-sacks endocarditis is ass. with?
Associated with SLE and antiphospholipid syndrome
- Microscopic findings:
Libman-Sacks lesions can mimic ——————————- due to the presence of fibrin, cores of fibrinous necrosis of the valve, inflammatory cell infiltrates
Infective endocarditis
Epidemiology of ENDOCARDITIS OF THE
CARCINOID SYNDROME
*Affects mainly right-sided valves (tricuspid and
pulmonic valve)
* Mitral and aortic valve are rarely involved
laboratory of endocarditis of the carcinoid syndrome
Secretory products of carcinoid
tumours (VAPs [Vaso Active Peptides] and Amines)
Macroscopic findings:
Thickened endocardial plaques (mural endocardium or valvular cusps)
are the features of which heart disease
Endocarditis of the carcinoid syndrome
Epidemiology of Rhabdomyoma
–> Most common in infants and young adults
–> benign tumour
disease ass. w/ Rhabdomyoma
Close association with Tuberous
Sclerosis Complex
clinical signs and symptoms of Rhabdomyoma
- depending on size, location, and number of masses:
- Congestive heart failure
- Conduction abnormalities
- Obstruction of flow across a valve
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?
Rhabdomyoma
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?
RHABDOMYOMA
epidemiology of Fibroma
- Second most common tumour in children
- Rarely spontaneously regress, unlike
rhabdomyomas - The majority are sporadic (infrequent) ; less than 5% are associated with Gorlin syndrome
clinical symptoms of Fibroma
1) Congestive heart failure,
2) obstruction,
3) arrhythmias, or sudden death
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?
Fibroma
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?
Fibroma
Epidemiology of cardiac Myaxoma
- Most common primary neoplasm (75-80% of
tumours) - part of the Carney complex or as a
non-syndromic familial cardiac myxoma
localisation of cardiac myxomas
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%)
clinical features of cardiac myxoma
Symptoms at presentation depend on location (left vs. right atrium), size, and mobility:
1) Atrio-ventricular valve obstruction,
2) embolisation,
3) congestive heart failure
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?
Myxoma
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?
Myxoma
Epidemiology/localization of cardiac Angiosarcoma
arise from the right atrium or pericardium
clinical symptoms ofAngiosarcoma
spreads inside the thoracic
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?
Angiosarcoma
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 ?
Angiosarcoma
