CVS l Flashcards by Elyaa Saleh

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

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Conditions Characterised by Tetralogy of Fallot

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

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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])

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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)

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Causes of Valvular heart disease

RF
–> could be congenital

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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

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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)

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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)

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Epidemiology of Mitral valve prolapse

Common in women and Marfan syndrome

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

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Complications of Mitral valve prolapse

Usually, asymptomatic
Can lead to mitral valve insufficiency
Associated with arrhythmias
Predisposes to infective endocarditis

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Treatment of Mitral valve prolapse

Surgical repair

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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)

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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

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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

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Histopathologic changes of Bicuspid aortic valve stenosis

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

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Complications of Bicuspid aortic valve stenosis

Increased risk for ascending aortic aneurysm and dissection

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Causes of senile (degenerative) Caclific tricuspid aortic stenosis

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

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Epidemiology of SENILE CALCIFIC
TRICUSPID AORTIC STENOSIS

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

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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

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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

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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

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complications of POST-INFLAMMATORY/POSTRHEUMATIC AORTIC STENOSIS

Infectious Endocarditis

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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

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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

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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