3.2b CVS Pathology 2

Question 0

What is the adaptive response in systemic HHD?

Answer 0

Concentric hypertrophy

Question 1

1. Stems from the increased demands placed on the heart by HPN
2. Refers to the effects on the heart as a result of increased pressure

Answer 1

Hypertensive heart disease (HHD)

Question 2

Pulmonary HHD is characterized by (2)

Answer 2

1. RV hypertrophy (chronic) or

2. RV dilatation (acute)

Question 3

1. Adapt over whose to PRESSURE overload

2. Can lead to: myocardial dysfunction, cardiac dilatation, CHF, sudden death

Answer 3

systemic HHD

Question 4

Diagnostic criteria of systemic HHD (3)

Answer 4

1. LVH (concentric thickening >2cm towards inner chamber)
2. History or pathologic evidence of HPN
3. Absence of other CVS lesions

Question 5

Its gross features include:

1. Concentric hypertrophy
2. Thickened LV wall
3. Thickened IV septum
4. Thick and robust papillary muscles
5. Initially without ventricular dilatation
6. Increased heat weight (>500g)
7. Stiffness and impairment of diastolic filling

Answer 5

Systemic HHD

Question 6

Its microscopic features include:

1. Increased transverse diameter of myocytes
2. Enlarged myocytes and nuclei (box-like)
3. Diffused interstitial fibrosis

Answer 6

Systemic HHD

Question 7

1. Pressure overload in RV due to increased pulmonary resistance usually caused by chronic lung disease that can lead to pulmonary vasculature fibrosis (ie. COPD)
2. Characterized by: RV hypertrophy, RV dilatation, (potentially) failure due to increased pulmonary resistance

Answer 7

Pulmonary HHD

Question 8

Types of pulmonary HHD (2)

Answer 8

1. Acute cor pulmonale (RV dilatation)

2. Chronic cor pulmonale (RV hypertrophy)

Question 9

Predisposing factors of pulmonary HHD (4)

Answer 9

1. Diseases of pulmonary PARENCHYMA
2. Diseases of pulmonary VESSELS
3. Disorders affecting chest movement
4. Disorders inducing pulmonary arterial constriction

Question 10

The ff are examples of which predisposing factor of pulmonary HHD?

1. COPD
2. Disuse pulmonary interstitial fibrosis
3. Pneumoconiosis
4. Cystic fibrosis
5. Bronchiectasias

Answer 10

Diseases of pulmonary parenchyma

Question 11

The ff are examples of which predisposing factor of pulmonary HHD?

1. Recurrent pulmonary thromboembolism
2. Primary pulmonary HPN
3. Extensive pulmonary arteritis
4. Drug-, toxin-, radiation-induced vascular obstruction
5. Extensive pulmonary tumor
6. Microembolism

Answer 11

Diseases of pulmonary vessels

Question 12

The ff are examples of which predisposing factor of pulmonary HHD?

1. Kyphoscoliosis
2. Marked obesity
3. Neuromuscular disease

Answer 12

Disorders affecting chest movement

Question 13

The ff are examples of which predisposing factor of pulmonary HHD?

1. Hypoxemia
2. Metabolic acidosis
3. Chronic altitude sickness
4. Obstruction to major airways
5. Idiopathic alveolar hypoventilation

Answer 13

Disorders inducing pulmonary arterial constriction

Question 14

(Pulmonary HHD) RV hypertrophy and dilatation can lead to (3)

Answer 14

1. Compression of LV
2. Tricuspid thickening
3. Regurgitation

Question 15

On cross section of acute cor pulmonale, what is the shape of RV?

Answer 15

Ovoid (normal is crescent)

Question 16

What are the microscopic feature of pulmonary HHD? (2)

Answer 16

1. Normal fat in the wall disappears

2. Circumferentially arranged myocytes (normal: haphazardly arranged)

Question 17

1. Heart muscle disease (problem in heart muscle)

2. INTRINSIC heart disease resulting from a primary abnormality in the myocardium

Answer 17

Cardiomyopathy (CMP)

Question 18

CMPs usually produce (2)

Answer 18

1. Abnormalities is cardiac wall thickness, chamber size

2. Mechanical and/or electrical dysfunction

Question 19

Clinical manifestations of CMP are either primary of secondary CMPs. Describe each.

Answer 19

1. Primary: confined to the heart

2. Secondary: part of a generalized systematic disorder

Question 20

Types of CMP (3)

Answer 20

1. Dilated CMP
2. Hypertrophic CMP
3. Restrictive CMP

Question 21

Type of CMP:

1. Progressive hypertrophy and global dilatation
2. Contractile and systolic dysfunction
3. HYPOcontracting heart
4. Can cause mitral regurgitation, abnormal cardiac rhythms

Answer 21

Dilated CMP

Question 22

S/Sx of dilated CMP (such as shortness of breath, easy fatigability, poor exertional capacity) are manifestations of?

Answer 22

Progressive CHF

Question 23

The ff are a causes of which CMP?

1. Idiopathic
2. Previous viral or complicated myocarditis (Coxsackie virus, enteroviruses)
3. Alcohol abuse, other toxins (cobalt, CO, Doxorubicin)
4. Pregnancy (peri- and postpartum CMP)
5. Genetic (autosomal dominant, defects in cytoskeleton)
6. Malnutrition (B12 and B1 deficiency)
7. Adriamycin therapy (dose related)
8. Cobalt added to beer

Answer 23

Dilated CMP

Question 24

This CMP is a disease of exclusion and it's negative criteria for its diagnosis include the ff: NO 1. Major coronary artery disease or anomaly 2. Valvular disease or anomaly 3. Systemic HPN 4. Shunts within or outside the heart

Answer 24

Dilated CMP

Question 25

Define dilated CMP

Answer 25

Progressive hypertrophy | Global dilatation

Question 26

The ff are gross features of which CMP? 1. Increased weight 2. Global enlargement 3. Generalized chamber dilatation (flabby) 4. Abnormal release of blood flow (due to mural thrombi) 5. Annular ring dilatation --> valvular dilatation --> regurgitation

Answer 26

Dilated CMP

Question 27

The ff are microscopic features of which CMP? 1. Hypertrophied muscle cells 2. Enlarged nuclei 3. Interstitial endocardium fibrosis 4. Subendocardial scars

Answer 27

Dilated CMP

Question 28

It is aka 1. Idiopathic Hypertrophic Subaortic Stenosis (IHSS) 2. Hypertrophic Obstructive CMP 3. Asymmetric Septal Hypertophy

Answer 28

Hypertrophic CMP

Question 29

What is the most important finding in hypertrophic CMP?

Answer 29

Myocardial hypertrophy

Question 30

In this type of CMP: | In the atrioventricular septum, the ventricular lumen is reduced --> abnormal diastolic filling, LV outflow obstruction

Answer 30

Hypertrophic CMP

Question 31

Hypertrophic CMP is characterized by? (2)

Answer 31

1. Heart is muscular and HYPERcontracting * remember that in dilated CMP the heart is HYPOcontracting 2. Increased/normal LV ejection

Question 32

Pathogenesis of Hypertrophic CMP (1)

Answer 32

MUTATION in at least one gene encoding proteins that are part of the sarcomere *most cases are familial, autosomal dominant

Question 33

What are the clinical features of hypertrophic CMP? (4)

Answer 33

1. Impaired diastolic filling 2. Exertional dyspnea 3. Harsh systolic murmur 4. Anginal pain

Question 34

The ff are major clinical problems seen in which CMP? 1. Atrial fibrillation with mural thrombus 2. Infective endocarditis of mitral valve 3. Intractable cardiac failure 4. Ventricular arrhythmias 5. Sudden death

Answer 34

Hypertrophic CMP

Question 35

Its gross features include the ff: 1. Massive myocardial hypertrophy WITHOUT DILATATION 2. Asymmetric septal hypertrophy (septum:wall ratio is >1.3) 3. BANANA-like ventricular cavity 4. Endocardium thickening 5. Mural plaque formation in the LV outflow tract 6. Anterior mitral valve thickening

Answer 35

Hypertrophic CMP

Question 36

Its microscopic features include the ff: 1. Extensive myocyte hypertrophy 2. HAPHAZARD myocardial fiber disarray 3. Interstitial fibrosis

Answer 36

Hypertrophic CMP

Question 37

1. Decreased cardiac compliance 2. Impaired ventricular filling 3. Decreased LV ejection fraction

Answer 37

Restrictive CMP (RCM)

Question 38

Its causes include the ff: 1. Idiopathic 2. Amyloidosis, Radiation, fibrosis, sarcoidosis 3. Immune or metabolic 4. Metastatic tumors 5. Deposition of metabolites (d/t inborn errors of metabolism)

Answer 38

Restrictive CMP

Question 39

Other types of RCM (3)

Answer 39

1. Endomyocardial fibrosis 2. Loeffler endomyocarditis 3. Endocardial fibroelastosis

Question 40

The ff describes which type of RCM? 1. Diffuse fibrosis of ventricular subendocardium 2. Markedly diminishes volume and compliance of affect chambers 3. Restrictive functional effect

Answer 40

Endomyocardial fibrosis

Question 41

The ff describes which type of RCM? 1. Eosinophilia 2. Endocardial fibrosis 3. Thrombosis

Answer 41

Loeffler endomyocarditis

Question 42

The ff describes which type of RCM? 1. Focal or diffuse fibroelastic thickening 2. Heart is infiltrated by fibroelastic tissue

Answer 42

Endocardial fibroelasatosis

Question 43

1. An inherited disease of cardiac muscle 2. Causes RV failure, ventricular tachycardia or fibrillation ---> sudden death 3. Autosomal-dominant inheritance 4. Variable penetrance 5. (+) defective cell adhesion proteins in desmosomes (link adjacent myocytes)

Answer 43

Arrythmogenic RV CMP/Dysplasia

Question 44

What disorder is characterized by 1. Arrythmogenic RV CMP 2. Hyperkeratosis of plantar and palmar skin surfaces associated with mutations in the gene encoding plakglobin

Answer 44

Naxos Syndrome

Question 45

The ff are gross characteristics of? 1. RV wall: severely thinner due to loss of myocytes 2. Extensive fatty infiltration and fibrosis

Answer 45

Arrhythmognic RV CMP/Dysplasia

Question 46

This includes inflammatory diseases of the myocardium that results in injury to cardiac myocytes but is NOT reflective of the injurious pattern related to IHD

Answer 46

Myocarditis

Question 47

What are the major causes of myocarditis? (4)

Answer 47

1. Infections 2. Immune-mediate reactions 3. Unknown 4. Cardiotoxic drugs

Question 48

The ff are clinical features of? 1. Can mimic AMI 2. Broad clinical manifestations 3. Entirely asymptomatic 4. Precipitous onset of heart failure or arrhythmias, occasionally with sudden death 5. May develop late CMP as a late complication

Answer 48

Myocarditis

Question 49

The ff symptoms are seen in? 1. Fatigue 2. Dyspnea 3. Palpitations 4. Precordial discomfort 5. Fever

Answer 49

Myocarditis

Question 50

The ff are gross features of? 1. Normal or dilated 2. Lesions are diffuse (if viral) or patchy (parasitic) 3. Hypertrophy (depends on disease duration) 4. Ventricular myocardium is flabby and mottled by focal foci/minute hemorrhagic lesions 5. Beefy red (d/t associated vascular congestion)

Answer 50

Myocarditis

Question 51

The ff are microscopic features of? 1. Interstitial inflammatory infiltrate associated with focal myocyte necrosis 2. Edema, vascular congestion 3. Focal necrosis, degeneration of myocytes adjacent to inflammatory cells 4. Viral inclusions, parasites may be present 5. A diffuse mononuclear, lymphocyte infiltrate is most common

Answer 51

Myocarditis

Question 52

Type of myocarditis: 1. Multinucleated giant cells 2. Lymphocytes, eosinophils, necrosis 3. Differential: sarcoid, hypersensitivity 4. Aggressive clinical course

Answer 52

Giant Cell Myocarditis (Fiedler's Myocarditis)

Question 53

Type of myocarditis: 1. Interstitial infiltrate: macrophages, eosinophils 2. No/little necrosis 3. No granuloma 4. Multinucleated giant cells may be present 5. Associated with adverse effects of methyldopa

Answer 53

Hypersensitivity myocarditis

Question 54

Types of myocarditis (2)

Answer 54

1. Giant cell myocarditis (Fiedler's Myocarditis) | 2. Hypersensitivity myocarditis

Question 55

1. Anomalies of the heart or great vessels present from birth 2. Arises from faulty embryogenesis (1st trimester: 3-8th week when major cv structure develop)

Answer 55

CHD

Question 56

Etiology of Coarctation of Aorta

Answer 56

Trisomy

Question 57

Etiology of ASD, VSD, PDA

Answer 57

Down's Trisomy 21

Question 58

Etiology of CHD (5)

Answer 58

1. Genetic 2. Infection (viruses) 3. Chemical substances 4. Multifactorial 5. Idiopathic

Question 59

In Holt-Oram Syndrome (CHD): 1. Gene affected? 2. Congenital cardiac disease (3)

Answer 59

1. TBX5 | 2. ASD, VSD, conduction defects

Question 60

In Di'George Syndrome (CHD): 1. Gene affected? 2. Congenital cardiac disease (1)

Answer 60

1. TBX1 | 2. Cardiac outflow tract defects

Question 61

In Alagille Syndrome (CHD): 1. Gene affected? 2. Congenital cardiac disease (2)

Answer 61

1. JAG1, NOTCH2 | 2. Pulmonary artery stenosis

Question 62

In Marfan Syndrome (CHD): 1. Gene affected? 2. Congenital cardiac disease (2)

Answer 62

1. Fibrillin | 2. Aortic aneurysm, valve abnormalities

Question 63

Th ff are clinical effects of? 1. Failure to thrive 2. Cyanosis at birth (R-L shunt) 3. Cardiac failure 4. Pulmonary HPN 5. Infective endocarditis

Answer 63

CHD

Question 64

What are the classifications of CHD? (3)

Answer 64

1. Left to right shunt anomaly (acyanotic) 2. Right to left shunt anomaly (cyanotic) 3. Obstructive anomaly

Question 65

Which defects are a categorized under Left to Right shunt anomaly? (4)

Answer 65

1. ASD 2. VSD 3. AVSD 4. PDA

Question 66

Which defects are a categorized under Right to Left shunt anomaly? (5)

Answer 66

1. Tetralogy of Fallot 2. Transposition of great vessels 3. Truncus arteriosus 4. Tricuspid atresia 5. Total anomalous pulmonary venous return

Question 67

Which defects are a categorized under obstructive anomaly? (3)

Answer 67

1. Coarctation of Aorta 2. Pulmonary stenosis or atresia 3. Aortic stenosis or atresia

Question 68

Type of LR shunt: 1. Opening in the atrial septum --> communication of blood between LA and RA 2. Acyanotic at birth 3. Most common is a patent foramen ovale 4. Its late effect is pulmonary HPN 5. The most common CHD that is asymptomatic

Answer 68

ASD

Question 69

It is the most common CHD that is ASYMPTOMATIC

Answer 69

ASD

Question 70

Types of ASD (3)

Answer 70

1. Septum Secundum ASD 2. Septum Primum ASD 3. Sinus Venosus Defect

Question 71

Type of ASD: 1. 90% of ASD 2. Defect in fossa ovale 3. May be solitary or with another anomaly

Answer 71

Septum Secundum ASD

Question 72

Type of ASD: 1. 5% ASD 2. Defect near AV valve 3. Associated with a clefted anterior mitral valve (partial ASD)

Answer 72

Septum Primum ASD

Question 73

Type of ASD: 1. 5% of ASD 2. Located at the top, near entrance of SVC 3. Associated with anomalous pulmonary venous return to RA 4. Associated with TAPVR

Answer 73

Sinus Venosus defect

Question 74

The ff are effects of which LR shunt anomaly? 1. Right atrial enlargement and hypertrophy 2. Right ventricular enlargement and hypertrophy 3. Increased pulmonary blood flow --> pulmonary HPN 4. Murmur due to increased blood flow to pulmonary valve

Answer 74

ASD

Question 75

1. Incomplete closure of ventricular septum --> free communication between RV and LV 2. Most common CHD 3. 30% isolated anomaly 4. Most are associated with other congenital cardiac defects such as Tetralogy of Fallot 5. ACYANOTIC at birth 6. Can have associated mitral valve abnormality

Answer 75

VSD

Question 76

Types of VSD (3)

Answer 76

1. Membranous VSD 2. Infundibular VSD 3. Muscular VSD

Question 77

Type of VSD: 1. 90% of VSD 2. Located in upper portion of interventricular septum

Answer 77

Membranous VSD

Question 78

Type of VSD: | 1. Located below pulmonary valve

Answer 78

Infundinular VSD

Question 79

Type of VSD: 1. Found in muscular wall of interventricular septum 2. Spontaneous closure in 50% of cases

Answer 79

Muscular VSD

Question 80

Type of CHD: 1. Abnormal development of AV canal 2. Failure of fusion of superior and inferior endocardial cushions 3. Incomplete closure of atrioventricular septum 4. Inadequate formation of tricuspid and mitral valves

Answer 80

Atrioventricular Septal Defect (AVSD)

Question 81

More than 1/3 of those with complete AVSD have what other disorder/syndrome?

Answer 81

Down's Syndrome

Question 82

Types of AVSD (2)

Answer 82

1. Partial AVSD | 2. Complete AVSD

Question 83

Type of AVSD: | 1. Combination of Primum ASD + a cleft anterior mitral valve

Answer 83

Partial AVSD

Question 84

Type of AVSD: | 1.Total absence of atrial and ventricular septum --> free communication of all four chambers

Answer 84

Complete AVSD

Question 85

Type of CHD: 1. Persistence of communication between pulmonary artery and aorta via ductus arteriosus 2. 90% isolated anomaly 3. 10% associated with VSD, coarctation, pulmonary or aortic stenosis 4. Classic sign: machinery murmur 5. Late effect: pulmonary HPN

Answer 85

PDA

Question 86

What is the classic sign of PDA?

Answer 86

Machinery murmur

Question 87

What is given to preserve ductal patency (for infants with various forms of CHD with obstruct pulmonary or systemic blood flow such as on aortic valve atresia)?

Answer 87

Prostaglandin E

Question 88

What are the (4) components of a Tetralogy of Fallot?

Answer 88

1. VSD 2. Subpulmonic stenosis 3. Aorta overriding VSD 4. Right ventricular hypertrophy

Question 89

What is the morphological feature of tetralogy of Fallot?

Answer 89

Boot-shaped heart | due to marked RV hypertrophy, particularly the apical region

Question 90

In tetralogy of Fallot, the degree of cyanosis is dependent on the degree of pulmonary stenosis. 1. Severe pulmonary stenosis is what type of shunt and leads to what? 2. Mild pulmonary stenosis is what type of shunt and leads to what?

Answer 90

1. Right to left shunt --> classic tetralogy of Fallot | 2. Left to right shunt --> no cyanosis --> pink tetralogy of Fallot

Question 91

In transposition of great vessels, there is ventriculo-arterial discordance. Define ventriculo-arterial discordance.

Answer 91

1. AoRta arises from the Right ventricle (it's normally a the LV) 2. PuLmonary artery arises from the Left ventricle (normally at RV)

Question 92

In this type of CHD, there is switching of the aorta and pulmonary artery

Answer 92

Transposition of great vessels

Question 93

What is the result of transposition of great vessels?

Answer 93

Separation of systemic and pulmonary circulations | incompatible with life

Question 94

What is the embryo logic deft in complete transposition?

Answer 94

Abnormal formation of the truncal and aortopulmonary septa

Question 95

Type of CHD: 1. Developmental failure of separation of the embryologic truncus arteriosus into the aorta and pulmonary artery 2. Result: single great artery that receives blood from both ventricles, gives rise to the systemic, pulmonary, and coronary circulations 3. Associated lesion: VSD 4. Incompatible with life unless combined with another defect (PDA, ASD, VSD)

Answer 95

Truncus Arteriosus

Question 96

Type of CHD: 1. Complete occlusion of tricuspid valve (d/t unequal division of atrioventricular canal) 2. Incompatible with life unless combined with another defect 3. Associated lesions: large mitral valve underdeveloped RV, ASD, VSD, PDA 4. Early cyanosis 5. High mortality rate

Answer 96

Tricuspid atresia

Question 97

Type or CHD: 1. Pulmonary veins drain into left innominate vein or coronary sinus (instead of LA) 2. Needs a patent foramen ovale or VSD for blood to go back to left side of heart

Answer 97

Total anomalous pulmonary venous return (TAPVR)

Question 98

Th ff are morphological features of? 1. Right atrial and ventricular dilatation 2. Pulmonary trunk dilatation 3. Hypoplastic LA 4. LV is normal in size

Answer 98

Total anomalous pulmonary venous return

Question 99

Type of Obstructive anomaly: | 1. (+) obstruction at a segment of the aorta

Answer 99

Coarctation of Aorta

Question 100

Types of coarctation of aorta (2)

Answer 100

1. Infantile coarctation (constriction Before ductus arteriosus = Baby) 2. Adult coarctation (constriction After ductus arteriosus = Adult)

Question 101

Type of coarctation of aorta: | 1. Tubular hypoplasia of aortic arch proximal to PDA

Answer 101

Infantile Coarctation

Question 102

Type of coarctation of aorta: | 1. Ridge-like coarctation of aorta at level of ductus arteriosus

Answer 102

Adult coarctation

Question 103

Type of Obstructive Anomaly: 1. Obstruction at pulmonary valve 2. With or without cyanosis 3. S/Sx dependent on size of pulmonary stenosisl 4. May be an isolated event or part of a complex anomaly

Answer 103

Pulmonary Stenosis or Atresia

Question 104

The ff are associated lesions of which obstructive anomaly? 1. RV hypertrophy 2. Pulmonary artery hypoplasia 3. RV hypoplasia in pulmonary valve atresia 4. ASD

Answer 104

Pulmonary Stenosis or atresia

Question 105

Type of obstructive anomaly: 1. Narrowing/obstruction of aorta 2. Adaptive response: LVH

Answer 105

Aortic stenosis or atresia

Question 106

What are the types of aortic stenosis or atresia? (3)

Answer 106

1. Valvular aortic stenosis 2. Subaortic stenosis 3. Supravalvular stenosis

Question 107

Adaptive response of aortic stenosis

Answer 107

LVH

Question 108

Type of aortic stenosis: | 1. Cusps may be hypoplastic, dysplastic, abnormal in number

Answer 108

Valvular aortic stenosis

Question 109

Type of aortic stenosis: 1. Thickened ring or collar of dense endocardial fibrous tissue below the level of the cusps 2. Associated with prominent systolic murmur

Answer 109

Subaortic stenosis

Question 110

Type of aortic stenosis: | 1. Represent an inherited form of aortic Dysplasia: ascending aortic wall is thickened --> luminal constriction

Answer 110

Supravalvular aortic stenosis

Question 111

Types of valvular disease (6)

Answer 111

1. Stenosis 2. Insufficiency 3. Functional regurgitation 4. Singular valvular disease 5. Mixed stenosis/regurgitation 6. Combined multiple valvular disease

Question 112

Type of Valvular Disease: | 1. Failure of a valve to OPEN completing thus impending blood flow

Answer 112

Stenosis

Question 113

Type of Valvular Disease: | 1. Failure of a valve to CLOSE completely thus allowing regurgitation

Answer 113

Insufficiency

Question 114

Type of Valvular Disease: | 1. Incompetence of a valve resulting from an abnormality on one of its support structures

Answer 114

Functional regurgitation

Question 115

Type of Valvular Disease: | 1. Mitral valve is commonly affected

Answer 115

Singular valvular disease

Question 116

Type of Valvular Disease: 1. Occurs when valvular stenosis and regurgitation coexist (Example. RHD)

Answer 116

Mixed stenosis/regurgitation

Question 117

Causes of valvular disease (2)

Answer 117

1. Congenital valvular heart disease | 2. Acquired valvular heart disease

Question 118

Examples of congenital valvular heart disease (3)

Answer 118

1. Congenital bicuspid valve 2. Congenital calcific aortic stenosis 3. Congenital mitral valve prolapse

Question 119

Examples of acquired valvular heart disease (2)

Answer 119

1. Rheumatic valvulitis | 2. Infective endocarditis

Question 120

Factors that affect clinical impact of valvular disease (4)

Answer 120

1. Valve impairment m 2. Degree of impairment 3. Rate/duration of rate of involvement 4. Rate and quality of compensatory mechanism

Question 121

Major valvular heart diseases (7)

Answer 121

1. Calcific aortic valve stenosis 2. Congenital bicuspid aortic valve calcification 3. Mitral annular calcification 4. Infective endocarditis 5. Non-bacterial thrombotic endocarditis 6. Endocarditis in systemic lupus erythematosus (SLE) 7. Carcinoid heart disease

Question 122

1. The most common of all valvular abnormalities 2. Acquired, age related 3. "Wear and tear"

Answer 122

Calcified aortic valve stenosis

Question 123

Morphologic hallmark of calcified aortic valve stenosi

Answer 123

Heaped-up calcified masses within aortic cusps | (No commissural fusion

Question 124

(3) Clinical manifestations of calcified aortic valve stenosis

Answer 124

1. LVH (Increased LV pressure 200mmHg) 2. Angina Pectoris (Diminished microcirculations of hypertrophied myocardium) 3. Chronic Heart Failure, Syncope (Impaired systolic and diastolic myocardial functions)

Question 125

1. The most frequent congenital cardiovascular malformation in humans 2. Cusps are usually of unequal size (larger one has a midline raphe due to incomplete commissural separation during development) 3. Associated with: aortic dissection, aortic coarctation, aneurysm

Answer 125

Congenital Bicuspid Aortic Valve Calcification

Question 126

This is frequently the major site for calcific deposits (Congenital Bicuspid Aortic Valve Calcification)

Answer 126

Raphe

Question 127

1. Degenerative calcific deposits that developed in the peripheral annulus of the mitral valve 2. Common in elderly women (60 years old), individuals with mitral valve prolapse, with elevated left ventricular pressure 3. Doesn't affect valvular function

Answer 127

Mitral Annular Calcification

Question 128

What is the gross appearance of Mitral Annular Calcification

Answer 128

Irregular, stony hard, ulcerated nodules behind the mitral leaflets

Question 129

What are the etiological agents in Infective Endocarditis? (5)

Answer 129

1. Streptococcus viridans 2. Staphylococcus aureus 3. HACEK Group 4. Rickettsia 5. Chlamydia

Question 130

The ff are predisposing disease to which Valvular Disease? 1. Rheumatic valvulitis 2. Degenerative atherosclerotic/syphilitic valve disease 3. Congenital defects 4. Prosthetic heart valves

Answer 130

Infective Endocarditis

Question 131

Types of Infective Endocarditis (2)

Answer 131

1. Acute IE | 2. Subacute IE

Question 132

The ff describe which type of valvular disease? 1. Occurs in previously normal valve 2. Bulky vegetation that may produce erosions/valvular ruptures 3. Produces Necrotizing, ulcerative, destructive lesions 4. Severe clinical s/sx, high morbidity 5. Difficult to cure, requires surgery

Answer 132

Acute IE

Question 133

The ff describe which type of valvular disease? 1. Occurs in previously diseased valve 2. Histology: granulation tissue indicative of healing (at base) 3. Caused by less virulent organisms, insidious clinical onset

Answer 133

Subacute IE

Question 134

1. A serious infection characterized by colonization/invasion of heart valves or mural endocardium by a microbe 2. Frequently involves AV or MV 3. Tricuspid valve involved in drug mainliners

Answer 134

Infective Endocarditis

Question 135

Hallmark of IE

Answer 135

Friable, bulky, potentially destructive vegetation (contain fibrin, inflammatory cells, bacteria)

Question 136

The ff are clinical features of: 1. Fever 2. Nonspecific signs: weight lodge, fatigue, flu-like symptoms, anorexia 3. Murmurs

Answer 136

Infective endocarditis

Question 137

What is the most consistent sign in IE? (Clinical feature)

Answer 137

Fever

Question 138

What are the embolic complications seen in IE? (3)

Answer 138

1. Janeway Lesions 2. Osler Nodes 3. Roth Spots

Question 139

Embolic Complication (IE): 1. Small erythematous or hemorrhagic, macular, non-tender lesions 2. Palms, soles 3. The consequence of septic embolic events

Answer 139

Janeway lesions

Question 140

Embolic Complication (IE): 1. Small tender subcutaneous nodules 2. Pulp of digits, fingers

Answer 140

Osler Nodes

Question 141

``` Embolic Complication (IE): 1. Oval retinal hemorrhages with pale centers ```

Answer 141

Roth spots

Question 142

Type of vascular disease: 1. Sterile, nondestructive, noninvasive vegetations 2. Does not elicit inflammation 3. Associated with debilitated patients, carcinoma, sepsis, cachexia 4. May be part of Trousseau Syndrome of migratory thrombophlebitis

Answer 142

Non-bacterial Thrombotic Endocarditis (NBTE)

Question 143

Pathogenesis of NBTE (3)

Answer 143

1. Hypercoagulable states 2. Malignancy 3. Istrumentation

Question 144

The ff are gross features of which valvular disease? 1. Small nodules along the lines of closure 2. Bulky and friable, may be single or multiple 3. Leaflets are often normal 4. Atrial and mitral valve

Answer 144

NBTE

Question 145

The ff are gross features of which valvular disease? 1. Bland thrombi loosely attached to underlying valve 2. No inflammation 3. No organism identified/present

Answer 145

NBTE

Question 146

Which valvular disease is associated with SLE?

Answer 146

Libman-Sacks Endocarditis

Question 147

Which valves are affected by Libman-Sacks Endocarditis? (2)

Answer 147

1. MV | 2. TV

Question 148

Type of valvular disease: 1. Sterile, small vegetations on the undersurface of cords, mural (sterile vegetations beneath valves) 2. Fibrinoid necrosis of valves

Answer 148

Libman-Sacks Endocarditis

Question 149

The are histologic features of which valvular disease? | 1. Finely granular, fibrinous eosinophilic material containing "hematoxylin bodies"

Answer 149

Libman-Sacks Endocarditis

Question 150

What constitutes Carcinoid Syndrome? (5)

Answer 150

1. Episodic skin flushing 2. Cramps 3. Nausea 4. Vomiting 5. Diarrhea

Question 151

Right sided endocarditis

Answer 151

Carcinoid endocarditis/heart disease

Question 152

Which valves are affected by carcinoid endocarditis? (2)

Answer 152

1. Pulmonary valve | 2. Tricuspid valve

Question 153

Lesion seen in carcinoid endocarditis (1)

Answer 153

Endocardial thickening/fibrosis

Question 154

In this valvular disease, there is elaboration of serotonin, histamine, bradykinin, prostaglandin, gastroactive amines

Answer 154

Carcinoid endocarditis

Question 155

An acute immunologically mediated, multiway stem inflammatory disease following group A streptococci pharyngitis

Answer 155

Rheumatic Fever

Question 156

Rheumatic fever is an effect of what?

Answer 156

An antibody against streptococcus | It is not a direct effect of strep

Question 157

What are the clinical features or rheumatic fever (major criteria of jones)? (5)

Answer 157

1. Carditis 2. Arthritis 3. Subcutaneous nodules 4. Erythema 5. Sydenham's Chorea (neurologic disorder, epileptic-like involuntary rapid, muscular, purposeless movement)

Question 158

What are the clinical features or rheumatic fever (minor criteria of jones)? (5)

Answer 158

1. Fever 2. Arthralgia 3. C-reactive protein 4. ESR (erythrocyte sedimentation rate) 5. Anti-streptolysin O

Question 159

In RF, what will you see if pericardium is affected? (Gross)

Answer 159

Fibrinous pericarditis or bread and butter appearance

Question 160

In RF, what will you see if myocardium is affected? (Histo)

Answer 160

Aschoff body in INTERSTITIUM (not muscle cells)

Question 161

In RF, what will you see if endocardium is affected? (Gross)

Answer 161

Verrucal vegetations (on lines of closure)

Question 162

Plump activated macrophages

Answer 162

Anitschkow cells aka caterpillar cells (zig zag pattern)

Question 163

Necrotic vegetations on lines of valve closure that resemble rosary beads Hint: RF

Answer 163

Verrucae

Question 164

Irregular thickenings, usually in LA caused by subendocardial lesions Hint: RF

Answer 164

MacCallum bodies

Question 165

It is a complication of recurrent bouts of RF

Answer 165

Rheumatic heart disease (RHD)

Question 166

The ff are Morphologic features of which valvular disease? 1. Multivalvular (MV --> AV --> TV) 2. Commissural fusion of cusps --> stenosis 3. Fish mouth deformity / buttonhole stenosis 4. Shortening/thickening of chordae 5. Fibrosis

Answer 166

RHD

Question 167

What are the four major forms of vegetative endocarditis?

Answer 167

1. RHD 2. IE 3. NBTE 4. LSE

Question 168

What is the key histologic change in mitral valve prolapse?

Answer 168

Myxomatous degeneration of mitral valve Notes 1. Mitral valve is enlarged, hooded, or floppy --> tendency to prolapse into LA during systole 2. Developmental anomaly of connective tissue 3. Most are idiopathic 4. Fibrillin 1 mutations (Marfan's Syndrome)

Question 169

The ff are gross features of which valvular disease? 1. Interchordal hooding or ballooning of MV into left atrium 2. MV leaflets are floppy, enlarged, redundant, thickend, rubbery, fibrotic, calcified 3. Associated tendinous cords may be elongated, thinned, ruptured 4. Annular dilatation is common

Answer 169

MVP

Question 170

The ff are clinical features if which valvular diseas? 1. Majority are asymptomatic 2. Mid-systolic click 3. Late systolic or holosystolic murmur from mitral regurgitation 4. Symptoms: chest pain, dyspnea, fatigue, depression

Answer 170

MVP

Question 171

Complications of MVP (4)

Answer 171

1. IE 2. Mitral insufficiency 3. Thrombo-embolic complications 4. Arrhythmias

Question 172

Disadvantage of a bioprosthetic valve (2)

Answer 172

1. Mechanical detioration | 2. Calcification

Question 173

Disadvantage of a man-made prosthetic valve (1)

Answer 173

1. Thrombi formation

Question 174

Types of pericardial disease (6)

Answer 174

1. Serous pericarditis 2. Fibrinous 3. Hemorrhagic 4. Purulent 5. Caseous 6. Healed

Question 175

Type of pericardial disease: 1. Causes: RHD, SLE, uremia, viral 2. Scanty inflammation 3. (+/-) pericardial serous effusion 4. Rare sequelae: organization fibrosis 5. Histology: mild inflammatory infiltrate in epipericardial fat (mostly lymphocytes)

Answer 175

Serous pericarditis

Question 176

Type of pericardial disease: 1. Causes: RHD, post-radiation, post-infarction, AMI, Dressler Syndrome 2. The most common type of pericarditis 3. Shaggy, rough pericardium (bread and butter) 4. Sequelae: resolution, lysis, organization 5. Most striking characteristic: loud pericardial rub

Answer 176

Fibrinous pericarditis

Question 177

It is a secondary form of pericarditis that occurs in th stating of injury to the heart or pericardium. It consists of a triad of features: 1. Fever 2. Pleuritic pain 3. Pericardial effusion

Answer 177

Dressler Syndrome

Question 178

What is the triad of features of Dressler Syndrome?

Answer 178

1. Fever 2. Pleuritic pain 3. Pericardial effusion

Question 179

Type of pericardial disease: 1. Causes: TB, tumor metastasis, bacteria 2. Mixture of a huge purulent and fibrinous effusion 3. Exudate: blood + fibrinous or suppurative effusion

Answer 179

Hemorrhagic pericarditis

Question 180

Pathways of spread of Purulent Endocarditis (3)

Answer 180

1. Hematogenous seeding 2. Lymphatic extension 3. Iatrogenic

Question 181

Type of pericardial disease: 1. Rare type 2. Tuberculous in origin 3. A frequent antecedent of disabling, fibrocalcific, chronic constrictive pericarditis

Answer 181

Caseous Pericarditis

Question 182

Type of pericardial disease: 1. Organization produces "soldier's plaque" (plaque-like fibrous thickenings) 2. Rarely causes impairment of cardiac function

Answer 182

Healed pericarditis

Question 183

(2) types of healed pericarditis

Answer 183

1. Adhesive mediastinopericarditis | 2. Constrictive pericarditis

Question 184

Type of healed pericarditis: | 1. Obliteration of pericardial sac with adhesion to parietal pericardium

Answer 184

Adhesive mediastinopericarditis

Question 185

Type of healed pericarditis: 1. Obliterated sac with encasement by dense fibrous tissue 2. Diastolic expansion

Answer 185

Constrictive pericarditis

Question 186

Types of cardiac neoplasms (2)

Answer 186

1. Myxoma | 2. Rhabdomyoma

Question 187

Type of cardiac neoplasm: 1. Most common primary cardiac tumor in adults 2. Associated with clonal abnormalities of chromosomes 12 and 17 3. 90% located in LA 4. Complication: ball-valve obstruction and embolization

Answer 187

Myxoma

Question 188

Favored site of origin of myxoma

Answer 188

Fossa ovalis in atrial septum

Question 189

The ff are gross features of which cardiac neoplasm? 1. Sessile or pedunculated masses (globular) 2. Soft, translucent, papillary, or villus lesions 3. Gelatinous appearance

Answer 189

Myxoma

Question 190

Type of cardiac neoplasm: 1. Most frequent primary cardiac tumor in children 2. Location: ventricular chamber 3. Causes valvular or outflow tract obstruction

Answer 190

Rhabdomyoma

Question 191

The ff are gross features of which cardiac neoplasm? 1. Gray-white, fleshy, myocardial masses 2. Location: either on left or right side of heart, extending in ventricular Chambers

Answer 191

Rhabdomyoma

Question 192

The ff are microscopic features of which cardiac neoplasm? 1. Large, rounded, or polygonal cells 2. Cytoplasmic glycogen separated by strands of cytoplasm running from plasma membrane to nucleus

Answer 192

Rhabdomyoma