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CV II Exam 2 > More heart pathology from day 2 > Flashcards

More heart pathology from day 2 Flashcards

1
Q

Carcinoid heart disease is fibrous thickening of the endocardial surfaces accompanying carcinoid syndrome. What do you see with the smooth muscle?

What is shown in the movat stain image on the right?

What is the key thing that metastatic carcinoid tumors secrete?

A

Smooth muscle proliferation

the histological slide shows intimal thickening with myocardial elastic tissue underlying the acid mucopolysaccharide-rich lesion.

Secrete serotonin (5HT)

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2
Q

What is a key consideration for those who are fitted with a cardiac valve prostheses?

A

Requires anticoagulation therapy to deal with mechanical hemolysis.

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3
Q

What are soem complications of cardiac valve prostheses?

A

Thrombosis/thromboembolism

Anticoagulant-related hemorrhage

Prosthetic valve endocarditis

Structural deterioration (intrinsic)

  • wear
  • fracture
  • poppet failure in ball valves
  • cuspal tear
  • calcification
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4
Q

What complication is shown here?

A

Thrombosis of a mechanical prosthetic valve

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5
Q

What complication is shown here?

A

Calcification with secondary tearing of a porcine bioprothetic heart valve

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6
Q

What is going on with this heart?

A

Dilated cardiomyopathy

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7
Q

What is going on with this heart?

A

Restrictive cardiomyopathy

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8
Q

What is wrong with this heart?

A

Hypertrophic cardiomyopathy

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9
Q

What does cardiomyopathy arise from?

A

Primary abnormality in the myocardium.

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10
Q

What are most cases of cardiomyopathy d/t?

A

Primarily idiopathic, genetic or acquired secondary as part of systemic or multiorgan disorder

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11
Q

90% of cardiomyopathies are of what type?

A

Dilated - Large, flabby heart

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12
Q

Hypertrophic hearts are only 6-8% of cases, and show what?

A

Markedly thickened left ventricle. (His example shows hypertrophy in both ventricles, I suppose left is just more significant/common)

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13
Q

Restrictive cardiomyopathy is relatively uncommon. What sort of disorders lead to this?

A

Infiltrative disorders

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14
Q

By what means do we assess the etiology of cardiomyopathy?

A

Endomyocardial biopsy

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15
Q

In dilated cardiomyopathy LVEF is less than 40% of normal. What is the mechanism of heart failure?

A

Impaired contractility leads to systolic dysfunction

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16
Q

What are some causes of dilated cardiomyopathy?

A

Genetic; alcohol; peripartum; myocarditis; hemochromatosis; chronic anemia; doxorubicin (Adriamycin); sarcoidosis; idiopathic

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17
Q

In hypertrophic cardiomyopathy the LVEF 50-80% nl is around 40-65%. What is the mechanism of heart failure in this condition?

A

Impairment of compliance

(diastolic dysfunction)

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18
Q

What are some causes of the hypertrophic cardiomyopathy phenotype?

A

Genetic; Friedreich ataxia; storage diseases; infants of diabetic mothers

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19
Q

In restrictive cardiomyopathy the LVEF is ssomewhere between 25% and 90%. What is the mechanism of heart failure?

A

Impairment of compliance - leading to diastolic dysfunction

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20
Q

What are some examples of things that cause restrictive cardiomyopathy?

A

Amyloidosis; radiation-induced fibrosis; idiopathic

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21
Q

What cardiac infections are associated with heart muscle diseases?

A

Viruses

\Chlamydia

Rickettsia

Bacteria (go fucking figure)

Fungi

Protozoa

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22
Q

Dilated cardiomyopathy is an insidious, slowly progressive, intractable congestive heart failure. What are the important etiologic associations?

A
  1. Myocarditis d/t coxsackie B and other enteroviruses
  2. ETOH both directly through toxicity and indirectly through Thiamine deficiency. (Beriberi Disease)
  3. Peripartum Cardiomyopathy
  4. Iron Overload
  5. Familial (genetic) via an autosomal dominant pattern most commonly. Presenting first in kids

1.

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23
Q

Dilated cardiomyopathy is frequently seen between 20 and 50 years of age in familial DCM. What do most of these patients have?

What is somewhat unique about this type of whatever the answer above is?

A

Symptomati congestive heart failure (CHF)

Poor response to traditional CHF treatments

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24
Q

In DCM, what do outcomes look like one LVEF is below 25%

A
  • ½ die in 2 years or less
  • only 25% survive >5 years

This is the most common indication for cardiac transplantation

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25
Q

What is the size of this heart likely to be?

What do you see indicated by the white arrow?

What do you see in the histology image?

A

700 - 1000 grams

Mural thrombus at the arrow

Histology reveals variable myocyte hypertrophy and interstitial fibrosis

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26
Q

What are the valves and coronary arteries like in DCM?

A

Normal on both counts, free from narrowing

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27
Q

On what chromosome do you find the coding sequence for B-myosin heavy chain?

A

Chromosome 14

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28
Q

What is the problem with this heart?

A

Arrythmogenic Right Ventricular Cardiomyopathy

Right sided heart failure, with rhythm disturbances. The myocytes are replaced with adipocytes and fibrous tissue.

(Arrythmogenic Right Ventricular Dysplasia)

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29
Q

What rhythm might you see in arrythmogenic right ventricular cardiomyopathy?

A

V-Tach

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30
Q

What are the mutations we need to be aware of regarding arrythmogenic right ventricular cardiomyopathy?

A

Autosomal dominant mutations in plakoglobin (chr 17) and desmoplakin (chr 16) (desmosomes)

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31
Q

A patient presents with palmoplantar keratoderma with arrhythmogenic right ventricular cardiomyopathy and woolly hair (recessive plakoglobin mutation) .

What do they have?

A

Naxos Syndome

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32
Q

Patient presents with palmoplantar keratoderma with left ventricular cardiomyopathy and woolly hair (recessive desmoplakin mutation)
What does this patient have?

A

Carvajal Syndrome

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33
Q

What are the four major causes of myocarditis in the US?

A
  1. Viral - coxsackie viruses A and B (and other enteroviruses)
  2. Lyme disease (borrelia burgdorferi)
  3. Hypersensitivity (eosiniphilic) myocarditis
  4. Trichinosis
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34
Q

What is the big cause of S. american myocarditises?

A

Chagas disease from T. Cruzii

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35
Q

What type of myocarditis is shown here?

A

Lymphocytic myocarditis, associated with myocyte injury

36
Q

What type of myocarditis is shown here?

A

Hypersensitivity myocarditis

Note the interstitial inflammatory infiltrate composed largely of eosinophils and mononuclear inflammatory cells.

37
Q

What is the myocarditis type shown here?

A

Giant-cell myocarditis

Note the mononuclear inflammatory infiltrate containing lymphocytes and macrophages with extensive loss of muscle and the presence of multinucleated giant cells (fused macrophages)

38
Q

What type of myocarditis does this man from costa rica have?

A

Myocarditis of Chagas disease

Note the arrow indicating the myofiber that is distended with Trypanosomes. Also visible is indivual myocyte necrosis.

39
Q

Hypertrophic cardiomyopathy may also be referred to as?

A
  • •Idiopathic hypertrophic subaortic stenosis (IHHS)
  • Hypertrophic obstructive cardiomyopathy
40
Q

The features of hypetrophic cardiomyopathy include myocardial hypertrophy, abnormal diastolic filling, and intermittent ventricular outflow obstruction with a systolic ejection murmur in 1/3 of cases.

What typically mutated in this cardiomyopathy?

A

Mostly dominant mutations in proteins of the sacromere. Specifically, mutations of β-myosin heavy chain gene on Chr. 14

41
Q

What is the course that most patients have in HCM?

A

Stable and non-progressive

42
Q

What is the major clinical limitation in HCM?

What do 10-20% develop though?

What occurs in a small percentage?

A

Exertional dyspnea

10-20% atrial fibrillation →mural thrombus →systemic emboli

Sudden cardiac death

43
Q

HCM is one of the most common causes of…

A

cardiovascular-related sudden cardiac death in young people, especially competitive athletes (most often in football and basketball)

44
Q

What are the gross pathological findings of hypertrophic cardiomyopathy?

A

•Massive myocardial hypertrophy without ventricular dilatation
•Disproportionate thickening of left ventricular septum in comparison to LV free wall (asymmetrical septal hypertrophy)
•Left ventricular chamber decreased in size and configuration by the hypertrophic septal wall
•Hypertrophic LV septum most prominent in subaortic region where it may cause obstruction to LV outflow in 1/3

45
Q

What are the histopathologic findings seen in HCM?

A

•Marked myocyte hypertrophy: Cross-sectional diameter of myocytes approach 40 microns (normal ~ 15 microns)

•Haphazard myofiber disarray
•Interstitial and replacement fibrosis in myocardium

46
Q

What is shown here?

A

HCM

47
Q

Compare the ventricle in HCM vs. DCM

A

HCM - Firm with a small lumen and thick wall

DCM - Dilated and flabby

48
Q

Compare the force generated by the heart in HCM vs DCM

A

HCM - Hypercontracting

DCM - hypocontracting

49
Q

Characterize the dysfunctional portion of the heart cycle for HCM vs DCM

A

HCM - diastolic dysfunction

DCM - Systolic dysfunction

50
Q

Compare the clinical presentation (most common) of HCM vs DCM

A

HCM - Exertional Dyspnea

DCM - CHF

51
Q

What are the mutated components in HCM? DCM?

A

HCM - Sarcomere proteins

DCM - Cytoskeleton proteins

52
Q

Restrictive cardiomyopathy is characterized by primary decrease in ventricular compliance, resulting in impaired ventricular filling during diastole with systolic LV function usually unaffected.

What are the heart findings in this condition?

A
  • Ventricles usually normal size or slightly enlarged
  • Ventricular chambers are normal size
  • Biatrial enlargement common
53
Q

What are the associated disorders with Restrictive cardiomyopathy?

A
  1. Amyloidosis
  2. endomyocardial fibrosis
  3. Loeffler Endomyocarditis
  4. Endocardial fibroelastosis
54
Q

Interstitial deposition of amyloid protein results in restrictive cardiomyopathy which may be complicated by arrhythmias. What are the two main types of amyloidosis that impact the heart?

What is the third, token one?

A
  1. Systemic senile (cardiac) amyloidosis - amyloidosis primarily in the heart. Occurs in “aged” patients over 60 yrs.
  2. Primary systemic amyloidosis - chronic disease that involves most visceral organs and almost always involves the interstitium of the myocardium.
  3. Isolated atrial amyloidosis - rare, ANP deposition
55
Q

What do you see here?

What is the stain used on the right?

A

Cardiac amyloidosis

Left - hematoxylin and eosin stain showing amyloid appearing as amorphous pink material around myocytes.

Right - Congo red stain showing characteristic apple-green birefringence of amyloid.

56
Q

What are the disorders that are considered channelopathies?

A
  1. Long QT syndrome
  2. Short QT syndrome
  3. Brugada syndrome
  4. CVPT syndrome
57
Q

What are the four genes involved in long qt syndrome? What is each responsible for?

A

KCNQ1 - K+ channel (LOF)

KCNH2 - K+ channel (LOF)

SCN5A - Na+ channel (GOF)

CAV3 - Caveolin, Na current (GOF)

58
Q

What are the genes associated with short QT syndrome? What ions are they associated with?

A

KCNQ1 - K+ channel (GOF)

KCNH2 - K+ channel (GOF)

59
Q

What genes are associated with Brugada Syndrome? What ions?

A

SCN5A - Na+ channel (LOF)

CACNB2b - Ca++ channel (LOF)

SCN1b - Na+ channel (LOF)

60
Q

What genes are associated with CVPT syndrome?

What is the function of these gene products?

A

RYR2 - Diastolic Ca++ release (GOF)

CASQ2 - Diastolic Ca++ release (LOF)

61
Q

________________syndrome manifests as arrhythmias associated with excessive prolongation of the cardiac repolarization; patients often present with stress-induced syncope or sudden cardiac death (SCD), and some forms are associated with swimming.

A

Long QT

62
Q

____________________ patients have arrhythmias associated with abbreviated repolarization intervals; they can present with palpitations, syncope, and SCD.

A

Short QT syndrome

63
Q

____________________________ syndrome manifests as ECG abnormalities (ST segment elevations and right bundle branch block) in the absence of structural heart disease; patients classically present with syncope or SCD during rest or sleep, or after large meals.

A

Brugada Syndrome

64
Q

______________ does not have characteristic ECG changes; patients often present in childhood with life-threatening arrhythmias due to adrenergic stimulation (stress-related).

A

CVPT

65
Q

What are the direct cardiac causes of myocardial disease?

A

Doxorubicin (Adriamycin)

Cyclophosphamide (cytoxan)

High doses of catecholamines (vasopressors)

Iron-Overload (hemochromatosis)

66
Q

The most common cardiac tumors are metastatic malignancies. What are the next six in order of occurance?

A
  1. Myxoma
  2. Fibroma
  3. Lipoma
  4. Papillary Fibroelastoma
  5. Rhabdomyoma
  6. Angiosarcoma
67
Q

What is shown here?

A

Atrial myxoma - large pedunculated lesion arises from the region of the fossa ovalis and extends into the mitral valve orifice.

Abundant amorphous extracellular matrix containing scattered multinucleate myxoma cells with abnormal vessel-like formations.

68
Q

What is a carney complex?

A

•myxomas, pigmented skin lesions and overactivity of endocrine organs (null mutation PRKAR1A Chr 17)

69
Q

What is mazabraud syndrome?

A

•GNAS1 mutation with monoostotic or polyostotic fibrous dysplasia with intramuscular myxomas in same region as the fibrous dysplasia

70
Q

What is McCune-Albright syndrome?

A

•activating mutation in GNAS1 with polyostotic fibrous dysplasia, café-au-lait lesions, and autonomous endocrine hyperfunction but no myxomas)

71
Q

In what patients do we see rhabdomyomas?

A

•(#1 in children and 50% associated with tuberous sclerosis/ TSC1 and TSC2 genes)

72
Q

What kind of cardiac tumor is this?

A

Lipoma

73
Q

What kind of cardiac tumor is this?

A

Fibroma

74
Q

What kind of tumor is this?

A

Rhabdomyoma

75
Q

What kind of tumor is this?

A

Papillary fibroelastoma

76
Q

What kind of tumor is this?

A

Rhabdomyosarcoma

77
Q

What are the direct consequences of noncardiac neoplasms?

A

Pericardial and myocardial metastases

Large vessel obstruction

Pulmonary tumor emboli

78
Q

What are the indirect consequences of non-cardiac neoplasms?

A

Nonbacterial thrombotic endocarditis (NBTE)

Carcinoid heart disease

Pheochromocytoma-associated heart disease

Myeloma-associated amyloidosis

79
Q

What aare the six types of pericardial effusions we covered?

A

Serous

Serosanguinous

Sanguinous

Purulent

Chylous

Malignant (neoplastic)

80
Q

What are the causes of serous pericardial effusions??

A

CHF

Hypoalbuminemia

81
Q

What are the causes of serosanguinous pericardial effusions?

A

malignancy

trauma

ruptured MI

Aortic dissection

82
Q

What are the causes of sanguinous pericardial effusions?

A

Hemopericardium (aortic/cardiac rupture)

83
Q

What are the causes of purulent pericardial effusions?

A

Infection

84
Q

What is the cause of chylous pericardial effusions?

A

Lymphatic obstructions

85
Q

At what level of fluid accumulation can you get a cardiac tamponade?

A

as little as 250 mL

86
Q

What are the types of pericarditis?

A
  1. Serous
  2. Fibrinour/serofibrinous
  3. purulent/supperative
  4. constrictive
  5. hemorrhagic
  6. Casseous
  7. Adhesive
  8. Malignant (neoplastic)
87
Q
A

CV II Exam 2 (8 decks)

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