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Cardiovascular II > Cardiac Path Robbins Part 4 > Flashcards

Cardiac Path Robbins Part 4 Flashcards

1
Q

what are cardiomyopathies?

A
  • diseases of the myocardium assoc with mechanical and/or electrical dysfxn that usually exhibit inappropriate ventricular hypertrophy or dilation
  • due to variety of causes, often genetic
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2
Q

cardiomyopathies- types

A
  • dilated (systolic dysfxn)- most common!! (90%)
  • hypertrophic (diastolic dysfxn)
  • restrictive (diastolic dysfxn)
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3
Q

dilated cardiomyopathy

A
  • progressive cardiac dilation and systolic dysfxn

- usually assoc with dilated hypertrophy

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4
Q

dilated cardiomyopathy- pathogenesis

A
  • familial (30-50% of cases)- TTN (titin) mutations (20%)- most autosomal dominant
  • X-linked- dystrophic protein
  • alcohol!!
  • myocarditis!!
  • cardiotoxic drugs/substances: doxorubicin, cobalt, iron overload
  • childbirth (peripartum cardiomyopathy)
  • supraphysiologic stress (excess catecholamines)
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5
Q

dilated cardiomyopathy- morphology

A
  • dilation of all chambers
  • mural thrombi are common
  • fxnal regurgitation of valves
  • interstitial fibrosis
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6
Q

dilated cardiomyopathy- presentation

A
  • ages 20-50
  • progressive CHF- dyspnea, exertional fatigue, dec EF
  • arrhythmias
  • embolism
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7
Q

Takotsubo cardiomyopathy

A

“broken heart syndrome”

  • excess catecholamines after emotional/psychological stress
  • > 90% women, ages 58-75
  • symptoms- similar to acute MI
  • apical ballooning of left ventricle with abnormal wall motion and contractile dysfxn
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8
Q

Arrhythmogenic right ventricular cardiomyopathy

A
  • right ventricular failure and arrhythmias!!!
  • myocardiu1m of right ventricular wall replaced by adipose and fibrosis
  • causes ventricular tachycardia and fibrillation, sudden death
  • familial (usually autosomal dominant)- defective cell adhesion proteins in desmosomes that link adj cardiac myocytes
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9
Q

Naxos syndrome

A
  • Arrhythmogenic right ventricular cardiomyopathy

- hyperkeratosis of plantar palmar skin surfaces (mutations in gene encoding the desmosome-assoc protein- plakoglobin)

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10
Q

hypertrophic cardiomyopathy- caused by?

A
  • genetic disorder- myocardial hypertrophy and diastolic dysfxn- reduced SV
  • ventricular outflow obstruction (25%)
  • mutations involving sarcomeric proteins (most common- B-myosin heavy chain)
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11
Q

must be distinguished clinically for hypertrophic cardiomyopathy (HCM)

A
  • deposition diseases

- HTN heart disease

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12
Q

hypertrophic cardiomyopathy- morphology

A
  • massive myocardial hypertrophy
  • often with marked septal hypertrophy!
  • microscopically- myocyte disarray
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13
Q

hypertrophic cardiomyopathy- central abnormality/clinical featuer

A

-reduced SV due to impaired diastolic filling!

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14
Q

hypertrophic cardiomyopathy- consequences of extensive hypertrophy

A
  • foci of MI may occur
  • left atrial dilation and mural thrombus
  • diminished CO and inc pulm congestion- leads to exertional dyspnea
  • arrhythmias
  • sudden death
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15
Q

most common cause of sudden, unexplained death in young athletes

A

HCM (hypertrophic cardiomyopathy_

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16
Q

restrictive cardiomyopathy- due to?

A
  • dec ventricular compliance (inc stiffness)- leads to diastolic dysfxn
  • may be secondary to deposition of material within the wall (amyloid) or inc fibrosis (radiation)
  • ventricles usually normal size, but both atria can be enlarged
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17
Q

Amyloid

A
  • extracellular deposition of proteins which form an insoluble B-pleated sheet
  • may be systemic (myeloma) or restricted to the heart (usually transthyretin)
  • certain mutated versions of transthyretin are more amyloidogenic
  • when amyloid deposits are in the interstitium of the myocardium- restrictive cardiomyopathy results
  • Congo red stain- apple-green bifringence ! amyloid!
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18
Q

other restrictive conditions

A
  • endomyocardial fibrosis
  • loeffler endomyocarditis
  • endocardial fibroelastosis
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19
Q

endomyocardial fibrosis

A
  • children/young adults in africa
  • fibrosis of ventricular endo and subendo- extends from apex upward- involves tricuspid and mitral valves
  • fibrous tissue- diminishes the volume and compliance of chambers- restrictive fxnal defect!!
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20
Q

loeffler endomyocarditis

A
  • endomyocardial fibrosis with large mural thrombi
  • peripheral eosinophilia and eosinophilic infiltrates in mult organs
  • toxic products of eosinophils (major basic protein)- initiate endomyocardial necrosis and then scarring
  • many have a myeloproliferative disorder assoc with chromosomal rearrangements involving PDGFR alpha or B genes
21
Q

endocardial fibroelastosis

A
  • uncommon
  • fibroelastic thickening involving the left ventricular endocardium
  • 1st 2 yrs of life
  • 1/3 of cases- aortic valve obstruction or other anomalies
22
Q

myocarditis- due to?

A

infl of the myocardium

  • most commonly due to a virus- Coxsackie A and B most common!
  • other viruses- CMV, HIV, influenza
23
Q

myocarditis- nonviral causes

A
  • Trypanosomes (chagas disease)
  • Trichinosis (Trichinella spiralis)- most common helminthic
  • Lyme disease (Borrelia Burgdorferi)
  • diphtheria (Corynebacterium diphtheriae)
24
Q

myocarditis- noninfectious causes

A
  • hypersensitivity myocarditis- immune mediated rxns (RF, SLE, drug hypersensitivity)
  • giant cell myocarditis- idiopathic
25
Q

myocarditis- morphology

A
  • interstitial infl infiltrate with focal myocyte necrosis
  • lymphocytes!!
  • hypersensitivity myocarditis
  • giant cell myocarditis
26
Q

acute pericarditis- types

A
  • serous
  • fibrinous and serofibrinous
  • purulent or suppurative
  • hemorrhagic
  • caseous
27
Q

serous pericarditis

A
  • produced by noninfectious infl diseases (rheumatic fever, SLE, scleroderma)
  • sterile serous effusion
28
Q

fibrinous and serofibrinous pericarditis- caused by? symptoms?

A
  • most frequent types of pericarditis!
  • serous fluid with fibrinous exudate
  • acute MI, postinfarction (dressler) syndrome, uremia, chest radiation, rheumatic fever, SLE, trauma
  • pain- sharp, pleuritic, position dependent
  • pericardial friction rub- most striking finding!
29
Q

purulent or suppurative pericarditis- caused by?

A
  • active infection caused by microbial invasion of pericardial space
  • serosal surfaces- reddened, granular, coated with exudate
  • acute infl rxn- produce mediastinopericarditis
  • outcome- scarring!- produce constrictive pericarditis
30
Q

hemorrhagic pericarditis

A
  • exudate composed of blood with fibrinous or suppurative effusion
  • most commonly caused by malignant neoplasm spread to pericardial space
  • also seen in bacterial infections in those with bleeding diathesis and tb
31
Q

caseous pericarditis

A
  • tb! (fungal infections- less common)

- antecedent of disabling, fibrocalcific, chronic constrictive pericarditis!

32
Q

adhesive mediastinopericarditis

A
  • may follow infectious pericarditis, cardiac surgery, or mediastinal irradiation
  • pericardial sac is obliterated
  • adherence to external aspect of parietal layer to surrounding structures- strains cardiac fxn
  • heart pulls against parietal pericardium and surrounding structures- rib cage and diaphragm (pulsus paradoxus)
33
Q

constrictive pericarditis

A
  • dense, fibrous or fibrocalcific scar- limits diastolic expansion and CO
  • cardiac hypertrophy and dilation cannot occur
  • muffled heart sounds, elevated jugular venous pressure, peripheral eema
34
Q

rheumatic arthritis

A
  • joint disorder
  • subcutaneous rheumatoid nodules
  • vasculitis
  • neutropenia
  • heart involved in 20-40% of cases
35
Q

rheumatic arthritis- heart involvement

A
  • most common- fibrinous pericarditis- may progress to fibrous thickening of visceral and parietal pericardium
  • granulomatous rheumatoid nodules
  • rheumatoid valvulitis- marked fibrous thickening and secondary calcification of aortic valve cusps
36
Q

primary tumors of heart

A

rare!

  • most common- myxoma, lipoma, papillary fibroelastoma, rhabdomyoma, angiosarcoma
  • benign!- 80-90% of primary tumors of herat
37
Q

metastatic tumors of heart

A

5% of ppl dying of cancer

38
Q

myxomas

A
  • most common primary tumor of adult heart!
  • benign
  • arise from primitive multipotent mesenchymal cells
  • familial- act mutations in GNAS1 gene or null mutations in PRKAR1A
  • 90% arise in atria; 4:1 left:right ratio!
39
Q

myxoma- morphology

A
  • usually single
  • fossa ovalis in atrial septum- favorited site!- can extend into mitral valve orifice
  • sessile or pedunculated lesions
  • clinical manifestations due to valvular “ball-valve” obstruction, embolization
40
Q

lipoma

A
  • localized, well-circumscribed, benign tumors
  • composed of mature fat cells that can occur in subendocardium, subepicardium, or myocardium
  • asymptomatic or can produce ball-valve obstructions or arrhythmias
  • most often in left ventricle, right atrium or atrial septum
  • in atrial septum- depositions of fat- “lipomatous hypertrophy”
41
Q

papillary fibroelastoma

A
  • incidental, sea-anemone-like lesions- most ID’ed at autopsy
  • may embolize!
  • resemble Lambl excrescences
  • usually on valves
  • distinctive cluster of hairlike projections
42
Q

rhabdomyoma

A
  • most frequent primary tumor of pediatric heart!
  • commonly found in 1st year- obstruction of valvular orifice or cardiac chamber
  • 1/2 sporadic
  • 1/2 assoc with tuberous sclerosis (TSC1/2 mutations- hamartin and tuberin)
  • regress spontaneously
43
Q

angiosarcoma

A

-not clinically or morphologically distinctive from their counterparts

44
Q

most frequent metastatic tumors involving the herart

A
  • carcinomas of lung and breast, melanomas, leukemias, lymphomas
  • retrograde lymphatic extension, hematogenous seeding, direct contiguous extension, venous extension
45
Q

noncardiac neoplasms- clinical syptoms

A

-pericardial spread- pericardial effusions or mass-effect that is sufficient to restrict cardiac filling

46
Q

major complication of cardiac transplantation

A
  • allograft rejection
  • endomyocardial biopsy- only reliable means of diagnosing acute cardiac rejection before substantial myocardial damage has occured
47
Q

cellular rejection- characterized by

A

-interstitial lymphocytic infl with assoc myocyte damage

48
Q

single most important long-term limitation for cardiac transplantation?

A

allograft arteriopathy

  • late, progressive, diffusely stenosing intimal proliferation in the coronary a’s- leading to ischemia
  • 5 yrs of transplantation- 50% of pts develop allograft arteriopathy; all pts by 10 yrs!\
  • can lead to a silent MI (pts have denervated hearts)

Cardiovascular II (13 decks)

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