Cardiomyopathies Flashcards

1
Q

What is the most common cardiomyopathy? What is the least common?

A

Most = Dilated Cardiomyopathy

Least = Restrictive cardiomyopathy

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2
Q

Describe Dilated cardiomyopathy. What is the ejection fraction? Mechanism of heart failure? Causes of phenotype? Indirect myocardial dysfunction (mimicking cardiomyopathy)?

A

EF < 40%

Mechanism = impairment of contractility (systolic dysfunction)

Causes of phenotype: genetic, alcohol, hemochromatosis, etc.

Mimicking = IHD, VHD, HHD, congenital HD

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3
Q

Describe Hypertrophic cardiomyopathy. What is the ejection fraction? Mechanism of heart failure? Causes of phenotype? Indirect myocardial dysfunction (mimicking cardiomyopathy)?

A

EF = 50-80%

Mechanism = Impairment of compliance (diastolic dysfunction)

Causes of phenotype: genetic, infants of diabetics, storage DO

Mimicking = HHD, aortic stenosis

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4
Q

Describe Restrictive cardiomyopathy. What is the ejection fraction? Mechanism of heart failure? Causes of phenotype? Indirect myocardial dysfunction (mimicking cardiomyopathy)?

A

EF = 45-90%

Mechanism = Impairment of compliance (diastolic dys)

Causes of phenotype: Amyloidosis, radiation induced fibrosis, idiopathic

Mimicking = pericardial constriction

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5
Q

What is Dilated cardiomyopathy (DCM)?

A

Progressive cardiac dilation and systolic dysfunction, usually with dilated hypertrophy

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6
Q

What is the genetic basis behind DCM?

A

Thought to be familial in 30-50% of cases

TTN mutations may account for 20% of all cases

Usually autosomal dominant

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7
Q

What cause is strongly linked to DCM? What else is related?

A

Alcohol

Myocarditis
Cardiotoxic drugs/substances = doxorubicin, cobalt, iron overload

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8
Q

Why does iron overload occur leading to DCM?

A

Iron overload from hereditary hemochromatosis (HFE mutation) or multiple transfusions

Use the prussian blue iron stain

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9
Q

What is the morphology of dilated cardiomyopathy?

A

Dilation of all chambers
Mural thrombi
Functional regurgitation of valves

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10
Q

What is the presentation of DCM?

A

Usually manifests b/w ages 20-50 yo

Progressive CHF –> dyspnea, exertional fatigue, decreased EF

Arrhythmias (sudden death)

Embolism

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11
Q

What is Takotsubo cardiomyopathy?

A

A dilated cardiomyopathy

“Broken heart syndrome”

Excess catecholamines following extreme emotional or psychological stress

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12
Q

How does takotsubo cardiomyopathy present?

A

> 90% in women
Ages 58-75

Sx and sign similar to acute MI

Apical ballooning of the left ventricle with abnormal wall motion and contractile dysfunction

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13
Q

Histologically, what can you observe with DCMs?

A

Dilation
Myocyte hypertrophy
Interstitial fibrosis

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14
Q

What is arrhythmogenic right ventricular cardiomyopathy (ARVC)? What can it lead to?

A

Right ventricular failure & arrhythmias

Myocardium of right ventricle wall replaced by adipose & fibrosis

Causes ventricular tachycardia or fibrillation –> sudden death

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15
Q

What is the genetics behind ARVC? What is the pathogenesis?

A

Familial, Autosomal dominant

Defective cell adhesion proteins in desmosomes that link adjacent cardiac myocytes

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16
Q

What is Naxos syndrome?

A

ARVC w/ hyperkeratosis of plantar palmar skin surfaces

Mutations in gene encoding the desmosome-associated protein Plakoglobin

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17
Q

What is hypertrophic cardiomyopathy? Describe the genetic changes seen with the dz?

A

Genetic DO leading to myocardial hypertrophy & diastolic dysfunction, leading to decreased SV & often ventricular outflow obstruction

Numerous mutations known, involving sarcomeric proteins
-most commonly B-myosin heavy chain (B-MHC)

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18
Q

What is the morphology of hypertrophic cardiomyopathy?

A

Massive myocardial hypertrophy, often with marked septal hypertrophy

Microscopically, myocyte disarray

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19
Q

What are Sx of hypertrophic cardiomyopathy?

A

Harsh systolic ejection murmur, caused by ventricular outflow obstruction as the anterior mitral leaflet moves toward the ventricular septum during systole

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20
Q

What are the consequences of extensive hypertrophy in hypertrophic cardiomyopathy?

A

Foci of myocardial ischemia may occur

Left atrial dilation and mural thrombus

Diminished cardiac output and increased pulmonary congestion leads to exertional dyspnea

Arrhythmias

Sudden death (sudden, otherwise unexplained death in young athletes)

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21
Q

What is Restrictive Cardiomyopathy?

A

Decreased ventricular compliance (increased stiffness), leading to diastolic dysfunction (impaired filling), while systolic function of the LV remains normal

22
Q

What may restrictive cardiomyopathy be secondary to? What changes to the heart will you see?

A

May be secondary to deposition of material within the wall (amyloid), or increased fibrosis (radiation)

Ventricles normal size, but BOTH atria can be enlarged

23
Q

What is amyloid and how does it relate to restrictive cardiomyopathy?

A

Extracellular deposition of proteins which form an insoluble B-pleated

It is a common cause of RC

May be systemic (myeloma) or restricted to the heart (usually transthyretin)
-certain mutated versions of transthyretin are more amyloidogenic

Amyloid can involve different parts of heart, but when deposits are in interstitium of myocardium = restrictive cardiomyopathy

24
Q

What is the classic stain associated with amyloid?

A

Congo red stain = apple green birefringence

25
Q

What is myocarditis?

A

Inflammation of the myocardium, most commonly due to virus in US

Coxsackie A/B viruses most common

26
Q

What other infection is associated with myocarditis?

A

Trypansoma cruzi (Chagas dz)

  • 10% dies during acute attack
  • may progress to cardiac insufficiency in 10-20 yrs
  • parasitization of scattered myofibers

Will see mixed inflammatory cell infiltrate (PMS, lymphs, macrophages, and occ eosinophils)

27
Q

What are noninfectious causes of myocarditis?

A

Immune mediated Rxns:

  • RF
  • SLE
  • Drug hypersensitivity
28
Q

Histologically, what will you see with Chagas Dz?

A

Amastigotes

29
Q

What type of fluid is seen with pericardial dz?

A

Normal = <50 mL clear, straw colored fluid

30
Q

What is pericardial dz?

A

Slow accumulation of fluid,

<500 ml - asymptomatic if slow enough

Globular enlargement of heart shadow on CXR

Acute = 200-300 mL rapid accumulation –> cardiac tamponade

31
Q

What is most common type of pericardial dz? When will you see it?

A

Fibrinous and serofibrinous

AMI, postinfarction (Dressler’s), uremia, chest irradiation, RF, SLE, trauma

32
Q

Describe what fibrinous and serofibrinous pericardial dz look like. What are the Sx seen with them?

A

Fibrinous = dry, finely granular

Serofibrinous = yellow-brown, turbid fluid with WBC, RBC, and fibrin

Sx: pain (sharp, pleuritic & position dependent), fever, +/- CHF

Loud pericardial friction rub = most striking feature

33
Q

What is serous pericarditis?

A

Characteristically produced by non infectious inflamm dz (RF, SLE, Scleroderma, tumors, and uremia)

34
Q

What is purulent or suppurative pericarditis?

A

Active infection caused by microbial invasion of pericardial space (direct extension, seeding form blood, lymphatic extension, direct introduction)

35
Q

What is hemorrhagic pericarditis?

A

Exudate composed of blood mixed with fibrinous or suppurative effusion

Most commonly caused by spread of a malignant neoplasm to pericardial space

36
Q

What is caseous pericarditis?

A

Tuberculous in origin; infrequently, fungal infections evoke a similar reaction

37
Q

What is constrictive pericarditis?

A

Heart is encased in dense, fibrinous or fibrocalcific scar that limits diastolic expansion and CO

Features that mimic restrictive cardiomyopathy

38
Q

What are primary cardiac tumors?

A

Rare, top 5 = benign

Myxoma, fibromas, lipomas, papillary fibroelastomas, rhabdomyomas,

Angiosarcomas

Metastatic tumors occur in 5% of people dying from CA

39
Q

What is a myxoma?

A

Most common primary, predunculated, usually in region of fossa ovalis

Familial syndrome - GNAS1 mutation (McCune-Albright syndrome)

PRKAR1A mutation (Carney complex)

40
Q

What is the appearance of a myxoma? What are the Sx?

A

Globular hard mass, mottled with hemorrhage to soft, translucent, papillary, or villous with a gelatinous appearance

Sx = Ball-valve obstruction, embolization or constitutional sx (fever and malaise)

Pedunculated form cause intermittent obstruction during systole of AV valve or wrecking ball causing damage to valve leaflets

Constitutional sx = elaboration of some myxomas of the IL-6, major mediator of the acute phase response

Auscultation = tumor “plop”

41
Q

What is a lipoma?

A

Localized, well-circumscribed, benign tumors composed of mature fat cells; subendocardium, subepicardium, or myocardium

42
Q

What is a papillary fibroelastoma?

A

Usually incidental, sea-anemone-like lesions, most often identifies at autopsy; usually (>80%) located on valves

Resemble smaller, trivial, labl excresences that may represent remotely organized thrombus on the aortic valves of older indivs

43
Q

What is a rhabdomyoma?

A

Most frequent primary tumor of the pediatric heart

  • 50% sporadic mutations
  • 50% assoc with tuberous sclerosis (TSC1/Hamartin or TSC2/tuberin mutations - TS genes)
44
Q

What is an angiosarcoma?

A

Malignant endothelial neoplasm that primarily affects older adults

45
Q

What are some direct consequences of tumors to the heart?

A

Pericardial and myocardial metastases
Large vessel obstruction
Pulmonary tumor emboli

46
Q

What are some indirect consequences of tumors to the heart?

A

Non-bacterial thrombotic endocarditis
Carcinoid heart dz
Pheochromocytoma-assoc heart dz
Myeloma-assoc amyloidosis

47
Q

How may cardiac transplants occur? Due to what?

A

~3000/yr worldwide due to DCM and IHD (most common)

48
Q

What is a major complication to cardiac transplantation?

What is the survival rate? Histologically, what changes to the heart are seen?

A

Allograft rejection
-routine endomyocardial bx to monitor

Overall survival = 90% at 1 yr, >60% at 5 yr

Histo = lymphocytic infiltrate, intimal thickening

49
Q

What is allograft arteriopathy?

A

Most impt long term limitation

Late, progressive, diffuse stenosing intimal proliferation

50% dev in 5 yr, virtually all pt within 10 yr

Silent MI = denervated transplanted heart = no angina

50
Q

What can develop in transplant pts due to chronic T-cell immunosuppression?

A

EBV assoc B-cell lymphoma