Cardiomyopathies Flashcards

1
Q

Genetic
Mixed
Acquired

A

Genetic - HCM, ARVC

Mixed - DCM, restirctive

Acquired - inflammatory myocarditis, stress provoked

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2
Q

Hypertrophic CM

Genetics
Path

A

Auto dom

Mut in sarcomeric proteins inc myofilament activity…beta-myosin heavy chain

Myocyte hypertrophy with cells disorganized and chatoic

Abnormal intramural coronary arteries…blunted BF during strss and small vessel ischemia

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3
Q

Hypertrophic CM anatomic effects and dx

A

Unexplained LV hypertrpohy

15 mm on Echo or MR or 13-14 with fam member

Most asymmetric septal

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4
Q

Hypertrophic famial and non-famial causes

A

Glycogen storage dz
Lysosomalsotrage dz

Obesity, athletic trainign, amyloid

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5
Q

Hypertrophic eval

A

Treat HTN and eval for regression

Family hx of premature HCM related death
unexplained syncope 
Nonsustained VT 
Hypotensive in exersive
Extreme LV hypertrophy (over 30)
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6
Q

Hypertrophic tx

A

Tx HTN…beta blocker and disoperamide

Surgical - implantable defib, alcohol septal abltaion, transplant

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7
Q

ARVC genetics and in general

A

Auto dom

Mostly in RV

Predominanty desmosomal dz

May be associated with hair/skin findings

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8
Q

ARVC imaging and dx

A

Fibrofatty replacement of myocardium

Fxn and structural abnoramlites of RV

Ventricular arrhythmias are HALLMARK

Dx difficult in asx unless naxos/carvajal syndome

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9
Q

ARVC causes familail and non

A

Intercalated disc protien mutations

Inflammation

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10
Q

ARVC eval

A

Determine idiopathic from secondary

Imaigng - ECHO, CMR

Tissue characterization

ECG

Fam Hx

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11
Q

ARVC tx

A

MEd - beta blocker, antiarrhythmics, HF manageemtn

Surgical - ICD, ablation ,HT

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12
Q

Dilated CM in general

A

Dystrophin mutations (x-linked)

Many different mutations

Impaired contraction, cellular compromise

Lamin (nuclear envelope protein) implicated in conductin dz

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13
Q

Dilated CM anatomy

A

Ventricular dilation with systolic dysfunctiin

Normal LV wall thickness

Asbcen of ID causes

Seocndary - long standing systemic HTN, chronic valve dz, drugs, myocarditis

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14
Q

Dilated CM eval

A

50% idiopathic

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15
Q

Dilated CM tx

A

Remove offneindg agennt

Tx HF (diuretics, B blocker, ACEIs, nitrates, hydralazine)

Tx tachy or PVCs

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16
Q

Restrictive CM

A

Reduced diastolic/systolic volumes
Normal wall thickness and systolic fxn
Biatral enlargment

Oftne caused by amyloid (AL or transthyretin)

May be HCM-grelated gene mutation

17
Q

Eval of restrictive

A

Need to determine if not constrictive pericarditis

Biopsy helpful for sarcoid

Cardiac MRI T2 for hemochromatosis

18
Q

Restrictive CM EKG

A

Low voltage

19
Q

Restrictive CM tx

A

Amyloid - immunosupp
Sarcoid - steroids
Enzyme def - replace enzymes
Endomyocardial fibrosis - immunosuppress

Stem cel transplant - amyloid

20
Q

Stress-induced CM

A

Sudden onset of sx and signs of ischemia

Occurs most frwquently in women postmenopauseal triggered by emotional or physical stressor

21
Q

Paht of stress-induced CM

A

Catecholamine hypothesis

Hypothal pituitary adrenal axis hypoth

Estrogen hypothesis

Myocardial ischemia

22
Q

Dx of stress induced CM

A

Acute onset

Abnormal LV contraction anatomically inconsistent with major coronary artery idstricbute

Absnce of plauqe

Ishcemic ECG with troponin elevation

Reversibility

23
Q

Myocarditis presentation

A

Inflammatory dz with wide rasnge of clinical presentation…often viral like illness

24
Q

Sarcoid
Acute RF
HS myo
Giant cell

A

LAD with arrhtymias and other organs involvement

Erythmia marginatum, polyarthrlagia, chorea, sub q nodules

Pruritic maculopapular rash and hx of offending drug

Sustained VT in rapidly progressing HF

25
Q

Dx of myocarditis

A

Full panels and endomyocardial biopsy

Intersitial inflalmamotyr

Esodino
T and B cells

26
Q

Myocarditis tx

A

Withdraw agent

Supportive managment

Immunosupp

Others for HF