Cardiomyopathies Flashcards
Genetic
Mixed
Acquired
Genetic - HCM, ARVC
Mixed - DCM, restirctive
Acquired - inflammatory myocarditis, stress provoked
Hypertrophic CM
Genetics
Path
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
Hypertrophic CM anatomic effects and dx
Unexplained LV hypertrpohy
15 mm on Echo or MR or 13-14 with fam member
Most asymmetric septal
Hypertrophic famial and non-famial causes
Glycogen storage dz
Lysosomalsotrage dz
Obesity, athletic trainign, amyloid
Hypertrophic eval
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)
Hypertrophic tx
Tx HTN…beta blocker and disoperamide
Surgical - implantable defib, alcohol septal abltaion, transplant
ARVC genetics and in general
Auto dom
Mostly in RV
Predominanty desmosomal dz
May be associated with hair/skin findings
ARVC imaging and dx
Fibrofatty replacement of myocardium
Fxn and structural abnoramlites of RV
Ventricular arrhythmias are HALLMARK
Dx difficult in asx unless naxos/carvajal syndome
ARVC causes familail and non
Intercalated disc protien mutations
Inflammation
ARVC eval
Determine idiopathic from secondary
Imaigng - ECHO, CMR
Tissue characterization
ECG
Fam Hx
ARVC tx
MEd - beta blocker, antiarrhythmics, HF manageemtn
Surgical - ICD, ablation ,HT
Dilated CM in general
Dystrophin mutations (x-linked)
Many different mutations
Impaired contraction, cellular compromise
Lamin (nuclear envelope protein) implicated in conductin dz
Dilated CM anatomy
Ventricular dilation with systolic dysfunctiin
Normal LV wall thickness
Asbcen of ID causes
Seocndary - long standing systemic HTN, chronic valve dz, drugs, myocarditis
Dilated CM eval
50% idiopathic
Dilated CM tx
Remove offneindg agennt
Tx HF (diuretics, B blocker, ACEIs, nitrates, hydralazine)
Tx tachy or PVCs
Restrictive CM
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
Eval of restrictive
Need to determine if not constrictive pericarditis
Biopsy helpful for sarcoid
Cardiac MRI T2 for hemochromatosis
Restrictive CM EKG
Low voltage
Restrictive CM tx
Amyloid - immunosupp
Sarcoid - steroids
Enzyme def - replace enzymes
Endomyocardial fibrosis - immunosuppress
Stem cel transplant - amyloid
Stress-induced CM
Sudden onset of sx and signs of ischemia
Occurs most frwquently in women postmenopauseal triggered by emotional or physical stressor
Paht of stress-induced CM
Catecholamine hypothesis
Hypothal pituitary adrenal axis hypoth
Estrogen hypothesis
Myocardial ischemia
Dx of stress induced CM
Acute onset
Abnormal LV contraction anatomically inconsistent with major coronary artery idstricbute
Absnce of plauqe
Ishcemic ECG with troponin elevation
Reversibility
Myocarditis presentation
Inflammatory dz with wide rasnge of clinical presentation…often viral like illness
Sarcoid
Acute RF
HS myo
Giant cell
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
