Terms in this set (25)

  • Restrictive (Infiltrative) Cardiomyopathy
    an increased resistance to ventricular filling due to increased myocardial stiffness (dec vent compliance). Assoc w elev ventricular diastolic pressure and increased atrial pressure. Global systolic function may be preserved. Overall this disease results in rigid vent walls which limit diastolic fxn -least common of all CMs
  • 5 types of Restrictive (Infiltrative) Cardiomyopathy
    amyloidosis, sarcoidosis, hemochromatosis, glycogen storage disease, endomyocardial fibrosis
  • amyloidosis
    MC Restrictive (Infiltrative) Cardiomyopathy multisystem disease w extracellular deposition of amyloid protein in heart, kids, liver, nerves skin, tongue. tissue damage and organ malfxn. mimic constrictive pericarditis bc of it limits the filling of the heart (dias dys). may have pericardial eff w dysrhythmias myocardium
  • sarcoidosis
    multisystemic granulomatous (inflamm disease prod tumors of lymphoid and epi cells) -disease which affects the heart in ~25% of cases -women:men 2:1 -lungs, lymph nodes, liver, spleen, skin, parotid glands -dysrhythmias and heart block myocardium
  • hemochromatosis
    multisystemic iron storage disease affects heart, panc, liver, skin BRONZE DM, iron deposited in myocardial tissue where it limits LV fxn dysrhythmias. pts who have mult transfusions, >40y/o, 10x MC in men myocardium
  • glycogen storage disease
    aka glycogenosis - abnl accumulation of glyocen (carbs stored in body for conversion into sugar) affects may tissues in body -in heart, causes enl and thickening. -hereditary factor; infancy, early childhood myocardium
  • endocardial fibrosis
    affects endocardium - occurs when there is a replacement of nl endo tissue w collagenous fibrotic tissue involves inflow tracts of RV and LV but may affect AV valves leading to TR and MR. -apical thrombus due to necrosis. nl LV fxn
  • Restrictive (Infiltrative) Cardiomyopathy
    Clinical hx of what disease? physical hx depends on the classification - CHF, dyspnea, orthonpnea, fatigue, weakness, anorexia, angina, poor exercise tolerance, peripheral edema, ascites, hepatomegally, hypotension, jugular venous distention, cardiac tumors
  • Restrictive (Infiltrative) Cardiomyopathy
    M-mode of what disease? - hypertrophy of RV and LV - small to nl LV size - RAE, LAE
  • Restrictive (Infiltrative) Cardiomyopathy
    2d of what disease? LVH, RVH GROUND GLASS appearance of endocardium and myocardium- speckled - nl or dec LV syst fxn - abnl diastolic filling fxn (dec compliance) - poss pericardial eff - w endomyocardial fibrosis- endocardium appears echogenic
  • Restrictive (Infiltrative) Cardiomyopathy
    color flow and dopp eval -MR, TR -abnl dias filling patterns via AV - reduced AV flow velocities during atrial contraction - less respiratory flow variation than tamponade (collapse of cardiac chambers during systole) or constrictive pericarditis) E/A waves, IVC, Pul Veins, Hep Veins) - low systolic vel of PV inflow
  • other types of CM
    - non-compaction cardiomyopathy (NCC) aka spongiform or LV non compaction - arrthymogenic RVC (ARVC or ARVD displasia) - Takotsubo aka transient apical ballooning syndrome, stress induced, broken heart
  • non-compaction cardiomyopathy
    -rare, genetic -first id'd in 1984 -failure of myocardial devel, heart muscle does not firm up, stays spongy, doesn't contract - excessively prom trabeculations
  • non-compaction cardiomyopathy
    Diagnosis of what disease: measurement of ratio of the distance from trough to peak, of trabeculations, to epicardial surface - non compaction, when trab are more than 2x the thickness of the underlying vent wall
  • non-compaction cardiomyopathy
    common symptoms of what disease? dyspnea, fatigue, pedal edema, exercise interolerance, tachyarrythmia (can lead to sudden death), intracardiac thrombus formation - often misdiagnosed as other CM - tx medication, pacemaker, valvular surgery, heart transplant
  • arrythmogenic RV displasia
    - genetic CM - hypokinetic areas of the RV free wall - fibrous and fatty tissues replaces RV myocardium (fibro-fatty infiltration) - assoc w arrythmias originate in RV - 90% of pts w ARVD have EKG abnl - pts have palmoplantar keratoderma and woolly heair
  • arrythmogenic RV displasia
    Sx of what disease? - syncope and sudden cardiac death - MCC of sudden cardiac death for youths in Italy - palps - RVOT tachy - exercise related sx
  • arrythmogenic RV displasia
    M-mode/2d/Dop of what disease? enl hypokinetic RV PAPER THIN RV free wall enl of TV annulus w TR paradoxial septal motion
  • arrythmogenic RV displasia
    management of what disease? - goal is to limit sudden death esp for young athletes - drugs, surgical, catheter ablation, placement of an implantable cardioverter-defribrillator
  • Takotsubo cardiomyopathy
    -non ischemic cm w sudden temporary weakening of the muscular portion of the heart -acute, transient, stress-induced
  • Takotsubo cardiomyopathy
    - apical ballooning w apical dilation and dyskinesia - cardiac base dimensions and fxn preserved - octopus pot: shape of LV during systole - broken heart syndrome, stress CM, apical ballooning syndrome
  • Takotsubo cardiomyopathy
    clinical presentation of what disease? chest pain, SOB, EKG changes, elevated cardiac enzymes consistent w myocardial infarction after episode of stress - poor syst fxn (EF decreased initially ~20-49%) - angiography shows no stenosis of CA -MI or Acute Coronary Syndrome "mimic" - occurs w excessive catecholamines - hormones released by adrenal glands during periods of stress - causes toxicity and stunning of myocardium
  • Takotsubo cardiomyopathy
    women >50y/o (post meno) wide range of stressors drugs: epi-pen, cymbalta, synthroid can cause CM -recovery w/in 1 month
  • Takotsubo cardiomyopathy
    echocardiographic signs: hyperkinetic base and akinetic or dyskinetic LV apex (apex alone bulges or bulges and elongates), BANANA SHAPE variations exist: reverse takotsubo (basal akinesia and apical hyperkinesia) mid ventricular ballooning w basal and apical hyperkinesia localized to any one segment
  • Takotsubo cardiomyopathy
    TX of what disease: meds to maintain heart fxn: beta blockers, asprin, blood pressure meds -alleviation of physical and emotional stressors