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Echocardiography Cardiomyopathies Cardiomyopathies Definition lPrimary disease of the myocardium, excluding myocardial dysfunction due to ischemia, chronic valvular disease, hypertensive heart disease and pulmonary heart disease. lDiagnosis by exclusion. Physiologic Classification lDilated lHypertrophic lRestrictive Cardiomyopathies Cardiomyopathies Echocardiographic Evaluation lConfirm the diagnoses and type. lDefine the physiologic consequences. Dilated Cardiomyopathy lCharacterized by four chamber enlargement. lImpaired systolic function of both ventricles Dilated Cardiomyopathies Dilated Cardiomyopathies Etiology lIdiopathic lToxins lMetabolic lPeripartum Dilated Cardiomyopathies Etiology lInfections lSystemic Disease lInherited disorders Dilated Cardiomyopathies Toxins lAlcohol lMedications lCobalt lSnake bite Dilated Cardiomyopathies Metabolic lThiamine deficiency lAcromegaly Dilated Cardiomyopathies Infections lChagas disease lPost viral Dilated Cardiomyopathies Physiology lImpaired contractility of the LV lReduced CO lElevated LV-EDP. Dilated Cardiomyopathies Symptoms lLow cardiac output lPulmonary venous congestion lSystemic venous congestion Dilated Cardiomyopathies Diastolic Dysfunction lLV diastolic dysfunction often coexists with systolic dysfunction but typically is not the predominant feature. Dilated Cardiomyopathies 2-D Echocardiographic Findings lEnlargement of the 4 chambers lGlobal decreased in wall motion Dilated Cardiomyopathies Dilated Cardiomyopathies M-mode findings lIncreased EPSS lReduced anteroposterior aortic root motion lB-bump on the AC shoulder Dilated Cardiomyopathies Dilated Cardiomyopathies Dilated Cardiomyopathies Doppler lReduced aortic ejection velocity lReduced aortic VTI lA slow rate of rise in the velocity of the MR jet (reduced rate of rise in LV pressure in early systole) Dilated Cardiomyopathies Dilated Cardiomyopathies Dilated Cardiomyopathies Dilated Cardiomyopathies Doppler lMitral and tricupid regurgitation Dilated Cardiomyopathies Dilated Cardiomyopathies Diastolic Filling Pattern Early - Impaired Relaxation lProlonged IVRT lReduced E lIncreased A Dilated Cardiomyopathies Further deterioration in LV function lIncreased E velocity (high LA pressure) lReduced A velocity (elevated LV- EDP) lPseudonormalization Dilated Cardiomyopathies LA Filling Pattern lIncreased atrial reversal velocity lIncreased ratio of antegrade diastolic to systolic flow. Dilated Cardiomyopathies Dilated Cardiomyopathies Cardiomyopathies Hypertrophic Cardiomyopathy Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathy lAutosomal dominant lAbnormalities in the Beta myosin heavy chain. Predominant Features lAsymmetric hypertrophy of the LV lNormal ventricular systolic function lImpaired LV diastolic function l+/- Subaortic dynamic obstruction Hypertrophic Cardiomyopathies Clinical Features lHigh risk of sudden death lSymptoms of angina lExercise intolerance lSyncope lSystolic murmur Hypertrophic Cardiomyopathies Type Feature IHypertrophy confined to Anterior segment of the IVS IIHypertrophy confined to Anterior and Inferior segments of the IVS IIIExtensive hypertrophy of LV with sparing of the basal segment of the posterior wall. IV Apical hypertrophy Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies lThe apical pattern of hypertrophy is associated with an inverted precordial T waves on the EKG Hypertrophic Cardiomyopathies The common feature of all of these hypertrophy patterns is normal thickness of the basal posterior LV wall. Hypertrophic Cardiomyopathies Subaortic obstruction lCaused by apposition of AMVL against the hypertrophied septum. lDynamic obstruction lOccurs in mid late systole Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies Subaortic obstruction lMaximum LV to aortic pressure gradient occurring in late systole. lPresence and severity of obstruction depends on the loading conditions. Hypertrophic Cardiomyopathies The obstruction can diminished by maneuvers that: Increase ventricular volume (increase in preload). Decrease contractility. Increase afterload. Hypertrophic Cardiomyopathies The obstruction can be increased by maneuvers that: Decrease ventricular volume (decrease in preload) Increase contractility Decrease afterload Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies Clinical useful maneuvers lExamination during a post premature contraction beat (Increased contraction). lValsalva (Decreased preload). lInhalation of amyl nitrate (Decreased afterload and preload) Hypertrophic Cardiomyopathies These maneuvers lead to lIncreased degree of dynamic obstruction lLouder murmur lIncreased in Doppler velocity. Hypertrophic Cardiomyopathies Mitral Regurgitation lThe SAM of the leaflets in systole disrupt the normal mitral valve coaptation. lResults in a posteriorly directed MR jet of mild to moderate severity. Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies LV function lNormal systolic function. lAbnormal diastolic function with impaired relaxation and decreased compliance. Hypertrophic Cardiomyopathies Echocardiographic Evaluation 2-D lUse multiple views to define pattern of LV hypertrophy. lQualitative and quantitation evaluation of LV systolic function. Hypertrophic Cardiomyopathies LV Diastolic Function lDoppler inflow velocity curve recorded at the mitral valve leaflet tips lMeasure E velocity, A velocity, E/A ratio, IVRT and deceleration slope Hypertrophic Cardiomyopathies Findings lProlonged IVRT lReduced E velocity lIncreased A velocity Hypertrophic Cardiomyopathies 2-D and M- mode Findings lSAM of the AMVL lApposition of AMVL and septum in late systole. lContact lesion on the IVS at point of AMVL impingement Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies lAortic valve show normal leaflet opening in early systole, followed by mid-systolic abrupt partial closure with coarse fluttering of the AV leaflets in late systole. Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies Doppler Evaluation lPresence of obstruction lLocation of obstruction (Pulsed Doppler Stepwise evaluation lSeverity of obstruction Hypertrophic Cardiomyopathies High Velocity Systolic flow High intracavitary gradient. 1. Apical hypertrophy. 2. Opposition of the papillary with the septum. Hypertrophic Cardiomyopathies Hypertensive heart disease/ hypovolemia lLVH lHyperdynamic systolic function lMid ventricular obstruction in late systole lLate-peaking high velocity systolic waveform. Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathies No evidence of outflow obstruction at rest. Maneuvers lSpontaneous PVC results in an increased LV contraction on the post PVC beat. lValsalva maneuver lAmyl nitrate Hypertrophic Cardiomyopathies Mitral valve abnormalities lIncreased length and surface area of MV especially the AMVL. lExcessive degree of coaptation with the coaptation place displaced posteriorly. lAnomalous papillary muscle anatomy with direction insertion of the papillary muscle into the leaflet. Hypertrophic Cardiomyopathies Differential Diagnosis lHypertension with hyper dynamic concentrically hypertrophy of the ventricle. Hypertrophic Cardiomyopathies Cardiomyopathies Restrictive Cardiomyopathies Restrictive Cardiomyopathies Restrictive Cardiomyopathy lNormal LV systolic function. lImpaired diastolic function due to a stiff, hypertrophied LV Restrictive Cardiomyopathies Symptoms of heart Failure lDue to elevated LV-EDP lInability to increase CO due to impaired diastolic filling Restrictive Cardiomyopathies Heart Failure lInability to maintain a normal CO. Or lMaintenance of a normal CO only with an elevated LV-EDP. Restrictive Cardiomyopathies Disease Progression lRestrictive Pattern lSome features of dilated cardiomyopathy lFeatures indistinguishable from dilated cardiomyopathy. Restrictive Cardiomyopathies Etiology lAmyloidosis lHemochromatosis lSarcoidosis lHyperesinophilic Syndrome lGlycogen storage diseases Restrictive Cardiomyopathies Hypereosinophilic Syndrome lHypereosinophilia involving the lungs, bone marrow, brain and heart. lCardiac involvement - LV apical thrombus with gradual obliteration of the apex and thrombus formation under the PMVL with adherence of the PMVL to the endocardium. Restrictive Cardiomyopathies Echo findings lNon dilated, thick- walled LV lNormal systolic function lAbnormal diastolic function lThick RV free wall lBiatrial enlargement Restrictive Cardiomyopathies Restrictive Cardiomyopathies Restrictive Cardiomyopathies Doppler Evaluation lModerate Pulmonary hypertension a. TR jet b. PR jet c. Time to peak velocity of the RVOT velocity. d. IVC evaluation Restrictive Cardiomyopathies Pulmonary Hypertension Pulmonary Hypertension Pattern of Diastolic Filling Early in the disease lImpaired relaxation a. Prolonged IVRT b. Reduced E c. Increased A Restrictive Cardiomyopathies Diastolic Dysfunction Pattern of Diastolic Filling d. Decreased early diastolic deceleration slope e. Reduced diastolic filling in the LA filling curve. Restrictive Cardiomyopathies Later in the Disease lLA pressure rises Increased Atrial to ventricle pressure gradient. lReduced diastolic compliance a. Increased E wave b. Rapid dece

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