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MyocardialDz
lecture 26 brickner
| Question | Answer |
|---|---|
| definition: cardiomyopathy | myocardial disorder in which the heart muscle is structurally or functionally abnormal in the absence of CAD, HTN, valve or congenital heart dz sufficient to have caused said abnormality |
| 3 types of cardiomyopathy | hypertrophic obstructive, restrictive and dilated |
| dilated cardiomyopathy | LV dilatation and dec systolic function in the absence of abnormal loading conditions like HTN or valve dz |
| primary vs secondary cardiomyopathy | primary: not associated with another type of heart dz // secondary: specific etiology is known like post-partum cardiomyopathy |
| common etiologies of dilated cardiomyopathy | up to 20% are familial or hereditary like Duchenne muscular dystrophy, idiopathic, post-partum, post-viral or caused by toxins like EtOH |
| presentation of pt with dilated cardiomyopathy | sx of heart failure: left - DOE, PND, orthopnea and fatigue // right - pedal edema, inc abd girth, anorexia and/or nausea |
| systolic vs diastolic dysfunction | inability to maintain sufficient CO vs inability of ventricles to receive incoming blood |
| sx seen occasionally in pt with dilated cardiomyopathy | palpitations or CP without evidence of CAD, syncope or embolic event from thrombus formation in LA or LV |
| PEx of pt with dilated cardiomyopathy | resting tachy, narrow pulse pressure, S3/4 JVD with prominent v waves (reflects tricuspid regurg - tender pulsatile liver if so), CM with lateral PMI. R heart failure sx 50% of the time |
| best prognostic indicator in pt with dilated cardiomyopathy who are in heart failure | NOT LVEF but maximal oxygen consumption at peak exercise (measures systemic oxygen delivery or consumption) |
| clinical course of pt with dilated cardiomyopathy | usually deteriorate until the point of sudden death from ventricular arrhythmias, progressive heart failure or thromboembolic events to pulmonary or systemic circulations |
| CXR and EKG of pt with dilated cardiomyopathy | CXR: mod-severe CM with pulmonary vascular congestion, pleural effusions // EKG: sinus tach with atrial or ventricular ectopy, Q waves ("pseudoinfarction pattern"), non-specific ST changes, LAE & LVH |
| findings on Echo for pt with dilated cardiomyopathy | large dilated and poorly contractile ventricle, possible thrombus in the ventricular apex, associated mitral/tricuspid regurg, elevated intracardiac filling pressures |
| why risk of thromboembolic events is so high in pts with dilated cardiomyopathy | LVEF is usually less than 45% and there is stasis of blood in the ventricle, predisposing to thrombus formation |
| hypertrophic cardiomyopathy | characterized by an increased left ventricular wall thickness in the absence of any apparent etiology that causes diastolic dysfunction. usually involved the interventricular septum, LV free wall or the apex |
| HOCM | 25% of hypertophic cardiomyopathies - usually asymmetric thickening and causes an obstruction to outflow from the LV and resultant pressure gradient btwn LV cavity and aorta. in addition to restriction of LV filling |
| 2 biggest causes of HCM | cardiac myosin heavy chain beta mutation and myosin binding protein C mutation (next are troponin I and tropomyosin mutations) |
| maneuvers and HCM | Valsalva or nitrates make HCM worse b/c the RV is filling more and providing less space for the LV to fill. isometric contraction or squatting makes it better b/c it stops venous return of blood to the LV |
| presenting signs and sx of pt with HCM | all typical sx from pulmonary congestion due to inc end-diastolic LV, LA and pulmonary vascular pressures. dyspnea, orthopnea, PND, angina, palpitations & syncope from ventricular or atrial arrhythmias |
| leading cause of sudden death in young athletes (<35 y/o) | hypertrophic cardiomyopathy - either from fatal ventricular arrhythmia or MI from inability to perfuse thickened myocardium especially when with exertion outflow vol can't inc appropriately |
| PEx of pt with HCM | bisfiriens pulse (double carotid), forceful and lateral PMI, apical systolic thrill, prominent S4, systolic murmur that changes with maneuvers |
| PEx in pt with aortic stenosis vs HOCM | both will have harsh crescendo-decrescendo systolic murmur and abnormal carotid pulses. AS: pulsus parvus et tardens, murmur radiates to carotids // HCM: murmur radiates to base of heart, bisfiriens pulse |
| structural phenomenon that worsens HOCM | anterior leaflet of mitral valve can be pulled into the outflow tract further obstructing flow of blood |
| common complications in pts with hypertrophic cardiomyopathy | 10-15% of pts develop dilated cardiomyopathy, ventricular or atrial arrhythmias, infective endocarditis from turbulent flow about the outflow tract |
| CXR & EKG of pt with hypertrophic cardiomyopathy | CXR: usually nl, maybe some pulmonary vascular redistribution // EKG: LVH with septal Q waves (due to myofibrillar disarray), giant inverted T waves in precordial leads |
| Echo findings in hypertrophic cardiomyopathy | LVH, a normal LVEF, LV outflow tract obstruction/MR possible. asymmetric septal hypertrophy is septum to LV free wall ratio exceeds 1.3:1, possible systolic anterior motion of mitral leaflet = HOCM |
| tx of hypertrophic cardiomyopathy | diuretics to relieve pulmonary congestion, beta blockers or CCBs help with ventricular filling, resection of septum, iatrogenic septal MI, treating arrhythmias with amiodarone |
Created by:
sirprakes
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