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סיכומי הריסון17 קרדיולוגיה וריאות סוכם ע" י יובל שוורץ, האונ' הע ברית, תשס" ט1

קרדיולוגיה וריאות

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722 :Heart Failure and Cor pulmonale ) (Heart failure=HF / . ) ( ) ( : . HF , -02 . - HF %2. -%01-6 56. HF , HF . , - Lifetime risk HF 5:1 04. , HF . HF , ) ,MI (, . - HF - ,LV EF -%05 HF EF ) 50 mmHg 60 mmHg (, - valvutomy / ) mild stenosis 2.(1.5 cm ,valvutomy : . -MR / . -%05 01 . ,MS - valvotomy . - PMBV TEE . (MVR) Mitral valve replacement : MS MR . . - MVR - chordae . MVR , - ,LV function ,CAD . -%5 2 ) ( ]3-032 .[Fig ) 0841(, , -) severe MS -2 (1 cm ) NYAH class III ( . -01 - %07. : . . - CO . -.RV dysfunction :(MR) Mitral Regurgitation 23 , 71 @ , ', "

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1. : MR -) Mitral valve apparatus , - ,annulus- chordae ,tendineae- papillary muscles-MR .[Table 230-1] (subjacent myocardium : acute MI ) 932(. . ,acute MI - ) -( . MR .angina pectoris - chordae tendineae - acute on chronic MR myxomatous degeneration . MR : Mitral valve prolapse -annulus ' . .Hypertrophic Obstructive cardiomyopaty (HOCM) .dilated cardiomyopathy - MR -%03 . : , -.cusps -commissure , -.chordae tendineae - MR MVP- HOCM . , - MR- HOCM ) (systolic anterior motion - LV outflow ) tract (. - annulus . MR ' ) 922(, - ) endocardial cushion .(atrioventricular cushion defects , .primum ASD MR : .ventricular remodeling . . ) -.(MI - annulus- Ventricular remodeling -MR dilated cardiomyopathy ) - (end-diastolic - 6 ". , - MR , :

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- LA , - dysfunction . , - LA- ,LV -.chordae . "."MR begets MR 2. : ) (LV afterload .MR , - LV - - LA , -LV , . - MR -) LV .(SV - LV ) ( - LV. , - LV diastolic pressure . : LV systolic pressure - , -.annulus - EF severe MR - LV , -) EF -%06( dysfunction. - ,early diastole - LA , -y descent ) .(MS ) -] mid-diastolic murmur [(MS pure MR ) ], [(. - ,PAP ,CO ,LVEF , -)regurgitation fraction (RF .Doppler echo " .cardiac MRI , contrast ventriculography . Severe MR : .60 mL/beat RF > 50% 2 .0.40 cm 3. : - severe MR, - LA . , - LA - .LA- v wave- LA pressure pulse " )3-322 .(Fig , . , - acute MR ,decrescendo - LV- .LA- LV systolic function- MR , , . severe MR, , , ) ( . - v wave . -MR

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,plateau - LV- .LA - CO, . AF . , . 4. : isolated MR, , . LV volume overload . - , - orthopnea severe MR. -.AF )(, , , , - ,secondary TR MR . , severe MR. 5. : severe MR: , - arterial pulse upstroke. Thrill -cardiac apex - LV Impulse . -) rapid-filling wave -3(S - apex . severe MR: . - pulse pressure ).(narrow - waves . - impulse . . 6. : : )1 (S " , - .chronic MR ,severe MR -2.S )3 (low pitched S - 0.12-0.17 sec ), - rapid-filling phase (, , -chordae tendineae . mid-diastolic ) .(MS )4 (S severe MR . :

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6/3 - severe MR. " )4-022 ,(Fig decrescendo - mid systole severe MR. MR - .axilla, - chordae tendineae ) (prolapse- regurgitant jet , - LA . , .AS - ,chordae tendinaea " ),"(seagull flial . - MR ,MVP ) ( - strain phase .Valsava maneuver 7. : ECG , . . - severe MR, .AF . , . ECHO TTE : -.MR . --LV end-diastolic & end systolic volumes-.EF , - ,chordae - LA- ,LV - ,annulus . : - MR jet . -) jet .(continouse wave Doopler signal . - LA- LV . -.RF . -.TR jet velocity - TTE chronic MR . - ECHO MVP . TEE .(Fig 222-3) TTE CXR- LA- LV - MR. , - LA . , , -.Kerley B lines MR+MS . - annulus , . severe MR, - -jet -.upper lobe pulmonary vein

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8. Mitral Regurgiration )4-032 :(Fig )2-032 :(Table - severe MR . , Warfarin AF INR 3-2. cardioversion . , - vasodilators - isolated severe MR . - MR ) dilated cardiomyopathy -( , - ACE inhibitors ,blockers-.digitalis severe MR -LV . severe MR ) (urgent . , ) IV vasodilators ,(sodium nitroprusside intraaortic balloon counterpulsation -) MI severe MR(. : severe MR , ) ( - MR . )3-032 .(Table " valvuloplasty .anuuloplasty ), , (. , , - valvuloplasty . - severe MR , )4-032 .(Fig AF ) 50 mmHg 06 mmHg(. , severe MR - LV dysfunction , LVEF - %06 / ) - (Echo 04 ". , .myxomatous disease, primary valvuloplasty repair 57 - LV ,CAD -%1 ) (. -%59 .myxomatous disease . -%1 01 . ,AF Maze procedure radiofrequency ablation - .pulmonary V osteia - AF. )%03 < :(EF .

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. , , . , ) CAD .(bypass , myocardial dysfunction , - .LVEF acute severe MR , . , Left ventriculography severe MR - TTE - TEE .Cardiac MRI coronary arteriography - . :(MVP) Mitral valve prolapse MVP : .systolic-click murmur syndrome .Barlows syndrome floopy-valve syndrome .billowing mitral leaflet syndrome . , myxomatous degeneration acid .mucopolysaccharide ,MVP , . type III , . MVP : ) Marfan syndrome 753(. osteogenesis imperfecta -.Ehaler-Danlos syndrome MVP - ,Marfan syndrome , ) .(Stright back syndrome ,MVP- myxomatous degeneration ) ( . ) (, - annulus . , , - chordae tendineae, . - MVP : -.acute rheumatic fever -.IHD -cardiomyopathies -%02 .ostium secundum ASD 83 , 71 @ , ', "

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- MVP , - dysfunction . - chordae tendineae - annulus , , " ."vicious cycle -) ECG ( - dysfunction . 1. : MVP 03-51, . , - MVP ) 05(, " , - MR . , . - MVP , + murmur + systolic click prolapse severe MR - chordae- prolapse . ) (. - chordae . . , - MVP - isolated severe MR . , : .ventricular premature contractions .paroxysmal supraventricular or ventricular tachycardia .AF , , . severe MR - .LV .flial leaflet , , -.angina pectoris Transient cerebral ischemia , . MR/ . 2. : - ,mid or late systolic click - 0.14 sec )1 (S -chordae tendineae - prolapse . - systolic clicks ) (multiple ,cresendo-decrescendo ) whooping ( ") "honking( - .apex- click : . ) (strain phase-.valsalva maneuver

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-) LV - prolapse (. , squatting ) -.(LV - MVP - click-murmur complex ) ( . mid-systolic click , - .click . 3. : - ECG : T -leads II,III, aVF .supraventricular or ventricular premature beats TTE - prolapse . MVP )- parasternal long (axis view 2 " - LA -.annulus - color flow- continous wave Doppler -.MR TEE , . - Left ventriculography - prolapse . 4. MVP: . blockers . - ,severe MR ) ( ]4-032 .[Fig ,TIAs .warfarin :(AS) Aortic Stenosis AS -%52 . -%08 AS . 1. )1-032 :(Table AS : -aortic cusps ' . ) Age related degenerative calcific AS senile AS (sclerocalcific AS - AS . -%03 56 sclerosis . ,AS , %2 . , - aortic stenosis - 04

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cusps ) 2.5 m/s" .(Doppler - aortic sclerosis .CAD , . ) , , , ,DM , , - ,LDL - HDL - (CRP . ' ) 922( , . ', " ,(BAV) bicuspid aortic valve . , , , . - ,commissures .bicuspid , . - LV outfolw , , ) ( . Rheumatic AS - Mitral valve-.AR 2. -:LV outflow - ,AS -:LV outflow ] (HOCM) Hypertrophic Obstructive Cardiomyopathy 132[ . .discrete congenital subvalvular AS ) supravalvular AS 922(. - LV outflow -.Doppler echo 3. : - LV outflow - LV. , - LV -.SV , / , - output . , - systolic stress , S=pr/h) Laplace ) r=radius ,p=pressure ,S=systolic wall stress (afterload-.(h=wall thickness -CO - ,LV . - 40 mmHg CO -2) 1.0 cm -] (0.6 cm2/m2 body surface area [( -LV outflow )4-322 .(Fig - LV end-diastolic pressure severe AS - LV/ -.LV - CO ,severe AS . - AF - ,AV dissocoation

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. , - CO - LV - PA ,LA- RV. - LV . , ,obstructive CAD . , ) ,(subendocardium . 4. : - AS - 2 .1.0 cm severe AS - LV SV. ) pure AS AS( -8-6 . : . .Angina pectoris . . - LV . Angina pectoris . , - CAD ) ,(AS . , - angina pectoris - severe AS .obstructive CAD ) (exertional syncope : - exercising muscles - nonexecrsicing CO. - CO . - CO - ,AS , , , cachexia CO . PND , Orthopnea ) - ,(LV . , , , - AF- ,TR .isolated severe AS AS- ,MS - CO - MS " -.AS 5. :

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. , AF . " . , - SV, ).(narrow pulse pressure ) pulsus parvus .([Fig 220-2] et tardus , . - a wave ' . . - impulse . impulse , . Thrill , - suprasternal notch . 6. : early systolic ejection sound AS '. . - AS , )2 (A )2 ,(P ) 2] S 022[(. AS , . , )4 (S - LV -.LV end diastolic pressure )3 (S - LV . AS ejection mid-systolic )1 ,(S - ,ejection )4-022 .(Fig : low pitched ).(rasping , . . )(, .(Gallavardin effect) MR CO, 6/3. , ) - SV ( . 7. : ECG severe AS - .(Fig 221-9) LV , ST depression ) T (LV strain- Leads I & aVL- .left precordial leads, -ECG , - ECG LVH .

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ECHO LVH ) AS(, )2-222 .(Fig - cusps .bicuspid - TEE , TEE . " Doppler . : 2 Severe AS - .1.0 cm moderate AS 21.0-1.5 cm mild AS 2.1.5-2.0 cm - LV . : MS ) AR -(AS AS .outflow obstruction ) 5.4 "( - ascending aorta -%02 ,bicuspid aortic valve . Dobutamine severe AS )%53 < .(EF CXR ) (. - apex AP . Severe AS - ascending aorta ) .(post-stenotic dilatation, , .Marfan syndrome " . -.severe AS , , , , - PA ,LA . AS . ) ( . " : - . noncalcific congenital AS - ,LV outflow ] [ -Percutaneous Aortic Balloon ) PABV (Valvoplasty ,AS . - Balloon valvotomy ) (, . - LV outflow . 44 , 71 @ , ', "

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CAD 54 severe AS . CAD ) (AVR %05 . 8. :AS severe AS . , : 3 Angina pectoris ) -5 ?( 3 Syncope . 2 Dyspnea . 1.5-2 CHF . , -%08 ,AS -4 . ,AS , , -%02-01. , , ) -%1 ( .severe AS , - Calcific AS , -annulus 0.1 cm2/year .7 mmHg/year 9. Aortic stenosis: )5-032 (Table 230-2, Fig : ,(1.0 cm2 >) severe AS , . -.CO ,CAD blockers- ,ACE inhibitors . Ntroglycerin -.angina pectoris calcific AS ) HMG-CoA reductase inhibitors( ) (. atrovastatin , rosuvastatin . - AS . : severe calcific AS " . : 2 2 2 ) severe AS - 1.0 cm - 0.6 cm /m body surface (area . )%05 < .(EF ) 4.5 cm - ,(0.5 cm/year . , ) (AVR -%3 )3-032 .(Table severe calcific AS

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)%05 > ,(EF . , AVR . . . -%03 .AVR - AVR severe stenosis .CABG, - AVR moderate AS CABG . ) ( , , -, CO- SV-) EF .(low gradient low output AS )%02-51(, . , - LV dysfunction . , , . ) dobutamine echocardiography -%02 - SV .(dobutamine AS- ,CAD - AS " ) (aortocoronary bypass grafting )3-032 .(Table calcific AS , , .AVR - .AVR . -01 AVR -%06. -%03 primary valve failure 01 , -%03 . :(PBAV) Percutaneous Balloon Aortic Valvuloplasty noncalcific AS '. , severe calcific AS restenosis , ) (bridge to operation LV dysfunction . :Aortic Regurgitation 1. )1-032 :(Table AR : . . : AR, , , - cusps

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. isolated AR . ,congenital bicuspid aortic valve AR. , ' - ,mild AR Membranous subaortic stenosis -.AR Prolapse - ,cusps AR, - %51 .VSD - ) myxomatous degeneration / (. AR , , , . syphillis ankylosing spondylitis - AR ) " , (. - AR, . AS- AR AR AR '. AR , - annulus . . : AR ) ,(aortic root disease . - annulus -) AR 242(. - annulus - AR : Cystic medial degeneration- ,ascending aorta ) ( .Marfan syndrome . .annulo-aortic ectasia .osteogenesis imperfecta . , - AR - annulus. syphillis-) Ankylosing spondylitis (, , . Syphilis ) 261(, , - intima , . 2. : - total SV " -) LV - forward SV ( .AR AR ) ,(free AR - Forward SV.

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- ,MR - LV SV , - AR - SV )(. -) LV end diastolic volume - (preload - .AR SV . , Severe AR forward SV EF ] - EF - total SV - ,[EDV - LV end ) diastolic pressure & volume ,Laplace - LV (. , AR - LV preload & afterload, , . , - End diastolic volume - Forward SV- EF. - LV . - AR, ) .(1000 g ) - ,(LV )5-322 ,(Fig - decrescendo . severe AR, . severe AR, - LV " " . ) (, - LV ) 04 .(mmHg , . severe AR, - forward CO , . LV dysfunction - .EF - ,LA- ,PA wedge pressure- PA- RV -) forward CO (. myocardial ischemia AR : , . , , -, . - ,MI - ,subendocardium .CAD 3. : -%57 ) pure AR AR( . , ) - (AR . ' , .

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severe AR, , , - LV - ,SV )- PA wedge (pressure / . severe AR, , 51-01 . , , , . , ) (, premature ventricular contractions ) .(Head pounding , . PND ,orthopnea . ,severe AR , .CAD . Nocturnal angina , . , - nitroglycerin . , , . 4. : - severe AR, ) ( , . - ,AR - ankylosing spondylitis ,Marfan syndrome-.VSD ):(Arterial pulse - free AR: )" ("water hammer ).(Corrigans pulse )(Quinckes pulse " " ) (pistol shot ) ,(Traubes sign ) (Duroziezs sign " . ) (widened ) - (300 mmHg . " sphyngomanometer . , - Korotkoff sounds" . - End diastolic pressure , ) - end diastolic pressure- .(LV , severe AR - .pulse pressure -.CO :

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severe AR, - impulse . , - apex . - Thrill - ,Left sternal border - thrill - suprasternal notch . - thrill AS. pure AR ,AS + AR bisferiens ] 2 ).[(Fig 220-2D : :severe AR )2 (A . )3 (S systolic ejection . )4.(S chronic AR: . high pitched, blowing, decrescendo -Left sternal border ).(Fig 220-4B ,mild AR , , -.holodiatolic , , . - AR , - sternal border . , - sternal border, - AR . " " ) (eversion - cusp . mid-systolic .isolated AR . . severe AR -,Austin Flint murmur , rumbling- .low pitch " - AR . , - AR , . - severe AR, - end-diastolic pressure : . ) ( . - pulse pressure .AR 5. :

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ECG severe AR, - ECG .LVH, ST depression T- ,leads I, aVL 6) V5 & V .(LV strain / QRS , . ECHO -, . - jet . - ,AR " - annulus )3-222 .(Fig . ,AR . - AR : - jet %56 -.LV outflow tract -.60 mL/beat - Regurgitant fraction %05. -.proximal descending aorta - continuous wave Doppler severe AR , - diastolic pressure . " 2D-echo dysfunction . CXR- severe AR, - apex . , left anterior oblique , . - AR , , -. - CT angiography - CXR . 6. : , contrast aortography . Coronary angiography . 7. Aortic Regurgitation )6-032 :(Fig - AR: severe AR IV ) sodium ,(nitroprusde " . - .Intraaortic balloon counterpulsation blockers - CO . , . - AR: ) ) CCB ,ACE inhibitors (dihydropyridine .(Hydralazine ) (.

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- severe AR dysfunction . ) -(140 mmHg AR, - . -.SV severe AR . - nitroglycerin- nitrates ,IHD . syphilitic aortitis course ) 261(. blockers ) marfan syndrome .(AR , severe AR . : , : 1. severe AR .myocardial dysfunction 2. ) - LV ,(dysfunction . , severe AR, 6 ), - LV dysfunction (. . ) (AVR severe AR . , severe AR : %05 < .LVEF 55 " - end-systolic volume 2.55 mL/m 57 ". severe AR 21-3 . . , AVR AR . , - annulus , . - AR - annulus-ascending aorta ) (, - annulus " . -%05 acute AR Type .A aortic dissection, , "

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, - ascending aorta ) AVR(. , - stage . - AVR -%3 )3-032 .(Table, - LV dysfunction -%01 - %5 ) (. , , . severe AR . :(TS) Tricuspid stenosis ,TS : - MS . . " .MS TS -%01-5 .severe MS TS " .TR - TS . 1. : - RA- RV - .TS . 4 mmHg" . , , . : - a wave ) (. - y descent . - CO " , . - CO ) ( - PA ,LA- RV .MS, TS MS ) -.(TS : MS" - ,TS . .TS , , . , - CO , , .TS , - TS .mitral valvotomy

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: - TS , . severe TS , : . . . anasarca . . ' , a. - v waves , , y descent. - . , - opening snap -0.06 sec . - ,TS - ,MS - TS ,MS -) TS (. , - left lower sternal margin - ,xiphoid process - . - TS , - strain - ,valsalva maneuver . : - ECG )8-122 (Fig: P -.lead II P -1.lead V - ECG ) (RVH MS . - CXR MS + TS - RA- SVC - PA ) MS (. , " , ) (dome . " .doppler echo -TTE , -.PA Tricuspid stenosis: TS . , , . ) (. : moderate to severe TS - 4 mmHg -2.1.5-2.0 cm 45 , 71 @ , ', "

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- TS - TR. . , ) (. ) (. :Tricuspid Regurgitation - TR TR - annulus . TR , . TR ' ) (PA systolic pressure > 55 mmHg TR - IHD-.dilated cardiomyopathy - TR . TR, .TS - TR : . .tricuspid valve prolapse .carcinoid heart disease .endomyocardial fibrosis ' - atrioventricular canal - Ebsteins malformation ) 922(. - TR . ,TS TR - .CO - TR , , . : v wave y descent. . . . . . - hepatojugular reflux. - Left parasternal region - ,Lower left sternal margin - strain valsalva maneuver ) AF .(Carvallos sign" . - ECG" - RV -.TR , .severe RVH - ,prolapse, '.

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TR " ,color flow Doopler " - Severe TR .(Fig 222-4) doppler . - continuous wave doppler . - RV-. RA :severe TR - CO " . - pulse pressure x descent c-v wave y descent. - end diastolic pressure " . Tricuspid Reurgitation: ,Isolated TR , , " . , - PA pressure . -TR , " - annulus. , TR , - PA ) ( - TR . , ,severe secondary TR , - annulus, tricuspid annuloplasty )" " ( open tricuspid valve repair )3-032 .(Table Tricuspid annuloplasty - severe TR . :Pulmonic Valve Disease " , . PR - .severe pulmonary hypertension - Graham Steell murmur High pitched, decrescendo - ,Left sternal border -) AR (. - PR " , , ) ( . : carcinoid syndrome - PS .PR PR Tetralogy of Fallot -.RV outflow tract PS ' 922. :

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: 1. . 2. . 3. , . : ) , , (. . : , . . ,stenosis regurgitation .mixed , . -3 , ,chronic AF . , , . -%03 01 , -%05 51 . . . : 56 ) (. ) 56( ) .(AF , ", ) 56(. : . . ] [. 56 . , 56 .

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56 ) (. : . . : ) Xenografts (. (allograft) Homograft ] [. Pulmonary autografts . , , 56, . - St. Jude ,prosthesis , , . : , , , ", . , . Rheumatic fever . 000,001/1 -, -000,001/051 . -%56-51 . : -.GAS pharyngitis , ) (, , 02. . ,TS , -, . 0002, -000,001/5.5, . , , . . -%21 57. : . - vascular grafts - . . . 85 , 71 @ , ', "

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bicuspid aortic valve -%2-1 . , ' .

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132 :Cardiomyopathy & myocarditis : . ischemic .cardiomyopathy cardiomyopathy , . : primary type / 1. . Secondary type 2. )1-132 .(Table , Restrictive ,Dilated Hypertrophic )3 & 2-132 .(Fig 231-1; Table , . ) ( . :(DCM) Dilated Cardiomyopathy CHF .DCM / , ).(remodeling - remodeling ) (. , - DCM ) ( , . - DCM acute viral ) myocarditis (, . - DCM , 4-3. DCM : alcohol abuse . 1. : -%03-02 DCM . -02 . ] heavy chain -tropomyosin , & myosin , cardiac actin - .[Troponin T,I,C " / .

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DCM , Duchenne- ) Emery-Dreyfuss muscular dystrophy (. - ) Lamin A/C ( , DCM - AV - .(SCD) sudden cardiac death X- linked recessive " - dystrophin . . 2. : ) CHF ( . . , angia pectoris ) .(IHD ) ( . 3. : CHF . , - pulse pressure ' . , MR/ .TR 4. : , . ) (. - ECG : AF low voltage ST-T / -.AVN , CT- CMRI , , . brain natriuretic peptide . - coronary angiography . - . , Cardiac CT .MR DCM , . Transvenous endomyocardial biopsy DCM , , , . 5. Dilated Cardiomyopathy:

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, , 55, 4 . - CHF, . -%52 . , -SCD . , . - ,HF 722 . , -CCB - .NSAIDs - - . - ICD - DCM HF . , . . :Alcoholic Cardiomyopathy ) (90 g/d - DCM . . ) ,(alcohol dehydrogenase - ACE . , , . -%52 3 . , . ) - .(Supraventricular ,holiday heart syndrome . ) AF AFl-.(VPBs , ) 03-02 (g/d ,cardioprotective - HDL cholesterol ,IHD . . :Peripartum cardiomyopathy - CHF 6 . , , . peripartum CM : multiparous 03 ) (. , DCM, ICD . -%01. - .CHF , , - .CHF, , / - LV EF 6 , , . ,

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- peripartum CM , - LV dysfunction . . :Neuromascular Disease .muscular dystrophies: - - Duchennes progressive muscular dystrophy ) (dystrophin . ECG R - right precordial leads 0.1 > ,R/S ratio Q -leads - .limb leads, - .supraventricular . Myotonic dystrophy - ,ECG ,AV . , . , ICD/ . . : " - .DCM - ,ECG - fulminant CHF. ,anthracycline ,(Adriamycin) doxorubicin - . ) 2] (450 mg/m 2 .[550 mg/m .ATP ) ( : . .cyclophosphamide ,doxorubicin . Doxorubicin 3 . -.doxorubicin " , ] radionucledie ventriculography [, " , - . , - doxorubicin .HF " , cardio-protective ) dexrazoxone ,(iron-chelator . , -.ACE inhibitors ) Trastuzumab(, , -%7 4 .doxorubicin

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cyclophosphamide - CHF . . nonreceptor tyrosine kinase ,(imatinib mesylate = Gleevec) inhibitor -.chronic myeloid Leukemia - ECG : tricyclic antidepressant . .Emetine .aerosol propellants , ,SCD, DCM -) acute MI / (. , ,CCB . . :(ARVC/D) Arrhythmogenic RV cardiomyopathy/Dysplasia - ARVC/D - ) (. -) desmosomes (. -. - 2-plakophilin )2- .(PKP - rynodine receptor gene... , , . , . - ECG QRS- right precordial leads .LBBB- CT- MRI , )2-132 .(Fig - beta blockers amiodarone . ICD . , . . :Tako-Tsubo (Stress) Cardiomyopathy .Apical ballooning syndrome . 05 - ST elevation/ T- .precordial leads, . akinesia - .EF . " "ballooning , . , , 7-3 . - Tako-tsubo CM , -surge ,

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. beta blockers, . . :(LVNC) Left Ventricular Noncompaction LVNC ' . ,CHF . , deep recesses- sinusoids ) (. - sinusoids ) (compaction , , -.LVNC " , , . , - ,CHF" -. :(HCM) Hypertrophic Cardiomyopathy HCM ) (, ) .(AS- HCM -005:1 . - HCM : 1. -, . 2. -) ,LV outflow tract (LVOT ) .(subaortic area, -%03 HCM - LVOT %03 . ,diastolic dysfunction Doppler -) LV EDP (. - HCM ) .(AS , . , . , , . 1. : -%05 HCM . -004 -11 - sarcomere, -%06 . - myosin heavy chain gene 41 ] -- myosin Light chain ,actin ,cardiac troponin (C,I,T) ,myosin heavy chains'[. . HCM . 02, " , -%05 HCM

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. , , - ,LVOT . , . screening" 02-21 42-21 , " . 2. : , HCM " -.sarcomere ,HCM " . , . 3. : - HCM . , . , ,SCD . , HCM - SCD . , , , ) (, , . : . angina pectoris . -.LVOT 4. : double or triple apical impulse . - LVOT . - hallmark - obstructive HCM , ,harsh , . - lower left sternal border , , - MR -.obstructive HCM 5. : , - ,AS - ,HCM , . - LVOT systolic anterior (SAM) movement . : 1. . 2. - preload. 3. - afterload.

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] [, - preload ] - ,Valsalva maneuver [nitroglycerin - LV EDV . , ]" squatting [ ]" [ ] [ , . 6. : - ECG , Q . . , ) SVT (AF ) ,(VT )(. , - . HCM , , " 3.1 . ,ground-glass . - SAM , - MR - .LVOT ,HCM . , , T - ECG ) .(spade shaped . - MRI . ,HCM - HCM: , , " - .subaortic , " : isoproterenol amyl nitrate valsalva maneuver .premature ventricular contraction 7. Hypertrophic Cardiomyopathy: - SCD , . . beta blockers - angina pectoris-%05-03 . " , . , - beta blockers .SCD

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amidarone SVT , -.SCD ) CCB Verapamil] nondihydropyridine ([diltiazem , -.LVOT Disopyramide - ,LVOT . : ) CCB (dihydropyridine -beta agonists -.LVOT , . ,AF , . beta blockers -AVN . myotomy/myectomy " -%57 . " -) septal artery (alcohol septal ablation . , " . ICD -) SCD (. 8. : HCM , . AF , - onset . -%01 . HCM . - HCM ) - Dilated (cardiomyopathy -) LVOT (burnt out HCM -%01-5 - CHF . - HCM - ,SCD . - SCD : . . VT ) 03 "(. 86 , 71 @ , ', "

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.SCD ) (.

. :Inherited Metabolic CMs with LV Hypertrophy :Cardiac Danon Disease -(LAMP2) lysosome-associated membrane protein .X inclusions .PAS CHF . - ECG - preexcitation. :Glycogen storage CM )2 (PRKAG - adenosine .(AMPK) monophosphate activated protein kinase - HCM . :Fabry disease X-linked autosomal recessive - lysosomal -galactoside A - , - .HCM , - .restrictive CM - AV conduction . MRI . .agalsidase :Friedreichs ataxia spinocerebellar ,frataxin . -%05 . - ECG - ST segment - .T ) CT (MRI - . - Friedrichs ataxia ,HCM ) Cellular .(disarray :(RCM) Restrictive Cardiomyopathy - hallmark RCMs . . . , ) (, . , - RCM. , -restriction : Sarcoidosis hypereosinophlic disease 96 , 71 @ , ', "

17

'. , . 1. : - CO . , . , ) ,(dependent, , . ' ) ( ) .(Kussmauls sign , . - ,constrictive pericarditis - RCM , - impulse " , MR . 2. : , - ECG" - ,ST-T wave low voltage . -,X-ray - ,constrictive pericarditis - .RCM, CT- MRI , . " . .diastolic dysfunction : -.CO - End diastolic pressures . dip and plateau -.constrictive pericarditis RCM- constrictive pericarditis " " . : ) RV transvenous endomyocardial biopsy -(RCM -) CT/MRI - constrictive pericarditis - .(RCM 3. Restrictive Cardiomyopathy: , ) (, - ) Fabrys disease (. - . . :Eosinophilic Endomyocardial disease Lofflers endocarditis Fibroplastic endocarditis . hypereosinophilic syndrome . , , .

27

07

, 71 @ , ', "

, - posteriorbasal . MR . , . . : -afterload -. - hydroxyurea . / . . :Cardiac amyloidosis -) primary amyloidosis (AL- ) ,hereditary amyloidosis (ATTR . , , , ).(waxy , . , ) senile cardiac ,(amyloidosis , . ) (: .diastolic dysfunction .Systolic dysfunction . .Orthostatic hypotension - 2D echo "". - MRI - gadolinium- .subendocardium ) ( -%57 . ) alkylating agents (melphalan , . ) -(hereditary amyloidosis . , , . . :Other Restrictive Cardiomyopathies ) Iron-Overload cardiomyopathy( : ) (-thalassemia ) ( , . " . - MRI -2 T .

37

17

, 71 @ , ', "

Phlebotomy . deferoxamine iron chelators . Myocardial sarcoidosis" . , . , High grade AV ,block. RV overload . . - carcinoid syndrome + / / . fenfluramine-.phentermine :Myocarditis ), (, , . ) ,tricyclic antidepressant - ( , . )1-132 ,(Table ) "( ] " - HCV ,Adenovirus ,Coxsacievirus B- .[HIV / . 1. : -upper respiratory tract , - nasopharyngitis .tonsillitis - ECG - ,ST-T , , . , acute coronary ,syndrome , - ,ECG , . , , .MR . , , . , , round-cells . MRI . - MRI . self-limited , . , - LVEF ) -%53(, -.DCM

47

27

, 71 @ , ', "

2. Myocarditis: , - ECG . CHF . . -,CHF - AV block - ECG . , , , . . :HIV HIV -, , , . -%01 .HIV LV dysfunction " - HIV. , : , ] .anti-HIV , - HIV myocarditis , , - anti-HIV[. . :Bacterial myocarditis , , Infective endocarditis - . Diphtheric myocarditis -%52 . . , -. . - CHF . anti-toxin . . . :Giant cell myocarditis - CHF . -4-3 . . : - giant cells . - giant cell myocarditis , . , . . :Lyme carditis - Lyme disease " tick-borne spirochete , - upper Midwest" . -%01

57

37

, 71 @ , ', "

. -AV conduction . , , . IV ceftriaxone ) lyme carditis amoxicillin doxycycline (. ECG .second or third degree AV block AV block, . , - AV block . - Lyme disease . :Global Considerations :Chagas Heart Disease , " .tryponosoma cruzi " - reduvid bug , . . , %57-02 . -81 , 000,002 . " . , . -%1 3-2 . , ) ,(indeterminate phase-%03 . " . , ) ( - .AV block , , , , )3-132 ,(Fig . CHF . CHF SCD . - ECG ,low voltage RBBB - ,Left anterior hemiblock - .complete AV block- CXR . . , . Chagas Heart Disease: - CHF . PO amiodarone . -AV block . - . / ) (, . :Cardiomyopathy in Africa

67

47

, 71 @ , ', "

:Endomyocardial fibrosis .RCM - ,tropical and subtropical Africa , - tropical Asia . - hypereosinophilic endomyocardial ) disease (, - .endomyocardial fibrosis - ,CHF -%52 . -) AV valves (. " . . , . " , . :Dilated Cardiomyopathy DCM -%05 .CHF DCM . - Coxsackievirus B DCM, African trypanosomiasis - .Cameroon %05 .DCM, - thiamine . Peripartum cardiomyopathy . . , ,multiparity , intake , -.selenium :Cardiomyopathy in Asia ) 1 (B , , ) (. . - wet beri-beri heart disease . high cardiac output . , , , . ECG ,low voltage T .QT-CXR . , . - Selenium . - ,Keshans disease DCM , .

77

57

, 71 @ , ', "

232 - :pericardial disease : " ) (15-50 mL ) ultrafiltrate (, . , " : , , . - kinking . ", , ' , . , - main pulmonary artery . , . :Acute Pericarditis , , )1-232 .(Table : friction rub -ECG -.paradoxical pulse . - . , : tuberculosis -.uremic pericarditis acute pericarditis , , - left ,precordial , . ) , (. . , Acute MI . , . AMI- acute pericarditis , ) creatin kinase- (troponin, ) (peri-myocarditis . , , , , - ST - ECG. . 67 , 71 @ , ', "

87

- pericardial friction rub -%58 . , , . - left lower .sternal border . - rub ), , (. - pericardial rub , - pleural rub . - ECG-, acute pericarditis , - .(Fig 232-1) subpericardial- ECG 4 : - stage I - ,ST segment upward concavity - standard limb leads 6 ,V2-V -) aVR -1 ,(V ] PR [. - .QRS- stage II , - ST segments , ) ( -T ) .(stage III, - onset , - ECG ).(stage IV , - ,AMI - ST segment ,convex . - ,QRS ,Q Notching - .R- ,AMI - T - ST segments . , - ,AMI - ST segments . , - ECG -.AMI Early repolarization - ST ,segment -) left precordial leads .(acute pericarditis - T - ST/T ratio -52.0. , -.acute pericarditis 1. :Pericardial Effusion , / - ECG , . ) (. , . , - friction rub - impulse ) (. , ,Ewarts sign dullness )- (egophony - angle . - CXR " " )2-232 (Fig . : , , , , , )3-232 .(Fig - TTE echo-free - LV epicardium , - anterior RV-) parietal pericardium ( . .

97

77

, 71 @ , ', "

, - .ECG, . " CT MRI )4-232 .(Fig : loculated pericardial effusion . 2. :Cardiac Tamponade - inflow . . : . .Idiopathic pericarditis . , : ) , PCI ( -tuberculosis - hemopericardium acute pericarditis -. ) (Becks triad: Hypotension ) ( x descent -.y descent , -.CO ) (200 mL , ) (2000 mL . , . , , ), orthopnea (. , , '. , , - ,QRS- electrical alternans - ,P- QRS- T . 2-232 acute cardiac tamponade- .constrictive pericarditis :Paradoxical Pulse )(10 mmHg . - paradoxical pulse , " , " Sphyngomanometric . 87 , 71 @ , ', "

08

) - ,(pericardial sac . , . , , . , ', - ,y descent .pulsus paradoxus 2-232. - paradoxical pulse ) constrictive pericarditis (. , , ) (, -.pulmonary embolus Low-pressure tamponade -) 5-10 mmHg (. , - CVP ) (, .paradoxical pulse . . .pericardiocentesis : , " )2-232 .(Fig , - Doppler US , , . ) (collapse . TEE loculated . Cardiac Tamponade: acute pericarditis . , . . :Pericardiocentesis , pericardiocentesis ) ,parasternal - .(subxiphoid . . , . . subxiphoid thoracotomy : recurrent tamponade loculated effusions

18

97

, 71 @ , ', "

/ . . : " -tuberculosis - ,rheumatic fever , ,MI - pericarditis . . : . DNA Mycobacterium tuberculosis PCR - ) adenosine deaminase activity (30 U/L tuberculosis .pericarditis 3. :Viral or Idiopathic Form of Acute Pericarditis , -,influenza ,coxsackievirus A or B EBV ,CMV ,adenovirus ,chickenpox ,HSV ,mumps ,echovirus ,HIV / . , . , , , / . - ,HIV ) (mycobacterial ) (. -full ,blown AIDS . , , idiopathic acute .pericarditis , - .pneumonitis , 21-01 , acute pericarditis- ,AMI . , - friction rub . . - ST segment-ECG , - T . Pleurtis- pneumonitis - .pericarditis , constrictive pericarditis . Relapsing pericarditis -%52 acute .idiopathic pericarditis , . Idiopathic Acute Pericarditis: Hyperimmune globuline pericarditis ,CMV adenovirus- ,parvovirus INF- -.coxsackie B pericarditis

28

08

, 71 @ , ', "

- acute idiopathic pericarditis , + - ) (2-4 mg/d . , - ,NSAIDs 4 X 400-800 mg) ibuprofen ( X 0.6 mg) colchicine (. ) (40-80 mg/d ,prednisone purulent bacterial pericarditis ) (. - . , - NSAID . colchicine , , , , , , pericardiotomy . 4. :Postcardiac Injury Syndrome . : )(postpericardiotomy syndrome ) ( . .AMI - .acute viral/idiopathic pericarditis - ,acute pericarditis 4-1 ) 3-1 (AMI . - pericarditis . 004, - Pleuritis- .Pneumonitis -. -pericarditis - fibrinous ) ] (serosanguineous [. , , - ECG - acute pericarditis . : - ,acute idiopathic pericarditis . , acute fibrinous pericarditis acute . viral/idiopathic pericarditis .AMI -acute fibrinous pericarditis ,AMI , - friction rub . -) ECG ,Q ST , T- (AMI pericarditis-.AMI Pericarditis - post cardiac injury - acute idiopathic pericarditis . ,AMI , , . , - pericarditis .

38

18

, 71 @ , ', "

pericarditis acute idiopathic .pericarditis, SLE drug ) induced Lupus procainamide .(hydralazine - ,SLE . - pericarditis , SLE .ANA , : rheumatoid arthritis -polyarteritis nodosa ) (. . - acute pericarditis : Procainamide hydralazine isoniazid cromolyn -minoxidil . - pericarditis- acute rheumatic fever - severe pericarditis . Pyogenic (purulent) pericarditis : - .aseptic pericarditis Pericarditis of renal failure -%03 ) ,(uremic pericarditis BUN, dialysis-associated .periscarditis fibrinous . ,friction rub . NSAID . , - .pericardiocentesis , pericardial window .pericardiotomy ) , , ( . , , . " . / - chronic .constrictive pericarditis : .Syphilis ), aspergillosis ,blastomycosis-.(candidiasis ) ,amebiasis, echinococcosis-.(trichinosis

48

28

, 71 @ , ', "

:Chronic Pericardial Effusions . , . .tuberculosis 1. : Myxedema ) ( . , . myxedema " . . : . .SLE .rheumatoid arthritis . . -.chylopericardium . : . Tuberculosis . - ,pericardiocentesis .pericardiotomy - ) (bleomycin . :Chronic constrictive Pericarditis acute fibrinous pericarditis serofibrinous pericarditis . . ) (, , . chronic constrictive pericarditis : ) , (. Rheumatoid arthritis SLE

58

38

, 71 @ , ', "

. , / . chronic constrictive pericarditis "" ) .(tense- ,constrictive pericarditis . , . : - end diastolic volume - SV - end diastolic pressure - mean pressures, , ) 5 .(mmHg , - .chronic constrictive pericarditis, , , . - ,constrictive pericarditis ,M - x descent- .y descent- ,y descent , -constrictive pericarditis . - y descent , . ', . - ,constrictive pericarditis- pressure pulses " "square root . , , - constrictive pericarditis )2-232 .(Table : : )(girth , ,anasarca . , - orthopnea , . ) ( . , ) Kussmaul's .(sign - chronic pericarditis : TS 48 , 71 @ , ', "

68

-.restrictive CM - pulse pressure . -%03 , pulsus .paradoxus . . - apical pulse ) .(Broadbent's sign . ) ,(pericardial knock 0.09-0.12 s ) (. .TR - ECG low voltage - QRS .T AF -%03 . - CXR . -.tuberculous pericarditis ) , '( - chronic constrictive pericarditis . . , , , - (Fig 222-8) CT .MRI ) (TTE : - IVC -.LV , , constrictive pericarditis . ) - .(Doppler - shift . . , - Vena cavae )5-232 .(Fig, .constrictive pericarditis MRI (Fig 232-6) CT . constrictive pericarditis . : - ,chronic constrictive pericarditis cor pulmonale ; , .pulsus paradoxus, - ,cor pulmonale ) kussmaul's sign (. TS .chronic constrictive pericarditis , , . , - ,TS MS . , - ,TS paradoxical pulse - ,y descent - TS- chronic .constrictive pericarditis

78

58

, 71 @ , ', "

- constrictive pericarditis , ,restrictive CM -) constrictive pericarditis (. )2-232 .(Table restrictive CM: Orthopnea ) Gallop sounds .(pericardial knock Bundle brunch block Q -.ECG ) constrictive pericarditis ( )5-232 .(Fig ) CT ( - MRI restrictive CM- .chronic constrictive pericarditis, , . , , , CT MRI constrictive pericarditis ) (. Constrictive pericarditis: - ,constrictive pericarditis . 05 . . : , / . %01-5 . , . :Subacute Effusive-Constrictive Pericarditis tense effusion . chronic pericardial effusion .pericardial constriction " ) tuberculosis (, acute ,idiopathic pericarditis, , , . , paradoxical pulse- x descent ) y descent(. ,pericardiocentesis ,pericardial constriction square root sign - pressure pulse, - y descent. , - CVP , . pericardiocentesis . chronic

88

68

, 71 @ , ', "

.constrictive pericarditis . :Tuberculous Pericardial disease , " , , , ) - active tuberculosis (. . tuberculosis HIV , , . , tuberculosis pericardial effusion . , , ) " limited .(thoracotomy ) ,(caseation .anti-tuberculosis , pericardiotomy constriction tuberculosis -%05 ) tuberculosis (. Tubercular cardiac constriction .anti-tuberculosis , -effusive .constrictive pericarditis : Pericardial cysts , - right .cardiophrenic angle , , . ) (, , , . - .mesothelioma ) ( . ) (.

98

78

, 71 @ , ', "

332 :Tumors and Trauma of the heart : 1. : . %57 , - %05 . , , -%52 )1-332 .(Table , , . ; , . . : , . , , , , , , , ) (. , . :Myxoma , -%05-03 postmortem-%57 . , - 6-3 , . -%09 ; . ) (Carney complex: 1. ), / ( 2. .lentigines and/or pigmented nevi 3. ) primary nodular adrenal cortical disease , , / .(acromegaly : Nevi + Atrial myxoma + Myxoid neurofibroma + ) NAME syndrome (Ephelides (Lentigines + Atrial myxoma + Blue nevi) LAMB syndrome .Carney complex - complex ; , -) PRKAR1A gene ,(tumor suppressor gene - protein kinase A type .I- regulatory subunit , - myxomas ' - .glycosaminoglycans )(pedunculated 8-4 ". , , - interatrial

09

88

, 71 @ , ', "

septum - .fossa ovalis , : . . . . . . ) stenosis prolapse , regurgitation (. - outflow obstruction sub-aortic stenosis .subpulmonic stenosis , . ,low pitched " ,"tumor plop ) (early or mid . , : -ESR . digital clubbing Raynaud's phenomenon , collagen vascular disease -. ) TTE (TEE . )1-332 .(Fig CT- MRI , , )2-332 .(Fig , ) ( . , . , screening , -.myxoma syndrome : cardiopulmonary bypass , , .curative -%22-21 -%2-1 . . :

19

98

, 71 @ , ', "

, , ; , 51 " , , . -.CXR Papillary fibroelastomas . , , - ,TIA - .MI, . - Rhabdomyomas- fibromas , , , , ) ( .pericardial constriction - Rhabdomyomas Hamartomatous .growth-%09 , .Tuberus sclerosis ; , . , , , . Hemangoimas- mesotheliomas , , -.AV node ,Neurilemoma ,Chemodectoma ,teratoma Granular cell myoblastoma-.bronchogenic cysts

1.

2.

3.

4. 5.

:Sarcoma , . , ) , , , (. : -.Vena cava . - : , . , , / , . - ,cardiac lymphosarcoma . 2. : , . -%02-1 ,

29

09

, 71 @ , ', "

. , , . , . , . . ; , . , ) (. -%01 , , . . , : acute pericarditis ectopic tachyarrhythmias heart block -.CXR , ) (. , , , ) (. - ECG . - ,CXR . , CT-) Radionuclide imaging gallium (Thallium . cardiac MRI . pericardiocentesis . . : ; , . .pericardiocentesis ) (tetracycline , pericardial window . 3. :Traumatic Cardiac Injury ) (penetrating : Penetrating injuries , .

39

19

, 71 @ , ', "

Non penetrating Injuries , rapid deceleration injury . . - Myocardial contusion non penetrating cardiac injury . Myocardial necrosis - laceration . , ) ,(bundle brunch block - ECG - MI .pericarditis, - ECG . -) creatine kinase (CK-MB -%02 ) (falsely . - cardiac troponin . / , , , , . - radionuclide scanning , , . ) (, . , , ) TTE .(TEE , : hemopericardium )(free wall rupture -.(VSD rupture) intracardiac shunting , %04 . - hemopericard . , - post-cardiac injury ,syndrome -.post pericadiotomy syndrome , , , - ventricular fibrillation . , ,commotion cordis ) , , (, -) Repolarization - .(T .defibrillation transient cardiomyopathy - dysfunction - apex . , Tako-Tsubo syndrome ,Apical ballooning syndrome , ST , ) (. , - vasospasm. , .

49

29

, 71 @ , ', "

, - ligamentum ,arteriosum , - .Vascular deceleration injury aortic .dissection - amplitude , - CXR . , - ,aortic adventitia pseudoaneurysm . - hemipericardium/Tamponade . , %05 . : . , , . -,hemothorax - .hemopericardium . AV fistula , - .high output CHF , ,CHF - mitral regurgitation ) intracardiac shunt VSD ,ASD aotropulmonary fistula ,(coronary AV fistula . , . , , . Traumatic Cardiac Injury: ,MI ) anticoagulation (. . . Thoracotomy / . Pericardiocentesis .

59

39

, 71 @ , ', "

532 , . : - premature disability . , 0202, , , . , , : - MI-.angina pectoris - CNS .TIA , - intermittent claudicaton . - splanchnic circulation -.mesenteric ischmia ) renal artery (stenosis , ) atheroembolic (. , , , : , -proximal Left anterior descending (LAD) artery . ).(renal arteries - extracranial circulation , - .carotid bifurcation , )(branching points . )(. ) (Ectasia ) (. , ) ( , - ) intravascular ultrasound (IVUS .Postmortem " )" (. , , , . , , . : , ) stable angina ( - .intermittent claudication , , ,MI , . . 1. : 49 , 71 @ , ', "

69

" -" "fatty streak . - .intima )" ("leakness )1-532 (Fig - intima - ,extracellular matrix . , -) intima" glycosaminoglycans ( -.intima , - ,intima , . - ) (, , Oxysterols ,lysophospholipids ,hydroperoxides . , hypochlorous acid" ) myeloperoxidase ( . " . 2. : )1-532 .(Fig, , . " - atheroma : . adhesion - .atheroma, LDL - Adhesion. . , ? . )- (Shear forces ] [ . , ) ( . ., NO . , , ) " adhesion( . , - shear stress -)2 Kruppel-like factor 2 (KLF ) thioredoxin-interacting Protein (Txnip thioerdoxin ) - thioerdoxin ] [(. , -2KLF - NO synthase ,

79

59

, 71 @ , ', "

- Txnip -) thioredoxin - Txnip ( - . .- shear stress - superoxide mutase -. " - shear stress. - .intima , ) ( - adhesion . , 1- IL- TNF- adhesion . , . , monocyte chemoattractant )1- protein 1 (MCP . 3. :foam cells - ,intima -) lipid-laden foam cells , (. - LDL - uptake , : " ) LDL receptors familial (hypercholesterolemia - Xanthomata -.foam cells , - ,LDL receptor, . ) -(foam cells : "" - "scavenger" receptors ) (. LDL .-VLDL - - ,fatty streak : . -intima -.lipid laden macrophages - Atheroma: - fatty streaks , - fatty streaks atheroma, ? . , " "" , - scavenger receptors ) (

89

69

, 71 @ , ', "

, . , ,atheroma " ) (. . , reverse cholesterol transport " ,HDL - .atheroma - HDL .ATP binding cassette (ABC) transporter- 1 ABCA ) , - (foam cells - HDL ] ) Tangier disease .[(HDL- " "reverse cholesterol transport " - ,ABC transporters - HDL . , . , - foam cells -HDL ] , - HDL [. - ,foam cells , . ) (. ) (necrotic core . foam cells - ,fatty streak " extracellular matrix . ? - extracellular matrix . .extracellular matrix - , 1- IL- TNF- ) fibroblast growth ,PDGF factors(, . ) INF- (T , ) "" (. , - extracellular matrix , - ] fibro-fatty -) foam cells ([. ? PDGF , - tunica media -.intima ) -.(media TGF- , . )" ( ) (.

99

79

, 71 @ , ', "

, " , - fatty streak -.extracellular matrix , - .atheroma atherothrombosis . ? . , - fatty streak . -fatty streaks , - - extracellular matrix- BM ) ( ) PDGF- (TGF- . . , - Thrombin fibrin .protease-activated receptors , extracellular matrix" . ) ,fibrinolysis - (endothelial repair , " )-532 Fig .(2D 1. :Microvessels , microvessels -vasa vasorum . : -.atheroma - Microvessels . , , - microvessels- atheroma . .in situ - thrombin )- ,(prothrombin , . , - ,hemosiderin . 2. : . : .Osteocalcin osteopontin -. (BMPs) bone morphogenetic proteins , . 3. : , , . , - atheroma

001

89

, 71 @ , ', "

-) hypercellularity (. - athroma : TGF--) INF- ( - , . , , : . . ) -( -.extracellular matrix ) ( ". : . - atheromas , . , . ? - Remodeling - (Fig 235-2A) atheroma . - ,atheroma " )] (outward, abluminal [. , , .compensatory enlargement, - athermoa -%04 - internal .elastic lamina, , - atheroma . , " . : ) demand-induced angina pectoris (. intermittent claudication . , , " atheroma , ? , . , ) ( ) (. " " ". - , MI ) -%03 ( ) angina pectoris (.

101

99

, 71 @ , ', "

: ) (. : / unstable angina -.(Fig 235-2B) acute MI ,carotid atheroma -nidus -.TIAs - fibrous cup ) (Fig 235-2C - foam cells ) (. , resting .angina , - ,acute MI . / , - fatty streak ) Fig .(235-2D , , extracellular matrix . Atheroma . - acute MI , , : fibrous cup . . . , : , ) (T . ], HLA-DR - .[atheroma, - .HLA-DR, . , T HLA-DR . , - acute coronary syndromes . , .HLA-DR - fibrous cup , : IFN- ,T " . T - extracellular matrix -] fibrous cup - fibrous cup [. 001 , 71 @ , ', "

201

, - extracellular matrix, - fibrous cup . : : . -) Framingham Heart Study ( , . . , 2 : " / . - Evidence-Based , : . , ) ,Lipoprotein (a . , , ,CRP . 1-532 " - .ATP III . 1. : . 053 . - guidelines -:ATP III screening 02. - screening : Total cholesterol Triglycerides LDL HDL 5 . - guidelines ATP III . , )2-532 .(Table - guidelines ) Table 1-532(: -2 )].(LDL goal < 4.1 mmol/L [ ,ipsilateral/contralateral , . " bilateral .adrenal hyperplasia adrenalectomy , , curative-%07-04 . . 3 , . , potassium-wasting .fludrocortisone bilateral hyperplasia . , , aldosterone antagonists .potassium-sparing diuretics Glucocorticoid remediable hyperaldosteronism , , " . " . , angiotensin II - zona glomerulosa, ACTH - zona fasciculata. , 8, - ACTH - .zona fasciculata , - zona fasciculata ) 18-hydroxycortisol- (18-oxocortisol . ": urine excretion rates 03-02 " . , - ACTH , . .spironolactone 5. :Cushings syndrome -%08-57 .cushings syndrome " . , , screening " 24h free cortisol " .overnight dexamethasone suppression test - .screening test . . 6. : ) (Pheochromocytoma - - )(Paraganglioma -%50.0 . %02 . - .(Table 241-4) MEN type 2A & 2B , . ) (, , . , " , hypotension .hypertension / )4-142 .(Table 49 , 71 @ , ', "

372

. . -%09 . 7. : obstructive sleep apnea . , -%05 .OSA - .sleep apnea %07 OSA . - OSA . ) polysomnography (. , - sleep apnea ". (Continuous Positive Airway Pressure) CPAP - .OSA - ,CPAP . Coarctation of the aorta ' . 0001/8-1 . " -%53 .Turners syndrome , %03 . . , : , , . -.left posterior intrascapular " CXR-.TEE ,balloon angioplasty -. , ,IHD . - ,acromegaly . , . , ) ,(primary hyperparathyroidism . ) .(OTC :Monogenic Hypertension )4-142 .(Table , " . mineralocorticoid-induced -Hypertension , : ,17-hydroxylase deficiency )3-142 .(Fig , , psudohermaphroditism primary amenorrhea . - negative feedback ACTH , " -) ACTH ( . 59 , 71 @ , ', "

472

, .deoxycorticosterone , , . - 11-hydroxylase deficiency - salt-retaining adrenogenital ,syndrome -000,001:1 . , ) ,(deoxycorticosterone . , , , - ambigious genitalia, , - precocious puberty . , , . ).(late onset 11-hydroxylase dehydrogenase deficiency , -,cortisone . , licorice .glyccherizic acid . - Liddles syndrome - amiloride-sensitive (ENaC) epithelial sodium channels - distal tubules, - reabsorption . " -.amiloride " .progesterone : 1. : : screening screening . . , . , . : . 5-142 . 2. : . ) (, ) .(aneroid

572

69

, 71 @ , ', "

. , 5 . - cuff , -bladder cuff %04 , %08 . - ,cuff , - deflation - .(2 mmHg/sec) cuff ) Kortokoff sounds ( , - Kortokoff sound . , ,oscillometric 03-51 . , -" ."white coat syndrome-7 JNC : .Treatment resistance .Symptomatic hypotension .Autonomic failure . 3. : , , . , , , postural .hypotension , 03. ) .(AF /. ) Funduscopic ,(examination , . . , : -arteriolar light reflex ) (malignant . , . " PMI, . , , - .renovascular hypertension polycystic kidney disease . , CHF . 4. : 6-142 . , , , - , . .

672

79

, 71 @ , ', "

Hypertension: 1. : . -pre hypertension . . , - ) (NaCl . , , . : - intake - intake )7-142 .(Table . . 6.3/3.1 mmHg 9.2 Kg . : . " 03 ) (, 7-6 , ) (. - ,NaCl . -, " -intake NaCl- 4.4-7.4 g 3.7-4.9/0.9-2.9 mmHg , . , . . - ) (, , . drinks ) drink 41 ( , . , . - DASH trial , , ) .(mild hypertension - intake - 6 . , . . 2. : .140/90 mmHg - . - 10-12 mmHg - 5-6 mmHg relative risk reduction %04-53 -%61-21 CHD 5 89 , 71 @ , ', "

772

. -%05. -, counterregulatory . - -, , , : , , )8-142 .(Table . : thiazide diuretics , - . - Na/Cl pump- distal convulated tubule . , . , , , . ACE inhibitors ,beta blockers .ARBs , - CCB . hydrochlorothiazide .6.25-50 mg/d ), (, . Potassium-sparing, amiloride- ,triamterene - epithelial sodium channels . - . - loop diuretics -Na/K/2Cl cotransporter -) thick ascending limb- .(Loop of Henle : -) GFR < .([220 mol/dL] 2.5 mg/dL CHF ) .(minoxidil . -:Renin-Angiotensin System -:ACE inhibitors ANG II -bradykinin . -) ANG II receptor blockers (ARBs : -AT1 receptors - ANG II - AT2 receptors . - , CCB- .-blockers ACE inhibitors- ARBs -efferent . :

872

99

, 71 @ , ', "

CHF -.NSAIDs -%51 , - angioedema -%1 .ACE inhibitors- angioedema - . - ACE inhibitors -.ARBs . :Aldosterone Antagoinsts Spironolactone . : .low-renin essential hypertension Resistance hypertension -.primary aldosteronism ,CHF spironolactone - ACE ,inhibitors - .loop diuretics - spironolactone , : gynecomastia . ,eplerenone . - Eplerenone " . . :Beta blockers - beta blockers -) CO (. beta blockers : . - beta blockers , - hypotensive potency . , - beta blockers -1 2 . , - potency- beta blockers . - beta blockers , . beta blockers : . . -.recurrent MI ,CHF- beta blockers . Carvedilol- Labetalol - - . , . . :Alpha blockers

972

001

, 71 @ , ', "

- - " . - , . , - blockers CHF - . .prostatic hypertrophy - - . . :Sympatholytic Agents 2 " - .sympathetic outflow autonomic neuropathy . : .Somnolence -) rebound hyprtension .(withdrawl " - .nerve terminals -, : .Orthostatic hypotension . . :Calcium Channel Blockers CCB -,L type channels . : ) Phenylalkylamines .(verapamil ) Benzothiazepines .(diltiazem -) 1,4 dihydropyridines .(nifedipine ) beta blockers ,ACE inhibitors- ( 1-blockers, - CCB . - dihydropyridines: .Flushing ] - ,transcapillary [. . :Direct Vasodilators , , --renin angiotensin . , - .beta blockers-Hydralazine - ,NO minoxidil

082

101

, 71 @ , ', "

. -hydralazine - ,lupus-like syndrome - minoxidil hypertrichosis-.pericardial effusion 3. -: , , : Beta blockers ACE inhibitors ARBs CCB -2 blockers , - - .8-10/4-7 mmHg, : - beta blockers- ,ACE inhibitors 05 -.CCB : - ACE inhibitors-ARBs -.CCB - ACE inhibitors ) ARBs ( , . - beta blockers - . - . -ACE inhibitors , , . - ACE inhibitors- ARBs - proteinuria . , - ACE inhibitors CHF .MI ,CHF .ARBs- ACE inhibitors ,CCB - CCB ) ACE inhibitors .(beta blockers systolic and/or diastolic ,dysfunction , - ,ACE inhibitors- ARBs - beta blockers . , . 51 - . : -31 ,

182

201

, 71 @ , ', "

-2 , , . : patient dropout crossover . , , - . 4. -: , - cardiovascular endpoints - 135-140 mmHg - 80-85 mmHg . , . ) -08/031( : . .CHD . , ) (. - risk reduction . , . . , -%02 . , ) (additive . , , - J curve ) phenomenon , (. ,isolated systolic hypertension . , / - 08, , . resistant hypertension - 140/90 mmHg -, ) (, . 06 . - resistant HT : ) pseudoresistance , (. . ) (. ) .(Table 241-3] (OTC , , pseudohypertension .

282

301

, 71 @ , ', "

" - .(Osler maneuver) cuff .intra-arterial resistance HT . -.resistance HT 5. :Hypertensive Emergencies -, " crisis levels . ,severe HT , , . - severe HT Hypertensive crises- Hypertensive Urgencies , , . 9-142 -01-142 . Malignant HT ) (. . , : diffuse necrotizing vasculitis . , , . , ": ) , , (, proteinuria microangiopathic hemolytic anemia . , - MAO inhibitors ) (. , . , shift , . . , - mean arterial BP -%52 .160/100-110 mmHg " ) IV nitroprusside short acting (. labetalol- nicardipine . malignant hypertension , . PO short acting clonidine ,captopril .labetalol , , . , . ,acute CVA 401 , 71 @ , ', "

382

. )" ,(CT . , , - guidlines - 220 mmHg .130 mmHg , - 185 mmHg - .100 mmHg , - guidelines - 180 mmHg .130 mmHg subarachnoid hemorrhage . - mean arterial pressure .130 mmHg , adrenergic crisis : overdose clonidine withdrawl tyramine .MAO inhibitors phentolamine .nitroprusside

482

501

, 71 @ , ', "

:Chapter 242 Diseases of the aorta : . , : -3 " - origin 2.5 " -2-8.1 " . : , , - internal .elastic lamina tunica -extracellular matrix adventitia - vasa vasorum -.nervi vascularis , , , - SV , . - shear stress, )1-242 .(Table , , , ) stress wall (, . :Aortic aneurysm . - ,pseudoaneurysm -) adventitia -.(perivascular clot : - fusiform aneurysm , . , - saccular aneurysm - outpouching . ) (. - descending thoracic aorta infradiaphragmatic aneurysms thoracoabdominal .aortic aneurysms : - elastin- .collagen : )1-242 .(Table - , ,proteolysis -

582

601

, 71 @ , ', "

.descending thoracic aorta ) B-,(T , , - metalloproteinases . , , . : . . , . Cystic medial necrosis - tunica media, . - cystic medial necrosis , , - fusiform aneurysm - ascending aorta -.sinuses of Valsalva : marfan syndrome Ehlers-Danlos syndrome type IV congenital bicuspid aortic valve -.familial thoracic aortic aneurysm syndrome . -%02 , . -1- fibrillin-type III procollagen marfan syndrome-,Ehlers-Danlos type IV syndrome . Linkage analysis loci 5, 11 -3 , . ,TB ,syphilis . - Syphillis . - syphilitic periaortitis , . -%09 . - tuberculosis aneurysm - thoracic aorta - ,hilar lymph nodes , . . - mycotic aneurysm , , , , . .saccular aneurysm . Vasculitides : Takayasus arteritis -giant cell arteritis

682

701

, 71 @ , ', "

-.descending thoracic aorta - ,spondyloarthropathy - ,ankylosing spondylitis-,Rheumatoid arthritis - ,psoriatic arthritis- relapsing polychondritis- ,Reiters syndrome - Behcets syndrome .ascending aorta . - traumatic aneurysms - descending thoracic aorta - insertion -.ligamentum arteriosum chronic aortic dissection . :Thoracic Aortic Aneurysms . Cystic medial necrosis - ,ascending aortic aneurysms - .descending thoracic aorta .0.1-0.2 cm/year marfan syndrome . , %3-2 -4 ", - 6 ". %7 , , , , , .dysphagia - ascending aorta - CHF - ,AR - SVC , . - CXR thoracic aortic aneurysm )1-242 .(Fig / - .left main bronchus ,2D echo ,TEE - ascending aorta - descending CT .thoracic aorta , ,MRI- invasive aortography - thoracic aorta )2-242 .(Fig , +) CT ( ,MRI 21-6 . Thoracic Aortic Aneurysms: , Marfan syndrome , -beta .blockers , , . : 6-5.5 " -1 " . marfan syndrome ,bicuspid aortic valve 5 ". :Abdominal Aortic Aneurysms , . 4 " -%2-1 05. 801 , 71 @ , ', "

782

%09 5 " , . . , -5 -5 " %2-1, %04-02 5 ". . . " , , X-ray US . , . . , . . , , . , . , . . , -%52 . US . - US , : . , - screening US 47-56 - risk reduction -%24. -) CT ( -MRI , , ) - ( ]3-242 .[Fig aortography , , , , - .atheroembolism , - aortography . Abdominal Aortic Aneurysms: graft ) ( . , , - 5.5 .cm - ,5.5 cm - US . , ) -5 "( . )(percutaneous (Fig 242-3) endovascular stent graft , . , . , ,CHF , . blockers 901 , 71 @ , ', "

882

. , -%2-1. , -%05-54. - , , , -%04. :Acute Aortic Syndromes - acute aortic syndromes : ) (. Intramural hematoma ).(penetrating atherosclerotic ulcer ) ] [( ) - .(intimal flap - ascending aorta - Shear stress . - descending thoracic aorta - ligamentum .arteriosum : -media -) intima .(intimal flap ) .(false lumen , . "" . , . - aortic dissection: - intramural hematoma .intimal flap -.penetrating atherosclerotic ulcer - vasa vasorum . - .descending thoracic aorta- acute intramural hematomas . , , . - ,descendng thoracic aorta . - ,internal elastic lamina - medial hematoma . . DeBakey : Type I - ascending aorta -.descending aorta Type II -.ascending aorta Type III - descending aorta )4-242 .(Fig ) (Stanford classification : Type A -) ascending aorta ( Type B -) descending aorta (. , -type A (Stanford classification) type B , - type I- type II - Debakey classification .

982

011

, 71 @ , ', "

: ) -%07 ( -.cystic medial necrosis Marfan syndrome .Ehlers-Danlos syndrome : ) inflammatory aortitis - Takayasus arteritis- giant cell ,(arteritis -) congenital aortic valve anomalies (bicuspid valve . , . : . 1:2. ) ( , , , . , "" . ) - ,(interscapular . , . : AR ), ( -) spinal cord(. , , - MI . . , ) - ,superior cervical ganglia- ,SVC ( , : Horners syndrome SVC syndrome . Hemopericardium type A . - Acute AR ) %05( ) .(type A - annulus . - AR: bounding pulses -right sternal border -.CHF 111 , 71 @ , ', "

092

. - ,ascending aorta- CXR . ) ( . - . - desecnding thoracic ,aorta - .CXR, - descending aorta -.ascending - ECG - MI - .MI -) coronary ostium ( -.acute MI , CT .MRI- aortography . - TTE %58-06 . - ascending ,aorta %08, - descending thoracic aorta . - TEE - ascending -) descending thoracic aorta (, %89, %09. AR . - CT- MRI -intimal flap ' ) %09(. . - MRI , . - ,TEE- CT- MRI , - CT- MRI . Aortic Dissection: . - ICU . , ) - .(Shear stress , blockers ) IV propranolol IV metoprolol ] IV esmolol [( 06 . sodium nitroprusside - .120 mmHg- ,Labetalol -blocker- blocker . - CCB verapamil- diltiazem IV - blockers . ACE inhibitor, ,enalaprilat- blocker . - ,direct vasodilators diazoxide ,hydralazine )( - hydraulic shear . : acute ascending aortic dissections-intramural hematomas )(type A type B, : o o ' o 211 , 71 @ , ', "

192

o . : -intimal flap .interpositional graft graft . %52-51. : MI . - endoluminal stent grafts type B . , - intimal flaps , . , ) type ,(B . type B dissection %02-01. intramural hematomas - + beta blockers - ) ACE inhibitors .(CCB type B , 21-6 CT MRI . Marfan syndrome . ) -01 -%06(. :Aortic occlusion 1. :Chronic atherosclerotic occlusive disease , . . - .iliac arteries , ).(Leriche syndrome . , . : )( ) - umbilicus ( - common .femoral arteries , , . , . , , ,Doppler velocity analysis , -.Duplex US CT ,MRI ,aortography -.

292

311

, 71 @ , ', "

. 2. : . . , - in situ thrombosis . , , . CT ,MRI .aortography thrombectomy - . :Aortitis - aortitis , : vasculitides - Takayasus arteritis giant cell arteritis rheumatic & HLA-B27-associated spondyloarthropathies Behcets syndrome ANCA-associated vasculitides Cogans syndrome tuberculosis ,syphilis -.retroperitoneal fibrosis - aortitis : -AR -.acute aortic syndromes :Takayasus arteritis - ascending aorta , . - Takayasus arteritis pulseless disease . - descending aorta- abdominal aorta . . ,panarteritis : -adventitia . . , , , , . - ESR- CRP . ) (, , , , . , . . surgical bypass .

392

411

, 71 @ , ', "

:Giant cell arteritis . . . .polymyalgia rheumatica : ) -. temporal aa -.(opthalmic aa aortitis .AR . :Rheumatic Aortitis ,psoriatic arthritis ,ankylosing spondylitis ,Rheumatoid arthritis Reiters syndrome- ,relapsing polychondritis- IBD aortitis - .ascending aorta - ,sinuses of Valsalva , . , ,AR . :Syphilitic Infective aortitis - Infective aortitis , , . - aortitis . , ,saccular aneurysm .mycotic aneurysm- mycotic aneurysms . , , . - mycotic aneurysms 3 . , , ), (, . . CT MRI .mycotic aneurysms : - graft . - syphilitc aortitis , , , . - syphilitic aortitis . saccular fusiform , . ) endarteritis ( -) vasa vasorum - .(adventitia - spirochetes - .adventitia - spirochetes ) - .(vasa vasorum , , . . , -03-51 . : 511 , 71 @ , ', "

492

AR - coronary ostia -syphilitic aortitis ) ( . ) - rapid plasmin regain ) (RPR- .(fluorescent treponemal antibody , .

592

611

, 71 @ , ', "

342 :Vascular Diseases of the Extremities : 1. :Peripheral arterial disease - (PAD) peripheral arterial disease . -PAD 04. : fibromuscular dysplasia entrapment syndromes cystic adventitial disease . - PAD . , PAD : DM -. . . : . : ) (thinning -internal elastic lamina . : - 30%) iliac arteries ( - 85%) femoral & popliteal arteries ( , -. 45%) tibial & peroneal aa (. , , - ,shear stress . .DM . : -%05 PAD . - ,intermittent claudication , , ) (numbness , . . , , ,aorto-iliac disease .femoral-popliteal disease . 711 , 71 @ , ', "

692

, . , numbness . , )- .(dependent position , . - PAD: . . , . , , , . . . Ischemic neuropathy - numbness-. . : - .PAD . - sphygmomanometer cuffs Doppler - dorsalis pedis artery-. posterior tibial artery , ) - .pulse-wave amplification , . , )] ,(ankle/brachial index [ABI -0.1 -0.1 .PAD -5.0 . : . pulse volume recordings Doppler flow velocity waveform analysis ) duplex US B-mode-(pulse-wave Doopler transcutaneous oximetry . - pneumatic cuff . . , ) - pulse volume .(recording- Doppler velocity contour

792

811

, 71 @ , ', "

) (blunted PAD. - Duplex US . ) ( . - ABI PAD . ,CT angiography ,MR angiography- contrast angiography - . - ) (. . : PAD . -%05-%03 PAD - ,ECG -%05 . ,PAD -5 %03-51, 6-2 . PAD. , - PAD . -%08-57 . -%02 , %2-1 . -%52 . .DM . Peripheral Arterial Disease: PAD ) MI(, , , . . . , , varenciline . . ACE inhibitors PAD. blockers , . - ,MI . - NCEPATP - PAD -LDL cholesterol - .100 mg/dL , , . ,Clopidogrel -) ADP receptor (, .PAD, ) (aspirin + clopidogrel .PAD warfarin PAD. - intermittent claudication : .

892

911

, 71 @ , ', "

. . . , -shock block ) (canopy . . 54-03 , 5-3 , 21 , . , . - PAD . , - vasodilators . , . , , . , ,CCB , blockers papaverine .PAD ,Cilostazol phosphodiesterase inhibitor , -%06-04 - claudiacation . . ,Pentoxifylline ,xanthine , . -pentoxifylline , . - ,proprionyl-L-carnitine , - intermittent claudication . vasodilator prostaglandins . . , DNA -basic fibroblastic ,endothelial growth factor hepatocyte growth factor ,growth factor ,hypoxia-inducible factor 1 ,endothelial progenitor cells . . -.PAD -: -, , , intermittent claudication , . CT ,MRA- angiography -. : percutaneous transluminal angiography (PTA) -.atherectomy PTA - iliac artery PTA -. femoral a -. .popliteal a-%59-09 - iliac PTAs , 3 - patency rate -%57. - patency rates - .iliac artery , 021 , 71 @ , ', "

992

- femoral-popliteal PTA %08 3 - patency rate %06. - patency rate . , . drug eluting stent- PDA , . aorto-iliac disease - .femoral-popliteal artery disease . - aorto-iliac disease: Aortobifemoral bypass Axillofemoral bypass Femoral-femoral bypass -.aorto-iliac endarterectomy - aortobifemoral bypass - Knitted Dacron .grafts- patency - graft %99, 5 -01 - graft patency %09 -%08, . : MI, - ,graft , - sexual dysfunction . %3-1, . - femoral-popliteal artery disease: autogenous saphenous vein bypass grafts .Placement of polytetrafluoroethylene (PTFE) or other synthetic grafts .Thromboendarterectomy %3-1. - patency rate : -graft - patency )-.(runoff vessels - patency- femoral-popliteal saphenous vein bypass grafts -%09 , -%57 5 . - patency 5 - infrapopliteal saphenous vein bypass grafts %07-06. , - patency 5 - infrapopliteal PTFE -%03. . : angina MI ventricular ectopy . , : ) dipyridamole adenosine ( dobutamine echocardiography . - . blocker . - , 121 , 71 @ , ', "

003

unstable angina-) refractory angina (, - left main artery . 2. :Fibromuscular dysplasia . - iliac artery- .subclavian artery : intimal fibroplasias medial dysplasia -.adventitial hyperplasia - medial dysplasia : medial fibroplasias perimedial fibroplasias -.medial hyperplasia - medial fibroplasias . - Internal elastic lamina . - iliac arteries - fibromuscular .dysplasia string of beads . , , . - PTA- surgical reconstruction . 3. :Thromboangiitis Obliterans - (Buergers disease) Thromboangiitis Obliterans ) (. , . 04. . - thromboangiitis obliterans , . , PMNs . - internal elastic lamina , , . , , - .PMNs ,perivascular fibrosis -.recanalization - thromboangiitis obliterans : Raynauds phenomenon -.migratory superficial vein thrombophlebtis . , , , ) (. -. brachial a- popliteal ,artery )..(radial/ulnar/tibial aa . , , , . . . 221 , 71 @ , ', "

103

. , . arterial bypass , , . , . " , . 4. :Vasculitis . - Takayasus arteritis- giant cell arteritis 913. 5. :Acute Arterial Occlusion - Acut