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  1. 1.
  2. 2. definition<br />Cardiomyopathy is a group of disorders that specifically affect the cardiac muscle.<br />It is distinctive because it does not involve valvular,hypertensive ,congenital disorders.<br />Clinically and hemodynamically distinctive.<br />
  3. 3. DILATED CARDIOMYOPATHY<br />Dilated cardiomyopathy<br />
  4. 4. Most common cause of clinical syndrome of heart failure<br />
  5. 5. BACK GROUND<br />The most common cause of the clinical syndrome of chronic heart failure.<br />Clinical outcome has not changed substantially despite the improvements in the treatment of heart failure.<br />Median survival for men is 1.7 yrs ,women 3.2 yrs.---mortality is high.<br />It might be secondary to ischemia,<br />valvular ,<br /> hypertensive or primary – genetic ,nongenetic ,acquired .<br />It is defined as a ventricular chamber exhibiting increased diastolic and systolic volumes and a low < 45% ejection fraction. <br />
  6. 6. Anatomical specimen<br />
  7. 7. Natural history of clinical syndrome of heart failure depends on the course of myocardial failure.<br />Most powerful single predictor of outcome is the degree of LV dysfunction as assessed by the LV ejection fraction.<br />Improvement in natural history of heart failure is by improving intrinsic ventricular function.<br />Adverse effect on outcome with impaired intrinsic function. <br />
  8. 8. Classification <br />1995-WHO/ISFC <br />Based on global anatomic description of chamber dimension in systole and diastole.<br />Mixed- DCM,RCM<br />Genetic –HOCM ,ARVD,ARVC<br />Acquired –peripartum,tachycardia induced cardiomyopathies.<br />Secondary –beckerduchennecardiomyopathy.<br />Valvular ,hypertensive,ischaemic are excluded.<br />Ischaemic dilated cardiomyopathy related to previous myocardial infarction and the subsequent remodelling process.<br />
  9. 9. Molecular mechanisms<br />3 GENERAL CATEGORIES:<br />A.a single gene defect<br />Lamin a/c gene<br />myosin heavy chain<br />B.Polymorhic variation in modifier genes –reninangiotensin,adrenergic,endothelin.<br />C.Maladaptive regulated expression of completely normal genes .<br />
  10. 10. Gene mutation<br />Cytoskeletal<br />Sarcolemmal<br />Nuclear envelope gene<br />Sarcomere genes<br />Signal pathway genes<br />Ion channels<br />Desmosomal genes<br />
  11. 11. Polymorphic variation<br />ACE<br /> adrenergic receptors<br />Endothelin type a receptors<br />
  12. 12. Altered expression<br />Decreased expression of the  adrenergic receptors<br />MYHC<br />SERCA2<br />Increased expression of ANP, MYHC,ACE,ENDOTHELIN,BARK<br />
  13. 13. IMPRESSION<br />Single gene defect ----pathway---hypertrophy-----late decompensation----ventricular dilation.<br />Mutiple gene mutations associated<br />LIM protein—knock out mice—decreased systolic,diastolic,b adrenergic pathway<br />Over expression of 3,2,1,2---myocyte hypertrophy, increased fibrosis, apoptosis, L.V dilation.<br />
  14. 14. ACE-DD,homozygous for the deletion variant--circulating cardiac ACE activity<br />DD genotype—early remodelling after MI,development of end stage ischaemic dilated cardiomyopathy. <br />
  15. 15. DCM<br />AD—56%<br />AR—16%<br />X LINKED -10%<br />AD with skeletal—7.7%<br />AD with conduction defects—2.6%<br />AR with retinitis pigmentosa<br />AR with wooly hair<br />X<br />Mitochondrial DCM<br />
  16. 16. DIFFERENT RESPONSE TO MEDICATION<br />ACE DD– worse prognosis<br />Only to beta blockers they are respondent<br />1 receptor gene—isoproterenol,bucindolol<br />
  17. 17. Pathophysiology<br />Progressive myocardial failure is most likely caused by myocardial cell loss than by increased load ischemia.<br />
  18. 18. POSSIBLE MECHAnisms for stabilization<br />Increased heart rate and contractility<br />-increased  adrenergic signaling<br />—within seconds.<br />Volume expansion—<br />frank starling—stroke volume---hours<br />Increased contractility <br />–cardiuacmyocyte hypertrophy—days<br />Chronic continued activation of RAS,ANS –<br />progressive myocardial dysfunction <br />RAS ,ACE inhibitors<br />blockers—reverse remodelling,progressive systolic dysfunction<br />
  19. 19. process<br />Virtualy all dilated cardiomyiopathies----progressive dilation—myocardial dysfunction in viable segments.—change in chamber shape—less elipitical ,more round.<br />Dilated phenotype—commonest form <br />Ischaemic<br />Following MI<br />Hypertensive <br />Valvular<br />
  20. 20. Clinical features<br />Pulsusalternans<br />Pulsustardus<br />Systolic blood pressure normal or less than normal.<br />Raised JVP<br />Prominent a and v waves tricuspid regurgitation<br />b/l basal crepts<br />Pedal edema<br />Hepatomegaly<br />Gallop rhythm<br />Systolic murmurs—Mitral,tricuspid reg.<br />
  21. 21. ecg<br />Sinus tachycardia in presence of heart failure.<br />Atrial and ventricular tachyarryhthmias<br />Poor r wave progression<br />Anterior q waves <br />Intaventricular conduction defects –mostly LBBB<br />Left atrial abnormality<br />Hypertensive changes by voltage criteria not evident<br />ST –T changes are seen. <br />
  22. 22. 2d echo<br />Dilated chambers<br />Left atrium is usualy enlarged <br />Left ventricle is enlarged.normal 3.8—5.0cm<br />Mitral and tricuspid regurgitation on doppler flow.<br />Stress testing --tachyarryhthmias<br />Dobutamine stress echo helpful in assessing the clinical prognosis.<br />
  23. 23. normal<br />
  24. 24.
  25. 25.
  26. 26. NORMAL<br />
  27. 27. 2d echo finding m mode<br />
  28. 28. Parasternal longitudinal view<br />
  29. 29. NORMAL<br />
  30. 30. Short axis view m mode<br />
  31. 31.
  32. 32. Chest x ray pa view<br />
  33. 33. Massive cardiomegalywater bottle shaped heart<br />
  34. 34. Ischaemiccardiomyopathy<br />
  35. 35. Ischaemiccardiomyopathy<br />h/o of MI<br />Clinically significant >70% narrowing of a major epicardialartery,CAD.<br />Degree of myocardial dysfuntion and VD, is not explained solely by previous infarction.<br />An ischaemicDCMis present when a post MI patient left ventrricle experiences remodelling and a drop in ejection fraction.<br />Dilation of LV—15-40%.<br />< 12 months of acute MI.<br />Less number in inferior MI .<br />
  36. 36. CONT..<br />Transmural,subendocardialscarrinng—represents old MI—50% of LV chambers.<br />Worse prognosis—because of ischaemic events<br />Treatment—<br />ACE, <br />B blockers in symptomatic,<br />Diuretics- vol. overload,<br />Spironolactone –advanced,<br />Digoxin is drug choice,<br />Biventricular pacing—plus ICDs—IVCDS,<br />Anticoagulation,<br />Amiodarone,<br />Potassium levels high level normal –4.3-5.0,<br />Digoxin <1.0 ng/ml .<br />
  37. 37. Hypertensive cardiomyopathy<br />Systolic function is depressed out of proportion to the increase in wall stress.<br />Not in a patient with hypertensive crisis—unless Ven.dilation and depressed ventricular function remained after correction of hypertension.<br />Major risk factor<br />Increased systolic wall stress.<br />A.No increased wall thickness<br />B.Concentric hypertrophy<br />C.Systolic dysfunction<br />
  38. 38.
  39. 39. Hypertensive cardiomyopathy<br />
  40. 40. CONT..<br />Prognosis is better in absence of other co morbid conditions ,in whom after load is controlled<br />Treatment—<br />afterloadvigourosly<br />Amlodipine<br />Hydralazine<br />Nitrates<br />blockers<br />
  41. 41. Valvularcardiomyopathy<br />Abnormal valve<br />MR.,AR,AS ……….never with severe pure MS. <br />Eccentric hypertrophy—inc. dias stress…MR<br />Aortic regurgitation—both sys and dias<br />AS- conc hyper.<br />After MVR,AVR – if preoperatively mild LVD.<br />Variable prognosis<br />Time of cardiac surgery .<br />Not in MR with LVEF-- <25%.<br />Treatment-surgical,cathetervalvuloplasty.<br />Medical– severeLVD<br />
  42. 42.
  43. 43. AMLODIPINE is another option for after load reduction –AR—ca blocker -- improved survival <br />
  44. 44. Idiopathic dcm<br />
  45. 45. idiopathic<br />
  46. 46. Idiopathic DCM<br />It is by exclusion.<br />Relatively common.<br />0.04%.<br />Increases with age.<br />M>F.<br />Myocardial cell hypertrophy,interstitial cell fibrosis<br />Etiology as Alcohol >80g/day-M,>40g/day-F-5 years.<br />HTN160/100 ,uncontrolled<br />Assess TSH levels<br />biopsy<br />
  47. 47. pathophysiology<br />35-50% are familial<br />CARVAJAL syndrome—DCM,woolyhair,keratoderma.<br />Altered signal transduction—phospholamban gene<br />Tafazzin gene—acyltransferase,mitochondrialmem.protein.<br />High mortality and morbidity—lamin A/C gene<br />Post mortem—dilation of the chambers<br />Weight of heart is increased<br />No inc in wall thickness<br />Visible scars<br />Mural endocardial plaque <br />
  48. 48. microscopy<br />Myocyte hypertrophy<br />Very large nuclei<br />Increased interstitial fibrosis<br />Myocyte atrophy<br />Inc. cell length individually<br />Ultrastructure– t tubular dilation<br />No adjacent myocyte damage with lymphocyte infiltration—dd– myocarditis.<br />HLA—B27,A2,DR4,DQ4<br />HLA DRW6<br />Adenovirus,herpes<br />
  49. 49. prognosis<br />Better than ischaemic<br />50% survival ->5 yrs without ACE<br />LAMIN A/C gene—worse prognosis<br />Treatment<br />NO issue of revascularization<br />Increased incidence of thromboembolic complications<br />B blockers are more useful—viable myocardium<br />All should receive B BLOCKERS unless contraindicated.<br />
  50. 50. Anthracyclinecardiomyopathy<br />Doxorubicin.daunorubicin<br />Dose related<br />Dilated<br />>450mg/m2 with no risk factors.<br />Prior mediastinal radiation is a risk factor.<br />Definitive diagnosis is by biopsy<br />Cell vacuolization 16-25% of sampled cells in biopsy<br />Presenting late is better prognosis<br />Carvedilol –beneficiary effect<br />
  51. 51. Post partum cardiomyopathy<br />
  52. 52. Post partum cardiomyopathy<br />Last trimester<br />< 6 months of delivery<br />Not common<br />Dilated category<br />Better prognosis<br />Recover completely in 50% cases.<br />Family planning counselling should never become pregnant even if full recover of myocardial function present<br />Treatment is aggressive.<br />
  53. 53.
  54. 54. Alcohol cardiomyopathy<br />Relatively high cardiac output for differentiation.<br />Not good candidates for cardiac transplantation.<br />
  55. 55. Chagascardiomyopathy<br />MC cause of NICM .<br />T. cruzi.<br />Triatoma or kissing bug.<br />Blood transfusions.<br />Initial myocarditis in childhood.<br />Recovery—DCM after 30 years.<br />Clinically diagnosed.<br />BBB—HEMIBLOCKS.<br />LVH.<br />Doppler tissue imaging.<br />Mononuclear infiltrates.<br />Apical aneurysm.<br />Ab cross react with myosin.<br />Prognosis-50%.<br />Death—arrhtyhmias.<br />Verapamil,amiodarone,no specific treatment.<br />
  56. 56. Trypanosoma,triatoma<br />
  57. 57. chagas<br />
  58. 58. conclusion<br />1.Most common cause of clinical syndrome of chronic heart failure.<br />2.Global anatomic description of chambers in diastole ans systole for classification.<br />3.Lamin a/c gene –single gene defect – worse prognosis.<br />4.DCM is mostly familial.<br />5. 56% AD.<br />6.ACE DD – worse prognosis,responds to b blocker therapy.<br />7. Progressive myocardial failure is due to cell loss than by increased ischemia overload.<br />
  59. 59. Cont..<br />8.RAS ,ACE inhibitors are useful in therapy.<br />9. All DCM are less ellipitical , more round.<br />10.Dilated phenotype is commom in cardiomyopathy.<br />11.Ischaemic after MI ,CAD <12-24 months.<br />12.Ischemic more worse prognosis than non ischemic.<br />13.Potassium leels to be maintained high normal in ischemic DCM.<br />14.Anticoagulation imp. in Isc.DCM.<br />15.After load to be decreased in htn DCM.<br />
  60. 60. CONT..<br />16.Amlodipine is good drug in AR.<br />17.Idiopathic is by exclusion.<br />18.tafazzin gene is the new gene in pathogenesis of DCM.<br />19.Lymphocyte infiltration nothing to do with damage of myocardial cells as in myocarditis.<br /> issue of revascularization in idiopathic DCM.<br />21.higher incidence of thrombotic complications.<br />22.carvedilol useful in drug induced DCM<br />23.most common cause of non isc.DCM is chagas. <br />
  61. 61. Take home message<br />All should receive beta blockers in DCM unless contraindicated.<br />Anti thrombotic drugs to be started in the treatment of cardiomyopathy<br />Amlodipine in case of AR useful.<br />Alcoholic patients are not good for cardiac transplantation.<br />Medical treatment in case of severe LVD in case of valvular DCM.<br />
  63. 63. Management of heart failure is ………<br />
  64. 64. Goal of management of heart failure<br />
  66. 66. references<br />HURST CARDIOLOGY<br />HARRISONS 17 thed<br />Internet<br />
  67. 67. sites<br /><br />
  68. 68. THANK YOU<br />Dr.PRAVEEN NAGULA<br />