Cardiomyopathy encompasses a group of disorders that affect the heart muscle, specifically excluding valvular, hypertensive, and congenital issues. Dilated cardiomyopathy is the most prevalent form, often leading to heart failure, with a poor prognosis and significant mortality rates. Treatment varies based on the type and underlying causes, with beta-blockers and ACE inhibitors commonly used, along with lifestyle modifications and monitoring for complications.

2. definitionCardiomyopathy is a group of disorders that specifically affect the cardiac muscle.It is distinctive because it does not involve valvular,hypertensive ,congenital disorders.Clinically and hemodynamically distinctive.

3. DILATED CARDIOMYOPATHYDilated cardiomyopathy

4. Most common cause of clinical syndrome of heart failure

5. BACK GROUNDThe most common cause of the clinical syndrome of chronic heart failure.Clinical outcome has not changed substantially despite the improvements in the treatment of heart failure.Median survival for men is 1.7 yrs ,women 3.2 yrs.---mortality is high.It might be secondary to ischemia,valvular , hypertensive or primary – genetic ,nongenetic ,acquired .It is defined as a ventricular chamber exhibiting increased diastolic and systolic volumes and a low < 45% ejection fraction.

6. Anatomical specimen

7. Natural history of clinical syndrome of heart failure depends on the course of myocardial failure.Most powerful single predictor of outcome is the degree of LV dysfunction as assessed by the LV ejection fraction.Improvement in natural history of heart failure is by improving intrinsic ventricular function.Adverse effect on outcome with impaired intrinsic function.

8. Classification 1995-WHO/ISFC Based on global anatomic description of chamber dimension in systole and diastole.Mixed- DCM,RCMGenetic –HOCM ,ARVD,ARVCAcquired –peripartum,tachycardia induced cardiomyopathies.Secondary –beckerduchennecardiomyopathy.Valvular ,hypertensive,ischaemic are excluded.Ischaemic dilated cardiomyopathy related to previous myocardial infarction and the subsequent remodelling process.

9. Molecular mechanisms3 GENERAL CATEGORIES:A.a single gene defectLamin a/c genemyosin heavy chainB.Polymorhic variation in modifier genes –reninangiotensin,adrenergic,endothelin.C.Maladaptive regulated expression of completely normal genes .

10. Gene mutationCytoskeletalSarcolemmalNuclear envelope geneSarcomere genesSignal pathway genesIon channelsDesmosomal genes

11. Polymorphic variationACE adrenergic receptorsEndothelin type a receptors

12. Altered expressionDecreased expression of the  adrenergic receptorsMYHCSERCA2Increased expression of ANP, MYHC,ACE,ENDOTHELIN,BARK

13. IMPRESSIONSingle gene defect ----pathway---hypertrophy-----late decompensation----ventricular dilation.Mutiple gene mutations associatedLIM protein—knock out mice—decreased systolic,diastolic,b adrenergic pathwayOver expression of 3,2,1,2---myocyte hypertrophy, increased fibrosis, apoptosis, L.V dilation.

14. ACE-DD,homozygous for the deletion variant--circulating cardiac ACE activityDD genotype—early remodelling after MI,development of end stage ischaemic dilated cardiomyopathy.

15. DCMAD—56%AR—16%X LINKED -10%AD with skeletal—7.7%AD with conduction defects—2.6%AR with retinitis pigmentosaAR with wooly hairXMitochondrial DCM

16. DIFFERENT RESPONSE TO MEDICATIONACE DD– worse prognosisOnly to beta blockers they are respondent1 receptor gene—isoproterenol,bucindolol

17. PathophysiologyProgressive myocardial failure is most likely caused by myocardial cell loss than by increased load ischemia.

18. POSSIBLE MECHAnisms for stabilizationIncreased heart rate and contractility-increased  adrenergic signaling—within seconds.Volume expansion—frank starling—stroke volume---hoursIncreased contractility –cardiuacmyocyte hypertrophy—daysChronic continued activation of RAS,ANS –progressive myocardial dysfunction RAS ,ACE inhibitorsblockers—reverse remodelling,progressive systolic dysfunction

19. processVirtualy all dilated cardiomyiopathies----progressive dilation—myocardial dysfunction in viable segments.—change in chamber shape—less elipitical ,more round.Dilated phenotype—commonest form IschaemicFollowing MIHypertensive Valvular

20. Clinical featuresPulsusalternansPulsustardusSystolic blood pressure normal or less than normal.Raised JVPProminent a and v waves tricuspid regurgitationb/l basal creptsPedal edemaHepatomegalyGallop rhythmSystolic murmurs—Mitral,tricuspid reg.

21. ecgSinus tachycardia in presence of heart failure.Atrial and ventricular tachyarryhthmiasPoor r wave progressionAnterior q waves Intaventricular conduction defects –mostly LBBBLeft atrial abnormalityHypertensive changes by voltage criteria not evidentST –T changes are seen.

22. 2d echoDilated chambersLeft atrium is usualy enlarged Left ventricle is enlarged.normal 3.8—5.0cmMitral and tricuspid regurgitation on doppler flow.Stress testing --tachyarryhthmiasDobutamine stress echo helpful in assessing the clinical prognosis.

23. normal

26. NORMAL

27. 2d echo finding m mode

28. Parasternal longitudinal view

29. NORMAL

30. Short axis view m mode

32. Chest x ray pa view

33. Massive cardiomegalywater bottle shaped heart

34. Ischaemiccardiomyopathy

35. Ischaemiccardiomyopathyh/o of MIClinically significant >70% narrowing of a major epicardialartery,CAD.Degree of myocardial dysfuntion and VD, is not explained solely by previous infarction.An ischaemicDCMis present when a post MI patient left ventrricle experiences remodelling and a drop in ejection fraction.Dilation of LV—15-40%.< 12 months of acute MI.Less number in inferior MI .

36. CONT..Transmural,subendocardialscarrinng—represents old MI—50% of LV chambers.Worse prognosis—because of ischaemic eventsTreatment—ACE, B blockers in symptomatic,Diuretics- vol. overload,Spironolactone –advanced,Digoxin is drug choice,Biventricular pacing—plus ICDs—IVCDS,Anticoagulation,Amiodarone,Potassium levels high level normal –4.3-5.0,Digoxin <1.0 ng/ml .

37. Hypertensive cardiomyopathySystolic function is depressed out of proportion to the increase in wall stress.Not in a patient with hypertensive crisis—unless Ven.dilation and depressed ventricular function remained after correction of hypertension.Major risk factorIncreased systolic wall stress.A.No increased wall thicknessB.Concentric hypertrophyC.Systolic dysfunction

39. Hypertensive cardiomyopathy

40. CONT..Prognosis is better in absence of other co morbid conditions ,in whom after load is controlledTreatment—afterloadvigouroslyAmlodipineHydralazineNitratesblockers

41. ValvularcardiomyopathyAbnormal valveMR.,AR,AS ……….never with severe pure MS. Eccentric hypertrophy—inc. dias stress…MRAortic regurgitation—both sys and diasAS- conc hyper.After MVR,AVR – if preoperatively mild LVD.Variable prognosisTime of cardiac surgery .Not in MR with LVEF-- <25%.Treatment-surgical,cathetervalvuloplasty.Medical– severeLVD

43. AMLODIPINE is another option for after load reduction –AR—ca blocker -- improved survival

44. Idiopathic dcm

45. idiopathic

46. Idiopathic DCMIt is by exclusion.Relatively common.0.04%.Increases with age.M>F.Myocardial cell hypertrophy,interstitial cell fibrosisEtiology as Alcohol >80g/day-M,>40g/day-F-5 years.HTN160/100 ,uncontrolledAssess TSH levelsbiopsy

47. pathophysiology35-50% are familialCARVAJAL syndrome—DCM,woolyhair,keratoderma.Altered signal transduction—phospholamban geneTafazzin gene—acyltransferase,mitochondrialmem.protein.High mortality and morbidity—lamin A/C genePost mortem—dilation of the chambersWeight of heart is increasedNo inc in wall thicknessVisible scarsMural endocardial plaque

48. microscopyMyocyte hypertrophyVery large nucleiIncreased interstitial fibrosisMyocyte atrophyInc. cell length individuallyUltrastructure– t tubular dilationNo adjacent myocyte damage with lymphocyte infiltration—dd– myocarditis.HLA—B27,A2,DR4,DQ4HLA DRW6Adenovirus,herpes

49. prognosisBetter than ischaemic50% survival ->5 yrs without ACELAMIN A/C gene—worse prognosisTreatmentNO issue of revascularizationIncreased incidence of thromboembolic complicationsB blockers are more useful—viable myocardiumAll should receive B BLOCKERS unless contraindicated.

50. AnthracyclinecardiomyopathyDoxorubicin.daunorubicinDose relatedDilated>450mg/m2 with no risk factors.Prior mediastinal radiation is a risk factor.Definitive diagnosis is by biopsyCell vacuolization 16-25% of sampled cells in biopsyPresenting late is better prognosisCarvedilol –beneficiary effect

51. Post partum cardiomyopathy

52. Post partum cardiomyopathyLast trimester< 6 months of deliveryNot commonDilated categoryBetter prognosisRecover completely in 50% cases.Family planning counselling should never become pregnant even if full recover of myocardial function presentTreatment is aggressive.

54. Alcohol cardiomyopathyRelatively high cardiac output for differentiation.Not good candidates for cardiac transplantation.

55. ChagascardiomyopathyMC cause of NICM .T. cruzi.Triatoma or kissing bug.Blood transfusions.Initial myocarditis in childhood.Recovery—DCM after 30 years.Clinically diagnosed.BBB—HEMIBLOCKS.LVH.Doppler tissue imaging.Mononuclear infiltrates.Apical aneurysm.Ab cross react with myosin.Prognosis-50%.Death—arrhtyhmias.Verapamil,amiodarone,no specific treatment.

56. Trypanosoma,triatoma

57. chagas

58. conclusion1.Most common cause of clinical syndrome of chronic heart failure.2.Global anatomic description of chambers in diastole ans systole for classification.3.Lamin a/c gene –single gene defect – worse prognosis.4.DCM is mostly familial.5. 56% AD.6.ACE DD – worse prognosis,responds to b blocker therapy.7. Progressive myocardial failure is due to cell loss than by increased ischemia overload.

59. Cont..8.RAS ,ACE inhibitors are useful in therapy.9. All DCM are less ellipitical , more round.10.Dilated phenotype is commom in cardiomyopathy.11.Ischaemic after MI ,CAD <12-24 months.12.Ischemic more worse prognosis than non ischemic.13.Potassium leels to be maintained high normal in ischemic DCM.14.Anticoagulation imp. in Isc.DCM.15.After load to be decreased in htn DCM.

60. CONT..16.Amlodipine is good drug in AR.17.Idiopathic is by exclusion.18.tafazzin gene is the new gene in pathogenesis of DCM.19.Lymphocyte infiltration nothing to do with damage of myocardial cells as in myocarditis.20.no issue of revascularization in idiopathic DCM.21.higher incidence of thrombotic complications.22.carvedilol useful in drug induced DCM23.most common cause of non isc.DCM is chagas.

61. Take home messageAll should receive beta blockers in DCM unless contraindicated.Anti thrombotic drugs to be started in the treatment of cardiomyopathyAmlodipine in case of AR useful.Alcoholic patients are not good for cardiac transplantation.Medical treatment in case of severe LVD in case of valvular DCM.

62. NEAR FUTURE in management of heart failureAQUARETICSADENOSINE RECEPTOR ANTAGONISTSMYOSIN ACTIVATORSNOVEL NATRIURETIC PEPTIDESDEVICE THERAPY

63. Management of heart failure is ………

64. Goal of management of heart failure

65. THE FOUR BLESSED LOOKS LOOK BACK AND GET EXPERIENCE LOOK FORWARD AND SEE FUTURELOOK AROUND AND FIND TRUTHLOOK WITHIN AND FIND CONFIDENCE

66. referencesHURST CARDIOLOGYHARRISONS 17 thedInternet

67. siteswww.heartfailurematters.org

68. THANK YOUDr.PRAVEEN NAGULA