Heart Failure and Intrinsic Myocardial Disease

1. Heart Failure and Intrinsic Myocardial Disease

2. Heart Failure Inability of the heart to pump blood at a rate that is adequate for the body’s needs. High mortality - >50% in less than 5 years Contributing cause of death in 300,000 annually, 2,000,000 being treated Leading discharge diagnosis in hospitalized patients over 65 years of age

3. Starling’s Law of the Heart • The stroke volume of the heart is a function of the diastolic fiber length and the heart will pump whatever volume of blood is brought to it. • Or the increased preload dilation helps to sustain cardiac performance by enhancing contractility

4. Principles of cardiac Dysfunction • Failure of the pump itself • An obstruction to flow - overworks the chamber behind the obstruction • Regurgitant flow - increased volume workload • Disorders of cardiac conduction - non uniform and inefficient contractions • Disruption of the continuity of the circulatory system

5. Congestive Heart Failure • Cardiogenic - myocardial weakness • High output - excessive workload • Thyroid toxicosis • Reduced return - inadequate filling (cardiac tamponade)

6. Heart Failure • Systolic dysfunction- most, deterioration of myocardial contractile function • Diastolic dysfunction- inability of the chamber to relax, expand or fill during diastole

7. Left Sided Heart Failure • Ischemic heart disease • Hypertension • Aortic and mitral valve disease • Non ischemic myocardial disease (cardiomyopathies)

8. Pulmonary edema- left heart failure

9. Pulmonary edema: left heart failure

10. Right - Sided Heart Failure • Left heart failure • Cor pulmonale - pure right heart failure due to pulmonary hypertension

11. Pitting edema- rt. Heart failure

12. Effects of Heart Failure • Dilation of the heart (LHF & RHF) • Pulmonary edema and congestion(LHF) • Pleural effusions (LHF & RHF) • Renal failure (pre-renal azotemia) LHF & RHF • Congestion of liver - “nutmeg” liver (RHF) • Splenic congestion(RHF) • Ascites (RHF) • Dependent edema (RHF)

13. Causes of CHF • Congenital HD • Ischemic HD • Valvular HD • Cardiomyopathy • Inflammatory disease of the heart • Nutritional, endocrine, metabolic

14. Cardiac Hypertrophy • Onset of heart failure is preceded by cardiac hypertrophy • Increase rate of protein synthesis, # of sarcomeres, # of mitochondria • Pressure (concentric) hypertrophy • At some point the heart can no longer accommodate the increased demand

15. Cardiac Hypertrophy • Heart failure preceded by hypertrophy • Pressure overloaded ventricles (HTN or AS) develop concentric hypertrophy- increased thickness and normal to reduced cavity diameter • Volume overloaded ventricles (AR or MR), develop hypertrophy and dilatation • IHD & pulmonary hypertension up to 600gm • HTN, AS, MR or DCM up to 800 gm • AR or HCM up to 1000 gm

16. Example of a hypertrophic heart from AS

17. Hypertrophy- normal dilated hypertrophic

18. Hypertrophy normal hypertrophy

19. Examples of box car nuclei

20. Myocardial Disease • Clinical features • Arrhythmias -EKG • Pain • Rapid cardiac enlargement • Rapid onset of CHF • Sudden Death • By definition no significant coronary artery disease, valvular HD, or hypertensive HD

21. Myocarditis - Infectious • Viral: coxsackie A, B, ECHO, influenza , polio, HIV • interstitial lymphocytes • isolated cell degeneration • Rickettsial- more of a vasculitis • Fungal and protozoan -toxoplasmosis and Chagas disease • Bacterial • Borrelia burgdorferi (Lyme disease)Corynebacterium diphtheriae- toxin

22. Lymphocytic myocarditis

23. Chaga’s disease toxoplasmosis

24. “Myocarditis”- Non-Infectious • Hypersensitivity diseases • Radiation • Sarcoidosis, uremia

25. Cardiomyopathy • Heart disease not due to: • ischemia, hypertension, congenital abnormalities, or valvular heart disease • Idiopathic(meaning not one of the above) • Dilated cardiomyopathy (DCM) • Hypertrophic cardiomyopathy (HCM) • Restrictive cardiomyopathy

26. Primary Cardiomyopathy • Dilated: cardiac dilation CHF, arrhythmias • Hypertrophic: myocardial hypertrophy with or without outflow obstruction • Restrictive: amyloidosis, endocardial fibroelastosis , endomyocardial fibrosis

28. Dilated Cardiomyopathy • Progressive hypertrophy, dilation and contractile (systolic)dysfunction • Residual from a myocarditis- some cases have shown viral nucleic acids in the myocytes • Alcohol or other toxicity • Pregnancy-associated • Genetic - familial in some (20%) • Most truly idiopathic

29. DCM • Heavy >600gms (2 to 3 times normal) • Large , flabby with dilated chambers • Sometimes mural thrombi • Coronaries clear • Hypertrophied fibers, but stretched , large nuclei, and interstitial fibrosis

30. Clinical Features of DCM • Any age but 20 to 50 most common • Progressive congestive heart failure • End stage have ejection fractions < 25% • 50% die within 2 years, • 25% 5 year survival rate • Die of heart failure, arrhythmia, or embolism

31. Right endocardial biopsy technique

32. Dilated cardiomyopathy

33. Dilated cardiomyopathy

34. Dilated cardiomyopathy

35. Dilated CM

36. Dilated cardiomyopathy– note coronary arteries show no ASVD

37. Dilated cardiomyopathy

38. Hypertrophic Cardiomyopathy • Myocardial hypertrophy • Abnormal diastolic filling • Intermittent left ventricular outflow obstruction • Hypercontracting heart • Large heart, asymmetric septal hypertrophy • Myofiber disarray

39. HCM • Over half are familial, auto. dominant • Any one of 4 genes that encode proteins of the sarcomeres • B-myosin heavy chain (most) • Cardiac troponin T • Alpha-tropomyosin • Myosin-binding protein C

41. HCM Clinical Features • Reduced chamber size and reduced stroke volume leads to massively hypertrophied left ventricle • DOE • Harsh systolic ejection murmur • Focal ischemia, anginal pain • Atrial fib, emboli, endocarditis & sudden death

42. Hypertrophic cardiomyopathy

43. Hypertrophic CM Note septal bulging

44. Hypertrophic cardiomyopathy

45. Secondary Cardiomyopathy • Alcoholic • Metabolic • thyroid, K+ • glycogen storage • nutritional • hemochromatosis

46. Iron stain- hemachromatosis

47. Restrictive CM • Decrease in ventricular compliance resulting in impaired ventricular filling during diastole - contractile function OK • Confused with constrictive pericarditis • Idiopathic or radiation fibrosis, amyloid, sarcoidosis, metastatic tumor • Heart normal size and usually not dilated

48. Restrictive cardiomyopathy –amyloid

49. 2o Cardiomyopathy • Connective tissue diseases: • SLE, PSS, PAN • Neuromuscular disease: MD, etc. • Mucopolysaccharidoses: Hunter’s • Toxic: emetine, arsenic, cobalt • Infiltrative: leukemia • Drugs- Anthracycline (doxorubicin and daunorubicin) dose dependent >500 mg/m2 lipid peroxidation of myocyte membranes

50. Leutic (Syphilis) Heart Disease • Obliterative endarteritis - plasma cells • Aneurysm of thoracic aorta • Tree barking • Narrowing of ostia of coronary arteries • Severe ASVD at root of aorta • Dilation of aorta valve ring - (aortic insufficency) • Marked LVH - cor bovinum