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

51. Syphilitic aortitis and
aortic insufficiency
Weaken aorta with
Aneurysm of ascending
aorta
Dilated aortic root

52. Carcinoid Heart Disease
Serotonin, 5-hydroxtryptophane, bradykinin, and histamine
Most on right side
Plaque-like thickenings
Fusion of tricuspid leaflets

53. Note thick cusps-
stenosis
Carcinoid valvular disease

54. Summary Mitral Stenosis : Rheumatic Heart Disease
Mitral insufficiency: myxomatous degeneration (mitral valve prolapse), damaged papillary muscle due to infarct
Aortic stenosis: calcification of normal and congenitally bicuspid aortic valves
Aortic insufficiency: dilation of the ascending aorta, related to hypertension and aging