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Heart Failure and Intrinsic Myocardial Disease. Heart Failure I nability of the heart to pump blood at a rate that is adequate for the body’s needs. I.

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Presentation on theme: "Heart Failure and Intrinsic Myocardial Disease. Heart Failure I nability of the heart to pump blood at a rate that is adequate for the body’s needs. I."— Presentation transcript:

1 Heart Failure and Intrinsic Myocardial Disease

2 Heart Failure I nability of the heart to pump blood at a rate that is adequate for the body’s needs. I nability 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 High mortality - >50% in less than 5 years Contributing cause of death in 300,000 annually, 2,000,000 being treated Contributing cause of death in 300,000 annually, 2,000,000 being treated Leading discharge diagnosis in hospitalized patients over 65 years of age 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. 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 Or the increased preload dilation helps to sustain cardiac performance by enhancing contractility

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

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

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

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

8 Pulmonary edema- left heart failure failure

9 Pulmonary edema: left heart failure

10 Right - Sided Heart Failure Left heart failure Left heart failure Cor pulmonale - pure right heart failure due to pulmonary hypertension 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) Dilation of the heart (LHF & RHF) Pulmonary edema and congestion(LHF) Pulmonary edema and congestion(LHF) Pleural effusions (LHF & RHF) Pleural effusions (LHF & RHF) Renal failure (pre-renal azotemia) LHF & RHF Renal failure (pre-renal azotemia) LHF & RHF Congestion of liver - “nutmeg” liver (RHF) Congestion of liver - “nutmeg” liver (RHF) Splenic congestion(RHF) Splenic congestion(RHF) Ascites (RHF) Ascites (RHF) Dependent edema (RHF) Dependent edema (RHF)

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

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

15 Cardiac Hypertrophy Heart failure preceded by hypertrophy Heart failure preceded by hypertrophy Pressure overloaded ventricles (HTN or AS) develop concentric hypertrophy- increased thickness and normal to reduced cavity diameter 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 Volume overloaded ventricles (AR or MR), develop hypertrophy and dilatation IHD & pulmonary hypertension up to 600gm IHD & pulmonary hypertension up to 600gm HTN, AS, MR or DCM up to 800 gm HTN, AS, MR or DCM up to 800 gm AR or HCM up to 1000 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 Clinical features Arrhythmias -EKG Arrhythmias -EKG Pain Pain Rapid cardiac enlargement Rapid cardiac enlargement Rapid onset of CHF Rapid onset of CHF Sudden Death Sudden Death By definition no significant coronary artery disease, valvular HD, or hypertensive HD By definition no significant coronary artery disease, valvular HD, or hypertensive HD

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

22 Lymphocytic myocarditis

23 Chaga’s disease toxoplasmosis

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

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

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

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

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

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

31 Right endocardial biopsy technique

32 Dilated cardiomyopathy

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35 Dilated CM

36 Dilated cardiomyopathy– note coronary arteries show no ASVD

37 Dilated cardiomyopathy

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

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

40

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

42 Hypertrophic cardiomyopathy

43 Hypertrophic CM Note septal bulging

44 Hypertrophic cardiomyopathy

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

46 Iron stain- hemachromatosis

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

48 Restrictive cardiomyopathy –amyloid

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

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

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

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

53 Carcinoid valvular disease Note thick cusps- stenosis

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


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