Heart Failure and Intrinsic Heart Failure and Intrinsic Myocardial DiseaseMyocardial Disease

Heart FailureHeart Failure• IInability of the heart to pump blood at a nability of the heart to pump blood at a

rate that is adequate for the body’s rate that is adequate for the body’s needs.needs.

• High mortality - >50% in less than 5 High mortality - >50% in less than 5 yearsyears

• Contributing cause of death in 300,000 Contributing cause of death in 300,000 annually, 2,000,000 being treatedannually, 2,000,000 being treated

• Leading discharge diagnosis in Leading discharge diagnosis in hospitalized patients over 65 years of hospitalized patients over 65 years of ageage

Starling’s Law of the Starling’s Law of the HeartHeart

• The stroke volume of the heart is a The stroke volume of the heart is a function of the diastolic fiber length and function of the diastolic fiber length and the heart will pump whatever volume of the heart will pump whatever volume of blood is brought to it.blood is brought to it.

• Or the increased preload dilation helps Or the increased preload dilation helps to sustain cardiac performance by to sustain cardiac performance by enhancing contractilityenhancing contractility

Principles of cardiac Principles of cardiac DysfunctionDysfunction

• Failure of the pump itselfFailure of the pump itself

• An obstruction to flow - overworks the An obstruction to flow - overworks the chamber behind the obstructionchamber behind the obstruction

• Regurgitant flow - increased volume Regurgitant flow - increased volume workloadworkload

• Disorders of cardiac conduction - non Disorders of cardiac conduction - non uniform and inefficient contractionsuniform and inefficient contractions

• Disruption of the continuity of the Disruption of the continuity of the circulatory systemcirculatory system

Congestive Heart FailureCongestive Heart Failure

• Cardiogenic - myocardial weakness Cardiogenic - myocardial weakness

• High output - excessive workloadHigh output - excessive workload

• Thyroid toxicosisThyroid toxicosis

• Reduced return - inadequate filling Reduced return - inadequate filling (cardiac tamponade)(cardiac tamponade)

Heart FailureHeart Failure• Systolic dysfunction- most, Systolic dysfunction- most,

deterioration of myocardial deterioration of myocardial contractile functioncontractile function

• Diastolic dysfunction- inability of the Diastolic dysfunction- inability of the chamber to relax, expand or fill chamber to relax, expand or fill during diastoleduring diastole

Left Sided Heart FailureLeft Sided Heart Failure

• Ischemic heart diseaseIschemic heart disease

• HypertensionHypertension

• Aortic and mitral valve diseaseAortic and mitral valve disease

• Non ischemic myocardial disease Non ischemic myocardial disease (cardiomyopathies)(cardiomyopathies)

Pulmonary edema- left heartPulmonary edema- left heart failurefailure

Pulmonary edema: left heart failure

Right - Sided Heart Right - Sided Heart FailureFailure

• Left heart failureLeft heart failure

• Cor pulmonale - pure right heart Cor pulmonale - pure right heart failure due to pulmonary failure due to pulmonary hypertensionhypertension

Pitting edema- rt. Heart failurePitting edema- rt. Heart failure

Effects of Heart FailureEffects 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 & Renal failure (pre-renal azotemia) LHF & RHFRHF

• Congestion of liver - “nutmeg” liver Congestion of liver - “nutmeg” liver (RHF)(RHF)

• Splenic congestion(RHF)Splenic congestion(RHF)

• Ascites (RHF)Ascites (RHF)

• Dependent edema (RHF)Dependent edema (RHF)

Causes of CHFCauses of CHF

• Congenital HDCongenital HD

• Ischemic HDIschemic HD

• Valvular HDValvular HD

• CardiomyopathyCardiomyopathy

• Inflammatory disease of the heartInflammatory disease of the heart

•Nutritional, endocrine, metabolicNutritional, endocrine, metabolic

Cardiac HypertrophyCardiac Hypertrophy

• Onset of heart failure is preceded by Onset of heart failure is preceded by cardiac hypertrophycardiac hypertrophy

• Increase rate of protein synthesis, # of Increase rate of protein synthesis, # of sarcomeres, # of mitochondriasarcomeres, # of mitochondria

• Pressure (concentric) hypertrophyPressure (concentric) hypertrophy

• At some point the heart can no longer At some point the heart can no longer accommodate the increased demandaccommodate the increased demand

Cardiac HypertrophyCardiac Hypertrophy• Heart failure preceded by hypertrophyHeart failure preceded by hypertrophy

• Pressure overloaded ventricles (HTN or Pressure overloaded ventricles (HTN or AS) develop concentric hypertrophy- AS) develop concentric hypertrophy- increased thickness and normal to increased thickness and normal to reduced cavity diameterreduced cavity diameter

• Volume overloaded ventricles (AR or MR), Volume overloaded ventricles (AR or MR), develop hypertrophy and dilatationdevelop hypertrophy and dilatation

• IHD & pulmonary hypertension up to IHD & pulmonary hypertension up to 600gm600gm

• HTN, AS, MR or DCM up to 800 gmHTN, AS, MR or DCM up to 800 gm

• AR or HCM up to 1000 gmAR or HCM up to 1000 gm

Example of a hypertrophic heart from ASExample of a hypertrophic heart from AS

Hypertrophy- normal dilated hypertrophicHypertrophy- normal dilated hypertrophic

Hypertrophy normal hypertrophyHypertrophy normal hypertrophy

Examples of box car nucleiExamples of box car nuclei

Myocardial DiseaseMyocardial Disease

• Clinical featuresClinical features

• Arrhythmias -EKGArrhythmias -EKG

• PainPain

• Rapid cardiac enlargementRapid cardiac enlargement

• Rapid onset of CHFRapid onset of CHF

• Sudden DeathSudden Death

• By definition no significant coronary By definition no significant coronary artery disease, valvular HD, or artery disease, valvular HD, or hypertensive HDhypertensive HD

Myocarditis - InfectiousMyocarditis - Infectious• Viral: coxsackie A, B, ECHO, influenza , Viral: coxsackie A, B, ECHO, influenza ,

polio, HIVpolio, HIV

• interstitial lymphocytesinterstitial lymphocytes

• isolated cell degenerationisolated cell degeneration

• Rickettsial- more of a vasculitisRickettsial- more of a vasculitis

• Fungal and protozoan -toxoplasmosis Fungal and protozoan -toxoplasmosis and Chagas diseaseand Chagas disease

• BacterialBacterial

•Borrelia burgdorferiBorrelia burgdorferi (Lyme disease) (Lyme disease)Corynebacterium diphtheriae- Corynebacterium diphtheriae- toxintoxin

Lymphocytic myocarditis

Chaga’s diseaseChaga’s disease toxoplasmosistoxoplasmosis

““Myocarditis”- Non-Myocarditis”- Non-InfectiousInfectious

• Hypersensitivity diseasesHypersensitivity diseases

• RadiationRadiation

• Sarcoidosis, uremiaSarcoidosis, uremia

CardiomyopathyCardiomyopathy• Heart disease not due to:Heart disease not due to:

• ischemia, hypertension, congenital ischemia, hypertension, congenital abnormalities, or valvular heart diseaseabnormalities, 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 cardiomyopathyRestrictive cardiomyopathy

Primary CardiomyopathyPrimary Cardiomyopathy

• Dilated: cardiac dilation CHF, Dilated: cardiac dilation CHF, arrhythmiasarrhythmias

• Hypertrophic: myocardial hypertrophy Hypertrophic: myocardial hypertrophy with or without outflow obstructionwith or without outflow obstruction

• Restrictive: amyloidosis, endocardial Restrictive: amyloidosis, endocardial fibroelastosis , endomyocardial fibrosisfibroelastosis , endomyocardial fibrosis

Dilated CardiomyopathyDilated Cardiomyopathy• Progressive hypertrophy, dilation and Progressive hypertrophy, dilation and

contractile (systolic)dysfunctioncontractile (systolic)dysfunction

• Residual from a myocarditis- some cases Residual from a myocarditis- some cases have shown viral nucleic acids in the have shown viral nucleic acids in the myocytesmyocytes

• Alcohol or other toxicityAlcohol or other toxicity

• Pregnancy-associatedPregnancy-associated

• Genetic - familial in some (20%)Genetic - familial in some (20%)

• Most truly idiopathicMost truly idiopathic

DCMDCM• Heavy >600gms (2 to 3 times normal)Heavy >600gms (2 to 3 times normal)

• Large , flabby with dilated chambersLarge , flabby with dilated chambers

• Sometimes mural thrombiSometimes mural thrombi

• Coronaries clearCoronaries clear

• Hypertrophied fibers, but stretched , Hypertrophied fibers, but stretched , large nuclei, and interstitial fibrosislarge nuclei, and interstitial fibrosis

Clinical Features of DCMClinical Features of DCM• Any age but 20 to 50 most commonAny age but 20 to 50 most common

• Progressive congestive heart failureProgressive 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 rate25% 5 year survival rate

• Die of heart failure, arrhythmia, or Die of heart failure, arrhythmia, or embolismembolism

Right endocardial biopsy techniqueRight endocardial biopsy technique

Dilated cardiomyopathy

Dilated cardiomyopathy

Dilated cardiomyopathy

Dilated CMDilated CM

Dilated cardiomyopathy– note coronary arteries show no ASVD

Dilated cardiomyopathy

Hypertrophic Hypertrophic CardiomyopathyCardiomyopathy

• Myocardial hypertrophyMyocardial hypertrophy

• Abnormal diastolic fillingAbnormal diastolic filling

• Intermittent left ventricular outflow Intermittent left ventricular outflow obstructionobstruction

• Hypercontracting heartHypercontracting heart

• Large heart, asymmetric septal Large heart, asymmetric septal hypertrophyhypertrophy

• Myofiber disarrayMyofiber disarray

HCMHCM

• Over half are familial, auto. dominantOver half are familial, auto. dominant

• Any one of 4 genes that encode proteins Any one of 4 genes that encode proteins of the sarcomeresof the sarcomeres

•B-myosin heavy chain (most)B-myosin heavy chain (most)

•Cardiac troponin TCardiac troponin T

•Alpha-tropomyosinAlpha-tropomyosin

•Myosin-binding protein CMyosin-binding protein C

HCM Clinical FeaturesHCM Clinical Features

• Reduced chamber size and reduced Reduced chamber size and reduced stroke volume leads to massively stroke volume leads to massively hypertrophied left ventriclehypertrophied left ventricle

• DOEDOE

• Harsh systolic ejection murmurHarsh systolic ejection murmur

• Focal ischemia, anginal painFocal ischemia, anginal pain

• Atrial fib, emboli, endocarditis & Atrial fib, emboli, endocarditis & sudden deathsudden death

Hypertrophic cardiomyopathy

Hypertrophic CM Hypertrophic CM Note septal bulgingNote septal bulging

Hypertrophic cardiomyopathy

Secondary Secondary CardiomyopathyCardiomyopathy

• AlcoholicAlcoholic

• MetabolicMetabolic

• thyroid, K+thyroid, K+

•glycogen storageglycogen storage

•nutritionalnutritional

•hemochromatosishemochromatosis

Iron stain- hemachromatosisIron stain- hemachromatosis

Restrictive CMRestrictive CM• Decrease in ventricular compliance Decrease in ventricular compliance

resulting in impaired ventricular filling resulting in impaired ventricular filling during diastole - contractile function OKduring diastole - contractile function OK

• Confused with constrictive pericarditisConfused with constrictive pericarditis

• Idiopathic or radiation fibrosis, amyloid, Idiopathic or radiation fibrosis, amyloid, sarcoidosis, metastatic tumorsarcoidosis, metastatic tumor

• Heart normal size and usually not Heart normal size and usually not dilateddilated

Restrictive cardiomyopathy –amyloid

22oo Cardiomyopathy Cardiomyopathy• Connective tissue diseases:Connective tissue diseases:

•SLE, PSS, PANSLE, PSS, PAN

• Neuromuscular disease: MD, etc.Neuromuscular disease: MD, etc.

• Mucopolysaccharidoses: Hunter’sMucopolysaccharidoses: Hunter’s

• Toxic: emetine, arsenic, cobaltToxic: emetine, arsenic, cobalt

• Infiltrative: leukemiaInfiltrative: leukemia

• Drugs- Anthracycline (doxorubicin and Drugs- Anthracycline (doxorubicin and daunorubicin) dose dependent >500 mg/mdaunorubicin) dose dependent >500 mg/m22 lipid peroxidation of myocyte membraneslipid peroxidation of myocyte membranes

Leutic (Syphilis) Heart Leutic (Syphilis) Heart DiseaseDisease

• Obliterative endarteritis - plasma cellsObliterative endarteritis - plasma cells

• Aneurysm of thoracic aortaAneurysm of thoracic aorta

• Tree barkingTree barking

• Narrowing of ostia of coronary arteriesNarrowing of ostia of coronary arteries

• Severe ASVD at root of aortaSevere ASVD at root of aorta

• Dilation of aorta valve ring - (aortic Dilation of aorta valve ring - (aortic insufficency)insufficency)

• Marked LVH - cor bovinumMarked LVH - cor bovinum

Syphilitic aortitis and aortic insufficiency

Dilated aortic rootDilated aortic root

Weaken aorta with Weaken aorta with Aneurysm of ascendingAneurysm of ascendingaortaaorta

Carcinoid Heart DiseaseCarcinoid Heart Disease

• Serotonin, 5-hydroxtryptophane, Serotonin, 5-hydroxtryptophane, bradykinin, and histaminebradykinin, and histamine

• Most on right sideMost on right side

• Plaque-like thickeningsPlaque-like thickenings

• Fusion of tricuspid leafletsFusion of tricuspid leaflets

Carcinoid valvular disease

Note thick cusps-stenosis

SummarySummary• Mitral Stenosis : Rheumatic Heart Mitral Stenosis : Rheumatic Heart

DiseaseDisease

• Mitral insufficiency: myxomatous Mitral insufficiency: myxomatous degeneration (mitral valve prolapse), degeneration (mitral valve prolapse), damaged papillary muscle due to infarctdamaged papillary muscle due to infarct

• Aortic stenosis: calcification of normal Aortic stenosis: calcification of normal and congenitally bicuspid aortic valvesand congenitally bicuspid aortic valves

• Aortic insufficiency: dilation of the Aortic insufficiency: dilation of the ascending aorta, related to hypertension ascending aorta, related to hypertension and agingand aging