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Saint Louis University

School of NursingGraduate Program

In partial fulfillment

On the requirements

In Adult Health Care

CARDIAC MYOPATHIES and

RHEUMATIC HEART DISEASE

Submitted to:

Prof. Carolina Pangwi

Submitted by:

Baniqued, Charmaine A.

April 10, 2012


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CARDIOMYOPATHY

A heterogeneous group of diseases of the myocardium associated with mechanical and/or

electrical dysfunction, which usually (but not invariably) exhibit inappropriate ventricular

hypertrophy or dilatation, and are due to a variety of etiologies that frequently are genetic.

Cardiomyopathies are either confined to the heart or are part of generalized systemic disorders,often leading to cardiovascular death or progressive heart failure- related disability. (Maron BJ,

2006)

CLASSIFICATION OF CARDIOMYOPATHIES

As new data emerges, this requires further review and revision in the future. Recently, under

the auspices of the American Heart Association, A contemporary classification of cardiomyopathies has

been presented, relying substantially on recent advances in the characterization of diseases affecting

the myocardium.

In particular, the popular hypertrophic-diated-restrictive cardiomyopathies classification has majorlimitations by virtue of mixing anatomic designations. (i.e., hypertrophic and dilated) with a functional

one (i.e., restrictive). Consequently, confusion frequently arises when the same disease could

legitimately appear in two or even three categories.

OVERVIEW

I. PRIMARY CARDIOMYOPATHIES

I.A GENETIC

I.A.1. HYPERTROPHIC CARDIOMYOPATHY (ASSYMETRIC SEPTAL HYPERTROPHY/ IDIOPATHIC

HYPERTROPHIC SUBAORTIC STENOSIS)

-massive hypertrophy of the ventricular septum

-primary muscle hypertrophy which may exist with or without a dynamic LV outflow

tract gradient- WHO

-genetic disease transmitted as an autosomal dominant trait

- most frequently occurring cardiomyopathy

-Unexplained hypertrophied and nondilated LV

-When LV wall thickness is mild, differential diagnosis with physiologic athletes


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-PATHOLOGY:

Defects or mutation that alter the actin-myosin crossbridge formation affects

movement and force generation of the thick and thin filamentsasymmetric

hypertrophy of septum with a small left ventricular cavity

Coronary arterioles in the septum gets smaller because of intimalhyperplasiaMI

Large and bizarre fibrosis present with degenerating muscle fiberswhorling

Endocardium thickened by fibrous tissue

Mitral valve is intrinsically normal however there is a displacement of the

hypertrophied papillary muscles alteration in load and contractility of the LV

arrythmias, LVO obstruction, diastolic dysfunction, mitral regurgitation

Either rhythm abnormality or activation of LV baroreceptors results in a reflex

vasodilation decrease both pre and afterload syncope

Left atrium is dilated by autopsy

-MANIFESTATIONS:

murmurs

Dyspnea

Angina

Syncope

Largely limited to the , with massive degrees of LV hypertrophy

Ventricular pre- excitation

-DIAGNOSTICS:

Echocardiogram: stiff, noncompliant, hypertrophied ventricle and abnormal

relaxation of the elevated LV filling pressure

LVO tract obstruction & mitral regurgitation diastolic filling abnormalities

:primary tool for defining presence of LVO

:diffuse hypertrophy of entire septum with convex septal contour

ECG:LV hypertrophy

T wave inversion

Abnormal Q waves: stimulating MI

Normal sinus rhythm

Increased wall thickness: consider infiltrative disorder or athlete;s

Ambulatory monitor setting: supraventricular tachycardia, PVCs, non-

sustained VTach, Afib

CXR: mild to moderate enlargement of cardiac silhouette

LV: round contour


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Enlarged left atrium and right-sided chambers

Doppler: dagger shaped signal on continuous wave

Lab DNA analysis: + HCM-causing mutant gene

-TREATMENT/ THERAPY GUIDELINES

Screening all first-degree relatives is recommended.

Low to moderate aerobic exercise- permitted as part of healthy lifestyle

A. MEDICAL THERAPY

Beta-adrenergic blocking agents

-initial drug of choice

-decreased rate response to exercise

-decreased outflow tract gradient with exercise

-relief of angina by a decrease in myocardial oxygen demand

-improvement in diastolic filling

-no proven reduction in the incidence of sudden cardiac death

-should be titrated to obtain 60bpm resting up to 400mEq of

metoprolol

Calcium channel blockers (Verapamil)

-decreases inotropy and chronotropy

-may improve angina better than beta blockers

-resting heart rate: 60 bpm; titrate up to 480 mg /d

-CAUTION! No Dihydropidine-class calcium channel blockers: purevasodilators that reduces afterload

Disopyramide: in obstructive HCM; negative inotropic effect

decreases the gradient and improve symptoms

*The corrected QT interval must be monitored at the initiation of

the medication

Diltiazem: also used to treat symptoms

B. SURGERY

Septal Myectomy

-gold standard therapy for patients with obstruction and severedrug refractory symptoms

-the procedure consists of transaortic resection of a small amount

of muscle from the proximal to midseptal region enlarging the LVO

tract significant decrease/ abolish LVO tract obstruction

-mitral regurgitation also disappears as a result


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Mitral Valvuloplasty/ plication

-in combination with myectomy: proposed for patients with

deformed or elongated mitral valve leaflets

Mitral Valve Replacement

-recommended in patients with the assumption that the anterior

leaflet of the mitral valve contributes to the outflow tract

obstruction.

-should be performed only if there is associated severe and

unrepairable organic disease of the mitral valve

Dual Chamber Pacing

- Implantation of dual chamber pacemaker

-pacing the right ventricular apex decrease the outflow tract

gradient presumably because of ventricular contraction alteration with

a decrease in systolic projection of the basal septum into the LVO tract

Septal Ablation

- Alcohol is infused in the septal perforator arteries (catheter-based)

contolled MI of the proximal septum subsequent wall thinning

and remodeling of the basal septum region reduction of LVO

tract obstruction

- Major complication: complete block.

- Other complications: coronary dissections, large MI from alcohol

leakage into another coronary arteries, ventricular septal defects,

and myocardial perforations- Issue: Controversy over whether the results of septal ablation are

comparable to septal myectomy

-

Implantation of an automatic defibrillator

- Most effective and reliable treatment option for protecting patients

with SCD.

TREATMENT OF NONOBSTRUCTIVE CARDIOMYOPATHY

-major pathophysiologic abnormality: severe diastolic dysfunction

-Diuretics: used to decrease elevated filling pressures

-Beta blockers and calcium channel blockers: improve diastolic filling. Verapamil:

greater effect on relaxation abnormalities.

-Cardiac transplantation: forpatients with severe symptoms unresponcive to

conventional therapies


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-Promising new therapies: ACE inhibitors, angiotensin II receptor blockers, statins and

calcium blockers

NURSING CONSIDERATIONS

HISTORY TAKING

Obtain comprehensive heredofamilial cardiovascular diseases to establishgenetic ties.

Establish the past medical history of the patient. Because many patients have a

history of HCM, thus medicating with diuretics and vasodilators aggravates

the symptoms.

HCM is asymptomatic to some patients, but upon chest auscultation, murmurs

could be heard. Conduct thorough PA and extensive history taking.

PHYSICAL EXAMINATION

Assess also for carotid pulsations. Spike and dome pattern is a classic physical

finding for HCM.

Spike and dome pattern-(briskly) a rapid rise followed by a midsystolic drop that

is in turn followed by a secondary wave. The midsystolic drop in amplitude of

the carotid pulse contour is caused by premature closure of the aortic valve and

coincides with systolic anterior motion of the mitral valve. The late peak is

caused by relief of the outflow tract gradient as the mitral valve leaflet returns

to its original position.

JVP: normal in most HCM patients

:decrease in the compliance of ventricle.

Assess apical impulse. This is always abnormal in patients with HCM reflects

myocardial hypertrophy. Apical impulse is a sustained systolic thrust.

Systolic thrill may be palpable at apex from severe mitral regurgitation

LVO tract obstruction: ascertain crescendo-decrescendo murmur located at left

sterrnal border

Aortic valve disease: diastolic decrescendo

As a nurse, dynamic ausculatation should be performed to differentiate the

murmur.

Most reliable method for diagnosing LVO tract obstruction is the response of

the murmur to stand-squat-stand position.

From stand-squat: increase afterload and pre loadmurmur decrease in

intensity

From squat-stand: reduced afterload increase in murmur intensity

Educate patient. Patients with HCM often describe increase in symptoms during

hot, humid weather, because of fluid loss and vasodilation decrease preload

and afterload.

Counsel patient who wants to be pregnant about the risk of transmission

Prevent SCD by identifying high risk patients


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I.A.2. ARRHYTMOGENIC RIGHT VENTRIICULAR CARDIOMYOPATHY/ ARRYHTMOGENIC RIGHT

VENTRICULAR DYSPLASIA

-Uncommon inheritable heart muscle disease

-Autosomal dominant inheritance

-Predominantly involves RV with progressive loss of myocytes and fibrofatty

tissue replacement

-Specific feature: Aneurysms of RV in the triangle of dysplasia (inflow, apex,

outflow)

-Most common cause of death among athletes

-Sudden death: may be the first manifestation

-Life threatening tachyarrythmias, palpitations

-CHF; Right or Biventricular pump failure

-DIAGNOSTICS:

Original task force criteria

12- lead ECG: abnormal repolarization and T wave inversion

Echocardiography

RV angiography

CT/MRI

Endomyocardial biopsy from RV wall

-TREATMENT:

Lifestyle alterations: avoid intense physical activities

Anti-arrythmic drugs

ICD

Catheter ablation

transplant: final option

I.A.3. LEFT VENTRICULAR NONCOMPACTION

- Non compaction of ventricular myocardium: congenital cardiomyopathy

-Natural Hx of LVNC is largely unresolved but includes:

LV systolic dysfunction

HF

Thromboemboli

Arrhythmias

Sudden death

Diverse remodeling


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-CHARACTERISTICS:

Spongy morphologic appearance of LV myocardium: distal (apical

portion of the LV chamber)

-DIAGNOSIS:

2D-echo

LV angiography

I.A.4 GLYCOGEN STORAGE (will be discussed at I.B.2.a3)

I.A. 5 CONDUCTION SYSTEM DISEASE

-Lenegre disease, also known as progressive cardiac conduction defect (PCCD)

-Characterized as:

Primary progressive development of cardiac conduction defects in the

Purkinje fibers system leading to widening QRS complex and AV block

with long pauses and bradycardia= may trigger syncope

Sick sinus syndrome: typically similar to PCCD

Familial occurrence of both syndromes has been reported with an

autosomal dominant pattern of inheritance

I.A.6. MITOCHONDRIAL MYOPATHIES

-caused by mutations encoding mitochondrial DNA

-ATP electron transport chain enzyme defects which alter mitochondrial morphology

-considered also in metaboloic myopathies involving production and use defects

abnormalities (deficiencies and glycogen storage diseases)

I.A.7. ION CHANNELOPATHIES

-Inherited arrhythmia disorders caused by mutations in genes encoding defective ionic

channel proteins governing transit of Sodium, Potassium and Calcium ions

Includes:

I.A.7a. LQTS

-most common of the ion channelopathies

-Characterized as:

Prolonged ventricular repolarization and QT interval on standard 12-

lead ECG

A specific form of polymorphic VTach (Torsade de Pointes)


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Risk of syncope

Sudden cardiac death

I.A.7b. Brugada Syndrome

-CHARACTERISTICS:

Identified by a distinct ECG pattern consisting right bundle-branch

block and coved ST segment elevation in the anterior precordial

leads.

The pattern is often concealed and may be unmasked with

administration of Sodium channel blockers (Procainamide)

Mutation in cardiac sodium channel gene

I.A.7c. Sudden Unexplained Nocturnal Death Syndrome

-Predominantly found in young Asian males (Thailand, Japan, Philippines, Cambodia)

-disorder causing death during sleep because of VTach/VFib

-this gene mutation and Brugada syndrome have been shown to phenotypically,

genetically and functionally the same disorder.

I.A.7d. Catecholaminergic Polymorphic Ventricular Tachycardia (Cpvt)

-CHARACTERIZED BY:

Syncope

Sudden death

PVT

Normal resting ECG: unremarkable with the exception of sinus

bradycardia and prominent u waves in some patients

Absence of structural cardiac disease

Most typical arrythmia: bidirectional VTach presenting with an

alternating QRS axis

-Triggered by vigorous physical exertion or acute emotion; CR exceeds 120-125

bpm threshold

-mutation in the gene essential for regulation of excitation-contraction coupling

and intracellular calcium levels


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I.A.7e. Short QT Syndrome

-CHAR. AS:

Short QT interval on ECG

High incidence of Sudden cardiac death caused by VTach/VFib

Appearance of tall peaked T waves on ECG, similar to hyperkalemia

I.B MIXED GENETIC AND NONGENETIC

I.B.1 DILATED CARDIOMYOPATHY

-defined as a ventricular chamber exhibiting increased diastolic and systemic volume

and a low EF (


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-DIAGNOSTICS:

2D-echo

I.B.1a. ISCHEMIC CARDIOMYOPATHY

-a dilated cardiomyopathy in a subject with a history of MI or evidence of clinically

significant (i.e greater than or equal to 70% narrowing of a major epicardial artery) CAD,

in which the degree of myocardial dysfunction and ventricular dilatation is not explained

solely by the extent of previous infarction or the degree of ongoing ischemia.

-an ischemic dilated cardiomyopathy is present when a post-MI LV experiences

remodeling and a drop in EF.

PATHOPHYSIOLOGY:

Heightened compensatory mechanisms remodeling process transmural or

subendocardial scarring (representing old MIs) attempt of compromised ventricle

increase stroke vol. adverse outcome in the long term ischemic dilated

cardiomyopathy

PROGNOSIS:

Patients with ischemic cardiomyopathy have a worse prognosis than with subjects with

a non-ischemic dilated cardiomyopathy.

TREATMENT:

PHARMACOLOGIC

1. ACE inhibitors

-improves LV dimensions and function

-progressive decrease in LV dimensions significant improvement in

shortening fraction

-prolong survival in patient with asymptomatic LV dysfunction following

MI- if with (+) fluid retention: ACE inhibitor + diuretic


> Assess cardiopulmonary status of patient before starting with

the therapy.


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> I & O monitoring especially if patient is on concomitant

diuretic therapy

> Carefully monitor patient for the major side effects:

a. hypotension

b. prerenal azotemia

c. hyperK

d. cough

Adverse effect: angioedema

>Cough should not be immediately be presumed r/t the

medication, as elevated LV filling pressure may commonly cause

coughing. Patient may need an increase in the diuretic dose or an in

increase in rather than discontinuation.

2. Beta-blockers

-increases LV function as measured by EF

-promotes reverse modeling

3. Diuretics

-affects both cardiac preload and afterload with an associated

improvement in LV performance

-decrease in LV filling pressure was generally associated with an

improvement in cardiac performance measured by increases in stroke

volume


>Review renal functions lab result: BUN, Crea,

>Assess cardiopulmonary status of patient and compare with

the patients reaction to the therapy

>Monitor F & E status

>Monitor I & O

>Watch out for deafness: ototoxic drugs

4. Spironolactone

- K+ sparing


> Monitor F & E, specifically for hyperkalemia

> Note any ECG abnormalities such as tall peaked T wave and a

presence of u wave in any diuretics


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5. Digoxin

-For patients who remain asymptomatic on ACE inhibitor/ ARB, beta

blocker and diuretics.

-inotropic effect inhibition of Na/K ADP sympthoinhibitory effect

sensitization of high-pressure baroreceptors redeces central

sympathethic outflow

NURSING CONSIDERATION

> Count cardiac rate before administering

110 bpm: hold

DEVICE:

>ICDs

-for patients without intraventricular conduction defects

ADJUNCTIVE THERAPY

>Anticoagulation: for subjects with lower LVEF to prevent thromboembolic

complications

>Amiodarone: treat symptomatic arrythmias

SURGERY:

>Revascularization: an aggressive approach to treat ischemia

NURSING CONSIDERATIONS:

>Review serum K+

-serum K+ levels should be maintained in the high normal (4.3- 5.0

mEq/L) range to prevent sudden death

>Review lab results for digoxin levels

-Normal: less than or equal to 1.0 ng/mL

I.B.1b HYPERTENSIVE CARDIOMYOPATHY

-diagnosed when myocardial systolic function is depressed out of proportion to the

increase in wall stress

-a subject presenting in heart failure with a hypertensive crisis would not carry this

diagnosis unless ventricular dilatation and depressed systolic function remained after

correction of the hypertension.


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PATHOPHYSIOLOGY

Hypertension Sustained increase in systolic wall stress (both systolic pressure-

overloaded RV & LV) systolic dysfunction Hypertensive cardiomyopathy

PROGNOSIS

Depends on the presence of other comorbid conditions

TREATMENT

Same as in IDCM except that overload must be vigorously controlled. Addition

of pure anti-hypertensive vasodilators such as amlodipine, hydralazine, nitrates

to modestly increase LVEF relief of symptoms: dyspnea.

I.B.1C VALVULAR CARDIOMYOPATHY

-occurs when a valvular abnormality is present and myocardial systolic functionis depressed out of proportion to the increase in wall stress

-commonly occurs with left-sided regurgitant lesions (mitral and aortic) less

commonly with aortic and never as a consequence of pure mitral stenosis

PATHOPHYSIOLOGY

Exposure to different types of wall stress pattern of hypertrophy derives

from increased diastolic wall stresscompensated eccentric hypertrophy

long-standing mitral regurgitation progress to a dilated failing phenotype

valvular cardiomyopathy

TREATMENT

>same as in ICM

>plus aggressive afterload reduction, usually hydralazine/ nitrates on

top of ACE inhibitors.

>Calcium channel blocker amlodipine: another option for afterload

reduction

>Surgical valve replacement/ repair as soon as cardiomyopathy is

detected

>Mitral valve replacement SHOULD NOT be attempted in most subjects

with severe mitral regurgitation and LVEF less than 25% because of of

prohibitively high operative/ perioperative mortality rates.

>Catheter Valvuloplasty: for severe aortic stenosis


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I.B.1d IDIOPATHIC DILATED CARDIOMYOPATHY

-increases with age, more males are affected

-excluding or ruling out significant other causes

PATHOPHYSIOLOGY

Familial

FEATURES: (post mortem exam)

>increased mean cardiacweight: women-551g ; men- 632g

>increased muscle mass and myocyte cell vol.

> LV wall thickness is not increased because of the marked dilatation of the

ventricular cavities

>Grossly visible scars in either ventricle

> Marked myocyte hypertrophy, very large, bizarrely shaped nuclei

>(+) intracardiac thrombi

DIAGNOSTICS:

>Endomyocardial biopsy

TREATMENT

>Anticoagulant because of the risk of thromboembolic complications

> Beta adrenergic blockade: improves and normalize LV function

I.B.2 PRIMARY RESTRICTIVE (NONHYPERTROPHIED) CARDIOMYOPATHY

-rare form of nonhypertrophied, non dilated muscles disease and a cause of HF

-Char. By:

Normal or decreased volume of both ventricles

Associated with biatrial enlargement

Normal LV wall thickness and AV valves

Impaired ventricular filling with restrictive physiology

And normal or near normal systolic function


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-Manifestation

Normal (or near normal) systolic function at rest, ventricular, diastolic, jugular,

and pulmonary venous pressures are increase.

Elevated atrial pressures produce symptoms of systemic and pulmonary venous

congestion (dyspnea, orthopnea, edema, abdominal discomfort)

TREATMENT

Directed toward the treatment of diastolic failure

Reduction in the elevated ventricular diastolic pressures improves pulmonary

and systemic congestion (diuretics)

Vasodilators jeopardize ventricular filling

ACE inhibitors

Digoxin

Beta blockers

Anticoagulants

I.B.2a MYOCARDIAL DISEASES

I.B.2.a1 NON-INFILTRATIVE

i. IDIOPATHIC and FAMILIAL RCM

-not generally recognized to have a familial predisposition

-autosomal dominant and recessive patterns

-genetic cause of HCM also cause RCM

-Char:

Myocyte hypertrophy and fibrosis on endomyocardial biopsy

-DIAGNOSTICS:

2D-echo: distinguishes primary RCM

CT/MRI scans: restrictive fillings

ii. PSEUDOXANTHOMA ELASTICUM

-Char:

Fragmentation and calcification of elastic fibers (endocardial

fibroelastosis) RCM

Genetic disordes


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iii. PROGRESSIVE SYSTEMIC SCLEROSIS

-Char:

Myocardial fibrosis, a patchy distribution in both ventricles is found on

autopsy

-DIAGNOSTICS:

Echocardiography: LV wall thickening in the absence of hypertension.

LV dysfunction; pericardial involvement

ECG: block, supraventricular and VTach, and pseudoinfarction

patterns

***Pulmonary hypertension- leading cause of morbidity and mortality

I.B.2.a2 INFILTRATIVE CARDIOMYOPATHIES

i. Amyloidosis

-systemic disorder

-Char. by:

Interstitial deposition of linear, rigid, non branching amyloid protein

fibrils in the (multi-organ)

Amyloids- protein precepitates

-FEATURES

Ventricular filling pattern of abnormal ventricular relaxation that

gradually advances to restrictive pattern accompanied by clinical s/s of

RSHF

HF/ arrythmia

AFib and conduction abnormalities

Amyloid deposits in interstitial or widespread RCM/ localized to (a

)conduction tissue resulting in block and ventricular arrhythmias

(b)cardiac valves causing valvular regurgitation (c) pericardiumproducing constriction (d)pulmonary vasculature causing pulmonary

hypertension and cor pulmonale

-MANIFESTATION:

Orthospatic hypotension

syncope


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-DIAGNOSTICS:

Chest radiograph: normal or moderately enlarged

ECG: a pseudoinfarction pattern, arrhythmias, conduction disturbances

Echocardiogram: wall thickness involving right and left ventricles, a

small or normal LV cavity, depressed systolic function, left atrialenlargement, pericardial effusion, thickening of theventricular

myocardium, interatrial septum and valves (AV valves), enlarged

papillary muscles and dilated atria and inferior vena cava.

Doppler:restrictive pattern of LV filling

**earliest sign of amyloid cardiomyopathy: LV relaxation

Radionuclide ventriculography: LV time activity abnormalities in LV

filling

MRI

Serum and urine protein electrophoresis

Endomyocardial biopsy of RV: quantifies myocardial damage andatrophy

TREATMENT:

Symptomatic therapy

Amiodarone: Afib

Pacemaker: for symptomatic bradycardia/ high-grade conduction system

disease

Immunosuppressive therapy (melphalan & prednisone)

Autologous stem-cell infusion: little effect on existing infiltrative amyloid

ii. Sarcoidosis

-disorder of unknown etiology

-Char: non- caseating granuloma granulomas involve in the

:localized thinning and dilatation of the basilar LV resembling ischemic

disease

PATHOPHYSIOLOGY:

Interstitial granulomatous inflammation produces diastolic dysfunction

sarcoid pulmonary involvement


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DIAGNOSTICS:

Echo & Doppler: pulmonary HPN and RSHF, high grade AV block: a result

of the involvement of conduction system; ventricular arrhythmias

Echo: systolic and diastolic LV dysfunctions, LV aneurysm

formation,abnormal ventricular wall thickness, pericardial effusion, corpulmonale

ECG: T wave and conduction abnormalities

Thallium 201: to indicate areas of myocardial involvement

MRI: detect lesions

Endomyocardial biopsy

MANIFESTATIONS:

Syncope, sudden cardiac death

TREATMENT:

Corticosteroids, Prednisone

Permanent pacemaker

AICD (automatic implantable cardioverter-defibrillator)

Calcium channel blocker: ameliorate diastolic function to RCM

Cardiac transplant

iii. Gaucher disease

-most common lysosomal strage disease, inherited enzyme deficiency

-diffuse interstitial infiltration of the LV with reduced LV wall compliance and cardiac

output

DIAGNOSTICS:

Echo: left sided valvular thickening, pericardial effusion

THERAPY:

Enzyme replacement therapy

I.B.2.a3 STORAGE DISEASES

i. Hemochromatosis

-autosomal iron-storage disease


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FEATURES:

Accumulation of iron in the CM

Arrhythmia and conduction disturbances

CHF

Supraventricular and ventricular arrythmiias Granular sparkling and atrial enlargement

DIAGNOSTICS:

MRI: tissue characterization

Ultrasonic analysis: echo reflectivity

Endomyocardial biopsy: confirmatory

TREATMENT:

Chelating agent: desferrioxamine

ii. Fabry Disease: disorder of the lysosomal metabolism

:if deficient accumulation of glycolipid in the RCM

DIAGNOSTICS:

Echo: same as in amyloid

Endomyocardial biopsy: definitive

MANIFESTATIONS:

MVP

HPN

HF

` TREATMENT

Enzyme replacement therapy

ii. Pompe Disease

-acid maltase deficiency glycogen deposition in the

DIAGNOSTICS

Echo: indistinguishable with HOCM

LVH


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MANIFESTATIONS

Resembles HCM

I.B.2b ENDOMYOCARDIAL DISEASE

I.B.2.b1 OBLITERATIVE ENDOMYOCARDIAL DISEASE

i. Endomyocardial Fibrosis

ii.Hypereosinophilic Syndrome

-cause restrictive obliterative cardiomyopathies (EMF & Loeffler)

-Loeffer same features with EMF but it affects mainly men; usually related to

parasitic infection, leukemia and immunologic reactions char. by intense

eosinophilia and thromboembolic phenomena

MANIFESTATIONS:

Unexplained eosinophilia for at least 6mos.

Cardiotoxic eosinophils (abnormal cells containing vacuoles central to

the pathogenesis)

PATHOLOGY:

Eosinophilic infiltration and mediator released damage

acute eosinophilic myocarditis, thrombosis formation,

endomyocardial fibrosis with ventricular obliteration, AV regurgitation

RCM

DIAGNOSTICS:

ECG: T wave abnormality

Endomyocardial biopsy:

Echo: densities in the myocardium

Doppler

TREATMENT:

Symptomatic relief: anti coagulants

Corticosteroids

Palliative surgery

Surgical excision of fibrotic endocardium

Valve replacement


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I.B.2.b2 NON OBLITERATIVE ENDOMYOCARDIAL DISEASES

i. Carcinoid syndrome

-results from metastatic carcinoid tumors production of serotonin,

bradykinin and other substances affects right structures fibrous

endocardial plaque formation comprising of smooth muscle cells on tricuspid

and pulmonic valves and right endocardium is characteristic. Tricuspid

and pulmonic stenosis are the dominant characteristics pulmonary outflow

tract obstruction as the result of constriction.

DIAGNOSTICS:

CXR: cardiomegally, pleural effusions and nodules

ECG: low voltage QRS complex

2D echo: thickened, retracted immobile tricuspid and pulmnic valves

and right AV enlargemen Transesophageal echo: right atrial wall thickening

Doppler: low velocity tricuspid and pulmonic regurgitation

Catheterization: pulmonic regurgitation

TREATMENT

Somatostatin

Valvular replacement

I.B.2.b3 MALIGNANT INFILTRATION

-Caused by infiltrating tumors involvement of pericardium RCM

DIAGNOSTIC:

Echo: localized increase in wall thickness associated with pericardial

effusion

i. Iatrogenic disease

Pericardial disease frequently complicates radiation therapy to the

chest and can produce constrictive pericarditis, however, endo and myocardial

involvement can produce RCM


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I.C. ACQUIRED

I. C. 1. INFLAMMATORY CARDIOMYOPATHY

I.C.1.a MYOCARDITIS

-acute/ chronic inflammatory process affecting the myocardium (frequently subclinical)

-can trigger autoimmune reaction that causes immunologic damage to the myocardium

-typically evolves through active healing and healed stages characterized progressively

by inflammatory cell infiltrates leading to interstitial edema and focal myocyte necrosis

and replacement of fibrosis. - electrically unstable substrate - potential

predisposition to VTach/ sudden death

-Causes:

Toxins and drugs Infectious agents

Viral: most common (coxsackie, HIV, parvovirus)

Rickettsial

Fungal

Parasitic

Immune (giant cell myocarditis) and Hypersensitivity reactions

-Diagnostics:

Histopathology/ histochemically

Endomyocardial biopsy: inflammatory (leukocyte) infiltrate and necrosis

ECG: ST-T changes; LV dilatation

Challenging to id clinically

-Manifestations:

Chest pain, exertional dyspnea, fatigue, syncope, palpitations,

VTachyarrythmias, conduction abnormalities, acute CHF/ cardiogenic shock

-Treatment:

Supportive care

Diuretics

ACE inhibitors

Beta blockers

Aldosterone antagonist

Digoxin: can increase expression of inflammatory cytokines


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RHEUMATIC CARDITIS

-result from direct toxic effect of Group A Beta Hemolytic Streptococcus product versus an

immunologic mechanism.

PATHOPHYSIOLOGY

-Inadequately treated GABHS infection (strep throat, scarlet fever, pharyngitis)

GABHS have a number of structural components similar to those of human tissue

antibodies to streptococci cross react with glycoproteins of valves serum of RF

patients contains autoantibodies persistent focal inflammatory lesions in the

myocardium can persist for years after an acute attackformation of Aschoff

bodies. Repeated episodes of ARF scarring the valves RHEUMATIC disease

chronic valvular disease rarely CHF possible cardiomyopathy

CLINICAL DIAGNOSIS: JONES CRITERIA (see handout)

PHYSICAL EXAM:

Notable for fever

Murmurs: acute valvulitis

Mitral regurgitation: middiastolic murmur over apical area can be heard (Carey

Coombs murmur)

Acute migratory polyarthritis of the large joints

DIAGNOSTICS:

ECG: PR prolongation

Endomyocardial biopsy

Diffuse cellular interstitial infiltrate

Elevated ESR

Elevated CRP

ASO titer

TREATMENT

Aspirin and penicillin as the mainstay of therapy

Corticosteroids

IVIG

Mitral valve replacement/ repair: not during the acute attack

Antibiotic prophylaxis


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***The most effective method is a single monthly IM injection of 1.2 million units of

benzathine penicillin G until age 21 or for 5 years whichever is longer.

RHEUMATIC DISEASE

-often associated with stenosis and fusion of commisures secondary dilatation of mitral

annulus decrease contract between leaflet

-lesion: retractile fibrosis of leaflets and chordate causing loss of coaptation valvular

disease

VALVULAR HEART DISEASE

MITRAL VALVE PROLAPSE (most common)-fibromyxomatous changes in mitral leaflets

-Manifestations

> palpitation: PVCs

>Chest pain: coronary artery spasms

>Dyspnea and fatigue

-Physical Examination:

>skeletal abnormalities may suggest diagnosis of MVP, most

common: scoliosis, pectus excavatum,, straightened thoracic

spine and narrowed A-P diameter of the chest>systolic click

-Diagnostics:

>ECG: ST- T wave depression or T wave inversion

>Echo: holosystolic posterior hammocking of more than 3 nn

>CXR: calcification of mitral annulus

>Myocardial perfusion Scintigraphy: imaging with thallium to

determine coexisting MI

>LV cineangiography: confirms presence of MVP

-Treatment:

>Antibiotic prophylaxis: prevention of infective myocarditis

>Beta blockers: palpitation, sinus tachycardia, chest pain,

anxiety, fatigue


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>Volume expanders: orthostatic symptoms

>Anti-arrhythmic drugs

NURSING MANAGEMENT

>Educate on cessation of catecholamine stimulants (caffeine,

alcohol, cigarettes)

>Liberalize fluid and salt intake

AORTIC VALVE STENOSIS

-obstruction to outflow of blood from the LV

-most common cause: rheumatic

PATHOPHYSIOLOGY

Rheumatic AS adhesions and fusion of commisures and cusps

leaflets and ring become vascularized retraction and stiffening of the

cusps calcification aortic valve orifice is reduced to small

triangular or round opening stenotic regurgitation LV pressure

rises increase ventricular wall stressimpaired LV function

hypertrophy

PHYSICAL FINDINGS

> Systolic thrill in the carotid artery>JVP is normal

DIAGNOSTICS

>CXR: normal-sized heart with a dilated proximal ascending

aorta (poststenotic dilatation); calcium in the aortic valve

>ECG: LVH

>Echo/ Doppler sound: Aortic valve: thickened as a result of

calcification and fibrosis; LV cavity: normal size

Transesophageal echo: defines aortic valve abnormality

>Electron Beam CT: detects AV calcium in AS>Myocardial Viability

>Cardiac catheterization and angiography

>Coronary Arteriography

>BNP


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MANAGEMENT

>antibiotic prophylaxis

> AFib: Avoid moderate to severe physical exertion and

competitive stress

>Pharmacologic:

a. Statins: prevention and/ or slowing of progression of calcific

AS

b. ACE inhibitor: if the cause is HF

c. Vasodilators: reduce filling pressures and increase cardiac

output

>Surgery:

a. Simple commisurotomy/valve repair: relieve outflow

obstruction

b. CABG

c. Catheter Balloon Valvuloplasty

d. Percutaneous Transcatheter Prosthetic Heart Valve Insertion

e. Aortic valve replacement

PHYSICAL EXAMINATION:

Brisk carotid upstroke

Cardiac palpation: laterally displaced, diffused and brief apical impulse with

enlarged LV

Left sternal border lift: RV dilatation

DIAGNOSTICS:

ECG: may be entirely normal; severe mitral regurgitation: notched P waves

CXR: cardiomegaly

Doppler echo: severity of regurgitation

Echo: quantification of LV end-diastolic dimensions, wall thickness and EF

Radionuclide angiography: LVEF


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Cardiac catheterization: severity of MR, LV function, and coronary anatomy

MANAGEMENT:

MEDICAL

Digoxin/ Beta blockers: AFib

Oral anticoagulation

SURGICAL

Mitral valve repair

Mitral valve replacement

Catheter Balloon Commisurotomy

Percutaneous Mitral Balloon Valvotomy

I.C.2 STRESS CARDIOMYOPATHY

I.C.2.a TAKO- TSUBO

-brokenheart syndrome

-Char. By:

Acute but rapidly reversible LV systolic dysfunction in the absence of

atherosclerotic CAD

Triggered by profound stress

Distinction: involves the distal portion of LV chamber apical ballooning with

the basal LV hypercontractile. The shape is similar to a Japanese tako-subo

pot with a narrow neck and round bottom used by fishermen to trap

octopus.

PATHOPHYSIOLOGY

Adrenergic stimulation differences in the density of beta adrenergic

receptors in the apex and base of the heart unusual ballooning

-PRESENTATION:

Mimics ST segment elevation MI: acute coronary ischemia generally

preceded by a stressful, emotional, physical, or psychological event such as

the death of the love one

-MANIFESTATIONS:

Chest pain


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Dyspnea

Syncope

-DIAGNOSTICS:

Elevated BNP

ECG: ant. ST elevations but ST depression and T wave inversion

Criteria:

a. Transient mid to apical LV aknesis or dyskinesis in areas involving

more than a single coronary artery

b. Absence of coronary artery disease

c. Acute ECG changes including ST segment elevation or depression

d. No recent head trauma, intracranial hemorrhage,

pheochromocytoma, myocarditis or hypertrophic cardiomegally

II. SECONDARY CARDIOMYOPATHIES (see reproduced copy for the list)

II.A TOXICITY (Chemotherpeutic)

Anthracycline and Doxurubicin cardiotoxic early manifestation: pericarditis-

myocarditis sndromme increased oxidative stress from the generation of free radicals

endogenous antioxidants reduced by treatment LV dysfunction/ arrhythmias

late/ chronic cardiotoxicity dose-dependent degenerative cardiomyopathy

Anthracycline cardiomyopathy

Cause: cumulative doses above 550mg/m2

DIAGNOSTICS:

Serial EF

BNP:

ECG: decreased QRS voltage, nonspecific ST segment, T wave abnormalities

NURSING MGT:

Discontinuation with therapy Lowering peak blood flow at the blood by giving continuous instead of

bolus

II.B. PERIPARTUM CARDIOMYOPATHY

-more commonly in obese multiparous black female


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Risk factors: twin pregnancies, pre eclampsia, tocolytics

-present HF in the last trimester or in the first 5mos. Post partum

-absence of demonstarable cause of heart failure and structural heart disease

- hemodynamic stress of pregnancy can unmask previously unknown cardiac disease

peripartum cardiomyopathy

MANIFESTATIONS:

SOB

Dyspnea on exertion

Edema

Palpitations

Syncope

S3, S4

DIAGNOSTICS:

ECG: LV hypertrophy

Echo: can range from single chamber LV enlargement to four chamber dilatation

Endomyocardial biopsy: myocarditis

TREATMENT

Pentoxofylline: inhibit proinflammatory cytokines

Transplant: for patients with refractory HF

II.C NEUROMUSCULAR DISEASE

II.C1. Duchenne

-X-linked cardioskeletal cardiomyopathy dystropin gene mutation

cardiorespiratory failure death

-myotonic dystrophy arrrythmias MP

IID. DIABETES

Metabolic abnormalities associated with diabetes affects the myocytes structure and

functioning diabetic cardiomyopathy


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PATHOLOGY

Nonesterified fatty acids trigger insuli resistance and myocardial contractile

dysfunction and apoptosis hyperinsulinemia cardiac hypertrophy

hyperglycemiamediates tissue injury increase oxidative process interstitial

fibrosissystolic and diastolic dysfunctionMP

DIAGNOSTIC:

Histology:

No evidence of epicardial atherosclerotic disease

GENERAL NURSING DIAGNOSES

Knowledge deficit Ineffective tissue perfusion: Cardiac

Anxiety

Acute Pain

Altered physical mobility

Fatigue

Decreased Cardiac output

Impaired gas exchange

Ineffective role performance

Altered comfort status


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JOURNAL UPDATES

DIAGNOSING MODALITIES

TITLE: CLINICAL MANAGEMENT OF ARVC: An UPDATE

ORIGINAL and REVISED TASK FORCE CRITERIA

TITLE: ALMANAC 2011: CARDIOMYOPATHIES. THE NATIONAL SOCIETY JOURNALS PRESENT SELECTED

RESEARCH THAT HAD DRIVEN RECENT ADVANCES IN CLINICAL CARDIOLOGY

New Technology: Next- generation sequencing (NGS)- number of

technologies that provide massively parallel, high through put DNA

sequencing. DNA (enriching and labeling) reduced cost and

improvements in automation

TREATMENT- RELATED

TITLE: THE FIRST SEPTAL UNIT in HOCM

A newly recognized anato-functional entity, identified during recent alcohol

septal ablation experience

NURSING RELATED

TITLE: REDUCED BONE DENSITY IN PATIENTS ON LONG-TERM WARFARIN

Warfarin, a vitamin K antagonist

References:

Fuster, et al (2008). Hursts the heart. 12th ed. Mc Graw Hill Publishing

Huether (2004). Understanding Pathophysiology 4th

ed., Singapore. Elsevier

http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=bcc594e5-8ea0-4c2d-9ab9-94c8107ef7c3%40sessionmgr104&vid=1&hid=119

http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ad0fd8d5-0703-49f2-9e3f-4b3c7c436c1c%40sessionmgr14&vid=1&hid=11

http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=47e3f9d0-46f0-4a47-8757ebb5fad83540%40sessionmgr114&vid=1&hid=111

http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ba822833-c163-4788-b5f4-5a44424783ce%40sessionmgr15&vid=1&hid=1
http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=bcc594e5-8ea0-4c2d-9ab9-94c8107ef7c3%40sessionmgr104&vid=1&hid=119http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=bcc594e5-8ea0-4c2d-9ab9-94c8107ef7c3%40sessionmgr104&vid=1&hid=119http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=bcc594e5-8ea0-4c2d-9ab9-94c8107ef7c3%40sessionmgr104&vid=1&hid=119http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ad0fd8d5-0703-49f2-9e3f-4b3c7c436c1c%40sessionmgr14&vid=1&hid=11http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ad0fd8d5-0703-49f2-9e3f-4b3c7c436c1c%40sessionmgr14&vid=1&hid=11http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ad0fd8d5-0703-49f2-9e3f-4b3c7c436c1c%40sessionmgr14&vid=1&hid=11http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=47e3f9d0-46f0-4a47-8757ebb5fad83540%40sessionmgr114&vid=1&hid=111http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=47e3f9d0-46f0-4a47-8757ebb5fad83540%40sessionmgr114&vid=1&hid=111http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=47e3f9d0-46f0-4a47-8757ebb5fad83540%40sessionmgr114&vid=1&hid=111http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ba822833-c163-4788-b5f4-5a44424783ce%40sessionmgr15&vid=1&hid=1http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ba822833-c163-4788-b5f4-5a44424783ce%40sessionmgr15&vid=1&hid=1http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ba822833-c163-4788-b5f4-5a44424783ce%40sessionmgr15&vid=1&hid=1http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ba822833-c163-4788-b5f4-5a44424783ce%40sessionmgr15&vid=1&hid=1http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ba822833-c163-4788-b5f4-5a44424783ce%40sessionmgr15&vid=1&hid=1http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=47e3f9d0-46f0-4a47-8757ebb5fad83540%40sessionmgr114&vid=1&hid=111http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=47e3f9d0-46f0-4a47-8757ebb5fad83540%40sessionmgr114&vid=1&hid=111http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ad0fd8d5-0703-49f2-9e3f-4b3c7c436c1c%40sessionmgr14&vid=1&hid=11http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ad0fd8d5-0703-49f2-9e3f-4b3c7c436c1c%40sessionmgr14&vid=1&hid=11http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=bcc594e5-8ea0-4c2d-9ab9-94c8107ef7c3%40sessionmgr104&vid=1&hid=119http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=bcc594e5-8ea0-4c2d-9ab9-94c8107ef7c3%40sessionmgr104&vid=1&hid=119


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SUMMARY DIAGRAM

CLASSIFICATION OF CARDIOMYOPATHIES

PRIMARY SECONDARY

Genetic Mixed genetic

and nongenetic

Acquired

Hypertrophic CM

ARVC

LV non-

compaction

Glycogen

storage

Conduction

defects

Mitochondrialmyopathies

Ion Channel

Disorders

LQTS

Brugada

syndrome

SUNDS

Catecholami

-nergic

polymorphic

VTach

SQTS

Dilated CM

Ischemic CM

Hypertensive

CM

Valvular CM

Idiopathic

dilated CM

Restrictive

CM

Myocardial

Non infiltrative

Infiltative

Storage

Endomyocardial

Obliterative

Non

obliterative

Malignant

infiltration

Inflammatory CM

Myocarditis

Rheumatic

carditis

Stress provoked

Tako-

tsubo

Peri partum

DCM

Anthracycline

Peri- partum

Neuromuscular

Duchenne

Diabetes

Documents

Report Output