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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
NURSING CONSIDERATIONS
> 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
NURSING CONSIDERATIONS
>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
NURSING CONSIDERATIONS
> 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
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http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?sid=ba822833-c163-4788-b5f4-5a44424783ce%40sessionmgr15&vid=1&hid=1
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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