Download Caring For Patients With Cardiomyopathy

Caring For
Patients With
Cardiomyopathy
J.O. Medina,RN, MSN,FNP,CCRN
Education Specialist / Nurse Practitioner
Critical Care & Emergency / Trauma
Services
California Hospital Medical Center
Objectives :


Define cardiomyopathy.
Differentiate between dilated,
restrictive , and hypertrophic
cardiomyopathy with regard to
etiology, pathophysiology, and
management.
Cardiomyopathy:
Overview




Disease of cardiac muscle myofibril
degeneration affecting heart globally
Not as a result of HTN, coronary
atherosclerosis, valvular dysfunction or
pericardial abnormalities
cause often unknown (idiopathic)
categorized into 3 groups based on
functional and structural abnormalities
 dilated(congestive)cardiomyopathy
 hypertrophy cardiomyopathy
 restrictive cardiomyopathy
Cardiomyopathy : Types

Dilated (congestive)
cardiomyopathy
 systolic
dysfunction related to
abnormal dilation of heart
chambers

Hypertrophic cardiomyopathy
 diastolic
dysfunction related to
abnormal hypertrophy of IVS /
ventricles

Restrictive cardiomyopathy
 diastolic
dysfunction related to
non-compliant stiff ventricles
Cardiomyopathy :
Major Consequences



Systolic or diastolic heart failure or
combination of both
arrhythmias
other problems specific to type of
disorder
Dilated (Congestive)
Cardiomyopathy





most common form of
cardiomyopathy
diffuse dilation of cardiac
chambers : ventricle(s) and atria
systolic dysfunction caused by
decreased contractility
pulmonary and systemic
congestion :  CO
embolic episodes
Dilated (Congestive)
Cardiomyopathy :
Causes






Often unknown
Alcohol (15 – 40%)
Pregnancy (last trimester) / post
partum (6 months post partum)
Collagen-viral infections
Oncologic agents : adriamycin
Hederofamillial neuromuscular
disease
Dilated (Congestive)
Cardiomyopathy :
Causes




Postmyocarditis
Toxins
Nutritional (beriberi, selineum
deficiency, thiamine deficiency)
Cocaine, heroine, organic solvents
 “glue-sniffer’s


heart”
Infection ( viral HIV, rickettsial,
myobacterial, toxoplasmosis )
Antiretroviral agents
Dilated (Congestive)
Cardiomyopathy :
Pathophysiology



diffuse dilation of ventricle(s)
causing decreased contractility
leads to  CO
compensatory mechanisms :
 ST
to maintain CO
 catecholamine release stimulating
renin-angiotensin system 
sodium/water retention and
vasoconstriction
(preload,afterload)
Dilated (Congestive)
Cardiomyopathy :
Pathophysiology

poor contractility :

LVEDV   LVEDP  dilates
annulus of AV valve  papillary
dysfunction  valve incompetency
 atrial enlargement  pulmonary
congestion
Dilated (Congestive)
Cardiomyopathy :
Clinical Presentation

LVF
 chronic
fatigue ; weakness
 orthopnea ; paroxysmal nocturnal
dyspnea (PND)
 cough ; chest pain
 weight gain
 palpitations
 dizziness ; syncope
 impotence
 insomnia
Dilated (Congestive)
Cardiomyopathy :
Physical Examination

Precordium
 tachycardia
 enlarged apical impulse, laterally
displaced (cardiomegaly)
 right ventricular impulse along LSB
 heart sounds: S, S, systolic murmur

Lungs
 tachypnea : if dyspnea present at rest
 end stage disease
 auscultation : clear  crackles /
wheezes
Dilated (Congestive)
Cardiomyopathy :
Physical Examination

LV Failure signs :

LOC
 cool, pale extremities
 pulsus alternans
 alternating
strong / weak pulse due
to severe LV failure

RV failure signs indicate severe
disease
Dilated (Congestive)
Cardiomyopathy :
Diagnosis

EKG
 arrhythmias
(compensatory for  CO)
 atrial; fibrillation (Af) : ominous sign
(due to dilated atria)
 atrial and ventricular arrhythmias
(high grade ectopy portent to
sudden death)
 ST
Q
waves : pseudoinfarction due to
fibrosis ; ST-T wave abnormalities
 QRS widened : LVH, LBBB
Dilated (Congestive)
Cardiomyopathy :
Diagnosis

CXR
 multichamber
enlargement,
pulmonary congestion, pleural
effusions

Echocardiogram
 LV
dysfunction
 chamber enlargement
 valve dysfunction
 hypokinesis and wall motion
abnormalities
  EF
Dilated (Congestive)
Cardiomyopathy :
Diagnosis

Medical history with emphasis on :
 Dyspnea
on exertion, orthopnea,
PND
 Palpitations
 Systemic and pulmonary embolism

Cardiac Troponin T
 Persistent
outcome
elevation marker of poor
Dilated (Congestive)
Cardiomyopathy :
Diagnosis

Exercise electrocardiogram
 determines
patient’s functional
status and if arrhythmias may
develop with exercise

Cardiac catheterization
 may
be helpful to identify
concomitant coronary artery
disease
Dilated (Congestive)
Cardiomyopathy :
Management

Goals
 cardiac workload
Limit
activity
Improve
symptoms
Treat underlying disease
Dilated (Congestive)
Cardiomyopathy :
Pharmacologic
Management

Treat CHF ( cause of death in 70%
of patients)
diuretics ; sodium restriction
 ACEI ; β-blockers, spirolactone,
and Digitalis
  preload ;  pulmonary and
systemic congestion
  wall tension   demand

Dilated (Congestive)
Cardiomyopathy :
Pharmacologic
Management vasodilators


afterload :  LV workload
 acute setting : NTG, SNP
 ACE inhibitors (first line oral
agents)

mortality rate
  afterload and preload
 Hydralazine
 second
(Apresoline) ; Isordil
line oral combination
 if unable to tolerate ACE inhibitor
Dilated (Congestive)
Cardiomyopathy :
Pharmacologic
Management

Inotropes

contractility and SV
 acute setting : dopamine,
dobutamine, amrinone,
epinephrine
 digoxin

Antiarrhythmias
 treat
symptomatic arrhythmias
 consider implanted defibrillator
Dilated (Congestive)
Cardiomyopathy :
Pharmacologic
Management

Low dose ß blockers
 Controversial
 Atenolol
 Metoprolol
 Carvedilol

Anticoagulation for patients :
 In
atrial fibrillation
 Moderate or severe failure
Dilated (Congestive)
Cardiomyopathy :
Management

Activity :
reduced physical activity during
period of decompensation
 cardiac rehab program to 
exercise tolerance


Diet :
 sodium
restriction
 small frequent meals during liver
congestion
  nutrition (prevent cachexia)
 vitamins ; no alcohol
Dilated (Congestive)
Cardiomyopathy :
Management


Growth Hormone : increase
myocardial mass (controversial)
Surgical Therapy
 cardiac
transplantation for end
stage disease (>50% of cardiac
transplants are DCM)
 latissimus dorsi muscle wrap
around heart with muscle pacing
synchronized to heart increase
contractility
Dilated (Congestive)
Cardiomyopathy :
Disposition

Annual mortality
20% in patients with moderate HF
 > 50% in severe HF



AICD with severe nonischemic
DCM
Referral
 Heart
transplant if < 60 years old
and no longer responding to
medical therapy
Hypertrophic
Cardiomyopathy (HCM)

formally referred to as
 idiopathic
hypertrophic subaortic
stenosis (IHSS)
 hypertrophic obstructive
cardiomyopathy (HOCM)
HCM : Characteristics




Asymmetrical hypertrophy of LV
with disproportional septum
enlargement as compared to free
wall
decreased LV cavity creates
diastolic stiffness impairing filling
thickened, elongated MV leaflets
are displaced and may obstruct LV
outflow tract
 LVSDP   atrial and pulmonary
pressure
HCM : Causes


1/3 familial
2/3 unknown
 sporadic

occurrence
Autosomal dominant trait causing
encoding of cardiac sarcomere
HCM : Pathophysiology


septum : disproportionately
enlarged creating narrow, long
cavity
excessive, early LV systole
displaces MV leaflets (along with
altered papillary muscle position)
toward IVS  preventing complete
closure of MV  obstruct LV
outflow tract . Septum can obstruct
outflow tract  ventricular wall
becomes rigid   LVEDP  
LAP  pulmonary congestion
HCM : Factors That
Aggravate Condition
 contractility (exercise, positive
inotropes)
  heart rate (exercise, fever, CO)
  preload (hypovolemia, sepsis,
fluid shifts)
 loss of atrial kick (atrial fibrillation,
AVB, ventricular arrhythmias)
Arrhythmias may occur and cause
sudden death !

HCM :
Clinical Presentation





Varies with degree of hypertrophy
dyspnea on exertion : pulmonary
congestion
dizziness / syncope : result of
ischemic induced arrhythmias:
CO
chest pain: due to  supply with 
demand; narrowed transluminal
coronary arteries
sudden death from arrhythmias
may be first sign
HCM :
Physical Examination

precordium
 sustained,
possibly lateral
displacement of ventricular impulse
- cardiomegaly
 presystolic atrial impulse felt
 harsh, mid systolic murmur at
apex, LSB, possible radiation to
axilla or base of heart
 S, S may be present


lungs : tachypnea
LV failure especially if atrial
fibrillation present
HCM : Diagnosis

EKG

voltage of LV hypertrophy
 ST-T wave abnormalities
 Q waves in inferior/lateral leads
due to septal hypertrophy
 PVC : 75%
 SVT : 25 - 50%
 atrial fib : 5 - 10%

CXR : normal or enlarged heart,
atrial enlargement, pulmonary
congestion
HCM : Diagnosis

Echocardiogram :
 septal
hypertrophy
 LA enlargement
 narrow outflow tract
 wall motion abnormalities
 MV leaflet abnormality

Cardiac Catheterization :

chamber pressures
 MR
 altered LV outflow gradient
HCM : Management

Goals :

ventricular filling by slowing HR
  contractility by reducing
obstruction
HCM : Management






maintain normal sinus rhythm
if atrial fibrillation : convert
pharmacologically / electrically
avoid hypotension, vasodilators,
dehydration, strenuous exercise,
sepsis, chemical withdrawal,
shivering, seizures
surgery : excise part of septum
implant defibrillator
avoid alcohol
HCM : Management


Avoid : digitalis, diuretics, nitrates
and vasodilators
Arrhythmia control
 Disopyramide
( Norpace )has
negative inotropic properties
 Amiodarone for atrial and
ventricular arrhythmias
HCM :
Pharmacologic Support


ß blockers
Propranolol 160mg – 240 mg/day
 for
dyspnea and chest pain
  HR ( provides longer filling)
  contractility (  outflow
obstruction;  demand )
 blocks SNS ( catecholamines
may be a causative factor)
 may  arrhythmias
HCM :
Pharmacologic Support


Calcium Channel Blockers :
Verapamil :  LV obstruction
line for β-blockers
 for hospital patients
  diastolic filling time
 promotes relaxation
  contractility
  outflow gradient
 second
HCM : Referral
Management

Myotomy-myectomy
 Resection
of basal septum
 For > 50% mmHg outflow gradient

Nonsurgical reduction of IVS
 Controversial
 Injection
of ethanol in septal
perforator branch of LAD
 Associated with high incidence of
heart block ; patient may require
permanent pacemaker
Restrictive
Cardiomyopathy :
Characteristics




uncommon type
restricted ventricular filling due to
replacement of ventricular muscle
with a non elastic material
diastolic dysfunction may develop
systolic dysfunction later in disease
symptoms of pulmonary / systemic
congestion
Restrictive
Cardiomyopathy :
Causes




90%
 Infiltrative and storage disorders
 amyloidosis deposits of insoluble
protein into muscle and connective
tissue
 sarcoidosis ; hemochromatosis
 myocardial fibrosis (after open heart)
radiation
scleroderma
diabetic cardiomyopathy
Restrictive
Cardiomyopathy :
Pathophysiology

stiff ventricles   ventricular
filling   CO  biatrial dilation
 pulmonary and systemic
congestion
Restrictive
Cardiomyopathy :
Clinical Presentation

subjective symptoms
 RUQ
discomfort ( right sided failure
symptoms predominate vs. left
sided symptoms )
 dyspnea : pulmonary congestion
 chronic fatigue :  CO
 poor exercise tolerance
Restrictive
Cardiomyopathy :
Physical Signs

right sided
failure :
JVD
 ascitis
 hepatic
enlargement
 edema

Restrictive
Cardiomyopathy :
Physical Signs

left sided failure
:
pulmonary
congestion
  BP
 narrowed
pulse
pressure
 weak, tired
 DOE

Restrictive
Cardiomyopathy :
Clinical Presentation

precordial exam :
 palpable
apical pulse; may be
displaced laterally
 cardiomegaly
 systolic murmur : TVR / MVR due
to atrial dilation or amyloid
infiltrates of papillary muscles
 S, S
Restrictive
Cardiomyopathy :
Diagnosis

EKG changes :
 low
voltage QRS
 sinus tachycardia, atrial fibrillation,
sinus bradycardia if SA node
infiltrated
 complex ventricular arrhythmias :
are poor prognostic sign
 Q waves : pseudo infarct from
fibrosis
 BBB, AVB
Restrictive
Cardiomyopathy :
Diagnosis

CXR :
 cardiomegaly
with biatrial
enlargement

Echocardiogram :
 normal
contractility
 no pericardial effusion
 biatrial enlargement
 LV hypertrophy with small
ventricular cavity

Myocardial Biopsy : amyloidosis,
hemochromatosis, etc.
Restrictive
Cardiomyopathy :
Management

Goal :
relief primarily by 
pulmonary / systemic congestion
 symptom
Restrictive
Cardiomyopathy :
Management

Pharmacological support :
 mild
diuretic therapy : prevent
excessive volume depletion to
prevent syncope from  SV
secondary to  ventricular filling
 vasodilator : NTG, ACE inhibitors
 No digoxin : prone to digitalis
induced arrhythmias and heart
block
 No calcium channel blockers :
predisposes to hypotension due to
amyloidosis
Restrictive
Cardiomyopathy :
Management


restrict sodium intake
Hemochromatosis CM
 repeated
phlebotomy to reduce
iron deposition in the heart


Sarcoidosis may respond to
corticosteroids
Eosinophilic CM
 corticosteroids
and cytotoxic drugs
Restrictive
Cardiomyopathy :
Management


there are no effective therapy for
other causes
questionable therapies :
 AV
sequential pacemaker
 antiarrhythmics
 surgical interventions
 mitral
valve replacement
 tricuspid valve replacement
 excision of thickened
endomyocardial plaque
Questions ?
Thank You !