1. 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

2. Objectives : Define cardiomyopathy.
Differentiate between dilated, restrictive , and hypertrophic cardiomyopathy with regard to etiology, pathophysiology, and management.

4. 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

5. 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

6. Cardiomyopathy : Major Consequences
Systolic or diastolic heart failure or combination of both
arrhythmias
other problems specific to type of disorder

7. 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

8. 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

9. 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

10. 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)

11. Dilated (Congestive) Cardiomyopathy : Pathophysiology
poor contractility :
 LVEDV   LVEDP  dilates annulus of AV valve  papillary dysfunction  valve incompetency  atrial enlargement  pulmonary congestion

12. Dilated (Congestive) Cardiomyopathy : Clinical Presentation
LVF
chronic fatigue ; weakness
orthopnea ; paroxysmal nocturnal dyspnea (PND)
cough ; chest pain
weight gain
palpitations
dizziness ; syncope
impotence
insomnia

13. 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

14. 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

15. Dilated (Congestive) Cardiomyopathy : Diagnosis
EKG
arrhythmias
ST (compensatory for  CO)
atrial; fibrillation (Af) : ominous sign (due to dilated atria)
atrial and ventricular arrhythmias (high grade ectopy portent to sudden death)
Q waves : pseudoinfarction due to fibrosis ; ST-T wave abnormalities
QRS widened : LVH, LBBB

16. Dilated (Congestive) Cardiomyopathy : Diagnosis
CXR
multichamber enlargement, pulmonary congestion, pleural effusions
Echocardiogram
LV dysfunction
chamber enlargement
valve dysfunction
hypokinesis and wall motion abnormalities
 EF

17. Dilated (Congestive) Cardiomyopathy : Diagnosis
Medical history with emphasis on :
Dyspnea on exertion, orthopnea, PND
Palpitations
Systemic and pulmonary embolism
Cardiac Troponin T
Persistent elevation marker of poor outcome

18. 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

19. Dilated (Congestive) Cardiomyopathy : Management
Goals
 cardiac workload
Limit activity
Improve symptoms
Treat underlying disease

20. 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

21. 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 (Apresoline) ; Isordil
second line oral combination
if unable to tolerate ACE inhibitor

22. Dilated (Congestive) Cardiomyopathy : Pharmacologic Management
Inotropes
 contractility and SV
acute setting : dopamine, dobutamine, amrinone, epinephrine
digoxin
Antiarrhythmias
treat symptomatic arrhythmias
consider implanted defibrillator

23. Dilated (Congestive) Cardiomyopathy : Pharmacologic Management
Low dose ß blockers
Controversial
Atenolol
Metoprolol
Carvedilol
Anticoagulation for patients :
In atrial fibrillation
Moderate or severe failure

24. 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

25. 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

26. 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

27. Hypertrophic Cardiomyopathy (HCM)
formally referred to as
idiopathic hypertrophic subaortic stenosis (IHSS)
hypertrophic obstructive cardiomyopathy (HOCM)

28. 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

29. HCM : Causes 1/3 familial 2/3 unknown
sporadic occurrence
Autosomal dominant trait causing encoding of cardiac sarcomere

30. 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

31. 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 !

32. 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

33. 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

34. HCM : Diagnosis
EKG
 voltage of LV hypertrophy
ST-T wave abnormalities
Q waves in inferior/lateral leads due to septal hypertrophy
PVC : 75%
SVT : %
atrial fib : %
CXR : normal or enlarged heart, atrial enlargement, pulmonary congestion

35. HCM : Diagnosis Echocardiogram : Cardiac Catheterization :
septal hypertrophy
LA enlargement
narrow outflow tract
wall motion abnormalities
MV leaflet abnormality
Cardiac Catheterization :
 chamber pressures MR
altered LV outflow gradient

36. HCM : Management Goals :  ventricular filling by slowing HR
 contractility by reducing obstruction

37. 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

38. HCM : Management
Avoid : digitalis, diuretics, nitrates and vasodilators
Arrhythmia control
Disopyramide ( Norpace )has negative inotropic properties
Amiodarone for atrial and ventricular arrhythmias

39. 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

40. HCM : Pharmacologic Support
Calcium Channel Blockers :
Verapamil :  LV obstruction
second line for β-blockers
for hospital patients
 diastolic filling time
promotes relaxation
 contractility
 outflow gradient

41. 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

43. 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

44. 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

45. Restrictive Cardiomyopathy : Pathophysiology
stiff ventricles   ventricular filling   CO  biatrial dilation  pulmonary and systemic congestion

46. 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

47. Restrictive Cardiomyopathy : Physical Signs
right sided failure :
JVD
ascitis
hepatic enlargement
edema

48. Restrictive Cardiomyopathy : Physical Signs
left sided failure :
pulmonary congestion
 BP
narrowed pulse pressure
weak, tired
DOE

49. 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

50. 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

51. 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.

52. Restrictive Cardiomyopathy : Management
Goal :
symptom relief primarily by  pulmonary / systemic congestion

53. 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

54. 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

55. 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

56. Questions ?
Thank You !