Caring For Patients With Cardiomyopathy

J.O. Medina,RN, MSN,FNP,CCRNEducation 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

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

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 elevation marker of poor

outcome

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

Inotropes contractility and SV acute setting : dopamine,

dobutamine, amrinone, epinephrine

digoxin Antiarrhythmias

treat symptomatic arrhythmias consider implanted defibrillator

Dilated (Congestive)Cardiomyopathy : Pharmacologic Management

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

second line for β-blockers for hospital patients diastolic filling time promotes relaxation contractility outflow gradient

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 : symptom relief primarily by

pulmonary / systemic congestion

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 !