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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 ? 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