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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.

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Caring For Patients With Cardiomyopathy

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  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.

  3. 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

  4. 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

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

  6. 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

  7. 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

  8. 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

  9. 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)

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

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

  12. 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

  13. 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

  14. 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

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

  16. 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

  17. 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

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

  19. 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

  20. 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

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

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

  23. 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

  24. 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

  25. 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

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

  27. 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

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

  29. 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

  30. 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 !

  31. 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

  32. 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

  33. 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

  34. 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

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

  36. 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

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

  38. 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

  39. 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

  40. 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

  41. 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

  42. 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

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

  44. 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

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

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

  47. 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

  48. 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

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