1. Cardiomyopathy, Myocarditis and Pericardial Disease Dr. Pace September 22, 2005 Chapter 55

2. Cardiomyopathy 3 rd most common form of heart disease in U.S. 2 nd most common cause of adolescent sudden death(IHSS or HOCM) Directly affects cardiac structure and impairs myocardial function

3. Cardiomyopathy Four types Dilated Cardiomyopathy(DCM) Hypertrophied Cardiomopathy(HCM) Restrictive Cardiomyopathy Dysrhythmic right ventricular cardiomyopathy

4. Dilated Cardiomyopathy Dilation and compensatory hypertrophy of myocardium Depressed systolic function and pump failure with low cardiac output 80% of DCM cases are idiopathic African Americans and males have 2.5x increased risk Most common age of diagnosis 20-50yrs

5. Dilated Cardiomyopathy Clinical Presentation Signs and symptoms of CHF-dyspnea on exertion, orthopnea and pnd. Chest pain can occur due to low coronary vascular reserve Mural thrombi formation can occur Holosystolic regurgitant murmur or gallop may be present Dependent edema, bibasilar rales

6. Dilated Cardiomyopathy Diagnosis CXR- enlarged heart, biventricular enlargement, and pulmonary vascular congestion(cephalization) ECG- LVH, Left atrial enlargement, Q waves, poor R wave progression, afib Echo-Confirms Dx.-ventricular enlargement, increased systolic and diastolic volumes, decreased EF

7. Dilated Cardiomyopathy Differential Acute MI Restrictive Pericarditis Acute valvular disruption Sepsis Any other condition that results in low cardiac output state

8. Dilated Cardiomyopathy ED care and disposition Newly diagnosed or symptomatic DCM- admit IV lasix and digoxin-improve symptoms Ace inhibitors and B-Blockers-improve survival Amiodarone- for complex venticular ectopy Anticoagulation can be considered

9. Hypertrophic Cardiomyopathy Asymmetric LVH and/or RVH-primarily involves septum-usually without dilation Abnormal compliance-impaired diastolic relaxation and filling-output usually normal 50% are hereditary Prevelence 1 in 500, Mortality 1% Mortality 4-6% in childhood/adolescence

10. Hypertrophic Cardiomyopathy Clinical Features Symptom severity progresses with age Dyspnea on exertion-most common initial or presenting symptom Angina-like chest pain, palpitations and syncope may also be present

11. Hypertrophic Cardiomyopathy Exam Fourth heart sound(S4) Hyperdynamic apical pulse Precordial lift Systolic ejection murmur at apex or lower left sternal border Murmur increased with valsalva maneuver

12. Hypertrophic Cardiomyopathy Diagnosis EKG-LVH in 30%, Left atrial enlargement In 25-50% Large septal Q waves-25% CXR-usually normal Echo-study of choice-demonstrates disproportionate septal hypertrophy

13. Hypertrophic Cardiomyopathy ED Care and Disposition Pts who c/o exercise intolerance or CP with typical HCM murmur-needs echo- refer to cardiology B-blocker-treatment of choice for HCM with CP Discourage vigorous exercise(not a problem for most ed patients) Admit HCM with syncopal episode

14. Restrictive Cardiomyopathy One of least common cardiomyopathies Ventricular volume and wall thickness is normal Decreased volume of both ventricles Mostly idiopathic- sometimes familial Systemic disorders-amyloidosis, sarcoidosis, hemochromatosis, scleroderma, and carcinoid.

15. Restrictive Cardiomyopathy Clinical Features Symptoms of CHF-dyspnea, orthopnea, pedal edema- rare chest pain Exam-may have S3 or S4 gallop, rales, jvd, Kussmaul’s sign(jvd with inspiration), hepatomegaly, pedal edema or ascites

16. Restrictive Cardiomyopathy Diagnosis CXR-signs of CHF without cardiomegaly EKG-nonspecific changes most likely Conduction disturbances and low-voltage QRS complexes are common with amyloidosis or sarcoidosis

17. Restrictive Cardiomyopathy Differential Diagnosis Constrictive pericarditis Diastolic left ventricular dysfunction(due to ischemic or hypertensive heart disease) Need to differentiate restrictive cardiomyopathy from constrictive pericarditis(surgical treatment)

18. Restrictive Cardiomyopathy ED Care and Disposition Symptom directed-diuretics and ace inhibitors Corticosteroids for sarcoidosis Chelation therapy for hematochromatosis Admission based on severity of symptoms and availability of prompt follow up

19. Dysrhythmogenic Right Ventricular Cardiomyopathy(DRVC) Most rare form of cardiomyopathy Progressive replacement of RV myocardium with fibrofatty tissue Typical presentation of sudden death in young or middle aged pt Exam usually normal EKG- RBBB may be present Echo-necessary for diagnosis

20. Myocarditis Inflammation of myocardium Can be result of systemic disorder or infectious agent Viral-Coxsackie B, echovirus, influenza, parainfluenza, Epstein-Bar, and HIV Bacterial-C. Diptheria, N. meningitidis, M. pneumonia, and beta-hemolytic strep Frequently accompanied with pericarditis

21. Myocarditis Clinical Feature Fever, tachycardia out of proportion to fever, myalgias, headache,rigors Chest pain due to coexisting pericarditis Pericardial friction rub Severe cases may have CHF symptoms

22. Myocarditis Diagnosis and Differential EKG-nonspecific changes, av block, prolonged QRS suration, or ST elevation(with pericarditis) CXR-Normal Cardiac Enzymes- may be elevated Differentail-ischemia or infarct, valvular disease, and sepsis

23. Myocarditis ED Care and Disposition Supportive care Blood cultures Antibiotics for bacterial cause Watch for signs of progressive heart failure

24. Acute Pericarditis Loose visceral pericardium and dense parietal pericardium surround heart Pericardial space may contain up to 50ml normally Etiologies of acute pericarditis-viral, bacterial, fungal, malignancy, drugs, radiation, connective tissue disorder, uremia, myxedema, post-MI, or idiopathic

25. Acute Pericarditis Clinical Features Most common-sudden or gradual onset of sharp or stabbing pain with radiation to back, neck, L shoulder or arm Radiation to L trapezial ridge is distinguishing Pain more severe with lying supine and relieved with sitting Low grade fever, dyspnea and dysphagia Transient, intermittent friction rub

26. Acute Pericarditis Diagnosis EKG-changes in four stages 1-ST elevation in I, V5 and V6, PR depression in II, aVF and V4-V6 2-ST segment normalizes, T wave decreases 3-Inverted T waves in leads with previous ST elevation 4-Return to normal ECG In I, V5, or V6 ST:Twave ratio >0.25 most likely acute pericarditis

27. Acute Pericarditis Diagnosis and Differential Chest Xray-normal and can help r/o other disease Other tests of value-CBC, bun and cr, streptococcal serology, viral serologies, antinuclear/anti-DNA abs, thyroid function, ESR, Cardiac Enzymes

28. Acute Pericarditis ED Care and Disposition Idiopathic or presumed viral etiology- outpatient with NSAIDs for 1-3 weeks Treat any indentified specific causes Admit for myocarditis or hemodynamic instability

29. Nontraumatic Cardiac Tamponade Pressure in pericardial sac exceeds normal filling pressure in RV-restricts filling and cardiac output Etiology-metastatic malignancy, uremia, hemorrhage(over-anticoagulation), bacterial or tubercular disorders, chronic pericarditis, SLE, post radiation, myxedema

30. Nontraumatic Cardiac Tamponade Clinical Features Dyspnea and decreased exercise tolerance-wt loss, pedal edema, ascites Tachycardia, Narrow pulse pressure Pulsus paradoxus JVD, Muffled heart sounds, Hypotension

31. Nontraumatic Cardiac Tamponade Diagnosis and Differential EKG-low voltage QRS with ST elevation and PR depression possible Electrical Alternans-classic finding—P and R wave beat to beat variability CXR-+/- enlarged cardiac silhoutte ECHO-diagnostic modality of choice

32. Nontraumatic Cardiac Tamponade ED Care and Disposition IV Fluid Bolus-improves RV filling and improves hemodynamics Pericardiocentesis-therapeutic and diagnostic Admission to ICU or monitored setting

33. Constrictive Pericarditis Occurs when fibrous thickening and loss of elasticity interfere with diastolic filling Cardiac trauma, pericardiotomy, intrapericardial hemmorhage, fungal or bacterial pericarditis, uremic pericarditis are most common causes

34. Constrictive Pericarditis Clinical Features Sx’s gradually develop-mimics restrictive CM- CHF, DOE, and decreased exercise tolerance Chest pain, orthopnea and pnd are uncommon Exam-Pedal edema, hepatomegaly, ascites, jvd, and Kussmaul’s sign. Pericardial “knock”-early diastolic sound may be heard at apex

35. Constrictive Pericarditis Diagnosis EKG-not very helpful-may show low voltage QRS and inverted T waves CXR-pericardial calcifications seen in 50% on lateral view(not diagnostic) ECHO, CT, MRI are diagnostic

36. Constrictive Pericarditis Differential Diagnosis Consider acute pericarditis, myocarditis, exacerbation of chronic ventricular dysfunction, or systemic process resulting in decreased cardiac performance(sepsis)

37. Constrictive Pericarditis ED Care and Disposition Supportive care Symptomatic patients require admission and pericardiectomy

38. QUESTIONS Which is the 2 nd most common cause of adolescent sudden death? A. Suicide B. MVA C. Overdose D. Hypertrophic cardiomyopathy D

39. Which of the following is not useful in cadiac tamponade? a.IV fluids b.Pericardiocentesis c.Bedside ultrasound d.Diuretics e.Ekg d. d.

40. Which of the following is not found on the EKG of acute pericarditis A. ST elevation and PR depression B. Normal EKG C. Inverted Twave D. Electrical Alternans d

41. Which is not a sign or symptom of acute pericarditis? A. Sharp stabbing chest pain with radiation to back, neck or L shoulder B. Osler’s Nodes C. Friction Rub D. Pain worse with lying supine B, B,

42. b Which is not one of the 4 types of cardiomyopathy? A. Infectious B. Dilated C. Hypertrophied D. Restrictive E. Dysrhythmic Right Ventricular A

43. Head CT Look at bony structure-fx? Look at periphery for epidural Or Subdural bleeding Look at parenchyma Is there bleeding or infarct? Is there a mass Look at ventricles- Are they normal size? Are they clear