CASE PRESENTATION

1. CASE PRESENTATION Patricia Baile – PL1 December 16, 2009

2. 16 year old male presents to the ER with chest pain

3. HPI • Chest pain x 6 days • Sharp, stabbing sensation on left side of chest • Constant • PS 7/10 • Worse when lying on left side and on inspiration • Radiating to back • Mild improvement with aspirin and acetaminophen

4. HPI • Chest pain • Associated with nausea, dizziness and blurring of vision 2 days prior • No diaphoresis • On day of admission, chest pain persistent, no improvement with acetaminophen hence BIB EMS to ER

5. ROS • Denies recent strenuous physical activity • No fever • No URI symptoms • No sick contacts • No joint pains • No dyspnea

6. Past Medical History • Asthma • 9 previous hospitalizations, last 1 year prior • No ICU, no intubation • Advair BID • Singulair 10 mg PO QD • Albuterol PRN

7. Family History • + Asthma • + DM • + Hypertension • + CAD • No sudden death • No CVA • No connective tissue disorders

8. Birth History • FT, NSVD, no complication • Immunization History • Up to date

9. Adolescent History • Currently in 9th grade (behind 2 years) • Lives with mother, stepfather and older brother • No sport or after-school activity • Denies drugs, alcohol, tobacco • Sexually active, has had 8 partners, uses condoms, no STD history (never tested)

10. Physical Examination • BP 102/58 HR 72 RR 18 O2 100%RA T 98.3 • Pain score: 6-7/10 • Wt: 76.9 kg (90%) Ht: 185 cm (90%) • GS: non-toxic appearing • HEENT: NCAT, congested turbinates, TMI, mild erythema OP, no CLAD • C/L: SCE, good air entry b/l, CTAB, no crackles, no rales, no tenderness on palpation of chest • CV: RRR, no m/r/g • Abdomen: soft, ND, NT, no organomegaly • Ext: well-perfused, good distal pulses

11. EKG

12. Labs • CBC: 6.9 > 13.7/39.1< 251 N 68 L 20 • BMP: 138/3.8/100/26/7/0.6/104/8.3 • LFT: 3.9/6.9/25/109/0.7/74 • Lipase: 12 • CPK: 1751 • CKMB: 168.8 • Troponin T: 8.77

13. Labs • Lipid profile • Cholesterol: 106 • TG: 49 • HDL: 31 • LDL: 65 • CRP: 7.5 • Urine Drug Screen: Negative

14. Labs • RSV/Flu: Negative • Rapid strep test: negative • Throat culture • ASO: 165 • Streptozyme: Positive • Respiratory viral panel: Negative

15. Ancillary Tests • Chest Radiograph • Normal chest

16. Ancillary Tests • Initial Echo • Normal LV ejection fraction • Normal RV function • Mild inferolateral akinesis • Inferior wall akinesis • Small pericardial effusion

17. Initial Impression • 16 year old male with Juvenile pattern pericarditis; Asthma exacerbation

18. Hospital Course • Admitted to PICU • Cardiac enymes gradually decreased • Repeat Echo done • Cardiac catheterization done

19. Ancillary Tests • Repeat Echo • Myocarditis with LV inferior/posterior wall motion abnormality: persistent abnormal LV wall motion • Suboptimal LV shortening fraction • Normal diastolic LV function • Trace inferior and posterior pericardial effusion

20. Ancillary Tests • Cardiac catheterization • Clear vessels • Decreased ejection fraction • Decreased motion of LV

21. FINAL DIAGNOSIS • 16 year old male with chest pain secondary to Myocarditis; Asthma exacerbation

22. MYOCARDITIS

23. Introduction • Clinical syndrome characterized by inflammation of myocytes resulting from infectious, toxic, and autoimmune etiologies. • Ongoing viral infection, myocardial destruction, and adverse remodeling can lead to persistent ventricular dysfunction and dilated cardiomyopathy.

24. Introduction • Infectious etiologies, particularly viral, are most common in children. • The most common causes of viral myocarditis are enterovirus (coxsackie group B) and adenovirus

25. Incidence • Incidence of myocarditis in children is unknown • Inflammatory infiltrates and myocardial cell damage were found at autopsy in 3 to 40% of infants and children who died suddenly unrelated to trauma • 17 percent of infants who died of SIDS had histopathologic evidence of myocarditis

26. Incidence • In one retrospective study from a single tertiary Canadian center, the estimated prevalence of myocarditis presenting to their emergency department was 0.5 cases per 10,000 visits • A review of all the autopsies performed at a single English pediatric tertiary center over a ten-year period (1996 to 2005) identified 28 of 1516 cases with myocarditis (1.8 percent)

27. Pathophysiology • In susceptible patients: • Viral RNA uptake  cytotoxic necrosis  rapid cell death • More common presentation • 4-14 days post-infection  immune response (macrophage activation and cytokine expression)  natural killer cells target myocardium expressing the viral RNA and continue myocyte necrosis

28. Pathophysiology • TNF is involved in rapidly clearing virus and signals additional proinflammatory cells, activates endothelial cells, and has direct negative inotropic effects • Cytotoxic T lymphocytes infiltrate myocytes and trigger lysis of these cells

29. Pathophysiology • In the chronic phases, the effects of either inadequate or inappropriately abundant immune response can lead to the long-term sequelae of dilated cardiomyopathy and heart failure

30. Pathophysiology • Ongoing study has demonstrated the presence of antimyosin autoantibodies and other immunomodulators long after initial viral infection

31. Clinical Manifestation • Nonspecific illness • Fatigue • Mild dyspnea • Myalgias • Fever (20%) • Chest pain (35%) • most commonly described as a pleuritic, sharp, stabbing precordial pain • may be substernal and squeezing

32. Clinical Manifestation • In a 6-year study of pediatric ED patients, the most common presenting symptom was dyspnea and more than half of patients were initially diagnosed with asthma or pneumonia. • May be asymptomatic

33. Clinical manifestation • Symptoms of heart failure • Dyspnea on exertion • Orthopnea • Shortness of breath • Palpitation

34. Physical Findings • Tachypnea and retractions • S3 and occasionally S4 gallops may be present and are important signs of impaired ventricular function • If the right or left ventricular dilation is severe, auscultation may reveal murmurs of functional mitral or tricuspid insufficiency

35. Physical Findings • Signs of low cardiac output • Pericardial friction rub and effusion may become evident in some patients with myopericarditis • A widely inflamed heart shows the classic signs of ventricular dysfunction including the following: • Jugular venous distention • Bibasilar crackles • Ascites • Peripheral edema

36. Causes • Infectious • Toxic • Immunologic

37. Infectious Causes • Viral myocarditis is the most common • Parvovirus B19, 36.6% • Enterovirus, 32.6% • Human herpesvirus 6 (HHV-6), 10.5% • Adenovirus, 8.1% • Co-infection with HHV-6 and parvovirus B19, 12.6% • HIV

38. Infectious Causes • Bacterial causes • Most common worldwide is Diphtheria • Streptococcal and staphylococcal species and Bartonella, Brucella, Leptospira, and Salmonella species can spread to the myocardium as a consequence of severe cases of endocarditis • Chagas disease • Parasitic myocarditis from trypanosomiasis

39. Toxic Causes • Numerous medications (eg, lithium, doxorubicin, cocaine, numerous catecholamines, acetaminophen) may exert a direct cytotoxic effect on the heart • Zidovudine (AZT) has been associated with myocarditis

40. Toxic causes • Environmental toxins include lead, arsenic, and carbon monoxide • Wasp and scorpion stings and spider bites, specifically black widows, may cause myocarditis • Radiation therapy

41. Immunologic Etiology • Connective tissue disorders • Systemic lupus erythematosus (SLE) • Rheumatoid arthritis • Scleroderma • Dermatomyositis • Idiopathic inflammatory and infiltrative disorders such as Kawasaki disease, sarcoidosis, and giant cell arteritis may be a cause

42. Differential Diagnoses • Acute Coronary Syndrome • Pneumonia • Congestive Heart Failure • Aortic Dissection • Pulmonary Embolism • Esophageal Perforation, Rupture and Tears • Viral syndrome

43. Diagnostic Studies • EKG • CXR • Cardiac enzymes • Echo • MRI with contrast • Cardiac catheterization • Other tests

44. EKG • Classical triad • Sinus tachycardia (>100 in a child; >120 in an infant; >150 in a neonate) • Low voltage complexes • ST segment and T wave changes • Other abnormalities like, varying AV blocks, bundle branch blocks, both supraventricular and ventricular arrhythmias and an even an anterior wall myocardial infarction pattern

46. Chest Radiograph • Typically include cardiomegaly, although heart size may be normal • Pulmonary vascular congestion is often present

47. Cardiac Enzymes • Elevation reflects myocardial necrosis • Seen in some patients with myocarditis • Experimental and clinical findings in adults suggest that elevations of cardiac troponin I or T (cTnI or cTnT) levels may be more common than CK-MB elevations in patients with biopsy-proven myocarditis

48. Echocardiography • Enlarged Left Ventricular (LV) dimensions, left atrial enlargement and impaired ejection fraction (EF) and shortening fraction • Normal EF in children is 64+4% • 2D echo reveals a large, hypo contractile LV which is globular, with thin walls, mild pericardial effusion and occasional regional wall motion abnormalities

49. MRI • Said to pick up earliest abnormality in Myocarditis • Document the location and extent of inflammation • Gadolinium enhancement was greater in patients with myocarditis than in normal controls

50. Cardiac Catheterization • Reveals depressed cardiac index, elevated left ventricular end diastolic pressure, and elevated mean atrial pressure • Angiography shows decreased left ventricular function with or without mitral regurgitation • Main purpose is to obtain samples by endomyocardial biopsy (EMB) for pathologic and microbiologic analysis