Sepsis, stroke or heart disease ?

1. Sepsis, stroke or heart disease? Melissa B. Jones MSN, APRN, CPNP-AC Nurse Practitioner Children’s National Medical Center Washington, DC

2. Case #1: RS • 1 year old with 2 day h/o fever, presents to OSH with tachypnea and wheezing • Intubated for respiratory failure prior to transport • Presentation to PICU • HR 197 • BP: 62/25 (29) • Intubated • Sedated and paralyzed • Dopa 10 • Epi 0.3

3. Case #2: JC 6 year old male presents from OSH with excruciating abdominal pain slurred speech left-sided facial drooping. Presentation Sleepy but arousable, no acute distress Intermittent abdominal pain x 2 weeks, with occasional vomiting Speech is slurred and left facial drooping noted Unlabored breathing No murmur, rubs or clicks

4. Case # 3: JD 10year old male with h/o asthma presents with 3 day h/o fever and headache. Presentation RR 30’s Wheezing 2L O2 by Nasal cannula Admitted to floor with Q2hour albuterol nebs Transferred to PICU for worsening respiratory symptoms despite albuterol and O2

5. So…. Sepsis Stroke Heart disease

6. Myocarditis: • Patchy inflammation of the myocardium caused by immune mediated response to: • Viral infection • Bacterial infection • Autoimmune disorders • Toxins

7. What we are talking about…. Infectious Etiology Toxicities Autoimmune Disorders Myocarditis Genetics/ Metabolic Disorders Arrhythmias Death Recovery Cardiomyopathy • Recovery • Transplant • Death

8. Challenges with diagnosis Cardiomyopathy Myocarditis

9. What we will cover: Presentation of myocarditis Management and therapeutic goals Outcomes

10. (PedEmerg Care, 2013)

11. (Circulation, 2012)

12. Time Course JACC, 2012

13. Myocarditis: Diagnostic clues and tools • Diagnostic clues • Tachycardia out of proportion to fever • Hepatomegaly • Pulmonary edema • Cardiomegaly • GI symptoms • Exposure to or h/o recent illness • +/- fever • Diagnostic Tools • PHYSICAL EXAM • Detailed history • Biomarkers • CXR • EKG • Echocardiogram • MRI • EndomyocardialBiopsy

14. Diagnosis • Inflammatory Markers • CRP • Sed Rate • Leukocytes • PCR: limited because of a potential delay between infectious process and presentation • EKG: non-specific T wave and ST segment changes, low voltage; conduction delays • Echocardioography • chamber size, wall thickness, systolic/diastolic function. • Rule out structural heart disease, valvar disease or coarctation Physical Exam Tachycardia out of proportion to fever Failure to thrive Hepatomegaly Gallop Tachypnea CXR Cardiomegaly Pulmonary edema History h/o recent illness h/o GI symptoms Poor PO intake Urine output Family history Biomarkers Troponin I Creatine kinase (CK) Brain Natriuretic Peptide

15. Endomyocardial Biopsy Dallas Criteria (1987) for diagnosis of Myocarditis Biopsy confirms: • myocyte necrosis AND • adjacent inflammatory infiltrate • +/- fibrosis Draw Back Patchy nature of disease - sampling error False negatives

16. MRI Evaluation for presence of acute myocardial inflammation/edema Early gadolinium enhancement  hyperemia and capillary leak Late gadolinium enhancement  myocardial fibrosis and irreversible disease Drawbacks • Artifact • Decrease sensitivity • Standardization needed (JAAC, 2009)

17. Myocarditis: Management and therapeutic goals

18. Opie, Drugs of the heart Compensatory Response Augment CO and preserve end organ perfusion • Long term consequences: • Increase preload and afterload • Increase myocardial O2 consumption • Myocardial hypertrophy and fibrosis • Coronary insufficiency risk of ischemia • Chronic elevation of catecholamine concentrationdownregulation of beta receptors

19. No Low perfusion at rest Yes Shock and Congestion Congestion at Rest Yes No Warm & Dry Normal CI Normal PCWP Warm & Wet PCWP CI normal Vasodilators & diuretics Cold & Dry CI PCWP or normal Cold & Wet PCWP CI Inotropes

20. Management Goals Immediate • Restore end organ perfusion and oxygen delivery Acute • Minimize end-organ damage including the heart • Reduce hospitalization stays Chronic • Long term survival • Prevent re-hospitalization • Prevent disease progression

21. Standard Heart Failure Treatment: Supportive Care Acute Phase/Hemodynamic Compromise Inotropic support Afterload reduction Diuretics Positive pressure ventilation Mechanical circulatory support Chronic Heart Failure Management ACE Inhibitors Beta-blockers Aldosterone antagonists Cardiac glycosides Diuretics Pacer/AICD

22. Results: IVIG group • Improved recovery of LV function at 3-6 months, 6-12 months and 1 year • More likely to achieve normal function after 1styear Circulation, 1994

23. Not enough evidence to support the use of IVIG on adults, no pediatric trials 2010

24. Myocarditis: Outcomes

25. (Circulation, 2012)

26. 37 deaths (7.2%) 21 transplants (4.1%) Myocardial recovery in 59.6% who received VAD or ECMO. Circulation, 2012

27. Biopsy-confirmed or probable myocarditis had similar proportions of death, transplantation, and echocardiographic normalization 3 years after presentation. • Cardiomyopathy with a myocarditis diagnosis has better outcomes than those in whom no cause can be found, regardless of endomyocardial biopsy findings. • Impaired LV ejection at presentation without LV dilation and with greater septal wall thickness is associated with normalization of echo findings.

28. 1-5 years infants neonates >5years Circulation, 2013

29. Case #1: RS • 12mo 2 day h/o fever, lethargy presents to OSH with tachypnea and wheezing • Intubated for respiratory failure prior to transport • Presentation to PICU • HR 197 • BP: 62/25 (29) • Intubated • Sedated and paralyzed • Dopa 10 • Epi 0.3

30. Case #1: RS Work up Echo: Severely decreased LV function, moderately decreased RV function Cath: normal coronaries, no biopsy obtained on ECMO Respiratory viral culture + adenovirus, all other cultures negative Follow Up 21 day ECMO course Required tracheostomy for failure to wean from ventilator Recovery of ventricular function, EF 65% at discharge

31. Case #2: JC 6 year old male presents from OSH with excruciating abdominal pain, slurred speech, left-sided facial drooping. asthma, recent exacerbation 2 weeks prior to admission Presentation Sleepy but arousable, no acute distress Intermittent abdominal pain x 2 weeks, with occasional vomiting Speech is slurred and left facial drooping noted, no syncope Unlabored breathing No murmur, rubs or clicks

32. Case # 2: JC Work Up Head CT: Left MCA infarct Echo: Severely decreased LV function. Moderately dilated LV, biatrial enlargement. Moderate MR and TR. MRI: myocarditis vs. fibrosis BNP: 9821 (nl<1100) Ejection Fraction: 31% Follow Up Milrinone x6 days, transitioned to Enalapril and started on Digoxin, Carvedilol, Aldactone and Coumadin CICU x 7 days Cardiology floor x 5 days, then discharged to rehab facility 2 readmissions within first year Second admission presented in decompensated shock Listed for heart transplant

33. Case # 3: JD 10yr old male with h/o asthma presents with 3 day h/o fever and headache. Diagnosed 2 days prior to admission with OM. Presentation RR 30’s Wheezing 2L O2 by Nasal cannula Admitted to floor with Q2hour albuterol nebs Transferred to PICU for worsening respiratory symptoms despite albuterol and O2

34. Case # 3: JD Work Up CRP 9 Creatinine 0.7 Cultures negative Echo: mod-severe LV dysfunction MRI: No late gadolinium enhancement Follow Up BiPap x 3 days, then HFNC Milrinone Lasix Transfer to cardiology floor after 1 week

35. Summary Myocarditis has a wide range of clinical presentations Clinical exam and detailed history are key to informing the diagnosis. A careful assessment of the risks and benefits of diagnostic procedures is needed, given the imperfect diagnostic tools available. Because of the various etiologies and presentations, there is no “magic bullet” for treatment—supportive care is the standard. Myocarditis has better outcomes than cardiomyopathy of unknown etiology, however it continues to have a significant risk of morbidity and mortality.

36. “Cardiac inflammation is difficult to diagnose, and even if it is diagnosed, can we treat it effectively?” Jean Baptiste Sénang, Physician to Louis XV 1773