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  1. CASE CONFERENCE Alexandra Duque, PGY2

  2. CASE • 14 y/o female • Partial deletion on chromosome 1, developmental delay and dilated cardiomyopathy (Last EF: 40%)

  3. CASE CC • Fever

  4. CASE HPI and ROS • 5 days of fever up to 102F • Associated with: malaise and poor PO intake • Mild URI symptoms and headache the previous days • 2 weeks ago: N/V/D • No UTI symptoms • No abdominal pain, sore throat or any rashes • No joint swelling

  5. CASE PMH: • 1p chromosomal partial deletion with dilated cardiomyopathy • Global developmental delay and Microcephaly BH: • Born FT, C-section delivery PSH: • None FH: • None contributory

  6. CASE Meds: • Carvedilol, Amiodarone, Enalapril, Furosemide, Enoxaparin, Digoxin and Ranitidine SH: • Lives in Long Island with parents, no pets, no travel history, no sick contacts at home

  7. CASE ER and Hospital Course (OSH): • VS on admission: T: 102.6 BP: 50/20 HR: 147 • Patient in shock, lethargic and dehydrated • IVF given 1Lt NS with improvement of BP • LABs: Showed increased troponin, lactate, BNP and BUN/Cr • Admitted to the PICU, fluids continued, antibiotics started (Ceftriaxone /Vancomycin) and BlCx positive for GPC in clusters • TTE (Transthoracic Echo): + vegetation in papillary muscle, +large pericardial effusion • DBP ~20’s, BUN and Creatinine increasing, U/O decreasing, with low StO2 not maintained in supplemental O2 • Subsequently intubated, dobutamine and dopamine drip started and transferred to CHONY


  9. INFECTIVE ENDOCARDITIS • Rare infection of the cardiac endothelium • Pathogens become enmeshed in fibrin and platelets, forming vegetations • Associated with significant morbidity and mortality • Its incidence, although rare, has been increasing in recent years • High complexity of intensive pediatric and neonatal care units, has increase the incidence of catheter-related IE

  10. Epidemiology • More frequent in adults than in children • Accounts for 1 in 1280 pediatric admissions per year • Between 1930 and 1972, 1:2000 to 1:5000 pediatric hospital admissions were due to IE • Between 1960 and 1980, 1:500 to 1:1000 hospitalizations were due to IE • The increased rate in children is most likely multifactorial • 90% cases are patients with heart disease, mainly congenital heart disease (CHD)

  11. Epidemiology • In developing countries rheumatic fever still the main cause of IE • Cyanotic heart diseases are most common associated with IE • Corrective surgery with no residual defect eliminates the attributable risk for IE in children with VSD, ASD and PDA 6 months after surgery • IE has increased in neonates and is associated with high mortality rate • Its incidence has increased due to the use of more invasive techniques to manage their medical problems

  12. Etiology • Beyond the 1st year of life, streptococci viridans is the most frequent isolated organism • S. viridans and other streptococci (S. sanguis, S. mitis, S. salivarius, S.mutans and S. oralis) are generally associated with rheumatic fever, unrepaired CHD and late postoperative IE • S. aureus is the second most common cause but the most common cause of acute IE • MSSA, MRSA and coagulase negative staphylococci cause IE in normal hearts and in the immediate postoperative period • Enterococcal endocarditis is much less frequent than in adults

  13. Etiology • Less frequently gram negative rods, known as the HACEK group (Haemophilus aphrophilus, Actinobacillus actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens and Kingella kingae) • Fungal pathogens, including Candida and Aspergillus spp. are increasingly common in hospital-acquired endocarditis, mainly neonates in intensive care units • Pneumococcal IE is rare, but is associated with high mortality rare • Other rare causes: Coxiella Burnetti (Q fever), Brucella, Legionella, Bartonella and Chlamydia

  14. Etiology

  15. Pathogenesis • Intact cardiac endothelium is a poor stimulator of blood coagulation & is weakly receptive to bacterial attachment • CHD that involve high velocity jets of blood flow and/or foreign material are associated with the highest risk of development of IE • Damaged endothelium is a potent inducer of thrombogenesis • At the site of damage, platelets, fibrin and occasionally RBCs  Nonbacterial trombotic endocarditis (NBTE) • Any episode of bacteremia that produces sufficient number of bacteria can adhere to the NBTE

  16. Pathogenesis • Bacteremia occurs in the postoperative setting, in immunocompromised patients and in non-hospital settings (after tooth brush, tattooing, body piercing, IV drug use) • If adherence is produced, platelets and fibrin deposited over the organisms  to enlargement of the vegetation • Organisms trapped within the vegetation are protected from the phagocytic cells and other immune defense mechanisms • Disruption of the endocardium in neonates, occurs commonly on the R side of the heart and is produced by Catheter-induced trauma

  17. Pathogenesis • Vegetations on valve leaflets  very destructive producing valve regurgitation and heart failure (HF) • Pieces of the vegetation can embolize and travel to the lungs, kidneys or extremities • Also bacteria can infiltrate deeper tissues of the heart producing abscesses

  18. Clinical findings • Always suspect it in any child with unexplained fever and known to have heart disease • Acute IE: fulminant, rapidly changing symptoms, high spiking fevers, acutely ill • Subacute IE: more indolent, with prolonged low grade fevers, and a variety of somatic complaints • Myalgia, arthralgia, rigors, diaphoresis, headache, generalized malaise, weight loss, h/o anorexia, hematuria • Almost all patients with IE have a heart murmur

  19. Clinical findings • As in adults IE findings relate to 4 underlying phenomena: • Bacteremia • Valvulitis: changing auscultatory findings or development of congestive HF • Immunologic responses: Extracardiac manifestations (Roth spots, Janeway lesions, Osler nodes, splinter hemorrhages, splenomegaly) and Renal abnormalities: glomerulonephritis, infarct • Emboli: to abdominal viscera, brain, heart, extremities

  20. Clinical findings • In neonates symptoms are nonspecific and variable • Septic embolic phenomena are common • Symptoms may resemble septicemia or CHF from other causes • Often can have feeding difficulties, respiratory distress and tachycardia • Can have new or changing murmur

  21. Clinical Findings

  22. Clinical findings • Immunologic manifestations

  23. Diagnosis • Duke criteria: Combines clinical, microbiological and echocardiographic findings to determine likelihood of IE • Its utility has been established in pediatrics • Effective blood culture technique is key for successful diagnosis using the Duke Criteria • Is not necessary to obtain cultures at any particular phase of the fever cycle • Usually 3 BlCx are obtained by separate venipunctures on the first day • If there is no growth on the 2nd day of incubation, 2 more may be obtained

  24. Diagnosis • In not acutely ill patients, with persistent negative cultures and high suspicion for IE  Antibiotics can be withheld for 48hrs while additional BlCx are obtained • In acute IE: 3 separate BlCx can be performed over a short period of time with empirical antibiotics started • Ask the lab to incubate the cultures for at least 2 weeks Culture-negative IE: • Clinical and/or echocardiographic evidence of IE but persistently negative BlCx • Cause by infection due to fastidious organisms that grow poorly in vitro • Prevalence ~ 5-7%

  25. Diagnosis

  26. Diagnosis

  27. Diagnosis Echocardiography (TTE) • Better sensitivity than in adults, ~81% • Main modality for detecting endocardial infection • Can determine the site of infection, the extent of valvular damage, cardiac function and can be used for monitoring • Findings include vegetations, abscesses, new valvular insufficiency and other acute changes in intracardiac flow patterns • The absence of vegetations on echo does not rule out IE • TEE: Considered for all patients with Ao valvular IE and changing Ao root dimensions

  28. Diagnosis

  29. Diagnosis Other miscellaneous tests: • Anemia, hemolytic or a. of chronic disease • Leukocytosis, not consistent feature of IE but immature forms can be seen • Hypergammaglobulinemia and acute-phase reactants are elevated in a large proportion of patients • Hematuria can occur and be associated with RBCs casts, proteinuria and renal insufficiency

  30. Treatment • Antibiotics empirically started to cover strep and staph • Penicillin or Ampicillin (Vancomycin: if allergic to penicillins) plus Gentamicin • If HACEK group organisms isolated: 4 week-course of Ceftriaxone or third generation cephalosporin alone, or ampicillin plus gentamicin • If organism isolated, therapy based on sensitivities • IV treatment preferred, to attain persistently high bactericidal concentrations in a relatively avascular site • Course of therapy usually 4-6 weeks, but infection to prosthetic valve and cardiac tissue require longer therapy • Fungal IE: Surgery + antifungal therapy

  31. Treatment

  32. Treatment

  33. Treatment

  34. Treatment Main Indications for surgery: • Microbiologic: Inability to sterilize blood > 7d, Fungal IE • Vegetations: 1 or more serious embolic event within the first 2 weeks of treatment, anterior MV leaflet vegetation >10mm • Valvular dysfunction: Cardiac failure unable to be managed medically • Intracardiac extension: Large abscess or extension, valve dehiscence, fistula formation, new heart block

  35. Prognosis • The course can be complicated by embolization of virtually any organ • The organs affected depend on which side of the heart is involved • Other complications: abscess formation, heart failure, heart block and mycotic aneurisms • Increase risk for complications: prosthetic valves, L sided IE, S. aureus or fungal IE, symptoms > 3months, cyanotic heart disease, poor clinical response to antibiotics and systemic artery-to-pulmonary shunts

  36. Prevention • The AHA published in 2007 the new guidelines for IE prophylaxis

  37. Prevention • Dental procedures: Amoxicillin 30 to 60 minutes before the procedure • Antibiotic prophylaxis to prevent IE is no longer recommended for GI and GU procedures

  38. BACK TO THE CASE… PICU course at CHONY: • Patient arrived intubated and sedated • VS: T:97.5, HR: 98, cuff BP: 97/35 arterial BP: 81/43, CVP: 10 mmHg. On dopa/dobutamine/fentanyl drip • PE: Tachycardic, S1/S2 normal, GII/VI holosystolic murmur, no galops or clicks, lungs CTA b/l, Abdomen soft, extremities well perfused with IV-line and A-line in place and pale skin • ECHO repeated: vegetation + abscess? In L atrium with pericardial effusion and mild myocardial dysfunction

  39. CASE • ID consulted: Vancomycin continued @1gr IV Q12hrs, Gentamicin/Rifampin added with CTX d/c + inflammatory markers ordered • OPTHO consulted: no Roth spots • Abd/Renal U/S: + hepatomegaly, no thrombi or abscess noted • BlCx (OSH): + MRSA • Pericardial Fluid: + MRSA • 3 Consecutive BlCx at CHONY: + MRSA • CT scan brain: + lucencies within the globus pallidus and putamen b/l R>L • MRI brain: 1. L frontal and R cerebellar subacute infarcts with slight hemorrhage and slight rim enhancement possibly septic: 2. R basal ganglionic infarcts • AngioMRI: no evidence of mycotic aneurism

  40. CASE OR Course: • Findings: Thick yellow pericardium with fluid underneath, R pericardium opened with tubular vegetation extending from PV through LA impinging MV causing valve damage • Procedure: Vegetation removed, MV repaired, #2 mediastinal tubes + R pleural CT placed. Bypass time 47 min, x-C 0.26, T: 34C coming off-pump, no complications, bleeding 25 cc, FFP given • Patient returned to PICU

  41. CASE Course: • Remained afebrile, with negative BlCx after the 5th day of admission • CRP and ESR slowly decreasing • Patient still intubated but vassopressors d/c • Transferred to OSH to continue IV antibiotics: 2 weeks of Vancomycin + Gentamicin/Rifampin from the day of surgery • Vancomycin 4 more weeks alone

  42. PREP QUESTIONS • You are evaluating a 15-year-old boy in the emergency department who presents with fever, chills, malaise, and blood in his urine. On physical examination, he appears comfortable and alert and has a temperature of 102.7°F (39.3°C), a blood pressure of 110/40 mm Hg, no rashes, and clear breath sounds. He has a diastolic murmur heard best in the sitting position (Item Q133). You elicit no abdominal or flank tenderness.Of the following, the BEST next step in the management of this patient is

  43. Administration of broad-spectrum antibiotics • Blood cultures • Renal ultrasonography • Transesophageal echocardiography • Urine culture

  44. The dentist in your community health center's clinic calls you with a question about a patient that he is seeing later that day. The child is 14 years old and underwent surgical repair of his congenital heart disease 5 years ago. The dentist wants to know if this patient's cardiac condition warrants antibiotic prophylaxis for a routine dental cleaning.Of the following, the condition for which antibiotic prophylaxis is MOST appropriate when the patient is at risk for bacteremia is

  45. Atrial septal defect transcatheter device closure with no residual shunt • Complete atrioventricular septal defect repair with moderate mitral regurgitation • Prosthetic aortic valve with no residual stenosis or regurgitation • Tetralogy of Fallot repair with mild pulmonary stenosis and regurgitation • Ventricular septal defect repair with aortic insufficiency