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CASE CONFERENCE. Alexandra Duque, PGY2. CASE. 14 y/o female Partial deletion on chromosome 1, developmental delay and dilated cardiomyopathy (Last EF: 40%) . CASE. CC Fever. CASE. HPI and ROS 5 days of fever up to 102F Associated with: malaise and poor PO intake

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Case conference

CASE CONFERENCE

Alexandra Duque, PGY2


CASE

  • 14 y/o female

  • Partial deletion on chromosome 1, developmental delay and dilated cardiomyopathy (Last EF: 40%)


CASE

CC

  • Fever


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


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


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


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


CASE

Diagnosis

INFECTIVE

ENDOCARDITIS (IE)


Infective endocarditis
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


Epidemiology
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)


Epidemiology1
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


Etiology
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


Etiology1
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



Pathogenesis
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


Pathogenesis1
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


Pathogenesis2
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


Clinical findings
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


Clinical findings1
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


Clinical findings2
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



Clinical findings4
Clinical findings

  • Immunologic manifestations


Diagnosis
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


Diagnosis1
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%




Diagnosis4
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



Diagnosis6
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


Treatment
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





Treatment4
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


Prognosis
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


Prevention
Prevention

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


Prevention1
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


Back to the case
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


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


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


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


Prep questions
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



  • 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


  • 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


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