CASE CONFERENCE

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CASE CONFERENCE
Alexandra Duque, PGY2
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CASE
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14 y/o female
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Partial deletion on chromosome 1, developmental delay and
dilated cardiomyopathy (Last EF: 40%)
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CASE
CC
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Fever
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CASE
HPI and ROS
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5 days of fever up to 102F
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Associated with: malaise and poor PO intake
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Mild URI symptoms and headache the previous days
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2 weeks ago: N/V/D
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No UTI symptoms
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No abdominal pain, sore throat or any rashes
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No joint swelling
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CASE
PMH:
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1p chromosomal partial deletion with dilated cardiomyopathy
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Global developmental delay and Microcephaly
BH:
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Born FT, C-section delivery
PSH:
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None
FH:
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None contributory
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CASE
Meds:
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Carvedilol, Amiodarone, Enalapril, Furosemide, Enoxaparin,
Digoxin and Ranitidine
SH:
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Lives in Long Island with parents, no pets, no travel history,
no sick contacts at home
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CASE
ER and Hospital Course (OSH):
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VS on admission: T: 102.6 BP: 50/20 HR: 147
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Patient in shock, lethargic and dehydrated
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IVF given 1Lt NS with improvement of BP
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LABs: Showed increased troponin, lactate, BNP and BUN/Cr
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Admitted to the PICU, fluids continued, antibiotics started (Ceftriaxone /Vancomycin)
and BlCx positive for GPC in clusters
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TTE (Transthoracic Echo): + vegetation in papillary muscle, +large pericardial effusion
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DBP ~20’s, BUN and Creatinine increasing, U/O decreasing, with low StO2 not
maintained in supplemental O2
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Subsequently intubated, dobutamine and dopamine drip started and transferred to
CHONY
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CASE
Diagnosis
INFECTIVE
ENDOCARDITIS (IE)
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INFECTIVE ENDOCARDITIS
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Rare infection of the cardiac endothelium
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Pathogens become enmeshed in fibrin and platelets, forming
vegetations
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Associated with significant morbidity and mortality
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Its incidence, although rare, has been increasing in recent
years
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High complexity of intensive pediatric and neonatal care
units, has increase the incidence of catheter-related IE
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Epidemiology
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More frequent in adults than in children
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Accounts for 1 in 1280 pediatric admissions per year
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Between 1930 and 1972, 1:2000 to 1:5000 pediatric hospital
admissions were due to IE
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Between 1960 and 1980, 1:500 to 1:1000 hospitalizations were
due to IE
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The increased rate in children is most likely multifactorial
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90% cases are patients with heart disease, mainly congenital
heart disease (CHD)
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Epidemiology
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In developing countries rheumatic fever still the main cause
of IE
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Cyanotic heart diseases are most common associated with IE
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Corrective surgery with no residual defect eliminates the
attributable risk for IE in children with VSD, ASD and PDA 6
months after surgery
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IE has increased in neonates and is associated with high
mortality rate
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Its incidence has increased due to the use of more invasive
techniques to manage their medical problems
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Etiology
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Beyond the 1st year of life, streptococci viridans is the most
frequent isolated organism
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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
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S. aureus is the second most common cause but the most
common cause of acute IE
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MSSA, MRSA and coagulase negative staphylococci cause IE in
normal hearts and in the immediate postoperative period
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Enterococcal endocarditis is much less frequent than in adults
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Etiology
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Less frequently gram negative rods, known as the HACEK
group (Haemophilus aphrophilus, Actinobacillus actinomycetemcomitans,
Cardiobacterium hominis, Eikenella corrodens and Kingella kingae)
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Fungal pathogens, including Candida and Aspergillus spp.
are increasingly common in hospital-acquired endocarditis,
mainly neonates in intensive care units
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Pneumococcal IE is rare, but is associated with high mortality
rare
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Other rare causes: Coxiella Burnetti (Q fever), Brucella,
Legionella, Bartonella and Chlamydia
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Etiology
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Pathogenesis
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Intact cardiac endothelium is a poor stimulator of blood
coagulation & is weakly receptive to bacterial attachment
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CHD that involve high velocity jets of blood flow and/or
foreign material are associated with the highest risk of
development of IE
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Damaged endothelium is a potent inducer of
thrombogenesis
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At the site of damage, platelets, fibrin and occasionally RBCs
 Nonbacterial trombotic endocarditis (NBTE)
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Any episode of bacteremia that produces sufficient number
of bacteria can adhere to the NBTE
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Pathogenesis
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Bacteremia occurs in the postoperative setting, in
immunocompromised patients and in non-hospital settings
(after tooth brush, tattooing, body piercing, IV drug use)
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If adherence is produced, platelets and fibrin deposited over
the organisms  to enlargement of the vegetation
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Organisms trapped within the vegetation are protected from
the phagocytic cells and other immune defense mechanisms
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Disruption of the endocardium in neonates, occurs
commonly on the R side of the heart and is produced by
Catheter-induced trauma
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Pathogenesis
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Vegetations on valve leaflets  very destructive producing
valve regurgitation and heart failure (HF)
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Pieces of the vegetation can embolize and travel to the lungs,
kidneys or extremities
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Also bacteria can infiltrate deeper tissues of the heart
producing abscesses
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Clinical findings
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Always suspect it in any child with unexplained fever and
known to have heart disease
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Acute IE: fulminant, rapidly changing symptoms, high spiking
fevers, acutely ill
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Subacute IE: more indolent, with prolonged low grade fevers,
and a variety of somatic complaints
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Myalgia, arthralgia, rigors, diaphoresis, headache,
generalized malaise, weight loss, h/o anorexia, hematuria
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Almost all patients with IE have a heart murmur
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Clinical findings
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As in adults IE findings relate to 4 underlying phenomena:
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Bacteremia
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Valvulitis: changing auscultatory findings or development
of congestive HF
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Immunologic responses: Extracardiac manifestations (Roth
spots, Janeway lesions, Osler nodes, splinter hemorrhages,
splenomegaly) and Renal abnormalities:
glomerulonephritis, infarct
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Emboli: to abdominal viscera, brain, heart, extremities
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Clinical findings
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In neonates symptoms are nonspecific and variable
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Septic embolic phenomena are common
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Symptoms may resemble septicemia or CHF from other
causes
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Often can have feeding difficulties, respiratory distress and
tachycardia
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Can have new or changing murmur
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Clinical Findings
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Clinical findings
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Immunologic manifestations
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Diagnosis
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Duke criteria: Combines clinical, microbiological and
echocardiographic findings to determine likelihood of IE
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Its utility has been established in pediatrics
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Effective blood culture technique is key for successful diagnosis
using the Duke Criteria
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Is not necessary to obtain cultures at any particular phase of the
fever cycle
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Usually 3 BlCx are obtained by separate venipunctures on the
first day
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If there is no growth on the 2nd day of incubation, 2 more may be
obtained
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Diagnosis
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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
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In acute IE: 3 separate BlCx can be performed over a short period of
time with empirical antibiotics started
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Ask the lab to incubate the cultures for at least 2 weeks
Culture-negative IE:
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Clinical and/or echocardiographic evidence of IE but persistently
negative BlCx
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Cause by infection due to fastidious organisms that grow poorly in vitro
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Prevalence ~ 5-7%
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Diagnosis
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Diagnosis
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Diagnosis
Echocardiography (TTE)
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Better sensitivity than in adults, ~81%
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Main modality for detecting endocardial infection
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Can determine the site of infection, the extent of valvular damage,
cardiac function and can be used for monitoring
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Findings include vegetations, abscesses, new valvular insufficiency and
other acute changes in intracardiac flow patterns
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The absence of vegetations on echo does not rule out IE
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TEE: Considered for all patients with Ao valvular IE and changing Ao
root dimensions
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Diagnosis
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Diagnosis
Other miscellaneous tests:
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Anemia, hemolytic or a. of chronic disease
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Leukocytosis, not consistent feature of IE but immature forms
can be seen
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Hypergammaglobulinemia and acute-phase reactants are
elevated in a large proportion of patients
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Hematuria can occur and be associated with RBCs casts,
proteinuria and renal insufficiency
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Treatment
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Antibiotics empirically started to cover strep and staph
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Penicillin or Ampicillin (Vancomycin: if allergic to penicillins) plus
Gentamicin
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If HACEK group organisms isolated: 4 week-course of Ceftriaxone or
third generation cephalosporin alone, or ampicillin plus gentamicin
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If organism isolated, therapy based on sensitivities
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IV treatment preferred, to attain persistently high bactericidal
concentrations in a relatively avascular site
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Course of therapy usually 4-6 weeks, but infection to prosthetic valve
and cardiac tissue require longer therapy
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Fungal IE: Surgery + antifungal therapy
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Treatment
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Treatment
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Treatment
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Treatment
Main Indications for surgery:
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Microbiologic: Inability to sterilize blood > 7d, Fungal IE
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Vegetations: 1 or more serious embolic event within the first
2 weeks of treatment, anterior MV leaflet vegetation >10mm
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Valvular dysfunction: Cardiac failure unable to be managed
medically
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Intracardiac extension: Large abscess or extension, valve
dehiscence, fistula formation, new heart block
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Prognosis
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The course can be complicated by embolization of virtually
any organ
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The organs affected depend on which side of the heart is
involved
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Other complications: abscess formation, heart failure, heart
block and mycotic aneurisms
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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
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Prevention
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The AHA published in 2007 the new guidelines for IE
prophylaxis
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Prevention
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Dental procedures: Amoxicillin 30 to 60 minutes before the
procedure
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Antibiotic prophylaxis to prevent IE is no longer
recommended for GI and GU procedures
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BACK TO THE CASE…
PICU course at CHONY:
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Patient arrived intubated and sedated
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VS: T:97.5, HR: 98, cuff BP: 97/35 arterial BP: 81/43, CVP: 10
mmHg. On dopa/dobutamine/fentanyl drip
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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
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ECHO repeated: vegetation + abscess? In L atrium with
pericardial effusion and mild myocardial dysfunction
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CASE
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ID consulted: Vancomycin continued @1gr IV Q12hrs, Gentamicin/Rifampin added
with CTX d/c + inflammatory markers ordered
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OPTHO consulted: no Roth spots
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Abd/Renal U/S: + hepatomegaly, no thrombi or abscess noted
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BlCx (OSH): + MRSA
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Pericardial Fluid: + MRSA
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3 Consecutive BlCx at CHONY: + MRSA
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CT scan brain: + lucencies within the globus pallidus and putamen b/l R>L
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MRI brain: 1. L frontal and R cerebellar subacute infarcts with slight hemorrhage and
slight rim enhancement possibly septic: 2. R basal ganglionic infarcts
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AngioMRI: no evidence of mycotic aneurism
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CASE
OR Course:
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Findings: Thick yellow pericardium with fluid underneath, R
pericardium opened with tubular vegetation extending from
PV through LA impinging MV causing valve damage
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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
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Patient returned to PICU
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CASE
Course:
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Remained afebrile, with negative BlCx after the 5th day of
admission
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CRP and ESR slowly decreasing
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Patient still intubated but vassopressors d/c
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Transferred to OSH to continue IV antibiotics: 2 weeks of
Vancomycin + Gentamicin/Rifampin from the day of surgery
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Vancomycin 4 more weeks alone
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PREP QUESTIONS
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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
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A.
Administration of broad-spectrum antibiotics
B.
Blood cultures
C.
Renal ultrasonography
D.
Transesophageal echocardiography
E.
Urine culture
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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
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A.
Atrial septal defect transcatheter device closure with no
residual shunt
B.
Complete atrioventricular septal defect repair with
moderate mitral regurgitation
C.
Prosthetic aortic valve with no residual stenosis or
regurgitation
D.
Tetralogy of Fallot repair with mild pulmonary stenosis and
regurgitation
E.
Ventricular septal defect repair with aortic insufficiency