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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

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

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

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)

+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

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

+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

+Etiology

+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

+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

+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

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 findings

As in adults IE findings relate to 4 underlying phenomena:

1 Bacteremia

2 Valvulitis: changing auscultatory findings or development of congestive HF

3 Immunologic responses: Extracardiac manifestations (Roth spots, Janeway lesions, Osler nodes, splinter hemorrhages, splenomegaly) and Renal abnormalities: glomerulonephritis, infarct

4 Emboli: to abdominal viscera, brain, heart, extremities

+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 Findings

+Clinical findings

Immunologic manifestations

+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

+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%

+Diagnosis

+Diagnosis

+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

+Diagnosis

+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

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

+Treatment

+Treatment

+Treatment

+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

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

The AHA published in 2007 the new guidelines for IE prophylaxis

+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…

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

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