Myocardial Failure in a 2-Month Old
Radiology Cases in Pediatric Emergency Medicine
Volume 3, Case 1
James J. Matsuda, MD, PhD
Kapiolani Medical Center For Women And Children
University of Hawaii John A. Burns School of Medicine
This is a 2 1/2 month old male who presented to the
ED with a 2 week history of intermittent low grade
fevers. He was seen by his primary physician 2 weeks
prior to this ED visit for chapped/bleeding lips and
mucocutaneous lesions. There was no significant rash
or fever and it was thought that he may have developed
a reaction to amoxicillin-clavulinate. Five days prior to
the ED visit, he received his first DPT shot and
developed fever and fussiness which subsequently
resolved. The day of the ED visit, he was seen by his
pediatrician for increased fussiness, decreased oral
intake and fever. His exam in the office was significant
for a temperature of 38.3, mild desquamation of his
finger tips, and mild meatitis. Laboratory evaluation
revealed: WBC 25,000 with 52% segs and 10% bands,
Hgb 8.2, Hct 24.3, platelet count 576,000, and ESR 54.
The patient was subsequently sent to the hospital for a
The patient had an essentially unremarkable birth
history and past medical history. His family history is
significant for an older brother who died of
Stevens-Johnson syndrome 3 years ago.
The patient arrived in the ED late that evening and
was noted to be pale with cool extremities. His initial
oxygen saturation was 80%. Approximately 20 minutes
after arrival, the patient went into respiratory arrest, with
a HR of 35 bpm, and CPR was initiated. He was
immediately intubated and chest compressions were
started. Intravenous access initially failed; therefore, an
intraosseous needle was immediately placed.
Epinephrine was given via the ET tube one minute into
the code with no increase in HR. A second epinephrine
dose was given one minute later without response.
This was followed by atropine via the ET tube and an
intraosseous NS bolus. Five minutes into the
resuscitation, the HR was up to 120 and chest
compressions were discontinued. His initial arterial pH
was 6.68 with a PaO2 134, PaCO2 51, base deficit 31
on 100% O2. However, his HR again dropped into the
70's five minutes later and chest compressions were
reinitiated. A chest radiograph is obtained.
This CXR reveals slight accentuation of the central
pulmonary markings, with a normal heart size. The ET
tube is in good position. There is a nasogastric tube
and a vascular catheter in satisfactory position .
During the course of the resuscitation, the patient
eventually received 9 doses of epinephrine, 11 doses of
atropine, 400 cc of normal saline, 50 cc of albumin, 30
cc of D25W, 50 meq of NaHCO3, 200 meq of CaCl2,
and 50 cc of PRBC's. After initial stabilization in the
ED, the patient was admitted to the PICU approximately
two hrs after arrival.
Upon arrival to the PICU his systolic BP was 70, HR
151, with extremely diminished pulses and poor
perfusion. Shortly thereafter, he became bradycardic,
After 20 minutes of chest compressions and other
resuscitation measures which were unsuccessful, the
patient was pronounced dead.
The working diagnosis at the time of death was
cardiopulmonary failure secondary to overwhelming
sepsis. The postmortem examination revealed findings
consistent with Kawasaki disease. Gross evaluation of
the heart showed mild chamber dilatation, mitral and
aortic valvulitis, focal myocarditis, and right coronary
arteritis. Microscopic examination of the heart showed
inflammatory infiltrates of the right coronary artery
consistent with Kawasaki disease. There was no
evidence of coronary artery thrombosis. Examination of
the brain showed diffuse cerebral edema, and
cerebellar arachnoid hemorrhages most likely due to
the prolonged cerebral ischemia.
Kawasaki disease is an acute febrile multisystem
vasculitis affecting children. It is diagnosed by the
presence of fever (classically greater than 5 days
duration) and 4 of the 5 following features:
1. Conjunctival injection
2. Oral changes
erythema, fissuring, and crusting of the lips
diffuse oropharyngeal erythema
3. Peripheral extremity changes
hand and feet induration
erythema of palms and soles
desquamation of fingertips and toes, about 2 wks
transverse grooves across fingernails, 2-3 months
4. Erythematous rash
5. Lymphadenopathy of greater than 1.5 cm in
diameter (classically cervical, non-suppurative).
Also consistent with the diagnosis are increases in
the ESR, WBC and platelet count. The disease is
usually self-limited and benign; however, complication
rates can be reduced if treated early with IVIG
(intravenous gamma globulin) and aspirin. The most
important clinical association of Kawasaki disease is
cardiac disease (incidence roughly 30%), and early
studies report a 2% mortality rate secondary to sudden
cardiac death. Autopsy studies report acute thrombosis
of coronary arteries in 80% of fatal cases. Risk factors
most often associated with coronary involvement are:
age under 1 yr, anemia, WBC > 30,000, elevated ESR
and/or C-reactive protein, and fever for 14 days or
longer. The patient described above had 4 of these 5
The diagnosis of Kawasaki disease in the ED is
often difficult because of atypical presentations (i.e.,
less than 4 of the 5 criteria). Young infants in particular
often have milder or more subtle signs and symptoms.
The term "atypical Kawasaki disease" describes
patients who do not have 4 of the 5 criteria but who
have evidence of coronary artery involvement. This
patient fits the diagnosis of atypical Kawasaki disease,
since he did not have either a rash or
lymphadenopathy at time of presentation. Kawasaki
syndrome is often difficult to distinguish from serum
sickness, Stevens-Johnson syndrome or erythema
multiforme. Because there is a risk of sudden cardiac
death in Kawasaki disease, the ED physician must be
able to recognize the clinical features of the disease so
that pharmacological therapy (IVIG and ASA) can be
Since Kawasaki syndrome is often misdiagnosed as
serum sickness, Stevens-Johnson syndrome, erythema
multiforme, or viral exanthems, it is important to
consider the diagnosis of Kawasaki syndrome
whenever any of the above diagnoses are entertained.
Of interest in this case is the family history of a sibling
who died from Stevens-Johnson syndrome.
Primary cardiac causes of bradycardia are rare in
the pediatric population, and such cases represent a
formidable challenge for ED personnel. The steps
taken in the attempted resuscitation outlined above
were in accordance with that outlined in the Pediatric
Advanced Life Support (PALS) manual (at that time).
After repeated doses of epinephrine and atropine,
another therapeutic modality that may be considered is
transcutaneous or esophageal pacing. The extent of
this patient's cardiac lesions suggests that he probably
would not have benefited from electrical pacing.
However, for bradycardic patients with Kawasaki
disease and less severe (and reversible) cardiac
lesions, external pacing might be beneficial while
waiting for IVIG and ASA therapy to take effect.
Yamamoto LG, Martin JG. Kawasaki syndrome in
the ED. Am J Emerg. Med 1994;12:178-182.
Melish ME. Hicks RV. Kawasaki Syndrome: Clinical
features, pathophysiology, etiology, and therapy. J
Rheumatoloty (suppl 24) `1990;17:2-10.
Gersony WM. Diagnosis and management of
Kawasaki disease. JAMA 1991;256(20):2699-2703.
Levy M, Koren G. Atypical Kawasaki disease:
analysis of cinical presentation and diagnostic clues.
Pediatr Infect Dis J 1990;9:122-126.
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