Pediatric emergency medicine trisk 1738 1738
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HCM. At times, the first symptom may be SCD. The typical murmur or family
history should be sought in all other patients presenting with these signs or
symptoms to assure that HCM is not overlooked. Cardiology should be consulted
when HCM is suspected or new symptoms are discovered.
Anomalous Coronary Arteries can also lead to SCD. The most common
variation is origin of the anomalous left coronary artery from the pulmonary
artery (ALCAPA). Syncope and sudden death may be the presenting symptoms.
Patients may be asymptomatic until exertion when flow to the coronaries is
impaired leading to ischemia, arrhythmia, or sudden death. Bland White Garland
Syndrome refers to presentation of ALCAPA in an infant around 10 weeks of age.
When the PVR drops significantly around this age, the anomalous LCA is not
perfused and ischemia causes the infant to cry with feeds, develop cardiac
wheezing, and respiratory distress. CHF and SCD may ensue. Some patients are
asymptomatic until later in childhood or adolescence. These older patients present
with chest pain, syncope, or SCD.
Diagnosing a child with ALCAPA requires a high index of suspicion. Clinical
assessment should include EKG and echocardiogram in a laboratory skilled in
imaging coronary arteries. Chest radiograph can be helpful. Treat with
supplemental oxygen, sedation, and analgesia for chest pain. Patients with
syncope concerning for cardiac etiology, should have consultation with a
cardiologist. Consult cardiology for definitive diagnosis.
About 0.1% of all patients with WPW (i.e., ventricular pre-excitation) will
experience SCD. The mechanism of death starts with atrial fibrillation, which
leads to VF if the atrial fibrillation is conducted rapidly via the accessory pathway
to the ventricle. Syncope in a patient with WPW is an indication for admission to
the hospital for electrophysiology study for risk assessment and/or catheter
ablation. Generally, the use of digoxin is avoided in these patients.
CPVT, long QT syndrome (LQTS), and Brugada syndrome are congenital ion
channelopathies that render the heart rhythm unstable. In pediatric patients with
any channelopathy, the first symptom may be sudden death.
Syncope is abrupt in onset with negligible prodrome and typically occurs
during or just after exercise or during acute auditory stimulation (alarm clock) (
Fig. 86.11 ).
The first presentation of SCD may be syncope or cardiac arrest. The EM
provider must be vigilant for clues which might suggest aborted SCD rather than
benign syncope. Early recognition of patients with cardiac syncope or aborted
SCD may save the patient, and prevent morbidity and mortality in close relatives
by commencing screening evaluations.
