Pediatric emergency medicine trisk 1737 1737
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TABLE 86.8
SUDDEN CARDIAC DEATH/SYNCOPE 5 S’S
Personal or family history of:
Syncope
Sudden cardiac death
Seizures (uncontrolled, unexplained)
“Swimming” (i.e., unexplained drowning)
Single car accidents (unexplained)
SCD is often is heralded by syncope. For detection of syncope that is more
likely to be high risk, obtain a thorough history of the patient’s and family’s prior
experience with similar episodes, syncope, heart disease, sudden death younger
than 50 years old, drowning, and/or single motor vehicle accidents. Some
syncope has been misdiagnosed as seizure. Carefully inquire about each incident
of syncope that the patient has experienced. Also, obtain an EKG for all patients
presenting with syncope ( Table 86.8 ). Restrict from exercise and refer to
cardiology if the episode was around the time of exertion. Admit to the hospital
on a monitored bed if patient received CPR.
HCM is a heterogeneous genetic disease involving 1,400 mutations in 11 or
more genes encoding proteins of the cardiac sarcomere. It is the most common
genetic heart disease with prevalence of 1:500. It manifests as increased LV wall
thickness without dilation of the LV cavity in the absence of any other
explanation (i.e., obstruction, hypertension, etc.). Hypertrophy develops over
time, and gains attention in the pediatric population because of its link to SCD in
people less than 35 years of age.
Death is due to arrhythmias, infarction, and heart failure. The onset of
symptomatic heart failure usually occurs between 20 and 40 years but can be at
any age. On examination, the EM provider should appreciate a LVOTO murmur
at the left sternal border or apex that is louder with standing or Valsalva maneuver
and softer when supine. EKG is almost always abnormal and may show LV
hypertrophy, ST and T wave changes, deep Q waves, or lack of R waves in the
lateral precordial leads. Echocardiogram is diagnostic. CXR is remarkable for
cardiomegaly and a globular shaped cardiac silhouette.
Patients with HCM can develop atrial fibrillation, SVT, and VT. These rhythms
are not well tolerated and even SVT can lead to cause SCD. Syncope, near
syncope, palpitations, exercise intolerance, and chest pain are all ominous signs in
