Posted by Catherine Brinkley Catherine Brinkley Saving American Hearts December 11, 2022 on Dec 12th 2022
How well do you know your PALS? Scenario for the week of December 12, 2022
You are working in the Pediatric ICU and you hear a nurse down the hall yell "I need a doctor in here, I need some help!"
Your initial impression as you enter the room and see the nurse performing CPR on a child. The child's lips are blue, face is pale and the child appears to be unresponsive. You connect the monitor and tell the nurse to stop CPR for a rhythm check. Many more nurses and a doctor comes to help. The monitor shows ventricular fibrillation.
The child is 8 years old with an approximate weight of 26kg.
You charge the defibrillator to 50 joules (because 2J/kg is 52 but you can only give 50 or 60 joules.)
You tell everyone to clear the patient and deliver a shock and resume CPR. Staff are directed to get in place to change compressors at the next rhythm check. Two providers give the child CPR with 15 compressions to 2 breaths, The doctor orders IV access and all attempts have failed. An IO is placed successfully, and 2 minutes of CPR have passed. The doctor calls rhythm check, staff change compressors, check for a pulse, there is no pulse and V-Fib remains on the monitor. You charge the defibrillator to 100 joules (4J/kg would be 104 joules). Everyone clears the patient and the shock is delivered, CPR is resumed. The doctor orders Epinephrine. The child is 26kg so Epinephrine 0.26mg is administered. The doctor asks for a S-A-M-P-L-E history and tries to determine the cause of the v-fib using the H's and T's. The child was admitted the day before yesterday after having symptoms of dizziness and fainting along with poor weight gain. Tests revealed a diagnosis of cardiomyopathy. The child ate 80% of breakfast and appeared to be doing much better, just watching TV when the nurse left the room about 30 minutes prior. The child's nurse was making rounds and noticed the child was pale and the lips were blue. The nurse rushed to the bedside, and began tapping and shouting with no response. The nurse checked a pulse, did not feel one, called for help and immediately began CPR. Two more minutes of CPR have passed and staff stop CPR, change compressors and check a pulse. There is no pulse and V-Fib remains on the monitor. The defibrillator is charged to 150 joules (6J/kg is 156 joules). The patient is cleared, the shock delivered and CPR is resumed. The doctor orders Amiodarone at 10mg/kg. You draw up 260mg and administer the Amiodarone. CPR continues for two minutes and staff stop CPR for a rhythm and pulse check as well as switch compressors. V-Fib remains on the monitor. The defibrillator is charged to 200 joules (8J/Kg is 208 joules). Staff clear the patient, the shock is delivered and CPR is resumed. The doctor orders Epinephrine and 0.26mg is administered. 2 more minutes have passed and the staff switch providers, check a pulse and see V-Fib on the monitor. CPR is resumed and the defibrillator is charged to 250 joules (10J/kg is 260 joules but the monitor jumps from 250 to 300 joules and 260 joules is not an option). The defibrillator is charged, staff clear the patient and the shock is delivered. Staff resume CPR and the doctor orders Amiodarone at 5mg/kg and 130mg of Amiodarone is administered. 2 more minutes of CPR have passed, the staff switch compressors and check a pulse. There is no pulse and V-Fib remains on the monitor. CPR is resumed, 10 joules per kg is the maximum for a child. The defibrillator is charged to 250 joules. Staff stop CPR, clear the patient and the shock is delivered. CPR is immediately resumed beginning with chest compressions. Epinephrine 0.26mg is administered IV as 2 more minutes of CPR has completed. Staff stop CPR, switch compressors and check for a pulse. The child now has a weak pulse at 80bpm. Resuscitation efforts should now focus on assessing breath sounds, making sure bilateral chest rise is equal with adequate spontaneous breaths, and maintaining an oxygen saturation between 94 and 99%.
Fluid boluses should be administered at 10ml/kg of normal saline over 10 to 20 minutes to improve the child's perfusion, with frequent reassessment.
Blood glucose levels should be checked along with labs, a chest x-ray, 12 lead EKG and a full set of vital signs.
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Cardiomyopathy in Children & Teens
- Source
- Copyright © 2016 American Academy of Pediatrics ＆ Children’s Cardiomyopathy Foundation
Last Updated 01/31/2021
Rare and often underdiagnosed, cardiomyopathy is the leading cause of sudden cardiac death in children under the age of 18. Because the disease can be present without symptoms, families should be aware of any family members who have heart disease or who died unexpectedly before age 50.
Read on for frequently asked questions from the American Academy of Pediatrics about cardiomyopathy in children.
What is cardiomyopathy?
Cardiomyopathy is a chronic heart condition that involves the deterioration of the heart muscle (myocardium). The heart muscle becomes abnormally enlarged, thickened and or stiffened. Eventually the heart is unable to pump effectively and cannot supply the body with enough blood to function. In severe cases, cardiomyopathy can lead to heart failure or sudden death.
There are 5 forms of cardiomyopathy:
- Dilated cardiomyopathy (DCM)
- Hypertrophic cardiomyopathy (HCM)
- Restrictive cardiomyopathy (RCM)
- Arrhythmogenic right ventricular cardiomyopathy (ARCM)
- Left ventricular non-compaction cardiomyopathy (LVNC)
Who gets cardiomyopathy?
Cardiomyopathy predominantly affects adults, but in rare instances affects infants and children.
According to the North American Pediatric Cardiomyopathy Registry, one out of 100,000 children in the U.S. is diagnosed with cardiomyopathy each year. However, this is a conservative number because it does not include all types of cardiomyopathies. Cardiomyopathy can occur in any child regardless of age, gender, race or economic background.
Causes of cardiomyopathy in children
There are many causes of cardiomyopathy in children, including some that are not fully understood. Common causes include:
- Inheriting the condition from one or both parents
- A viral infection
- Toxins affecting other organs
- Using chemotherapy drugs
- Metabolic, mitochondrial or systemic diseases in parts of the body other than the heart
Despite advances in genetic testing, the exact cause remains unknown in 2/3 diagnosed children.
Symptoms of pediatric cardiomyopathy
Occasionally, symptoms of pediatric cardiomyopathy can be mistaken as a cold, flu, asthma, or stomachache. Some affected children have no symptoms while others may experience any of the following symptoms:
- Shortness of breath or rapid breathing
- Dizziness and fainting
- Irregular or rapid heartbeat
- Abdominal bloating or swelling
- Chest pain
- Extreme fatigue
Infants may experience poor weight gain, extreme sleepiness, difficulty feeding or excessive sweating.
Warning signs of sudden cardiac arrest:
Recognizing the warning signs of sudden cardiac arrest is also important. If your child experiences any of the following, he or she should see a pediatric cardiologist for an extensive cardiac evaluation:
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How is pediatric cardiomyopathy diagnosed?
A pediatric cardiologist confirms the diagnosis through an echocardiogram (echo) and electrocardiogram (EKG, or ECG). In some cases, other medical tests are necessary. Genetic testing may be recommended to verify the cause of the disease.
Since pediatric cardiomyopathy is rare, it is recommended to locate a children's medical center with a wide range of experience in managing children with cardiomyopathy. Early diagnosis and treatment of the disease is essential to preventing complications and progression to heart failure.
Treatment options for cardiomyopathy
There is no cure for cardiomyopathy, but the disease is treatable. Symptoms and complications can be well-controlled by medications, surgery, and implantable devices. The type of cardiomyopathy and its severity determines the treatment plan.
- Medication is usually the first treatment option to improve the functioning of the heart.
- An implantable cardioverter defibrillator (ICD) may be needed to regulate heartbeat in children with irregular heart rhythms (arrhythmia).
- A surgical procedure called a myectomy may relieve some symptoms in children with HCM.
- A heart transplant may be necessary if medical management is ineffective in controlling symptoms and preventing heart failure.
Genetic considerations
Cardiomyopathy is often genetic, so it is important to assess a family's inheritance risk and discuss family screening. A pediatric cardiologist and a pediatric geneticist should work together to form a screening plan and discuss genetic testing options.
How does the diagnosis impact a child's lifestyle?
Although cardiomyopathy is a chronic condition, many children with cardiomyopathy lead a relatively normal life with few limitations. A diagnosis will likely involve more visits to the cardiologist and taking daily medications. Other adjustments may include restriction from competitive and contact sports, diet changes, and school accommodations.