©️ Cincinnati Children's
To get you started: an incredibly brewed overview (2:07mins)
Truncus Arteriosus
In truncus , we have a ventricular septal defect, that leads to a valve where, instead of the great vessels being separated into the aorta and the pulmonary arteries that originate from seperate ventricles, we have instead one joined artery that splits into both the aorta and the pulmonary artery.
The valve sits, as you can see, between the R) and L) ventricles, and the upper portion of these chambers is missing.
Signs & Symptoms
As I'm sure you can imagine, with mixing of oxygen rich and poor blood, we will have decreased oxygen delivery to the body, and thus, cyanosis.However because there is a large amount of blood flowing to the lungs, babies may develop congestive heart failure (CHF) in the first 1-2 weeks of life. The heart will look large on chest x-ray, and the lungs hazy, indicating pulmonary over-circulation.
CHF signs & symptoms include:
☞ Tachypnea
☞ Wheezing
☞ Dyspnea
☞ Grunting or noisy breathing,
☞ Increased work of breathing (incl. nasal flaring & pulling under the ribs)Additionally, the liver may be large and the baby may not be feeding well/symptoms worsen when the infant feeds.
Diagnosis is achieved through physical examination of above symptoms and cyanosis, echocardiogram to confirm the presence of a truncus arteriosus, and cardiac catheterisation that may be done in rare situations if the anatomy appears very unusual.
Treatment
Initially, we work to stabilise the infant. Treating cyanosis, medication to control CHF, and surgery in the first few weeks of life after the infant is stable and well.Surgery involves using a heart-lung bypass machine for support, and the procedure works to:
☞ seperate pulmonary arteries from main truncus,
☞ close VSD using a patch, and
☞ create a connection between right ventricle and pulmonary arteries using a conduit (tube).Babies will be in Neonatal Cardiac ICU for recovery, and in this time, babies will be on mechanical ventilation, special monitoring lines and strong IV medications.
Support and intervention will be slowly peeled back as the infant recovers, and time in hospital may vary from one to three weeks in most cases.
Results
Approximately 90% of infants survive this procedure, and those will need cardiology follow up lifelong. There should not be physical restrictions, however as the child grows, the conduit may need to be replaced through minimally invasive procedures in the cardiac cath lab until they will require open heart surgery to replace the conduit with a larger one. These are usually followed by one week of hospitalisation thereafter.~~~
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