To remember the five common types of congenital heart disease that present with early cyanosis, simply count to five.
One: truncus arteriosus
There is no separation between the aorta and pulmonary artery, leaving one great artery instead of two. This one vessel sits over a large VSD and carries blood to both the body and the lungs, eventually causing pulmonary hypertension if unrepaired.
Two: transposition of the great arteries
In this condition, the two great arteries are transposed, with the left ventricle abnormally connected to the pulmonary artery and the right ventricle abnormally connected to the aorta. Pulmonary venous return, however, is normal. This results in parallel circulation - blood goes from right heart to body to right heart, and from left heart to lungs back to left heart.
Three: tricuspid atresia
The three-leaflet triscuspid valve normally allows deoxygenated blood to flow from the right atrium to the right ventricle. When this valve is atretic (absent or seriously malformed), blood cannot flow easily to the lungs.
Four: tetralogy of Fallot
This is a cluster of four interrelated cardiac defects - pulmonary stenosis, right ventricular hypertrophy, overriding aorta and a ventricular septal defect.
Five: total anomalous pulmonary venous return
TAPVR is when the pulmonary veins carry oxygenated blood back to the right heart instead of the left heart. There are five words in this condition.
I first saw this somewhere on the web a while back. If you know who deserves credit, please let me know.
My daughter baffled the NICU staff by presenting with marked cyanotic spells but revealing only a medium VSD on the echo. Supposedly that shouldn't be possible so I don't know what she was up to, there.
ReplyDeleteInteresting. Newborns in general can be pretty unpredictable and sometimes we just throw up our hands. Most cases of cyanosis are related to a lung/oxygenation issue, but we suspect cyanotic cardiac defects if oxygen administration doesn't cause a rise in the blood's oxygen level.
ReplyDeleteA VSD is typically acyanotic, but if you have a VSD and your pulmonary artery pressures go up, that will reverse the direction of shunting of blood through the defect and can cause cyanosis.
Another interesting thing: A newborn needs 3-5 g/dL of desaturated hemoglobin in order to cause cyanosis. As a result, babies with high hemoglobin/hematocrit counts could turn blue at near normal saturations, while anemic babies might not develop cyanosis until their sats are ridiculously low.
Well I think you just solved the mystery, because she also had an *extremely* high red cell count! When she wasn't blue or yellow (she had bad jaundice too) she was bright red.
ReplyDeleteWow... so her skin had covered every color in the neonatal rainbow.
ReplyDeleteUsually in the NICU we see the opposite end of your daughter's situation. We'll have a scrawny, anemic preemie who is saturating 70% and we're wondering if the reading is accurate because the baby isn't cyanotic.
Thanks for your comments!
Thanks for posting this! As an echo tech I find it useful...
ReplyDelete