Tetraology of Fallot

Specialties Ob/Gyn

Published

I have never seen a kid so vigourous that was so blue. Two questions:

Is Tetralogy of Fallot something that should have be picked up on screening OB ultrasound?

We had this kid on 86% + Hood O2 prior to getting him shipped. If I remember correctly, with this disorder isn't it better to keep kiddos sats lower to preserve fetal circulation until surgery? Of course, we did not know what was wrong with him when we shipped him out - which is why I am a little upset with not having any hint about the problem prior to delivery.

Love to hear your responses - you folks have taught me an awful lot and I really do appreciate that.

Specializes in NICU, PICU, PCVICU and peds oncology.

This defect could have been picked up prenatally, but we've seen lots of Tets, hypoplasts and TGAs that got missed and went home at X hours of age, only to return half dead and the colour of a grape. You're right about wanting to keep the sats lowish with these kids for a few reasons, preserving fetal circulation being one. Another good reason is to limit the development of pulmonary hypertension. One of the features of TOF is pulmonary stenosis, which contributes to the infamous "Tet Spell" of paroxysmal hyperpnea, profound cyanosis, right-to-left shunting, acidosis and syncope/seizure/arrest. Unless the pulmonary stenosis is severe or there's pulmonary atresia, many Tets do go home and come back later for correction. Your little guy must have had a significant stenosis. But then he should also have had a very audible high-pitched pulmonic murmur, unless the pulmonary valve was simply not there. The murmur of a PDA is usually very machinery-like and heard best just under the left clavicle, so it's not impossible to distinguish one from the other. Maybe for future reference, if you have a baby who isn't pinking up as expected, listen for a murmur. If you hear one, do four limb BPs and arrange for a stat echo. While you're waiting you could maybe mix up some prostaglandin E1...

Specializes in NICU.

Oh my, is this the baby from your Did I Underreact thread?

Specializes in Specializes in L/D, newborn, GYN, LTC, Dialysis.

Jan, excellent post. Thank you for your help.

This defect could have been picked up prenatally, but we've seen lots of Tets, hypoplasts and TGAs that got missed and went home at X hours of age, only to return half dead and the colour of a grape. You're right about wanting to keep the sats lowish with these kids for a few reasons, preserving fetal circulation being one. Another good reason is to limit the development of pulmonary hypertension. One of the features of TOF is pulmonary stenosis, which contributes to the infamous "Tet Spell" of paroxysmal hyperpnea, profound cyanosis, right-to-left shunting, acidosis and syncope/seizure/arrest. Unless the pulmonary stenosis is severe or there's pulmonary atresia, many Tets do go home and come back later for correction. Your little guy must have had a significant stenosis. But then he should also have had a very audible high-pitched pulmonic murmur, unless the pulmonary valve was simply not there. The murmur of a PDA is usually very machinery-like and heard best just under the left clavicle, so it's not impossible to distinguish one from the other. Maybe for future reference, if you have a baby who isn't pinking up as expected, listen for a murmur. If you hear one, do four limb BPs and arrange for a stat echo. While you're waiting you could maybe mix up some prostaglandin E1...

i have never worked peds, i have done adult critical care and er, but i am amazed at your thorough and intelligent response!

EXCELLENT POST!

:bow: :bow: :bow: :bow:

to all the new nurses out there:

see, you are NOT glorified maids!

go strut your stuff!!

We let our Tet kids sat between 85-93%.Most go home until they are big enough for surgery.

Are you still beating yourself up about this? From what you told us, he did not present like a Tet kid.

IRT an early post - nope not the same kid re: "Did I underreact?" We shipped 4 babies in 5 days and this little TOF was #4. I have still not heard how my 'undreacting' baby is doing (? - septal valve defect) I think he was going to follow-up with a peds cardiologist after discharge.

I'm not sure if I feel better or not now that I know TOF isn't usually picked up on OB ultrasound. Unfortunately we do not have the capability to diagnose the TOF and therefore we wouldn't start them on the prostaglandins prior to shipping them. This guy did have a pretty significant mumur I was able to hear when he (finally) quit screaming his head off.

:uhoh21: screaming bloody murder and blue :uhoh21: Still floors me!!

Basically, we do the oxygen and stare therapy (stare at them until they declare they are going to get better or worst). We do CXR and blood work, EKGS, but not echos. If the O2 doesn't help, they keep working harder to breath despite increasing FiO2, they need more than we can give - so they get a ride. If there are any infection risk factors then we do the abx. I think that covers the questions since I was last on.

Oh yea, we did do the 4 pt. BP's and the MD did not seem to be worried about them.

Thanks again for your responses - I have really learned sooo much.

Basically, we do the oxygen and stare therapy (stare at them until they declare they are going to get better or worst). .

:lol2: :lol2: :lol2:

Specializes in NICU, PICU, PCVICU and peds oncology.

Hmm let me see, what to comment on first... your babe you wondered about under-reacting on probably had a ventricular septal defect based on the possible dx you've given. There would be a murmur associated with that defect as well; the pitch and volume would depend on how big the hole is... bigger means quieter.

TOF definitely can be picked up on prenatal U/S, it just sometimes is missed. And any time you have a screamer who is blue, it's heart related. They start to sunt right-to-left and just get bluer and bluer... and bluer. Case in point is my kiddie from today. She was born with pulmonary atresia and a VSD, so not a complete TOF. She had a central shunt done (Gore-tex conduit from the ascending aorta to the pumonary artery) at age 4 days, then came back earlier this month for repair, only to be found to have mediastinitis and only got cleaned out instead. So she's still in PICU following a rocky course with sats usually in the 70's until she snits. Then they drop to the 30s and about the only thing that helps is heavy sedation. Crying and fussing gone, sats are better. CXR will often reveal a classic boot-shaped heart with TOF. There are some good images on the Web that show this effect.

Four limb BPs are usually not significant unless the kid has a coarctation of the aorta and even then they're not always helpful. What you would see is a marked difference between upper limb pressures and lower limb pressure. But four limb BP is always included in the "this kid has a murmur" routine.

Another thing you can look at is the difference between pre- and post-ductal sats to help clue in where the defect might be. Lots of right-to-left shunting will give you a noticeable difference with sats taken on the right hand being lower than on the left. I've given a brief inservice on this at allnurses.com/forums/f95/pulse-oximetry-48755.html.

Boy, keep this up and soon you'll know as much as me!:wink2:

janfrn I have a question for you-since you are so knowledgeable. I had a term c/s baby who became blue, about 15 min of age, pre-ductal o2 sats were in low 80's and post-ductal sats low 50's. She pinked up after 2 mins of bagging with 100% o2 and then maintained under 50% fio2 oxy-hood. She was very active/alert the entire time. The difference in the pre-ductal and post-ductal sats was 20-30% difference for the next 2 hours with the pre-ductal being the higher value. Then it began to become the same and infant was able to be weaned off the o2. We never could figure this one out:nurse:

Jan - thanks for all the good info. I'm going to link to the page you referenced. I've never thought of doing pulse ox on two limbs at the same time. Does it work the same if you do it on their feet?

Funny that you mentioned that. The MD had the RT come in and try the pulse ox on the other foot to see if there was something wrong with the sensor - originally on right then put on the left.

Oh cripes, now you've got the gears jamming in my brain :lol2:

Thanks for the good info

Specializes in OR, ER.
This defect could have been picked up prenatally, but we've seen lots of Tets, hypoplasts and TGAs that got missed and went home at X hours of age, only to return half dead and the colour of a grape. You're right about wanting to keep the sats lowish with these kids for a few reasons, preserving fetal circulation being one. Another good reason is to limit the development of pulmonary hypertension. One of the features of TOF is pulmonary stenosis, which contributes to the infamous "Tet Spell" of paroxysmal hyperpnea, profound cyanosis, right-to-left shunting, acidosis and syncope/seizure/arrest. Unless the pulmonary stenosis is severe or there's pulmonary atresia, many Tets do go home and come back later for correction. Your little guy must have had a significant stenosis. But then he should also have had a very audible high-pitched pulmonic murmur, unless the pulmonary valve was simply not there. The murmur of a PDA is usually very machinery-like and heard best just under the left clavicle, so it's not impossible to distinguish one from the other. Maybe for future reference, if you have a baby who isn't pinking up as expected, listen for a murmur. If you hear one, do four limb BPs and arrange for a stat echo. While you're waiting you could maybe mix up some prostaglandin E1...

:yelclap: wow this is an excellent post!..thank you very much

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