HLHS & Norwood-Glenn-Fontan

Dr. Amy Fan is a Harvard affiliated pediatrician and founder of Kinder, the first and only online primary care clinic for children.
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This video is for educational purposes only. Without a physician-patient relationship, content of the video is not to be taken as medical advice.
Created by Dr. Amy Fan for Khan Academy

[@espenbraa]

My wife is 13 week pregnant with identical twins, and we learned yesterday that one of the babies has HLHS. I am so lost, but this was a good bit of information to have. Thank you for a great demonstration

[@chaitanyakotapati2419]

The best of the teaching material I have come across so far on this topic What a great simplification of such complex anatomical patahology

[@asmahafiz7396]

Excellent demonstration ❤❤❤

[@poonamsambhaji4464]

really great explanation. I came up with a mnemonic to remember the Norwood-Glenn-Fontan in that order New Girl Friend .thanks ones again

[@ArcherAdventures]

my daughter is 13, and best friend is 21 with HLHS :)

[@columbiashoes]

Amy, thanks for this informative video. You obviously have a good understanding of this topic and provide a good explanation.

[@michaelsauder]

Great explanation. Thank you

[@rebec7063_]

Great explanation thank you

[@Jimray5818]

Beautifully explained…Thank you !

[@MWtzl]

I am preparing for Radiology final exit exams - this is the best, to-the-point, easy to remember and time efficient overview I found to make sense of this complex topic. Thanks for your effort, it makes a difference!

[@AskDoctorAmy]

So glad to hear that! Best of luck to you.

[@bluecamuflase]

Thanks! I understand better now!!

[@kadinapawpaw5000]

Excellent explanation! As a CT intern this is very helpful 🤍

[@sundastahir1933]

thank you, very good explanation

[@gelilawitasefa6247]

Excellent explanation!

[@dr.leonardoeugenio8698]

Realy liked your video. Going to watch the others. I’m a cardiac surgeon in Brazil but i have no experience in pediatric surgery. Its a whole new universe. But i have to study for compliance reasons. What about the coronary perfusion? . I think this is the only point missed. Congratulations, your didactics is amazing!

[@stephenevans7884]

419 likes. I cannot believe this. This video is the best on the Internet.

[@samehsherbiny2020]

Excellent

[@icycream1406]

Excellent videos! could you please do one on Damus-Kaye-Stansel procedure. It's for HLHS babies as well. Many thanks =)

[@ramanareddy1000]

Great presentation, thank you

[@AskDoctorAmy]

Thanks!

[@carolynhigdon1214]

Hello, thank you for the info! My soon to be first grand daughter will be born and have to have these surgeries. Why is the time between the first & second surgeries she will be considered high risk? Why do they wait for a few days for the first surgery?

[@saimakousar7721]

But when connect pul artery and aorta then mixing of blood occur?

[@nerwus1987]

Hi! Great video, I know that hlhs happens more often, but could you explain HRHS ? Is it treated the same way? And what about hemi-fontan surgery? My daughter had Norwood, later hemi-fontan and 'left-side glenn procedure. I just want to understand it better. Another bothering me question, however still keeping in mind that at the moment this 3 stages are the best we've got, could you tell us what is being done to find new procedures to help these children? Any ideas that are being developed? Once again thanks for a great video.

[@AskDoctorAmy]

Hello, thanks for your comment. I'm very sorry to hear that your daughter had HRHS, but it sounds like she got the procedures she needed and I hope she is doing well. I am not a pediatric cardiologist or cardiac surgeon, so my understanding is coming from a pediatrician's perspective. HLHS is indeed more common than HRHS, but congenital defects are actually more individual conditions, meaning everyone is different, even though we have these large groups of the diseases. In other words, it depends on your daughter's exact anatomy and the way her system is working. I am guessing that HRHS involves problems with getting pulmonary circulation, and it sounds like the procedures worked to establish flow to the pulmonary artery. Since these staged procedures have been developed, the survival has improved so much that we see many more of these patients with a Fontan anatomy, living well into adulthood. Like you said, these procedures are the best we've got at the moment, and there is much focus on finding the best post-operative care for this population including long-term monitoring, nutrition, medications if any, etc. Patients now have a much better chance to live a full and healthy life. Here is a summary of the current therapies and research: www.ncbi.nlm.nih.gov/pmc/articles/PMC5119545/

[@rjyepez21]

Hi, not sure if this is the appropriate place to ask a professional question but my son has HLHS and had the Norwood done at about 4 days old. Months later, they did the Glenn procedure when he was about 6 months old. But for some reason, he was not doing well. He was on ECMO for some small time. But doctors never had answers as to why it didn’t work. They did cath procedures, and found nothing. They ended putting him back with the Norwood. It was a long recovery of 6 months and yet he still is at the hospital. Heart transplant is not an option since he will reject it. Any chance the Glenn and Fontan cane be done at the same time? Just trying to think outside the box

[@renosmith1837]

Good explanation, thanks for taking the time. You must be a good teacher and know your subject well to make it simple for the layman to understand. I wonder why they haven’t tried some sort of treatment in utero to help the left ventricle develop? Steroids or hormones or something

[@annabellefarrington5807]

I have left plastic heart disease

[@jeanantonio2907]

O tubo do fontan pode sair

[@ayushpradhan8839]

I don't understand the fontan heart how does the blood in the ivc have enough pressure to travel up the body to the pulmonary arteries

[@AskDoctorAmy]

Hello Ayush, sorry for the late reply. You're right, in this system we are missing the pump of the right ventricle that would normally push blood into the pulmonary arteries. It's not a perfect system, but it's the best compromise in this anatomy, to continue the passive movement of blood from the IVC (and SVC) directly into the pulmonary artery. The venous system has much lower pressure than the systemic side, but it does have some pressure and valves to keep the blood moving forward. With that being said, we do have to always consider the pulmonary circulation in the Fontan anatomy to make sure it is adequate.