#AsktheMayoMom about Pediatric Congenital Heart Defects

Hy. Ebstein anomaly with pulmonar atresy hipoplastico is this sever? We had BT SHUNT after 2 days of birth and 4mm, and atrioseptostomia rashkind after 9 days.. home after 12 days with furosemida espironolactona and acetilsalicilato.. now is 3 months old and using just the last one.. we go this month to our cardiologist for check... question is this sever? What should we do.. thank you from Portugal

Thank you for re-posting your questions here, Julio.

Hello @julioborhidan I forwarded your question to Dr. Dearani's team. While we can't diagnose a medical condition via this platform, if you could send your medical records to Dr. Joseph Dearani, he can review and make a recommendation based on those records. You may call 1-507-255-2000 or mail to Dr. Joseph Dearani at Mayo Clinic, 200 1st Street SW, Rochester, MN 55905. Thank you.

What kind of experiences and data do both of you have about donor tissue valve replacements?
If a 1 year old has had both tricuspid valve AND pulmonary valve replacements done at 18 months old what would your future treatment concerns be?
Could you explain why having both replacement valves may be favorable, or even why it isn't favorable to have both replacements compared to other surgical repairs?

Thank you!

Welcome to Connect, @dorothyma3. What a beautiful profile image of you with your babies. I'm trying to understand the timeline of your question. Your child had a tricuspid valve and pulmonary valve replacements done at 18 months old. How old is your child now? What was done at 1 year old?

While we can't speak to an individual's medical condition on Connect, your opening and closing questions can be addressed in general. @deannaconstans will forward them to Dr. Dearani. Thank you!

Our daughter is 7 months old, has severe displacement of the tricuspid valve with severe regurgitation and an ASD which had reduced in size since she was born. She has no symptoms of her condition (so far) and is not on any medication. Our cardiologists believe that waiting to see what happens is the best option, and would prefer her to be in her teens before they operate - so at the moment there is no surgical plan. They can perform the cone, however the youngest patient they have performed the operation on is 9 years old - they have said it is too technical an operation to be completed on very young children.

We are concerned that their ability to operate is a factor on their surgical plan. I'd like to understand:
- what are the factors Dr Dearani uses to determine if/when surgery should be performed on a patient
- the questions patients/parents should be asking of their cardiologist to get the best understanding of their specific circumstances/condition.

Thanks so much x

Mi hijo de 13 años ,fue hallazgo por ecodopler,ebstein leve,insuficiencia tricuspide moderado ,insuficiencia pulmonar leve,puede empeorar su diagnostico con los años o mantenerse siempre estable.no tiene sintomas ,solo un poco de falta de aire y ahora se le escucha un soplo ,

Thank you. My son was diagnosed at birth with severe Ebsteins anatomy, ASD, PDA, and pulmonary atresia with severe valve stenosis.
My son had his first open heart surgery at 7 months old (may 2015). He had a pulmonary valve patch placed for his severe pulmonary stenosis that kept returning after his caths. During this surgery his surgeon also did a tricuspid valve repair. He was doing great the first week post op but while in recovery he coded and the team discovered he had endocarditis and he went septic. The infection distroyed what tricuspid valve tissue he did have.
At 15 months old he had his second OHS (January 2016) where he went to the O.R an we planned for him to only have the Glenn procedure. They was not okay with him coming off the heart and lung machine when they trailed him off; his heart was not keeping up... So both his tricuspid and pulmonary valves was replaced with tissue valves and also he received a full pulmonary conduit. The Glenn was reversed after the replacements was successfully placed. He came off ecmo with ease.
Fast forward today he is now 24 months old (2 years old yayyy an 10 month post op) and doing amazing! He's like any other toddler! I was just curious to how may other doctors/surgeons experienced this type of repair? I really didn't want to explain his journey because I find some doctors tend to hold back on explaining the ugly/bad outcomes to parents.
The question about future treatment... My sons last check up was three months ago and they decided he now be seen every 6 months by his team. I feel that time span between appointments is to long considering all the risks possible with two valve replacements. I was just curious if there are different follow up plans for kids with two replacements valves, or is there no true plan and it's different with every case?

My 9 year old daughter has EA, as well as a PFO and WPW. Her valve is not leaking a whole lot so we have decided to wait for surgery. Her rhythm issues are her biggest . She is on a high dose of Atenolol. But still goes into svt. She has now started blacking out and getting dizzy. She had an ablation last year and it appeared to be successful. However a month later at follow up her delta wave was back. Our cardiologist said he would not try the ablation again until she was bigger because her AP is on the outside of her heart in a posterior septal location. My guess is that she needs to be bigger because the size of tools, but my question is, if a catheter is threaded through her femoral artery into the inside of her heart, how is the AP ablated when it's on the outside? Does the radio frequency burn through her heart? Would he use Cryo ablation instead?

Hello, our 14 month old daughter has a complex combination of CHDs. She has been diagnosed with Ebstein’s anomaly with trivial TR, Bicuspid Aortic Valve with small Aortic Annulus and severe Subaortic narrowing with a sub-aortic membrane, abnormal Mitral Valve with mild MR, small ASD and VSD. When she was 4 days old she had surgery to repair severe Coarctation of the Aorta. The next surgery she will require is for her LVOT.

Her surgeon has talked about a modified Konno however, they have never experienced her combination of CHDs before. In your response to their consultation letter on 4th September 2015 you mentioned the possibility of a transaortic resection.

The results from her latest Echo a couple of weeks ago show her LVOT Peak Velocity at 3.34 m/s and Aortic Valve Diameter 0.80 cm. Previously in July it was 2.66 m/s and 0.77cm. For the July echo she was sleeping however for the latest echo she was awake.

Our questions are:
• Have you experienced the combination of Ebstein’s anomaly and severe subaortic narrowing before?
• If so, what type of surgery did you preform and what were the outcomes?
• What indicators do you use to time your intervention?

Thank you.