My daughter was diagnosed at the age of 2, and so far the only impact has been annual visits to the consultant.  She is now 7 and we are about to go again, and I know she is going to ask more questions.  Her father explained last year that she had a ‘squeaky door’ but I am not sure she really understood that, and I don’t think it will suffice this year.  I am worried that we need to give her enough knowledge to equip her to be able to tell us/or a teacher if she feels anything unusual.  Also, my brother always knew he had a heart complaint and I think he accepted it much more readily because he had ‘always known it’.  I am also worried that she may look it up on the internet and find out information which would be risky.  At the same time we don’t want to burden her with too much information and make her worry.

Anyone experienced similar debates?  How did you resolve them?

Thanks, Caroline

My Baby Girl

23/07/09

My babygirl, Ellie, was diagnosed with Ebstein’s in utero about 3 days before her due date. She was born via emergency c-section. She spent a few days in the Neonatal Intensive Care Unit for monitoring and an eating issue. Now, at almost 10 months, she is just like any other baby. Her cardiologist prescribed her Enalapril. I have done some reading up on the medicine and everything looks good. What I was wondering is if we should limit her sodium and cholesteral intake. Any information would be helpful.

Going blue

24/08/09

Hello,

My daughter, the youngest of six (my eldest daughter having died of Aicardi Syndrome, another rare anomoly) was diagnosed with EA when she was a day old.  We have once/twice yearly vists to the cardio and, touch wood, they seem to be happy to keep an eye on her.  She has moderate regurge through the tricuspid valve and also has a hole in the heart, which they prefer to leave as they feel it relieves some pressure from the valve. She will be 8 in September and although we have always had episodes of turning blue – most noticably round the mouth and under her eyes, it seems to becoming more often.  So far the cardio has been happy again, just to keep an eye on this.  I, however, am feeling more anxious.  Has anyone else had experience of this.

Thank you

Susie

P.S. What a marvellous site, thank you

Does anyone know if EA is genetic?  My ex-husband had corrective surgery at UCLA for Ebstein’s Anomaly and WPW.  Our son is now 13 and having short of breath problems.

Our son, Bill, was born with Ebstein Anomaly. For nine years, he had been constantly followed up by his cardiologist. Recently, his cardiologist, suggested an operation and referred us to a cardiothoracic & vascular surgeon. According to the surgeon, he will correct the Trascupid Valve (TV) and patch the Atrial Septal Defect (ASD). Besides that, Bill has a loose Mitral Valve that need to be fixed as well. His heart was also swollen by a little.

Throughout the nine years, Bill did not show any symptoms of blue or exhaustion, except for complaints of tiredness once in a while when performing sports activities. We are quite brothered by whether we should send him to the surgical table. In addition, the surgeon we approached had done only 5 cases and he was considered as the most experience in our country to have performed Ebstein Anomaly operation. So far all 5 cases are still alive.

The surgeon said that this would be an elective operation. We went to another surgeon and he too suggested that an operation if not done now will be necessary in the future. However, the operation risk and complication will increase as he aged.

Because Bill doesn’t really look that sick to be operated at all. That is why we are in a dilemma. I would like to seek any advise or views on whether we should go for the operation.

Cone Procedure

03/06/10

Hi all,
My daughter, Katie, a twin, was diagnosed at birth with EA. She is compensating well without meds but we have always known she would need surgery. The plan being, as with most, wait until she isn’t coping, as close to adult size heart as we can get, and then go in for repair/replacement.  Like all of you, I hit the info highway as hard as I could and have been stopping in for updates for the past 6 years. Two years ago, there was a post about the cone procedure….and the research I did following that made my heart skip…really!….a procedure that has such incredible success rates is truly more than we were ever allowed to hope fore.  So, began the long process of trying to change minds….what if we just sent her stuff to Dearani and the Mayo, just to see if she is a candidate.  Fine, our cardiologist did so, but our surgeon is reluctant.  Mayo says yes, she is a candidate.  So mom says, “We’re going”.  Cardiologist says just wait, lets talk to the surgeon and see what we can come up with.  Dr. Ross, our surgeon from Stollery in Alberta Canada decided that this was a good procedure and western Canada should learn it so would we be willing to have Katie operated on with Dr. Ross assisting Dearani in Edmonton.  Sure, whatever, just get it done.  Now Dr. Dearani has not responded to repeated requests for contact but a Dr. del Nido from Boston has agreed to do Katie’s procedure in Boston.  I have done a lot of research on Dearani but can find less on Dr. del Nido and his results.   Does anyone out there have some information on Harvard and/or del Nido’s publications/results.

Thanks, Alison in Canada

Help for Rino

01/07/10

Hi,

My name is Emma and I have a small school on the island of Koh Samui in Thailand.  One of my students, a little boy called Rino, has just been (after literally years of doctors telling his family he would grow out of it) diagnosed with Ebsteins.  He saw a specialist in Bangkok who told him he will need surgery within a year.  His parents don’t have any insurance and the operation will cost somewhere in the region of 2 million baht, or 40,000 pounds.  We are setting up a website for him and are starting to organize fund raising events.  The reason I am putting this post up here is to ask for ideas and help.  He is an amazing little boy and I have come to love him very much over the last five years that I have known him.  He hasn’t had the easiest of lives and it breaks my heart to have to see him now go through this as well.  Any ideas will be gratefully received and when we have his blog set up I’ll post details of that too.

Thank you, Emma

 

My Felisha

27/12/10

My daughter Felisha was born in 1997 in Asheville, NC with moderate/severe EA. She ran and played like all the other kids but just tired a little sooner but she rested a little then went again. At age 5 Dr. Tripp at Asheville Cardialogy and Dr. Darani at the Mayo Clinic decided it was time to fix her valve so we got on the road to MN. She had her valve replaced with a pig valve and she has been doing great ever since. She now is 13 and the valve is leaking a little as expected, Dr. Darani said maybe it would last for 6 or 7 years. Darani and the guys at Mayo are alsome, and if anyone out there is trying to decide were to go for surgery, there is no better place to go in my opinion.

I am looking for some advice as feel very unsure what the future holds! Georgina is 3yrs and was diagnosed in Feb after many trips to hospital. She has been a very sick child and is now under the care of Great Ormond Street. she is seen every 8weeks and things have started to worsen! she has now developed an irregular heart beat. She has been put on medication but I can’t get her to take it (even tried hiding in food). She is very anxious about hospitals and I’am finding it a real battle to do the best for her. Her consultant has said he wants her to take the medication to buy us time before they do the valve repair but he is now worried her heart may be tiring as she has prollonged episods of fast heart rate which they are trying to controll
I was just looking for some who has experienced any of the above and can offer me some help.
Alex x

Is this normal with Ebstein’s that babies hate tummy time? He cries the whole time he is on his tummy. I only can deal with him crying for just a few minutes. I would love to know if anyone has had this experience. Does it hurt their chest? Is it too much “work” for them? His Ebstein’s isn’t severe but significant the cardiologist say. Anyone have any answers or any thoughts on this please email me or post on here! tommygirl_26201 [at] yahoo [dot] com

Thanks Melissa Ferrell

I recently ran across a site that said that one of the symptoms of EA was coughing. My almost 11 month old son has had a cough ever since he was born and was just wondering if this was true. Or is it just a coincedence? Just interested to hear of others that may have the “cough”.

Hi all, we are in New Zealand and my 4 year old son has been recently diagnosed with mild EA. I am trying to learn all we can about this and find any others in a similar situation but as it appears to be so rare there aren’t many out there who I can talk to! Patrick is under supervision of the cardiologists here and he is coping quite well so far. But this is all so new and I spend an awful lot of time worrying about him. He can still run around fairly well but tires easily and I have to work hard to get enough food into him sometimes (unless is chocolate of course!). Anyway, just wondering if there are any others in NZ?

 

nathan’s story

09/04/12

Hi my son nathan is 16 yrs old this may and this is his story.

nathan was diagnosed at age of 8 yrs, despite having an echo at 6 weeks, looking back the signs had been there but nobody put them together, nathan had stopped breathing at 12 hrs old but after having oxygen he was deemed ok, he didnt feed well, screamed for 3 hours each day with what we were told was colic and didnt grow we spent the first 5 yrs back and forth to see paediatricians as health visitors had concerns at 5 yrs old he wore size 3-4 yrs size, nathan also on occassion would fall to the floor when out playing with his older brother (we thought he was a bit of a drama queen) it was only when he had a displaced fracture of his radius manipulated under GA that things reallystarted to show up, he began to complain of his heart pounding out of his chest GP sent us straight to A&E and he was diagnosed with WPW (wolfe parkinson white syndrome) we were told take him home we’ll send you a cardiology referral, a number of attendences to A&E left me feeling like i was being an over anxious mother, 3 months later he came down with what we thought was stomach bug 3 weeks later i started to worry he couldnt even manage to walk 200 yards, we saw gp and he sent us again to A&E when we got there he was rushed to resus as his heart rate was over 300 bpm he was eventually admitted to PICU and  had cardioversion to stabilise him the next day he was moved to a general childrens ward and on mothers day 2005 the nurses told me i was being over anxious but on insisting on him being reviewed he was blue lighted to Glenfield hospital in Leicester Uk, we arrived there at 2am and i was told not only did he have wpw but ebsteins anomaly and heart failure a pleurel effusion and the next 24 hrs would show if he would survive, he did survive but i was left shell shocked, in 2006 nathan chose to have RF ablation as he didnt want to take medicine to regulate his heart rate, his first ablation didnt work so he underwent another ablation an accessory pathway was found in his AV node and by trying to ablate it he was put into complete heart block, 2 weeks later he collapsed and was again rushed to glenfield and had a pacemaker inserted, we were told this would be for life amazingly after a year his heart function had returned and there was talk of removing the pacemaker. As far as his wpw was concerned it was cured his ebsteins depending which consultant we see goes from being labelled as mild to severe, but we know he has a large atrialised ventricle his only outward signs however are that he often looks very pale and blue around his lips and finger nails and his exercise tollerence is not like that of a healthy active 16 yr old. Two years ago nathan suddenly collapsed at school and stopped breathing he was given mouth to mouth by a teacher and taken to A&E he took 45 minutes to come around and had amnesia, his cardiologist was adamant it wasnt his heart, he had neurologist input and was investigated for epliepy which was ruled out, he then had a CT head scan (he cant have an MRI) looking for chiari malformation again this was ruled out, he then collapsed again a year later with much badgering the cardiologist agreed to do EP studies incase it was another wpw pathway, the consultant telling me it couldnt possibly be his ebsteins (even though i told him a USA site talks about clots and mini strokes) the EP studies came back normal, again we had no answers! 3 weeks ago he collapsed again whilst out playing football with his friends and stopped breathing his friends panicked and whilst one called an ambulance the other shock him and he spontanuously started breathing again when i got to him he was grey and his lips and nails cyanosed (blue) he was also clearly hypoxic (short of oxygen) as he was confused disorientated and again had amnesia on arrival at A&E we saw a polish reg who said oh its his ebsteins it must be but again the cardiologist is saying no its not, through more badgering his cardiologist referred him for a tilt table test and exercise tollerence test both normal, we are constantly worried he is an active 16 yr old who isnt having much of a life right now as we are terrified that his next collapse could be whilst he’s alone and what could happen.

Does anyone have any ideas or has anyone else known these symptoms in someone with EA

 

Hello

 

I don’t know if anyone would be able to offer any advice. I’m 30 and I have suffered with EA since birth. I’ve miscarried several times. My doctor’s always say it is down to my EA. But I have never been investigated for this, until now. I got admitted to A&E last week and met a lovely gynaecologist who is going to help us and give me all the investigate me. We have recently got married and would love to start a family and I am just wondering if there is anyone out there who suffers from EA and has sustained a successful pregnancy. I’m after an information that people feel may help. I am hoping that the more information I can take to see my gynaecologist and cardiologist then the bigger help we have in our fight to have a family.

Thanks

 

Hi,

My son (With EA) has just turned 4 and has had migraines since he was 2. The consultants say it isn’t linked to his heart but how do they know that?   No tests have been carried out and he has at least 2 a month. Does anybody know if heart rate or EA affects migrains? Thank you very much, i hope to hear from you!

 

My daughter Florence has Ebstein’s Anomaly, she was diagnosed at 3 months old after I took her to hospital because she had a cough that got gradually worse and she was very weezy. They told me that she had bronchiolitis but they kept her in because her oxygen saturation levels (SATS) were a considerable amount lower than they should have been. As she got over her illness in hospital they remained concerned because her SATS were not improving. She was then transferred to the Birmingham Children’s Hospital for further tests and it was there that she was diagnosed with Ebstein’s Anomaly.

Since her diagnosis she has been monitored at Birmingham Children’s Hospital every 4 months. In the very first appointment we was told her condition was mild although in the last appointment they told us that her condition was severe. They also told us in the last appointment that they are looking at doing keyhole surgery to repair the ASD (Atrial septal defect). They also told us that at some stage of her life she would need open heart surgery to correct the displaced and perforated valve.

We wanted to know more information about the cone procedure which we found on the website of Boston Children’s Hospital. We have since found more information on this website about the cone procedure being performed at Bristol Children’s Hospital and I was wondering if anyone has any more information on this procedure? We would also like to know if this procedure is available in any other hospitals in the UK?

Thank You

 

My daughter was born on January 7, 2014 and diagnosed with EA on January 9. I am looking for advice on what should I expect. How do I even comprehend what is going on? This is suppose to be the happiest time of our lives. Don’t get me wrong, I am still very thankful for my daughter. I just want her to have a normal life.  I have read a lot of information since she was diagnosed but wanted to hear from parents that have gone through this. We met with a Pediatric Cardiologist Specialist from The University of Louisville on January 9. He  was very helpful and had a lot of information but those few day were somewhat of a blur. He said that my daughter would probably have to have surgery within the first or second year of her life.  My wife and I went from being the happiest we have ever been to getting are life turned upside down. He explained the oxygenated/de-oxygenated blood mix was small (and had apparently improved since she was last checked). He also told us that it is perfectly safe for a newborn’s pulse ox to drop down into the 70s without any harmful side effects.

We were released from the hospital this Saturday, January 11. The pediatric doctor told us to act as if there is nothing wrong with our daughter. How in the heck can we do that?   I am trying to stay strong for my wife and daughter but it is hard at times because just like a lot of you; I ask, why us? Any advice on how to comfort my wife? She is an emotional wreck  right now. She is scared to sleep or take her eyes off of Avalynn for the fear of something happing to our little princess. This coming up Friday we are meeting with another Pediatric Cardiologist Specialist from the University of Vanderbilt. What questions should I ask? I don’t want to miss anything.

From what I understand from the last specialist, the issue is with her right ventricle and tricuspid valve. Doctors were unable to diagnose our baby in the womb because her heart wasn’t enlarged and apparently, nothing abnormal was spotted. When the hospital took the baby out of our room and to the nursery to do the standard state testing on her hearing and heart rate/pulse ox/respirations, they noticed that her pulse ox was lower than normal. From this, she had some cyanosis; blue tint around her lips and in her feet and hands. This was when they notified us and explained that they would need to move her to the NICU to monitor her pulse ox. Anyone else’s EA experience similar to ours? Was your child’s particular “type” of EA spotted after birth and not during gestation?   Any help that anyone can give would be greatly appreciated.

Thanks,

Patrick

 

Our daughter was diagnosed with severe EA and ASD when she was born in Bath (UK) 2007. She spent a week or so in PICU in Bristol before we finally got her home.  She had a glen shunt procedure in 2009, but since then her oxygen saturations have been in the mid-70s, she has had little exercise tolerance and is quite blue.   About 2 years ago, our consultant in Bath  Andy Tomeztki told us what we had long suspected, that Emmeline would definitely need a second operation. Although it wasn’t clear what the best way forward was to be, as the valve was in a pretty bad way and really too severe to attempt to repair. The most likely outcome seemed to be the “fontan”, which is a bit of a last resort and doesn’t have a great prognosis. So, we had two years of scans and waiting, then had an appointment with Andy Parry last September when he told us they thought it was worth trying a repair afterall  (the “cone” procedure), and if this didn’t work they would go for an artificial valve, or else a fontan. We then had word on Valentines day2014 that the operation was scheduled for the 28th Feb, but this was postponed on the day due to lack of beds in ICU and re-scheduled for the 12 March. The night before (11 March) Massimo Caputo came to see us and explained that both he and Andy Parry would carry out the operation. Massimo explained had done 12 cone procedures previously, 11 of which were successful – Emmeline was number 13. The youngest patient he had carried out this operation on previously was 14, Emmeline is 6. He estimated that the chances of a successful repair were 70%. Anyway, after 5 agonising hours, Masimo came to see us and told us the operation had gone well, and the valve had good function, and they had largely closed the ASD -the only nuisance was that she would need a pacemaker (this, he explained, was a small price to pay). She had the pacemaker fitted last Thursday (20 March), and we got home from hospital yesterday (24 March). Emmeline is doing incredibly well so far; it really feels like a miracle. Her O2 sats are now 98-99%, and she is a completely different colour – her fingers and toes look pink for the first time in her life! She should be back at school after Easter. Massimo has now gone, or is about to go, to Chicago for a years sabbatical  – Andy Parry will now be carrying out the operation in Bristol his absence (I think this is also why he was present at Emmelines operation). We owe a huge debt of gratitude for the pivotal part played by the Ebsteins Society in disseminating the expertise on the cone technique from Brazil to Bristol and beyond.  We had long been under the impression that there was little chance this procedure could ever help Emmeline, but all being well she should now be able to live a basically normal life. I also want to big-up the Bristol Childrens Hospital which has had such bad press recently – in our experience it is full of the most amazing doctors, nurses and other support staff.

 

Our story

13/05/14

My little boy, Toby was diagnosed with EA at GOSH at 18 days old, due to weight loss. He was monitored at Frimley CC every 6 months for the first few years then yearly. Last February his cardiologist said he wanted to carry out a stress test. This was done in September, in November we received a letter advising us that the Doctors felt they should perform the cone procedure as his leak was quite bad,  before complications began to arise. I spoke to the cardiologist who assured me it was in Toby’s best interest for us to elect to have the surgery done asap. Surgery was scheduled for the end of March. The operation was performed at GOSH on the Thursday morning, Toby could not be stabilized during the night so was given a blood transfusion, still not stable so an ECHO was requested, and they found that there was still a leak. He was taken back down to theatre Friday morning where it was found that one of the stitches in his heart had come undone. We were sent from ICU to the ward Saturday evening and discharged from the ward on Monday. The treatment received was poor, and I do not believe that he should have been discharged so early. We were all very traumatized by the whole experience, to see your child lying in ICU with all those tubes is awful, when something goes wrong words cannot explain. Our appointment on the Friday started with a massive nose bleed which the clinical nurses assured us was not related to the op but most likely stress. We have transferred Toby’s follow-up care elsewhere at his request and with our support. At 4 weeks post op he had a mild leak, the right side of his heart is not functioning properly and he has extra and skipped heart beats, these things will hopefully improve over time. At 5 weeks post op he has very little stamina and there are still many things he cannot do, it is heart breaking to see a little boy who walked in to a hospital make such slow progress that 5 weeks later he is weaker and lighter than before he went in. I am not convinced that we made the right decision, maybe over time I will see things differently.