Hello,

My Daughter “Hannah” turned 5 in February.  We were diagnosed with Ebsteins at our 20 week pregnancy Scan.  We were recommended to terminate our pregnancy.  Hannah has mild Ebsteins with moderate tricuspid valve regurgitation.  Hannah goes to the cardiologist several times a year and is about to go again to have a stress test done.  She is just like any other normal 5 yr old but does tire quite easily.  I know it can be daunting awaiting the birth of a bub not knowing the outcome.  Hannah was taken straight away to the ICU and given a drug to keep the shunt open while her oxygen levels stabilised.  She was fine to move to the ward after 6 days.  So far she has not had to have any surgery but this may change in the future.  The cardiologist has advised us not to let her participate in any competitve sports which is a big challenge as Hannah strives to be the best at everything she does.

At the end of the day she is just a normal kid.  We tell her all the time the truth about her condition and she knows her body better than anyone else does.  She knows when she gets palputations to stop and chill out for a while.  I fully believe in telling kids all about their illnesses so they can learn what is important.

We feel lucky to have Hannah.  There are many other conditions out there that would have taken her from this world by now.

Have a nice day.

Gaylene from Oz.

Experiences

27/08/09

My name is Darlene Staimpel and my son John was born with Ebstein’s Anomaly.  I had no idea until he was born (March 2005).  That was one of the scariest days of my life.  I gave birth and then the nurses took him.  I never saw him.  Everyone left me in the room by myself while they were trying to figure out what was wrong.  After 20 minutes my husband walked in my room and said that “something” was wrong and they have literally every doctor and nurse in the hospital helping him.  They stuck him over 100 times that day just to get an iv started.  A larger hospital’s transfer team came to take him.  I got to touch his hand and foot but never saw his face or held him until the next day.  I had to stay in one hospital while my newborn baby, so sick, was in another hospital 45 miles away. I found out later that night that the transfer team had to revive him twice. 

Dr Chris Knott-Craig

The doctors ran several tests and diagnosed him with Ebsteins.  The hospital released me the next morning to be with John.  I remember seeing him for the first time and I was so scared. He was so blue he was almost black.  We spoke to his cardiologist and he said that they would have to transfer him to yet another hospital because they could not give him the proper treatment.  The hospital team Lear jetted him to a larger hospital where he stayed 3 days.  We were told again that they could not help him.  The staff, my husband, myself, and our family and friends made calls to every hospital in the US and NO ONE could or would help.  By the grace of GOD we found the only surgeon in the WORLD that has perfected Ebstein’s Anomaly in newborns.  He agreed to take our son if he could make the flight.  John had been intubated and was now grey and pretty much lifeless. We flew 8 hours to find Dr. Christopher Knott-Craig.  Our first meeting he explained that he wanted to wait until John was a month old before he could do the surgery. He said that he was way to sick and his survival rate would be less than 10%.  At this point John was in complete renal failure and heart failure. He also had a small brain bleed. Taking the advice of Dr. Knott-Craig we agreed to wait. 

The next morning Dr. Knott-Craig called us in and said tomorrow morning is the surgery, we have no choice.  This is his only chance for survival.  That was a hard day for myself and family.  Later that night, Dr. Knott-Craig said it would be a great idea to express our love to our son, make peace and say our goodbyes. That was the hardest thing I have ever done.  The next morning Dr. Knott-Craig performed the most AMAZING surgery on John.  He had made it off the by-pass and was doing great.  From that moment on, John never had a set back or hick-up.  He grew to become an extremely strong, smart, beautiful, tough and hyper 4 year old. Dr. Knott-Craig is now in Memphis, TN and I HIGHLY recommend him if anyone needs this procedure done.  Because of Dr. Knott-Craig and his skills, my son will need no future surgeries for Ebsteins.  HE IS CURED!!!! I would love to say to any parent going through this right now, I know what your going through.  I know the pain, heartache and deepen sadness and helplessness you feel. God will see you through as He did my son and my family. My email address is staimpeld <at> yahoo.com if anyone would like to talk.

New to site

25/09/09

Hi. My name is Miranda and I’m new to this site. I am 22 years old and have had two tricuspid valve replacements. Is there anybody else out there with more than one replacement?

[apologies – needed to shorten the long title – Ed]
hi, I am the Mother of a child who was diagnosed with Ebsteins at Great Ormond Street 27 years ago.

Hi, Nattikins here mother of 27 year old Laura who has Ebsteins, Wolff Parkinson, narrow pulmonary artery.  She was diagnosed at Great Ormond Street the day after her birth when at the “check” they heard a funny heart sound – they didn’t know what it was so sent her, with a police escort, up the M1 to London.  She was sent back 3 days later with a diagnosis and an appointment to attend at GOS.  That’s where her diagnosis was explained to us and we were told by our GP when we asked for a prognosis that “she could not run round the playground or she could not run a 4 minute mile” it would just be wait and see.  Anyway she was very well throughout her childhood and didn’t need any medication or interventions (apart from developing Migraine at 7).  At 20 she started to have rapid heart beat due to the extra electrical pulse in her heart and she was admitted to The Heart Hospital in London for a “simple, quick ablation to put it right”.  Unfortunately ablating this extra pulse put her into complete heart block and a pacemaker was installed.  She found this very traumatic and hard to deal with, particularly when she went for checks of the pacemaker and felt they were able to control her heart with an external magnet but she has just about got over this.  However some 6 months ago she experienced a “black dot” in her right eye – she went to the Optician and her Doctor who told her to go to the Local Eye Dept of the Hospital which she did – she was told her vision field was affected but to go back in a week.  She duly went back and was told it was still affected and was given a leaflet on Detached Retina, they also kept asking to give her an MRI scan which of course she had to explain to all of them that she could not have because of the pacemaker.  They therefore decided to make an appt to do a CT Scan.  Luckily, oh so luckily, my elder Daughter is a Doctor (currently a Registrar Radiologist) and when I told her the situation she immediately said that I should contact our Heart Specialist as it could be a clot from the hole in her heart.  I did so immediately and she was seen in January this year.  He was most concerned and arranged for a “Contrast/Bubble Test” and a letter was written to the Eye Consultant regarding his concerns and asking him to see her before her next heart appointment on 17 March.  She duly went for the bubble test and was told by the Physician who carried it out that they had never had a more positive result and it was also showing bleeps some 10 minutes after the injection of the solution – the Physician was concerned and contaced her Heart Specialist immediately.  She finally managed to get an appt to see someone at the Eye Department on the 16th March – they kept offering her appts in ~April and May, and all that was done was yet another vision test when she was told that her sight in her right eye was 60% compared to 77% and 67% on previous occasions.  She was not seen by the Consultant and was just told she had a “defective retina”.

We duly saw the Heart Specialist on the 17th when it was decided she needs to have an operation to close the hole in her heart as soon as possible as it is obviously throwing off clots and presumably has resulted in retinal emobolisms – this means she won’t recover the loss of sight and is very lucky really that they ended up in her eye and not giving her a stroke in her brain – however we still have no diagnosis from an Eye Consultant.  I am concerned and so are my daughters about the hole being closed because of how Laura will cope and if her heart will enlarge too much but they have said they will try on the table and see how it affects her.  She has a 3 year old daughter so is very concerned as is understable.

I am just so angry without my elder daughter having had medical training and suggesting that I contact the Heart Consultant this would still be going on as neither Laura nor myself would have necessarily mentioned this eye problem to him at her appointment – not putting the heart and eye together.  I, myself, had a stroke at 46 so know the devastation this can cause.  I just feel totally let down by the Eye Department and wonder if anyone else has had such an apalling experience.

I also wonder if  any other adult has had to have their hole closed due to the leakage of blood and clots and how they are coping?

Thanks for reading

For all the people that have had open heart surgery for their Ebstein Anomaly… Were you a sick child?  Did you have alot of problems?  What made them do open heart surgery on you … I am just asking because I want to know what to look for in my son… I want to know all details of things so I know if I gotta get him in and be seen or if he is just normal… What are signs of things that I should watch and look for in my son? Please anyone who reads this please help me understand things that I need to know and watch for .. Oh yeah and I seen some post about the tummy time… My son also does not like tummy time at all .. So I do other things with him to help him learn he is 4months old and he can hold his head up he can sit on his own and when he is on his tummy he gets really mad and he pushes himself backwards … he loves to play when he is awake he is full of energy but he wears down really fast… but I heard that it is normal for him to be more tired then normal babies his age… Thanks for all the help and support…

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

 

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

 

Introduction

29/01/14

Hi my name is Abbie,

I am 21 years old and have Ebsteins anomaly and a hole in my heart. I was lucky in that my condition was discovered by a midwife the day after I was born. My birth went well it wasn’t until the following morning that the midwife noticed that my lips were blue and called a doctor. Shortly afterwards I was rushed to Great Ormand Street Hospital where they realised what the problem was. I spent the following few weeks in the Special Care Baby Unit at Frimley Park Hospital. In the years that followed I visited Great Ormand Street twice a year for scans, echo’s, ecgs etc… And was told I was coping well no need for surgery every time.

Things changed in 2011 when I turned 19 and became a GUCH patient at the London Heart Hospital. I was told they thought they could improve things by surgery. In August 2011 I had open heart surgery performed by Dr Tsang, they repaired the valve using Cone technique and I also had a pacemaker fitted. I was very scared before hand but after the surgery I immediately noticed the difference. I didn’t struggle in the cold and didn’t get out of breath so easily. However recently I haven’t been feeling so good! The breathlessness has come back and my fingers are turning blue again in the cold. It would now seem that I need further surgery, they want to try and close the hole in my heart as they believe if they can successfully do this it will stop the problems.

I haven’t been given the date for my surgery yet as they want to do further tests. I seem to be stressing out a lot about this and have seen my doctor about anxiety and she pointed me towards this site. I am hoping that I may find some help, support or even offer support to others going through the same kind of thing.

Abbie.

 

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.

 
I am 61 and have Ebstein’s Anomaly since birth. In 1997 the tricuspid valve was reconstructed. Recently, within the past 2 years, there has been greater leakage of blood. I’m also in atrial fibrillation. At this point we are trying to evaluate surgery now or wait. I would like to know if anyone else is going through this or has gone through this with 2nd surgery.
Thank you,
Richard Oake
 

I am 67 years old and was diagnosed with Ebsteins Anomaly (EA) in March 2014.

In late 2013 I was having problems with fuzzy vision and MRI indicated abnormal blood vessels in my eyes. I was referred for cardiovascular investigation and in early 2014 atrial fibrillation was found and EA diagnosed by echocardiogram. The EA diagnosis was a complete surprise. The doctor said there was something unusual in my echocardiogram and went to consult a colleague. He came back and said I had EA and was only the second adult case he had seen in his long career. I was told to avoid vigorous exercise – his words were: don’t run for a bus! I’m a retired biomedical scientist and looked on the internet for information about EA and was quite alarmed at the seriousness of the condition and the apparent low incidence of survival to my age.

In subsequent weeks I had instances of palpitations and shortness of breath in the night resulting in emergency visits to A&E and became very aware of right jugular pulse due to tricuspid valve regurgitation. Simply going upstairs to bed causes shortness of breath with a strong jugular pulse making it difficult to go to sleep because of hearing blood flow in my head.
Over my whole life I have avoided vigorous sporting activities (did not have the energy!) and had several instances of transient blackouts in recent years. However, as a scientist I have travelled widely round the world without health issues. The recent emergence of EA symptoms may be stress related. My father died in 2013 and I have no siblings to help me support my 90 year old mother who has memory loss and lives at the other end of England.

I have been told that although tricuspid valve repair is possible, for me the risk outweighs the benefit at this time. Taking daily beta blocker, histamine H2 antagonist and warfarin tablets I am now feeling much better but have to carefully manage my daily amount of exercise. Since starting warfarin in January 2015 the jugular pulse seems less intrusive.

I would be very interested to hear about the experiences of anyone who has been diagnosed with Ebsteins Anomaly late in life!

Thanks,
Trevor