Surgery or No Surgery?
15/10/09
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.
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Hi there
You don’t say which country you live in. As far as I know in my experience surgery is usually done as the person deterioates, becomes unable to function properly – such as being breathless going upstairs. I have often heard that surgeons do not intervene with a ‘well’ child, although one of the journal papers (see our ‘research’ section) around the cone technique mentions that it may be in some cases where this technique is used that surgery should be just prior to deterioation (if you can tell when that is).
As you say Ebsteins surgery is rare – again I am not sure where you are but assume not the USA where such surgery is concentrated in specific hospitals such as Boston or the Mayo.
Let us know where you are and perhaps there will be people who visit this site with more specific knowledge of your area.
Caroline
Comment by caroline — October 15, 2009 @ 9:26 pm
Hi Caroline,
Yes. You are right that I am not in USA. I am living in Singapore.
Comment by kohbh2000 — October 18, 2009 @ 6:15 am
Hi,
I just want to say that after reading the above comment I don’t feel so alone. I live in the UK and my daughter is awaiting cardiac surgery at present, as you understand I am terrified.
Reading your comment, you could be talking about my daughter Sophie, she is also nine years old and has experienced the exact same symptoms as Bill with a recent deterioration in her condition.
I have to say tht we had not noticed a change in her symptoms so it came like a bolt out of the blue when we were told in May that Sophie needed surgery. Is is so hard to make the decision to opt for this knowing that the mortality rate is 5%.
As you know the condition is so rare that very few operations have been carried out at Birmingham Childrens Hospital, although we have also been told that the success rate is 100%.
I can’t advise you what to do, we are going ahead with the planned valve replacement but it is not a decision made easily. I have to be honest and tell you that I feel guity at making the decision and want to change my mind on most days.
Stay in touch and keep me informed on decision.
With very best wishes, Jill
Comment by Jill Garrett — November 19, 2009 @ 3:04 pm
Hi Jill (and Bill’s dad)
My goodness your stories are so similar to ours. My son also reached 9 years old with a severe misplacement of the tricuspid valve, asd and enlarged heart and we were suddenly told that he needed surgery when as far as we were concerned he had been a bit blue at times but otherwise seemed fine and had been biking up hills the week before. We were so, so scared – it felt like I couldn’t breathe for months – but he had his surgery, in Southampton Hospital (Wessex heartbeat), and despite a few tough days in intensive care, it has been really good. He still has a moderate regurgitation but despite this is very fit and well and plays team sports for his school.
I hope that things are going well for you and if I can help let me know.
Best wishes
Emma
Comment by Emma — February 1, 2010 @ 7:18 pm
Hi there,
We live in Tulsa, OK. I wanted to add my comments, as my experience has been as Caroline wrote — not to do the surgery on “well child” and wait until it’s really needed — not elective. My daughter, Marjorie has always been “looks good see you next year” for almost 12 years. We have always had conservative cardiologists — Seattle, Minneapolis, now Tulsa, but this past July, it was obvious she needed surgery. Her pulse ox went from a steady 94-95% (her entire life) to 88%. She would get winded running 5 yards. It was a terribly difficult time, but her color was off and her heart was moderately enlarged, as opposed to slightly. We did research the surgeons who did the procedure and were very confident in them (one was from Dallas, one from Chicago Childrens). Since this is such a scary time for a parent, I just wanted to share Marjorie’s success with you. The day after her surgery (they were able to tuck in the ventricle and re-flip a flap — took them two tries), she told me, “Mom, even though I’m really sore and hurt, I actually feel better.” She sometimes takes off running because now she can. Know that you aren’t alone, and I wish I had known of this site BEFORE her surgery. Good luck and my best to you all. Keep us informed, and you take care as well, Jill
Comment by Anne Sheaff — February 1, 2010 @ 11:50 pm
Hi everyone,
I’m so glad I found this site. I’m having second thoughts on my daughter’s coming surgery in April. Currently my daughter, Janna, is 4.5 years old and showing no symptoms related to her heart condition (she has Cerebral Palsy and yet to walk so maybe that’s why we don’t know if she does get tired easily). She has mild EA but with severe tricuspid regurgitation.
The doctors always said that Janna might need a surgery when she’s older (between 8-12 years old). So, when we came for her annual checkup last September, they suddenly told us that they’ve already took her case to a conference with other surgeons and agreed that she should have the surgery in March (later postponed to April or May), we were in utter shock. And as the time getting nearer, I’m getting more and more nervous about it.
We’re in Malaysia and she’s going to have her surgery at the National Heart Institute (apparently the best hospital here for heart procedures). Her procedure will be done by the head of cardiothoracic surgeon there (I’m assuming he’s also the best since he’s the head surgeon).
But, I’m still worried sick about this as I keep on thinking about watching her in pain post-surgery.. she’s still too small to bear the pain but old enough to speak & act about it. Would appreciate some advise. Thanks
Comment by Fiz — February 11, 2010 @ 10:21 am
We live in Wales and our litttle boy was diagnosed at two days old. Alhough he spent his first year on amiodorone for his fast heartrate but since then has had no medication or treatment. He has check ups every 18 months and is now 9 years old. He shows no symptoms whatsoever and we so hope he continues well, he is very active. It’s interesting to note quite a few people have noted symptoms appearing at this age.
Comment by lisa — February 22, 2010 @ 4:11 pm
Hi Fizz and everyone,
It’s been a while since my last posting, just before Sophie’s surgery in November in fact.
I have a long tale to tell and will try to make it brief. As you all know I found it difficult to consent to Sophie having surgery as she had appeared well to us and the consultants decision about the need for surgical intervention came completely out of the blue.
Sophie being in intensive care was awful and she had a few setbacks during that week. I beat myself up constantly about giving consent for surgery. After this she gradually recovered and was discharged from Birmingham Childrens Hospital just before Christmas.
I have to say that those few weeks were the worst in my life but as I was told by one consultant while Sophie was in intensive care, really I had no alternative to make this decision.
Sophie’s heart was in a worst state that than the tests showed and aswell as a tricuspid valve replacement, she had her ASD closed and a Cardio-pulmonary shunt. I just cant believe that she went through all this when I look at her now. The scars for all of us are emotional but they are healing.
Sophie did develop a fast heart rate a month following surgery but this is well controlled by medication.
This site is so valuable for us all to explain how we really feel,I know that it seems like we are going mad at times so lets use each other. The surgeons look after our children for a brief time only, they can’t really understand how it is for us as parents.
I know that I made the right decision for Sophie to have surgery, she is healthy and happy and no longer has blue lips and cheeks.
I wish you the very best of luck Fizz. Please don’t hesitate to contact me is I can offer help in any way.
Love to you all, Jill
Comment by Jill — February 24, 2010 @ 2:33 pm
Hi All,
I am so happy i found this website, and after reading all the stories, it is striking how similar they are with our son Michael. Michael was diagnosed with EA when he was born, and Amioderone was the life saving medicine during his first year. He is now 11 years old, and has been living a very normal life. He plays baseball and tennis. He does tend to quickly tire if doing a lot of running, but other than that shows no symptoms. All of this while he has severe right atrial dialation, and severe regurgitation. We are blessed to live in Boston, so local to some of the best doctors in the world. I am told there have been ~30 Cone Procedures done in Boston in the past 2 years with no fatalities. Michael will be having his 2nd MRI this week, and we will be discussing the Cone Procedure with our cardiologist and hopefully the surgeon after we get the MRI results.
Joe
Comment by Joe — September 20, 2010 @ 2:53 am
Hi Joe, wish that all is smooth and well.
It has been 10 months since I last updated. So far, my son never went for surgery.
Previously we are still in a dilemma of whether to send him for surgery in Nov 2009. We have even engaged the best surgeon here in Singapore. To save costs, we decided to approach the polyclinic (a government clinic) here to get referred to National University Hospital (NUH) in Singapore where the surgeon is practising there. By doing so, we will only be charged subsidised rate.
As we are now under government subsidy, everything will have to go according to process. This would mean that my son would have to go through all the checks right from the start again. As we waited for his appointment, we decided to let him try on some nutritional supplement that claims to be good for the heart as well as help him build up his immunity.
We waited for a few months for his appointment and finally he went for his exercise testing. After the exercise test, the doctor told us that our son is doing fine like a normal kid. Compared to a normal kid, he has no difference and is considered above average performer. As for the risk, it is high because he has a abnormal heart. When we were seen by another surgeon, partner of our intended surgeon, he told us that he had seen many such cases in UK and not necessarily all needs to be fix. Even if fix, there is nothing called perfect. He said that the cardios and surgeons would decide in a conference whether to perform this surgery based on the data gathered.
Another few months of waiting past as we waiting for the doctors to convene a conference. All this while my son is taking this nutritional supplement. We were called up to go back for review and the head cardiologist of NUH told us that there is no need for my son to be operated for the time being as he fairs well. No point performing surgery on someone who is coping well with possibility of making it worse even if it is a successful operation.
So far, my son seldom complains of any discomfort in the chest and no signs of tiredness. In fact, he seems to be getting better than ever. Sometimes I wonder if the nutritional supplement really helps here or is it because we are lucky till now.
Comment by kohbh2000 — September 21, 2010 @ 3:47 pm
I can’t believe others out there know what I’m going through. My daughter Isha was diagnosed with EA while I was pregnant. She was born blue and stayed in ICU for 8 days. Her oxygen level was at 96-98 for her fist year. Then she had the PDA closed the day after her 1st B-Day and the oxygen level dropped to 86-88. she’s now 5 years old and although she’s never had a lot of problems her oxygen level keeps dropping. Now she’s at 79-81. Cardiologist (at San Diego’s Children’s Hospital)wants to do heart surgery to correct the valve and close the ASD. She also has an arrhythmia and tachycardia. Surgery is a frightening thought. She seems fine to me and she’s so petite that I’m very scared for her. I want to help her have a normal life but I’m afraid of loosing her.
Comment by Maria — October 27, 2010 @ 3:28 pm
Make sure they want to do the Cone Procedure, it is the newest most successful treatment to EA.
Comment by Joe — November 1, 2010 @ 4:15 pm
For UK readers – we were recommended Great Ormond Street by our cardiologist in Warsaw, Poland (we are living here on an expat contract for my husband’s job and this is where our daughter was born and diagnosed. The medical staff is extremely competent but facilities and nurses lack TLC!) as being the best place for Ebstein’s in the whole of Europe. Our daughter was seen last July and the head of cardiology recommended to wait as long as possible (i.e. as long as she is not suffering from her condition don’t operate) and if/when an operation is envisaged then the current most recent and effective technique is the cone technique which he confirmed could be carried out at Great Ormond Street.
Comment by Celine — January 19, 2011 @ 10:45 pm
Reading all of your experiences, I found that mine is slightly different. My daughter has EA and WPW (Wollf Parkinson White), however she was not diagnosed until she was 18 years old. At this time, a terrific surgeon from Los Angeles came to the city where I live (35 miles southeast of LA) and did what is called a Radioablation procedure in the Radiology Department with a
3-pronged tubing in the femoral artery. The first to stimuate the heart to go into SVT’s (beating very fast), the second to look for areas that needed ablating and the third to ablate the areas of thicker tissue below the tricuspid valve. She was awake off and on, as she was not put asleep for the procedure. The procedure itself lasted almost 8 difficult hours, when we were told it would be 2-3 hours. It was the worst 8 hours of her life because the probe that stimulated the heart was very painful. She told me that she would never have surgery again. She is now 31 years old and her recent Echocardiagram showed changes in the pulmonary artery and the valve, which I assume is the tricuspid valve. We don’t have the actual reading from the cardiologist, but both our imaginations are running wild. She has re-affirmed the decision not to have any surgery. I’m just hoping I can convince her to take a medication. As I write this, I am dying inside. It’s touch enough for a parent to make the decision to allow the surgery, but can you imagine if the decision were taken out of your control? I love her so much and want to know that she will always be with me. I pray that everything is alright with your children, but study up because there can be changes in this condition. By the way, I am an Registered Nurse, because this very same daughter was also a premature baby and I currently work in a busy Newborn Intensive Care Unit in California. My daughter lives in Hawaii.
Comment by Cindy — May 26, 2011 @ 9:00 am
My husband was born in 1958, he was diagnosed at birth with Ebstein’s. Since there was no surgery available at that time and his condition was severe he wasn’t expected to survive past 18 yrs. He survived and was evaluated at the Mayo for a transplant in 2003. They did 4 procedures instead of a transplant and he is doing well. The surgical advances made in just the last 20 years are amazing! As late as 1992 the only option available was a transplant! Good luck to all of the families going through this.
Comment by dee — January 4, 2012 @ 7:07 am