Hello Web visitors,

A post from one of the first patients in England to have tricuspid valve repair by the cone procedure.

I first looked at this site in September last year, having been told of its existence by doctors at the Bristol Heart Institute. I want to share my story with you for all the same reasons that Caroline and Tim have set up the site.

In October 2007 I went to see my General Practitioner. I was breathless walking up slopes and hills, and exercising generally, compared with friends of similar age and fitness. I was 56 then. My G.P. suggested I may have asthma. He arranged lung capacity tests, an ECG and chest X-ray. A couple of weeks later I had a letter from him saying that, the X-ray showed my heart to be enlarged and misshapen. I was completely dismayed, an enlarged heart suggested heart failure to me.

The radiologist had advised I have an echocardiogram.I went to Southmead hospital in Bristol for this. The technician and consultant cardiologist were excited by what they saw. The first, never having seen the condition and the latter, not for 30 yrs. They were surprised I had been active all my life, and no symptoms recognised when I was young. I was shocked and bewildered. My husband joined me to be shown pictures in a text book; and be told I had a a congenital heart defect called Ebstein’s Anomaly. ” What did it mean? What should I do?” The consultant said I needed to see the GUCH (  Grown Up Congenital Heart defect ) people at Bristol Royal Infirmary. Feeling quite worried I phoned my G.P., clearly consulting his p.c. he then said “you’ve survived well!”

I was seen at BRI on 30th Jan.2008. The echo. and ECG were repeated. A consultant cardiologist discussed the mechanics of  E A with us. He explained that I had mild to moderate regurgitation and there was no question of any intervention. He said my life style was fine, no changes needed, they would see me in 12 months time. Before leaving I was given a GUCH info. file by a cardiac liaison nurse.

Notification of my second appointment came for June 2010. I attended at the newly built Bristol Heart Institute. After ECG and echo. a specialist registrar spoke to us. He asked about my current symptoms. My breathlessness had become much more marked, I had regular feelings of light-headedness and palpitations at times. I was beginning to really believe I had something wrong with me. The Dr. said he’d like to do further investigations, and he’d like me to consider the possibility of undergoing surgery. I was surprised and, of course, worried. Within the following eight weeks I had a 24hr. ECG, a 3D echocardiogram, an exercise test and a cardiac MRI scan. At the end of Aug.2010 I received a letter from B.H.I., saying,multiple heart specialists and surgeons had discussed my case in detail and agreed that I needed the tricuspid valve operated on, and also a pace maker fitted at the time of surgery.

My husband and I subsequently met with Mr. Massimo Caputo, consultant cardiothoracic surgeon at the BHI., but based at Bristol Children’s Hospital. He explained the right side of my heart was enlarged and becoming larger. If the valve was not repaired or replaced there would be irreparable damage and my heart would cease to pump efficiently. Also, the sinus node may well be affected at the op. so an epicardial pacemaker would be placed. Lots of information and the prospect of major open heart surgery, a lot to cope with! Mr. Caputo said he would very much like to carry out a repair method called “the cone procedure”. He explained that Dr. Da Silva had been invited to come to the BHI, where surgeons would observe him operating. The intention, he told us, is to learn and perfect the cone repair procedure for EA so this operation can be available at the BHI in future.

My op. was arranged for 20th Sept. 2010. The pre assessment took about 2hrs. with physical examinations, detailed information from a cardiac liaison nurse, and an opportunity to visit the Cardiac Intensive Care Unit. This turned out to be particularly helpful for my husband, who visited me there, when I was surrounded by machinery, with many tubes and lines coming from neck, chest and arms.( I remember nothing of being in CITCU). I went to hospital on 19th Sept. as my coronary arteries had to checked with an angiogram. Sadly, I returned home that day. Dr. Da Silva was unable to come to Bristol, because the GMC had not dealt with his application. This cancellation was psychologically difficult for myself and family.

My op. was rescheduled for Jan. 13th 2011. I was admitted to BHI on the 12th, my first experience of being an in- patient. I was visited by doctors, the anaesthetist and nurses who spent time asking and aswering questions, and explaining what would happen the next day. Mr. Caputo came with news that Dr. Da Silva had arrived. It was reassuring to see a face I knew. I left the ward at 8 a.m. and my husband tells me I was in CITCU by about 2 p.m. with an individual dedicated nurse caring for me.(I remember nothing of being in CITU).  Mr. Caputo came to CITCU, shook hands with my husband and said everything had gone according to plan and he was pleased  The following day I was moved to the Cardiac High Dependency Unit, where I was encouraged to wash my face at 6.10 a.m.! I was more awake here and believe Dr. Da Silva came with Mr. Caputo to see me. It’s all rather hazy, but I know I met him and thanked him at some point! By the evening of the 15th I was back on the cardiology ward. I felt absolutely awful, and sure I was going to die. I had a very pleasant room all to myself, large window, own toilet and shower. The staff were very attentive. The morning of the 16th arrived, I was so pleased I hadn’t died!

I spent 2 weeks at the BHI. getting better all the time. Doctors came every day and spent time with me. All the staff were patient and efficient. I was stunned by the volume of surgery being carried out there, and the obvious expertise needed by all staff  for such successful outcomes. My chest, at first numb, became painful, my heart thumped, my limbs and abdomen were bloated due to water retention, and fluid collection around my  heart   caused  concern. Though I longed to sleep, getting comfortable in bed was impossible. Food tasted like cardboard. My haemoglobin level had dropped so I had symptoms of anaemia. I was encouraged to take a gentle walk every hour or so, and soon made the acquaintance of fellow patients as we slowly paraded our breathless way up and down the corridor. I was often wired up to a heart monitor, the nurses constantly monitored my blood pressure and oxygen levels. The oxygen supply was a great help to me. I had several echos. and regular ECGs. The possibility of being discharged was under consideration when I developed Atrial Flutter and A/V block. The electrical impulses in my heart had gone awry due to the disturbance of the surgery, not unusual I was told. This was sorted immediately, in a clever way, through my pace maker, but Amiodarone has been prescribed for a while until my heart settles down.

It was so good to come home! I was warned of the short term emotional and physical effects of the op. and I suffered many of them. Mr. Caputo also told me I would feel better for having the valve repair. Well, I’ve had no light-headedness since leaving hospital, I am already walking as well as just before I went in and my legs no longer feel exhausted when I reach the top of the stairs.

The reported prognoses for adults and children who have had  the cone repair procedure is, I understand, amazingly good. It’s a bugger when your body goes wrong, but it’s no-ones fault. I am very fortunate that my condition could be diagnosed, and that something could be done to help me by such willingly dedicated people.

For ethical reasons the Drs. could not tell me much about the other 2 people Dr. Da Silva operated on in January. It would be good to know how you are getting on.

 

My daughter, Alice Eva-June Sagan Krivas was born on March 19, 2011 at 33 weeks, and passed away on March 21, 2011.  My husband Peter and I were aware that she had Ebstein’s Anomaly (at 28 weeks) but were given an optimistic diagnosis.  As it is commonly known with Ebstein’s, doctors were unable to give us much information about Alice’s particular condition until she was born.  After a routine followup (at 33 weeks), it was decided that an immediate cesarean would be necessary, as doctors found she had developed hydrops in her abdomen, chest, and heart.  Less than 24 hours later I gave birth to Alice.  She cracked a few cries and the pediatrician was optimistic.  She held tightly onto Peter’s finger as they weighed and measured her, while giving her oxygen.  It was only decided after a few hours that Alice’s condition was very severe.  A kind doctor from the Children’s hospital here in Montreal informed us that she did not have much hope.  We spent all of the next day reading to her and cuddling, though I did not get to hold her until it was decided that nothing more could be done.  We were given the opportunity to hold her and be with here in a more cozy and private environment for the last 6 hours of her life.  As she passed away, we held her in our arms as I sang her softly to sleep.  This was the most difficult thing I’ve ever lived through.  I wouldn’t wish it on anyone.

As we now look back, our minds are questioning all that happened. We wonder if there was anything at all anyone could have done to save her.  We are desperate but know that it is all too late.  It’s so frustrating to have those feelings.

I decided to join this website because I think it is very important to encourage at least one other ultrasound during pregnancy, as here in Canada women only get one, around 12-20 weeks.  If it weren’t for the many ultrasounds we had (for other reasons), this anomaly would not have been found and we would not have had the time with her that we did.  She would have more than likely passed away inside of me.

What a tragedy.

 

My Future Health

24/04/11

Hi,I”d like to give some information about my illness.And ask questions about it.I m 28 years old.I have the same anomaly Ebstein ,but I have already been operated WPW close operation in 2007 succesfully in Russia.I have no such a problem with my health now, have my check up normally , live as other even sometimes better than they are,play football , sometimes run.What do you think will happen with my health as I get older?