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Repair of Tetralogy of Fallot

danjou family picture

This story is about my friend, Shari's son, Michael. Shari and I met each other in the hospital, when my 3rd baby was recouperating from open heart surgery.

Tetralogy of fallot is a common congenital heart defect, and thankfully one that can often be successfully repaired. Shari has done a wonderful job of documenting Michael's experience, and I'm pleased that she has graciously allowed me to share their story with you.

I had a normal pregnancy without complications. Michael was born 2 weeks early. While being monitored, Michael's heart rate dipped to 70 a couple of times, and they lost the heart rate at one point. In a panic, after twelve hours of labor, I was induced (Pitocin). They thought the baby was in distress so they upped the Pitocin drip and I delivered at 6:05 p.m. We stayed 2 days and went home.

At the 10 day check-up and circumcision the pediatrician told me he had a heart murmur, probably a VSD. He referred us to a pediatric cardiologist who sent us to Children's Hospital Oakland for an echo and it confirmed her theory. Our little boy had moderate Tetralogy of Fallot. ToF consists of 4 components: The first major one is a ventricular septal defect (VSD). This is a large hole (his was roughly about 1/2 inch) between the two ventricles that lets venous (bluish) blood pass from the right ventricle to the left one. From there it goes to the aorta and on to the body without passing through the lungs to be refreshed with oxygen. The second major component of Tetralogy of Fallot is a stenosis (narrowing) at, or just beneath, the pulmonary valve. The narrowing partly blocks the flow of venous blood into the lungs. This varies in severity from child to child (his being moderate). The last two components are the right ventricle is more muscular than normal, and the aorta lies directly over the VSD (ventricular septal defect). The operation involves closing the VSD and removing the obstructing muscle. If the pulmonary valve is narrow, it is opened, by trimming away the muscle above and below the pulmonary valve. Our world was turned upside down. The pediatric cardiologist at Children's suggested open-heart surgery by 3 months of age to correct this congenital heart defect. He never had a tet or blue spell or any sign of having this condition, so it was a complete shock to us. We thought he was fine. We kept him away from people so he wouldn't catch any infections before the surgery. His sats were in the low 90's.

On September 1, 1998,Michael was admitted to Children's Hospital Oakland. This is a day that will be etched in my memory. Our lives changed forever. We had to be there by 6:15 a.m. His last feeding was at 4:45 a.m., with surgery scheduled for 7:45 a.m. It was very difficult to hand your child over to someone knowing what they were about to endure. We put him in little scrubs and handed him to a nurse in the holding area. Then we talked with the surgeon, Dr Hanley. I wondered if we would ever see him alive again. Our little boy left us with a smile on his beautiful face. It takes the anesthesiologist about an hour to put all of Michael's lines in.

We went to the surgical waiting room on the fourth floor with tears streaming down our faces. We waited for updates from the cardiology nurse, Susan. Dr. Frank Hanley performed the surgery. He is based at U.C.S.F. but travels to the local hospitals to perform his early, lifesaving surgeries that he and his team pioneered.

When we handed Michael over to the nurse, we were under the impression that he would be "out" in minutes. Later, however, we found out that he was given Chloral Hydrate which had an opposite effect on him (it made him really irritable instead of relaxing him).During the surgery they would make a long incision down the middle of his chest and open up the ribcage. The surgery required the atrium to be opened also to do the repair. Our first update came about 9 a.m. as they had just started surgery. At about 11:00 a.m. all was going as scheduled so we waited and waited. It was taking longer than expected. Then Susan (our nurse) came with such a negative look on her face (I knew it wasn't going to be good news). She said that things weren't going very well and please follow her. I couldn't even feel myself walking with her as we went to the 2nd floor Cardiology office. She said they were having complications. Our hearts fell to the ground. Dr. Christian Hardy, Chief of Cardiology, and the social worker met us there. The surgical team was having trouble weaning Michael off of the heart-lung bypass machine. They had already tried 2 times, unsuccessfully. They were going to let him rest for another hour and try again at 2:00 p.m. I was ready to faint, and I had to lay down and they took my vitals. We waited. There was talk by the surgical team of hooking Michael up to a machine called ECMO, a type of bypass machine that would take up a whole bed space next to his bed. It would give his heart a rest. However, he could only be on this machine for 7 days. Fortunately, they were able to wean him off bypass on the third try. He's such a little fighter. Michael was on bypass a total of 4 hours, and they had his heart completely stopped for 90 minutes. They said his VSD was 11 mm.

At around 4:30 p.m. they wheeled Michael to P.I.C.U. There we spoke with Dr. Hanley and met Dr Fenton, Dr. Hanley's surgical assistant. Dr. Fenton stayed with Michael until the next morning. She sat at the foot of his bed monitoring him all night. He was in critical condition. He was on the ventilator, had 3 chest tubes, a stomach drainage tube, and external pacemaker along with several (7+++) little boxes (medication drips),which administered medications systemically. We were so happy to see him alive, even though he had so many lines going in and coming out of his tiny little body. He had several drips and monitors. He was pale, cold and swollen.

Michael had to have a few blood transfusions during and after surgery ( I gave one unit myself hoping that would be all he would need, but that wasn't the case). He was heavily sedated the first couple of days. We didn't realize at the time just how critical he was. The machines kept him going. When they turned down the external pacemaker each day his own heart was a flat line. When he was in pain, we would notice a grunting noise under his breath. He couldn't cry while on the vent. We were by his side 24 hours a day, 7 days per week.

Michael's total correction went well thanks to Dr Hanley, his assistants (Dr. Fenton) and the many doctors, nurses and cardiologists. Our pediatric cardiologist., Dr. Newkumet, was on vacation so Dr. Culbertson cared for Michael after surgery.

Day 3 was the hardest. The stomach drain tube was removed, so was the vent. Michael was able to have a bottle of sugar water that night and he guzzled that right down. He also accepted some bottled breast milk and a pacifier that night. Before surgery he wouldn't accept any type of bottle or pacifier. Thank God he took that bottle, because they thought about putting in a feeding tube. He was strictly a breastfed baby. He was alert but still receiving Morphine (he became slightlyaddicted to the pain meds.). He would have a fever for a few days following each of the two surgeries.

I kept a diary of daily events. We drove ourselves crazy trying to read the monitors and what they meant (even though we were told not to pay attention to them that they would drive us crazy). We could tell when something didn't seem right and we brought it to someone's attention. We never left his side.

After a week off of work, Dad went back to work and took care of our 4 year old son. I did the day shift and grandma did the night shift staying with Michael continuously. After the ventilator was taken out, I was able to breastfeed. Michael would not take a pacifier or bottle (he was 3 months old at the time). When I was able to nurse him, I would slide Michael over toward the edge of the bed, on his cushion, and lean over him so he could nurse. It was a little uncomfortable, but it worked, and Michael loved it.

Over the next couple of days we were told his blood was too thin so they would have to transfuse him with fresh, frozen plasma, platelets and give him vitamin K. His liver was distressed from the surgery and machines. In time, that corrected itself, and the liver started functioning properly. They had to remove his arterial line early because of excessive bleeding. Michael would not accept anymore bottles so I continued to lean over the edge of the bed and feed him. He was a happy camper. They removed one chest tube (ouch). His blood was thicker and there was less drainage so they were able to remove the other two chest tubes. Michael was back to kicking and smiling. Great to see that happy face again.

We were allowed to change his diaper when the chest tubes came out (as long as we saved them so they could weigh them). We asked if we could take his temperature in his armpit, because whenever the nurses pulled the thermometer out of the armpit, he would scream. We would lift his arm and remove it carefully (it usually sticks to the skin in the armpit). They also measured the Tylenol and let us give it to him orally. We also learned how to give Tylenol, rectally, in suppository form. Michael liked suppositories best, since he wouldn't take anything by mouth, except breastmilk.

On approximately the eighth day, the external pacemaker was removed because he was in normal sinus rhythm. We thought we were going home that day. He had his first echo (day 7 after surgery) done that day also, and it showed a blood clot in his right atrium. They started him on low molecular heparin shots, 2 times per day, to try and dissolve the clot. They could break the clot apart, but there would a risk of having a stroke. All Michael wants to do now is breastfeed. It really comforts him. He lost 2 lbs. and gained one back by the time we left the hospital.

We rented a room at the Family House across the street from the hospital. We didn't spend much time there, but it was nice to be able to take a shower and change there. The charge was $20.00 per day.

After we were released from P.I.C.U., we went to M.C.U. to recoup. They were always drawing blood. On the 12th day after surgery, and since Michael was having some blood clotting problems (this can happen after transfusions; he was also anemic), the lab wanted a large amount of blood for tests (11 cc). The doctor on-call (not a p.c.) decided she would take blood from our Michael's artery in his groin (with no pain meds). The blood kept clotting during the first try, so she said she would try the left side of the groin (his arteries were very deep and he was a hard stick also). We demanded some pain meds (he got Tylenol, Benadryl which can constrict the vessels, and a cream to numb the area). After this try, our baby looked like he was in shock, sweat beads on his forehead, pale and motionless. On Sunday, September 13, at about 5:00 a.m. (5 hours after his blood drawing experience), a nurse noticed Michael didn't look right. Michael's heart rate dropped from 140 beats per min to 60 as he went into 2nd degree heart block. We returned to P.I.C.U. and his body tolerated the low heart rate, but he was cold, pale and clammy. We felt as if we were losing him all over again. We thought the worst was behind us. They kept the crash cart close to his bed that night. He was scheduled for surgery to implant a pacemaker the very next morning. They couldn't wait any longer. At 12:30 p.m., on Monday, Dr. Thompson, Dr. Fenton, Dr. Avasarala, an electro physiologist, and a team of surgeons implanted a permanent pacemaker in his tummy. They had to completely open up the old incision and sternum to stitch the 2 leads (one to the atrium/one to the ventricle) on the heart. This time they put in 4 wires instead of 3 to hold the ribcage together. The doctors said there was too much scar tissue from the first surgery to get the leads on correctly without completely opening his chest again, so he was reopened to do this which was a shock to us. The pacemaker was placed between the muscles in the stomach area. The surgery ended about 3:30 or 4:00. All went well, and he was off the vent before leaving the recovery room. He had just one chest tube this time. The next few days were bad again like the first time. Coming out of surgery he would moan in pain and we knew he needed Morphine. After the first surgery while he was on the vent, we couldn't hear him cry. We only saw the crying expression on his face. Michael became very anemic after this surgery.

On September 15, it was discovered that Michael was very anemic. The doctors don't want to transfuse him again, so he is put on liquid iron and 1/2 baby aspirin to keep the blood thin. On Saturday, September 19, another echo was done to check the status of the blood clot. Guess what, it's gone!. This is the first good news we've had since we've been here. The heparin shots helped to dissolve it. We got to go home on Monday, September 21, after 21 days in the hospital. Yahoo!! It was a little scary not having any doctors, nurses or monitors to help watch over him at home. We did have a visiting nurse come to our home 2 days per week to check on him. What an experience this has been for all of us. The whole ordeal has changed our lives forever. We're sure glad we were able to come home with Michael. He came home on two medications, 1/2 a baby aspirin and iron for anemia.

On October 4, Michael developed a staph infection in the top of his incision under his chin. His incision is about 5-1/3 inches long. Strong antibiotics taken for 10 days took care of the infection. A suture came up and out of the incision, also. He seemed to need the lights on in the bedroom when he slept. I guess it's because he was used to lights and lots of noise in the hospital.

As of October 15, Michael weighed 14 lbs. 11 oz. and his heart is beating on its own, 100% of the time. The doctors said they don't know why he went into 2nd and 3rd degree heartblock, since his own heart rate returned to a regular rhythm a week after the first surgery. Dr. Hanley said as soon as the heart rate returns to normal and they are taken off of the external pacemaker, the heart usually never goes back into heart block (.01% does) and that was us.We would like ot thank everyone for their support and encouragement. We couldn't have done it without them. Big brother Brandon did great through the whole ordeal, and we couldn't have made it without grandma who stayed by Michael's side through it all. Many family members and friends visited, watched and waited with us. I would like to thank Dr. Hanley for pioneering this new early repair technique in infants and neonates with incredible results. In our eyes he and his team are the best in the World. These surgeons are performing miracles today. I wish all the surgeons in the World could share their techniques with each other so that every doctor would be able to perform the best, up-to-date surgery on our children.

Updates

During our November 1998 p.c. appointment Michael had a residual leak between the 2 stitches in the VSD where they patched it with his own heart tissue (pericardium). His pulmonary valve gradient was 26 when we left the hospital and it was now 21 to 24. He weighed 15 lbs. 10 oz. with a diaper on. Michael has to take the aspirin and iron for one more month. His atrial is pumping fine, but the pacemaker was pacing the lower chamber 90% of the time. So the doctor adjusted it so his heart can do it's own pumping. The pacemaker was overriding his own normal sinus rhythm, because it was set to high. The pediatric cardiologist will see him in 2 months.

During our January, 1999 visit, Dr Newkumet had Michael wear a holter monitor for 24 hours to find out how often his pacemaker was pacing his heart for him. We learned he wasn't pacing during his waking hours, but when he slept he was pacing 5% of the time because his heart rate was dropping down to 65 bpm during sleep (this could be normal for Michael while he sleeps). The p.c. feels when he is a toddler the pacemaker will probably be taken out. Surgery won't be fun, but we have to do what we have to do. We also found out the leak between the 2 stitches had sealed itself, yeah! Michael is almost 8 months now. He rolls all over the place to get where he wants to go. He just got his third tooth. He's such a good baby, so mellow. Michael has had very frequent ear infections and has been on antibiotics quite a bit since surgery. He is now on a maintenance antibiotic (off and on again).

During our May 6, 1999 visit, the p.c. said the pulmonary valve is growing with him. He is only using his pacemaker 5% of the time (while sleeping because when he's in deep sleep, his heart would slow down to under 80 beats per min.). The p.c. also said the pulmonary artery is a little narrower than the last visit and it shouldn't be. His pulmonary valve gradient is now about 30. So the flow of blood through the valve is not quite as good as last time. The doctor said maybe when Michael's growth slows at about 2-4 years of age this will correct itself somewhat.

Michael was so good during his echocardiogram, ekg and pacer check. We thought he wouldn't lay still. He loved to watch his heart beat on the monitor of the echocardiogram. He got tired toward the end of our 2 hour appointment and slept on the way home. Our next appointment is in 6 months.

Michael weighs 18 pounds and is doing well. Michael is almost a year old and he has started to crawl on hands and knees, pull himself up on furniture sometimes, and he runs behind brother, Brandon, in his walker. He doesn't talk much, but babbles. He has 7 teeth. He loves Blues Clues (on t.v.). His birthday is May 31, he'll be a one year old. The family will really celebrate this milestone. Miracles do happen.

Michael finally learned to walk at 14 months old. All of these milestones we thought we'd never see, have happened. Michael and his brother are the best of buddies. Brandon is always watching over his brother. Michael loves it when Brandon chases him. He giggles and takes off running.

During our October, 1999 visit all went well. Michael is 16 months old now. He had his echo, ekg, and pacemaker interrogation. Michael weighed 19 lbs. 11 oz. (last visit in May he weighed 18 lbs.).

His pulmonary valve gradient (flow through the pulmonary valve) is now 26. O is normal for you and me. We're headed in the right direction, but Michael's gradient will probably never be 0. He could live a normal life with a gradient of 26. The doctor usually refers to the mean gradient figure.

The Pulmonary Valve Annulus (diameter of the valve) is 12 mm today. It was 8 mm at our visit last May. This is great news. That means his pulmonary valve is growing as his body grows. He has a slight valve leak. He can live with this quite awhile before any surgery might be needed. They would replace the pulmonary valve if it becomes very leaky. Maybe when he's a man. But hopefully, this won't be necessary.

Michael hasn't used the pacemaker at all during the past 6 months. They turned the pacemaker down to 50 bpm and in 6 mo. they will turn it down again. Yahoo!!! Snoopy dance!!!!!! The doctor said we can leave the pacemaker in or take it out when he is about 8 years old. It's okay to just leave it in once it is turned down and he doesn't use it.

Our next appointment will be in 6 months for another echo, EKG, and pacemaker interrogation, and maybe once a year from that time forward, just to keep an eye on things. This is really good news, too.

I'm still breastfeeding Michael. It still comforts him and he loves to be close to me. He still doesn't sleep through the night. I have become very protective of my boys (probably over-protective). We wash our hands constantly, don't cough or sneeze on each other, etc. We're very careful. Big Brother Brandon started kindergarten on September 2, 1999. He has adjusted to it and likes going to school now. It was hard for him to leave us. We just thank Heaven for these two little boys everyday!

On April 10, 2000 we went for our semi-annual pediatric cardiologist appointment at Children's Hospital Oakland. Unfortunately, due to Michael's brief stay in the hospital in February for a virus, he wasn't very happy with any tests or doctors. He cried through all of it. This visit was pretty uneventful. His gradient (pressure) through his pulmonary valve went up a little, which isn't good, but he can live a normal life with this pressure. Six months ago it was 26, this time it was 29. He still has a mild pulmonary valve leak and his valve didn't grow this time (it's the same as last time, which was expected). The doctor found out she can't turn his pacemaker off, but she lowered it to 30 bpm (this is the lowest) and it is set to sense so we will get a history of what the pacer will be doing in the next 6 months. Michael will have a future blood test for DiGeorge Syndrome. This condition can go along with his heart defect. The doctor doesn't believe he has it, because he doesn't have the facial features, etc. Michael's speech is a little delayed, but he is making his thoughts and wishes known to us (usually one word). He says lots of one word phrases now, so we know what he wants. He does get agitated sometimes when he tries to communicate, and we don't understand him. We go back for another check-up in 6 months. All in all, everything is going very well and this was a good check-up.

During our 10/9/00 visit, Brandon and Michael were both seen at Children's Hospital in Oakland by Michael's Pediatric Cardiologist to check both their hearts. Brandon had a routine check-up with an EKG and the cardiologist listened to Brandon's heart. She said he definitely had a healthy heart, not even an innocent murmur (yeah). This was great news since they don't know if Michael's defect is genetic or environmental. I just wanted to have him checked out for piece of mind. Brandon weighed 41 lbs. and was 45 inches tall. Yahoo Brandon's fine. When Michael was born he seemed fine also and obviously he wasn't. Michael weighs 22 lbs. and is 33 inches tall. He has only gained a little over 1 pound since our last visit 6 months ago, so everything is about the same as last time. The numbers haven't really changed which is okay because he hasn't had a major growth spurt, if I understood her right. Everything is still mild. He also has a trace tricuspid leak, but that is nothing to worry about at this time. He had a fit everytime they put the leads on his chest for the EKG. He wanted to grab them and pull them OFF! During his echo he was fussy, so the echo tech had me lay on the table with him and as long as he had a Starburst candy in each hand, he was okay. He would hold one and eat lil pieces off of the other and he watched Nick Jr. (Franklin) on T.V., instead of trying to grab the wand. Other than that, he was pretty good for a 2 year old. His pacemaker hasn't been used at all this time and the cardiologist said we can have it taken out when he is 3 or 4 years old because after that age he would worry and be nervous about the whole ordeal. So we go back in the usual 6 months. All in all, it was a good visit. Whew, I'm so glad it's over. I get so worked up over it all. Brandon is doing great at school also.

I have learned so much from all of the parents/friends on the PDHeart List and Hearts for Hearts Sake list. They have helped me become more prepared for each p.c. appointment. I now have a voice and I do speak up and ask lots of questions. I'm a lot more knowledgeable and confident because them.

We went for our usual 6 month check-up on April 9, 2001. Michael was wonderful throughout the whole appointment. He was so cooperative, we couldn't believe it. His pulmonary valve gradient was 33 instead of 34, which is good. Michael weighed 24 lbs. and was 35-3/4 inches tall. The pulmonary valve annulus was 13 mm this time (so it grew) and for the past 1-1/2 years it's been 12 mm. We just found out Michael has no enamel on his back teeth (2 year old molars). The dentist says maybe it's from the antibiotics he was on when he had all those ear infections. The cardiologist said it wasn't the antibiotics he was on in the hospital, but it could be genetic. So we don't know why this happened. Michael will need to have some dental work to save these molars from decaying. The cardiologist also told us that maybe we should wait until he is about 7 or 8 years old and then have the pacemaker taken out. I think she knows we're not ready for another surgery. We hope this decision will be the right one. It's traumatic for a child, when they know they are going to go to the hospital to have surgery. The pacer doctor said it does need to come out in the future. This was a very good appointment. Oh I almost forgot to mention that Michael also has a slight leak of his tricuspid valve. He had this at the last visit also but they said not to worry it is only a trace and it hasn't gotten any worse.

On another note, Michael is almost 3 years old and has finally started sleeping through the night. I've been waiting for this moment for a long time.

Michael has a new baby brother, Matthew, born June 16, 2002 and he's heart-healthy. Michael started pre-kindergarten classes in September 2002 and loves it. He also had 2 root canals and 2 big fillings on all four of his two year molars. In January 2003 he started speech therapy for thought processing (he's a year behind).

June 2004: Michael is doing well. He goes to speech once a week and will be graduating from kindergarten. He has annual cardiology appointments now, instead of every 6 months. He has mild to moderate pulmonary stenosis and he still doesn't use his pacemaker which will be removed in the future. Michael turns 6 years old on 5/31. He currently weighs 31 lbs. and is 41-1/2 in. tall. Michael loves school very much. He also loves playing with his brothers, swinging, riding his bike, reading, playing his gamecube and he really enjoys nature and being outside especially camping.


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