Pat Pearce tells the story of Adam at Left Heart Matters.

My husband and I found out at 20 weeks into my pregnancy that Adam had HLHS. It's funny but everything that we heard prior to going for the amnio was that the 2 weeks waiting for the amnio results to come back was going to be the hardest part of the procedure. WRONG! We actually were dealt with some of the hardest news during the amnio procedure itself. It was diagnosed via an ultrasound taken when we went for our Amnio. We feel we were very fortunate. The ultrasound tech who was on that morning was working double shifts and was rather rushed. It was her job to perform the appropriate measurements on the baby and to locate a spot for the needle to go to get the amniotic fluid. However when she couldn't visualize the left heart chamber she took the time to call in the Chief of Radiology. The Chief of Radiology came into the room and spent another 3 hours trying to visualize the heart structures and basically couldn't give my husband and I the satisfaction that things were OK. I have a cardiac nursing background and my husband used to sell ultrasound equipment so we had a fairly good understanding of what we were supposed to see in a heart. We asked a lot of questions during the procedure but the Chief Radiologist was not giving a lot of answers. He was hesitant about what he was seeing and didn't want to frighten us too much. I spent many years caring for adult patients in Cardiac Recovery, so I knew that when they said that they couldn't see the left chamber and an aortic valve that something big was wrong. In adult cardiology we never deal with those types of problems since HLHS is mainly known in the pediatric cardiology arena. While we drove home I intuitively knew that we were faced with a potential transplantation situation since I knew from my adult cardiology understanding that people can't survive without a left ventricle. Boy have I learned a lot since then!!! A couple friends of the family who are in the medical field actually pressured my husband and I to consider terminating the pregnancy. However after waiting 14 years for this child to come into our lives, and since the amnio results came back showing that the baby was normal, we chose to do everything we could to help this baby survive. We read up as much as we could about this thing called HLHS and began investigating options and names of Pediatric Cardiologists in town. We returned during week 22 to reconfirm the diagnosis via echocardiogram and to have a Pediatric Cardiologist review the echocardiogram with us.

We went in 2 more times during my pregnancy for echocardiograms. The last was done at week 34. These were done to check to see if there were any new developments. They found that the aortic valve did slowly develop somewhat during the 2nd trimester, however due to the babies' position and bone growth, they still weren't able to sufficiently diagnose whether the aorta had a coarctation or an interruption until after birth.

Because we knew that Adam would have to undergo surgery right away, I was able to have family members donate blood to be held for the surgery, have a priest on hand at the time of his birth to baptize Adam, and meet the NICU nurses who were going to take care of Adam after his birth. We attempted a normal delivery, however 20 hours into labor his heart began to show signs of decompensation due to the pitocin drip so we agreed to have a C-section performed. He was born April 14, 1997 at Emanual Hospital in Portland, Oregon. In the resuscitation room the Pediatric Cardiologist performed another echocardiogram while they began the infusion of "Prostaglandin E1" to keep the patent ductus open. He also was able to be baptized before an audience of the entire L&D staff. The medical team had a chance to review the echocardiograms and review final surgical options. I remember that the toughest part of the surgery was having to send Adam off to surgery when he looked so healthy. Because they knew about his problems in utero, they had time to keep all of his organs perfusing and he went to surgery "healthy".

The plan was to stage the surgery so that the team could evaluate in phases if Adam's heart could succeed in maintaining adequate right and left chamber and lung pressures without needing to undergo the Norwood Procedure. This was done by making repairs to other heart defects which he had before attempting the Stage 1 Norwood.

The first stage included putting Adam on the bypass machine, making a repair or patch to the coarctation of the aorta which he had and closing up the patent ductus arteriosis. Once this was done, they went onto stage two which included taking Adam off the bypass machine to observed filling pressures. Adam still had a small ASD and a small VSD but after observing him off bypass they chose not to correct these at that time since the holes between the chambers actually assisted in relieving some of the pressures while keeping sufficient pressures inside the left chamber to help it expand in size. They chose at this point in the surgery not to go to the next stage of the surgery which was the first stage Norwood procedure. While in still in surgery, the team observed the systemic pressures and heart pressures for quite a while before deciding to "close". The overall surgery time took about 4 1/2 - 5 hours. However, Adam was only on bypass for about 30 minutes of that time. He was placed in Pediatric ICU with various IV drips to help maintain pressures. An echocardiogram was done 24 hours later, demonstrating that the left chamber was filling appropriately and that it was expanding in size.

Over the next 5 days the echos continued to show improvement in his left heart chamber size, however the ASD which they choose to not repair during the first surgery was growing in size and this condition affected the output of his kidneys which began to fail. He gained 2 pounds of fluids in those 5 days post op. They returned Adam to surgery where they dialyzed off the excess fluid, closed the ASD, and put in a peritoneal catheter to allow dialysis to continue post operatively. After the second surgery Adam began to visually show signs of improvement. He was weaned off of the IV drips, his dialysis catheter was removed, he was slowly weaned from the ventilator (breathing machine), and moved to the Pediatric unit 10 days after the second surgery. His echocardiogram continued to show a developing left ventricle, and the mitral and aortic valves continued to grow proportionally to the rest of the left side of the heart.

He was discharged from the hospital for the first time 3 weeks after his birth. It took him two days to get used to the quiet house - we played music and kept lights on and weaned him from these stimulations over birth. It took him two days to get used to the quiet house - we played music and kept lights on and weaned him from these stimulations over time. We went back a month post discharge to have a follow up echocardiogram, and again at 3 months later. Both showed that the left chamber was developing - it is now within normal size. The mitral valve is now of normal size, the patch on the aortic valve looks good , the left ventricle is small but within normal size, the right ventricle has reduced in size (atrophied) to become a more normal looking right ventricle, and the aortic valve is still small and bicuspid rather than tricuspid, but they feel that it will be a while before Adam needs to have this replaced. Our next echo is scheduled for February 20th - 6 months after the last one. He will be 10 1/2 months old.

He is now 9 months old, and is a very happy little boy. He loves his music and loves looking and playing with his books. He has 4 teeth and is working on 2 more which our 14 year old cat is not happy about since Adam continually tries to nibble on her ears. He strings syllables together and loves to stand up and sit down. I have a feeling we are only a month away from seeing him walk. He is enrolled in a Gymboree together and loves to stand up and sit down. I have a feeling we are only a month away from seeing him walk. He is enrolled in a Gymboree play class and he is developmentally up to speed with where other children his age are. Only our friends and family know the ordeal that we have gone through over the past 18 months. We continue to pray for support of other "angel babies" and their families who live with the HLHS diagnosis, and pray that in the future all children with HLHS have such positive outcomes.

Pat Pearce