Headrush - The Average Guy

The life, times, and struggles of an Average Joe. And I don't even get to kiss the babe on TV.

Monday, August 29, 2005

Hypoplastic Left Heart Syndrome (HLHS)

What is Hypoplastic left heart syndrome?

Hypoplastic left heart syndrome (HLHS) is a serious problem that involves several parts of the left side of the heart. It is quite rare and occurs in about 1 out of every five thousand babies born. In the United States, about 1000 babies with HLHS are born each year. Two thirds of the babies affected are boys. Most babies with HLHS are otherwise healthy but some have other medical problems including other heart problems, neurologic problems, and Turner's syndrome.

In this condition, for unknown reasons, the left side of the heart does not develop properly while the baby is in the mother's womb. The parts of the heart that are usually affected are the mitral valve (1), the left ventricle (2), the aortic valve (3), and the aorta (4) In the normal heart, red blood returning from the lungs, flows from the heart's left upper chamber called the left atrium through the mitral valve to the left ventricle where it is pumped through the aortic valve and out to the body. In babies with HLHS, the left side of the heart is underdeveloped and cannot pump enough blood to meet the body's needs.

Without treatment, 95% of babies with HLHS die within the first month of life. Treatment means either three heart surgeries during the first two years of life or a heart transplant. Due to dramatic improvements in surgery and medical care, many children born with HLHS now do very well. However, these treatments involve many stressful experiences and risks. The treatments are not a "cure" and the children will need expert medical attention for the rest of their lives.

What are the effects of this defect on my child's health?

Like most heart defects, HLHS does not have an adverse affect until after the baby is born. Prior to birth, the baby's oxygen comes from the mother and the baby's lungs are filled with fluid. Blood pumped from the baby's right side of the heart bypasses the lungs by flowing through a blood vessel called a patent ductus arteriosus. Usually, one to two days after birth, this vessel closes. In a baby with HLHS, closure of the ductus arteriosus removes the baby's means of supplying blood to the body and results in shock and death. If the diagnosis of HLHS is made, a medicine given through the vein called prostaglandin is used to keep the ductus arteriosus from closing until the time of surgery. See the sections on heart transplantation and three stage surgical repair for information on health outcomes after treatment.

Participation in physical activities and sports: Children who undergo surgical repair of HLHS can participate in recreational physical activities but are restricted from competitive and vigorous athletic activities.

How is this defect diagnosed?

Prenatal diagnosis: The diagnosis of HLHS is made by an echocardiogram or ultrasound of the baby's heart and can be made as early as 16 weeks into the pregnancy. An echocardiogram of the heart is done when a possible problem is identified during a routine prenatal ultrasound or because of a family history of congenital heart disease. Left sided heart problems tend to recur in families where one child is affected. Estimates of having another child affected range from 4.5 to 13% (Boughman et al. 1993, Brenner et al. 1989). If there is a family history or if concern is raised during a routine ultrasound, the family is referred to a pediatric cardiology center where a detailed ultrasound of the heart is performed.

Clinical features: Most newborns with HLHS have mild heart related symptoms until the patent ductus arteriosus closes. This usually occurs within 48 hours of birth. Prior to closure of the ductus arteriosus, the baby's lips or fingernails may look slightly blue, especially when the baby cries. Babies with HLHS often breathe fast, have low energy levels, and/or have feeding problems. These changes can be subtle and can be difficult to detect in a newborn baby. When the ductus arteriosus does close, the baby may get very ill very quickly and develop symptoms of shock

Physical findings: The presence of a heart murmur, mild cyanosis (blue color from low oxygen levels in the blood), fast heart rate, and fast breathing rate are the first clues that an infant has HLHS.

Medical tests: The first tests often ordered are an electrocardiogram, chest x-ray and oxygen saturation test. The electrocardiogram may show decreased left-sided forces. The oxygen level in the blood is usually a little low. The chest x-ray often shows a bigger than normal heart and extra blood flow to the lungs. The gold standard for diagnosis is an echocardiogram. Cardiac catheterization is done if there are any questions not clearly answered by the echocardiogram.

How is hypoplastic left heart treated?
Treatment of HLHS means either a three-staged surgical repair or heart transplantation.

Three-stage surgical repair for HLHS

The surgical repair for HLHS is a series of three heart operations done during the first two years of life. The goal of these operations is to rebuild the heart so that the right side can be used to pump blood out to the body. The first operation is done during the first week of life, the second one is done when the child is four to six months old, and the last one is done when the child is about two years old.

In addition to the three operations, two or three heart catheterizations must be done. A heart catheterization is a heart test that is done by a cardiologist with the help of cardiovascular technicians. Soft, thin plastic catheters (tubes) are placed in the large blood vessels in the leg and threaded carefully to the heart. The catheters are used to take pressure measurements inside the heart and to inject contrast or dye so pictures of the heart can be taken. Overall, this is a very safe test and children can go home the same day.

Heart Transplantation for Hypoplastic Left Heart Syndrome

Heart transplantation is one option for treatment of children with hypoplastic left heart syndrome and remains an option even after staged surgical repair. Heart transplantation means that the heart from a child who died is removed and used to replace the defective heart in someone with a very serious heart problem. The first heart transplant was done in an adult in 1967. While early results were encouraging, most people died within one or two years after transplantation because the immune system rejected the new heart. In the early 1980’s, a new anti-rejection drug called cyclosporine became available and proved to be very effective in preventing rejection of transplanted hearts as well as other transplanted organs. In 1985, Dr. Leonard Bailey at Loma Linda University demonstrated that heart transplantation could be successfully done in infants with HLHS and other serious heart problems.

Heart transplantation offers advantages and disadvantages in the treatment of children with HLHS. Unlike children who undergo the three stage reconstructive approach, children who undergo transplant need one operation during early childhood that results in a "normal heart", that is, a heart with four chambers that pumps in the normal way. The risks associated with the operation itself are quite low at most major centers. Operative risk with the three stage surgeries varies considerably across institutions and in some centers it is very high.

Disadvantages of heart transplantation include the lack of availability of donated hearts of a size suitable for an infant. Due to the numbers of children awaiting heart transplantation and the lack of availability, about 10-25% of children die awaiting transplantation. Other concerns include side effects from the life long need for anti-rejection medications, growth problems, and early onset coronary artery disease. Also, low-grade rejection is almost inevitable so that the average "lifespan" of a transplanted heart is about ten years. For more information on heart transplantation, click here.

What is the prognosis for children with hypoplastic left heart syndrome?

The outlook for children with HLHS has improved dramatically. While once uniformly fatal (with 95% mortality by one month of age) survival at 3 to 5 years of age is now about 60%. Most children enjoy a good quality of life without significant developmental problems Although the outlook has improved, decision-making about treatment is difficult due to the potential for suffering and distress associated with multiple surgeries and heart catheterizations and high medical costs, particularly in light of an uncertain long term outlook for the children. Our experience so far indicates that most children will reach adulthood in good health. Some children will need other surgical procedures such as a pacemaker for abnormal heart rhythms. Some children will need a heart transplant at some point in the future. Children are restricted from competitive sports and from very demanding physical activities but can otherwise do all the things that children enjoy doing. Overall, it is expected that these children have a good quality of life: have friends, play, and go to school just like other children.

Stage 1: The Norwood Operation

The Norwood is the first of the three heart operations. It is most often done during the first week of life.

Why is the Norwood operation done?

Since the heart's left side does not pump well, the heart is rebuilt so the right side of the heart becomes the main pumping chamber. After the Norwood, the right ventricle pumps blood to both the lungs and the body.

How is the Norwood operation done?

The blood vessel leaving the right side of the heart called the pulmonary artery is divided. The far end (the end closest to the lungs) is sewn shut. The near end (the end closest to the heart is sewn into the aorta, which is the large blood vessel leaving the left side of the heart. A patch is sewn in this area to make the "new aorta" or neo-aorta bigger and stronger. Now all the blood leaving the heart goes from the right side of the heart through the pulmonary valve and out to the body through the new aorta (1).

A hollow tube that connects the new aorta to the pulmonary artery is sewn in place (2). The tube is called a Blalock-Taussig (BT) shunt. The BT shunt lets some of the blood pumped to the body go to the lungs.

The wall between the heart's two upper chambers is removed (3). This allows red blood coming back from the lungs to flow from the left upper chamber to the right upper chamber. The blood then goes to the right lower chamber and out to the body.

After the Norwood operation

After the Norwood operation, the right heart pumps a mixture of red and blue blood out to the body. It pumps blue blood coming back from the body and red blood coming back from the lungs. Since mixed blood is going out to the body, the oxygen level in the blood is lower than normal. This makes the baby's lips look a little "dusky" or blue and is more noticeable when the baby cries. Since the baby's body is used to a lower oxygen level, it does not hurt him to cry even he looks bluer.

Most babies go home ten days to two weeks after the Norwood operation is done. They often go home on three or four heart medicines. Some babies need to use a feeding tube for part of their feedings, at least for the first several weeks. Babies with heart problems often need extra calories in their milk. Breast milk is very good for children with heart problems as it is for all babies. Mothers of babies with heart defects can breast feed and breast milk can be given in the tube feedings. Supplements can be added to the breast milk if the baby needs more calories.

Parents learn about their baby's care before discharge from the hospital. They will be able to feed their baby and give the medicines that will be needed at home. In this way, parents come to know that they can care for their baby's special needs. Many parents choose to have a nurse visit their home after the baby leaves the hospital. The nurse can answer questions, assess how the baby is doing, and help parents learn more about their baby's care.

After the Norwood, most babies grow well and learn to smile, roll over, and play with their toys like other babies. Some babies have trouble gaining weight and need richer milk to drink. Some are slow to learn motor skills. Babies who are slow to develop often benefit from special help that can be arranged through the local school district, among other places.

The first time into the unknown

You have to understand, this blind-sided us; there was no advanced warning; there was no way to tell that this was going to happen. It was by sear luck that the doctor was on his toes and signs were shown before we went home from the hospital. Many parents go home and notice their baby turning blue, or in the worst case, their baby goes to sleep and does not wake up.

We also had to go on state insurance and were blind-sided by that. After we had spent nearly a month and a half in Phoenix Children’s Hospital we were contacted by this agency and told either we went into their CRS program or Michael would not be covered (Read do this or your baby dies. Though they were CAREFUL not to use those exact words). Gloria assured me, over and over, that nothing would change. We would have the same doctor and care we had before. Do not worry about a thing; it was merely a budgetary and Administrative matter. Little did we know, after a year or so of calling there never was, never is, never will be a woman named “Gloria” working at this organization. Imagine that. Not only did our provider change, but we were moved to St. Joseph’s cause of “politics” and we had to fight to keep the same surgeon. Understand Dr. Teodori is a gift from god and has worked a miracle on our child. Imagine yourself under a knife, your chest and heart is laying open and you wake up to find:

  1. They are moving to you to a new hospital. Not one specializing in your care, but you are a big ticket item and they want your money.
  2. You have all new nurses and none of them really care if you live or die, except for the paperwork involved.
  3. Best party, the guy who saved your life is going to be changed in the middle of the procedure.

Feeling a bit queasy? We were and we were helpless. Any answer we got was “Who is Gloria?” “Our office would NEVER say that.” “Ah that’s a shame, but that’s the way it is” etc. Nothing helpful but a lot of, to bad, so sad, you’re stuck.

Since our son’s life was the issue here, we had no choice. When it came time for the heart catheter at St. Joseph’s, my wife packed the kids up and headed to Phoenix (A 6 hour drive from our town). My wife did write a letter to the powers that be, but you already know how much action we got on that. I am still trying to get the smoke out of my hinny. The second time was not much better, but I was there to be a complete pain in everyone’s side. Meaning I did not just sit there HOPING my son was taken care of, I bug the nurses when the bad dings happened, and I force questions to be answered. Funny how people will actually pay attention to you when you don’t leave them alone, just watch a 3 year old in action, they are masters of it. Okay so here is the short story on our 2 trips to St. Joseph’s in Phoenix Arizona:

First time, when he was getting a heart catheter, he was shoved into a single room made double by a sliding current on the general floor with children who has all type of repertory and other illnesses. You have to understand, heart baby immunities are down any way and you through on a repertory illness and it can kill them. Every Doctor, Nurse or heart care specialist I have ever read or talked to says this is a BIG no no. Yeah the kids can get ill, they will get ill, they are kids, but you DO NOT take a well heart patient and sick them in the general floor and EXPOSE them to UNNECCESSARY illnesses.

What follows is part of the letter my wife wrote to the powers that be. As the event were so traumatic she never quite got the whole adventure retold. I will fill in the blanks from t he point I came in.

I was told by Dr. Vincent’s office that Michael would be admitted to the Pediatric ICU and to report there when I arrived at the hospital. I parked on the 3rd Ave parking garage, carried baby, toddler and three bags all the way to the desk at the ICU tower. Nobody was at the desk. I asked a security guard when someone would be back. They didn’t know. I stopped a nurse that was rushing past. She informed me that admitting was downstairs and around the front of the building. So I lug everything down to the admissions desk. Nobody was at the desk. I use the courtesy phone and let the operator know that no one is at the desk. She connects me with the voicemail of the front desk manager. I leave a message and wait. After a while, I call the operator back and ask to talk to a live human. She connects me with a nurse. The nurse tells me that Michael is to be admitted to the Pediatric Floor, not the ICU. I tell her there must be a mistake because he is supposed to be in the ICU. I realized quickly that this nurse could not help me with this problem so I head up to the Pediatric Floor (don’t forget with 2 kids and luggage). I address my concern to the admitting nurses on the Pediatric Floor, which they quickly dismissed. When I asked again, the called the doctor on call to appease me. And he said Michael was stable enough to be on the floor. This is a problem for two reasons. First, Michael is not allowed to catch anything more serious then a cold. It will stress his heart. Second, Dr. Vincent’s orders said that Michael was to be admitted into the Pediatric ICU, and I tend to go along with the doctor who is doing the procedure to know what is best. I guess her orders were disregarded. There wasn’t much I could do at this point, so we went to the room.

When we got to the room, there was another patient that would be sharing the room with us. I know that the staff can not let me know any information on the roommate, and a language barrier did exist. I don’t mind sharing a room except this room was obviously intended for one patient. The nurses could barely maneuver. If there were an emergency, they would probably need to roll the crib to the lobby in order for more than 2 people to help.

Since I also had my toddler with me, I thought we might go check out the playroom I read about on line. It was closed for remolding. I asked the nurses if they had any toys and after a while the child life person dropped off a box of small toys. He played with those for a little bit but seemed to be more interested in the examination equipment. Day turned to night and I put Michael to bed and hooked up his feeding pump (which I had brought from home).

My wife in no way left the hospital. I showed up a little after this, and camped right in the boy’s room. Mind you things were so tight I had to leave the room when the nurse came in to take his temperature. They attempted to get me to leave on multiple times and finally had me escorted out. Ever feel like you are leaving someone you love to the slaughter?

The following day I played with our 2 ½ year old to allow mom to watch the little guy, who did make it through his first night. Our family showed up and took the 2 year old so we could be with Michael while he went to have monitoring devices shoved up his main arteries in his legs up to his heart. We were not allowed in the room while this “fairly” routine procedure was done, and handed a pager to go to the waiting room. We were told it would take a few hours, 3 or so, and so we settled in for the duration. Well about 1 ½ hours later the pager goes off, and we raced back to the room. No Miiichael….. So some person slid a chair at us and we sat down and waited…. And waited…. We listened to medical talked and how hard the procedure was on the “patient”. No one talked to us….. we waited….. a life time later our son was wheeled out of the operating room, whizzed past us … still we sat… did we see him breathing as he went by… a person came up to us “The Doctor is just about done, she wants to talk to you.”….. nothing….  Turned to my wife and said “Well we haven’t seen a priest yet?” …. We waited…… By now I am sure you can imagine the anxiety, the pain, the confusion, the fear that was going through us….. and still we waited…. The doctor came out with a surprised look on her face (BTW Dr. Vincent was great with us and we have no complaints in regards to her.) and asked us why were still there and not with Michael. She wanted to talk to us in the room and let us know just how good Michael had done. That everything looked good, she did a slight procedure to enlarge one of the stitched areas of his artery, etc. We didn’t have to wait, and no one, besides the Doctor, cared what we were feeling or what was going on with us. We made it back to ICU just before Michael woke up and remained with him until he was released. Which was interesting because at Phoenix Children’s I had someone walk me out to make sure everything was okay, I got through security, and common courtesy. When it came to leave (Mom was nearly unconscious and had gone with friends and family to get some rest) someone stuck there head in and said that someone would be by in a little bit to check me out. After about 3 hours, when I figure “someone” must have come by when I blinked. I picked up my son in his carrier and left.

My boy Michael

Michael D. Szabo
Hypoplastic Left Heart Syndrome (Congenital Heart Defect)

Michael was born February 20, 2004 at Page Hospital in Page Arizona. Up to and after his birth he seemed to be a perfectly healthy boy. The ultrasound imaging showed no problems, his after birth check showed no difficulties, everything looked well. Nearly 8 hours later, things began to change. Michael began turning blue around his lips, feet and hands, a clear sign he was not getting clear circulation. Thanks to the prompt action of his doctor, he was immediately put on oxygen and Phoenix Children’s Hospital was contacted. Because his mother was still recovering from an emergency cesarean, Michael and his father <me> was air evacuated to Phoenix. Thanks to the wonderful care of the Neonatal and Pediatric Intensive Care Units, Michael was diagnosed with Hypoplastic Left Heart Syndrome and was able to receive his first open-heart surgery by the 27th of February.

We chose to have the Norwood three-stage procedure, rather than the other alternatives because we felt it gave him the best opportunity for life and health. The first surgery went well, Michael recovered quickly and was able to come back to us here in Page by mid-March. He was on a feeding tube and oxygen until his second stage surgery in mid July 2004. This surgery began the process of a full bypass to allow him to live a fairly normal life.

Michael came through it with flying colors and impressed everyone. His Pediatric Cardiothoracic Surgeon, Dr. Michael Teodori MB who is based out of Phoenix Children's Hospital in Phoenix Arizona, was wonderful and took great care of our son the whole time. Though Dr. Teodori was wonderful to him and took great care of him, his mother and I refused to leave his side during the 16-day stay. We did not feel the hospital staff was up to the care and needs of an infant. This time we were not at Phoenix Children’s hospital, were we forced to go to a hospital known for it ADULT care facilities. Their infant ICU (Intensive Care Unit: Were you take a person who needs around the clock supervision. You know the place they send people in the movie cause they are near death.) facilities reminded his mother and I of the general ward at Phoenix Children’s Hospital. There were times where I would have to FIND a nurse (remember this is the ICU) to get Michael checked on. There were times when I had to stop people from touching my son for cleanliness and because no one knew if they were suppose to be with him. St. Joseph’s hospital in Phoenix Arizona may be a wonderful ADULT care facility, but I would NEVER recommend it to anyone who was having health care issues with their children.

After being in the hospital most of 5 or 6 month and as I am the type to ask question, the Phoenix Children’s Hospital staff answered everyone with compassion and understanding. I was never ignored, never made to feel inferior, and my questions were answered every time. It was like we were people and someone cared about our child and us. Because I asked these questions and got answers my wife and I were very aware of the sites and sounds around our baby. We could tell a good ding from a bad ding and while we were at Phoenix Children’s Hospital we never had a bad ding go unanswered. This was different at St. Joseph’s both times we have been there.