Hypoplastic Left Heart Syndrome (HLHS)
What is hypoplastic left heart syndrome?
Hypoplastic Left Heart Syndrome (HLHS) is a congenital birth defect that affects the left side of the heart. HLHS is a condition in which the left side of the heart, which pumps blood to the body, fails to develop properly. It is the most common fatal congenital heart disease in newborns; without treatment greater than 95% die before one month of age. Most babies die by ten days of age if the condition is not diagnosed.
HLHS is not easily defined. Unlike other heart defects like a Ventricular Septal Defect (VSD) or a coarctation of the aorta, this defect is not easily defined. This defect affects the left side of the heart, specifically the left ventricle. It also frequently (although not always) affects the aortic valve and/or the mitral valve. Additionally, this defect has numerous other characteristics commonly associated with it. Because of this fact, there is not any one hypoplastic left heart definition. Instead there is a wide range of defects, any of which, when taken together, can be considered Hypoplastic Left Heart Syndrome.
Until recently (1983) this heart condition was considered lethal. Parents were given the diagnosis, and the baby was taken home to die, often in its sleep. Today, we parents are fortunate to have two other options available to us: the Norwood procedure or a heart transplant. In my opinion, the success that cardiothoracic surgeons are achieving with these two options makes the option to do nothing practically unethical.
Under the Anatomy section of this book are diagrams and explanations of the defects common to HLHS and to congenital heart disease in general. Immediately following this section is a page-length chart which the cardiothoracic surgeon and/or your baby’s cardiologist can use to help show you exactly what defects your baby has.
Did something happen during the pregnancy to cause HLHS?
No. There is nothing that a mother does during pregnancy to cause HLHS. No one is really sure what causes HLHS. It seems to be a fluke of nature.
My child’s pediatrician labeled my child Failure to Thrive. What did that mean?
Failure to Thrive usually occurs when babies are neglected or unloved. This is not the case with a critically ill baby. Babies with HLHS don’t grow for a variety of reasons. Feeding problems often occur because eating requires a lot of energy, and these infants tire easily. The infant has to interrupt sucking in order to breathe and may fall asleep, then awaken minutes later irritable and hungry. Weight gain is poor because the extra work of breathing burns most of the calories they intake. There is no reserve on which they can grow.
What other signs are common with HLHS?
You may notice your baby excessively sweating, perhaps because of the increased work of breathing. Cyanosis (blue skin) is often a sign. Pallor (paleness) is another common sign. At first, the doctor may suspect anemia due to the paleness of skin, but this is a symptom of congestive heart failure. Congestive heart failure is common in children with congenital heart defects.
Tachypnea (fast breathing) is also a sign of HLHS. Tachypnea is rapid, shallow breathing with a persistently fast heartbeat. The reason the breathing and heartbeat are so labored is simply because the body perceives a need for more oxygenated blood. Due to the defects in the heart, oxygenation is not optimal; therefore, the heart works harder, and the baby breathes faster in order to compensate.
Now that my child has been diagnosed with HLHS what are my options?
Once your child has been diagnosed with HLHS you have three options: 1) a series of operations known as the Norwood procedure, 2) heart transplantation, or 3) do nothing and let the child die.
I have specifically chosen not to include any statistics on HLHS in this book. Statistics are changing more rapidly than they can be printed, so any statistic I might quote would be obsolete. The most important “statistic” for you to know is that your baby has a 0% chance of survival without intervention. Due to the fact that your child’s heart is probably not exactly like any other heart anyway, statistics have no meaning.
A more meaningful way for you to obtain numerical data to help you make the decision on which option to choose would be to ask your baby’s cardiothoracic surgeon his own level of success in performing the operations. The best way for you to select your option should not be based on numbers. You must discuss your baby’s special set of circumstances surrounding the diagnosis of HLHS. Any additional problems the baby may be experiencing and your own intuitive sense of what is best for your baby will be you best guide.
The first option, the Norwood procedure, is a series of operations changing the heart from a four-chamber pumping heart into a two-chamber pumping heart. The advantages to selecting this option are: 1) the surgeon utilizes the baby’s own anatomy to redesign it into a working heart; 2) the procedure can be performed as soon as the surgeon deems it advisable; 3) it is a staged procedure which allows your child time to grow before proceeding to the next surgery and; 4) if the surgery is unsuccessful, the baby may become a good candidate for a heart transplant.
The disadvantages to this procedure are: 1) it is a staged or sequential procedure which carries its own set of risks for each surgery; 2) at any given time the baby may become an unsuitable candidate for the next surgery; 3) if any of the procedures do not work, the takedown of the procedure could be lethal and; 4) a suitable heart may not become available quickly enough to save the baby (due to a severe shortage of available donor organs) should the baby be unsuitable for the Fontan procedure. This is a procedure which changes the physiology of the heart so that the heart only pumps blood to the body; the blood travels passively to the lungs. This decreases the blood pressure in the lungs.
One thing to keep in mind is that cardiothoracic surgeons are continually improving and updating surgical techniques so that fewer babies are unsuitable candidates for the Fontan surgery. Now there are a number of different kinds of Hemi-Fontan procedures as well as completion Fontan procedures. Because of this fact, surgeries are tailored to suit each individual. Thankfully, the morbidity (disabling) and mortality (death) rates of these procedures are declining, especially over the past five years.
One other thing to keep in mind is that it is becoming a more common practice to do the Norwood procedure even if cardiac transplantation is the ultimate option selected by the parents. The Norwood procedure is a palliative (temporary fix) surgery which, if nothing else, buys the baby some time. By having the Norwood procedure, the baby will 1) have more time to be on the heart transplant list, 2) have time to grow and 3) give the parents more time to consider what is the next best step for their baby.
Another option is heart (cardiac) transplantation. There are certain advantages to this option, as well. One very promising advantage is the increasing data available on reduction of rejection episodes among neonates (babies 30 days of age and under). More women are finding out during pregnancy that their baby will be born with HLHS which gives them and the baby’s doctor time to do some planning. I do not think it is common practice to put a baby on the United Network for Organ Sharing list until the baby is born. Perhaps, when a mother reaches 35 weeks gestation, the baby could then be put on the list and labor induced should a matching heart become available. This might prevent the need for a Norwood procedure while waiting for a donor heart. Another advantage to cardiac transplantation is that the baby will (hopefully) receive a normal heart. Perhaps the most becoming advantage is that the parents and the baby only have to endure one heart surgery and not three.
While the advantages all look wonderful, they are a little misleading. Yes, the episodes of graft rejection are less in the neonate than in an older baby. But, over the course of years, the heart might be rejected anyway. This means that at a later date the child might have to have another donor heart and go through another open heart surgery. Since successful cardiac transplantation has only been conducted in babies since 1985, there is not sufficient data available to know how many times a person will need additional heart transplants.
One of the most serious disadvantages to the heart transplant option, though, is the use of very strong anti-rejection drugs that the baby must take for life. These drugs lower the body’s immune system. Because of this, the baby is at a much greater risk for infections. Daycare facilities represent a higher risk of infection than home care; therefore, it may be more advisable for one of the parents to stay home full-time or to hire a babysitter for in-home care.
There is also the necessity of moving within a certain distance of a treatment center. If you already live in a large city that has a treatment facility, then this is not a problem. If you live too far away, though, you will have to move, may need to find a new job, new school for siblings and possibly move away from the family and friends who form your support group.
If cardiac transplantation is your child’s only option, do not be discouraged. At least your baby has this option available. Even if you do have to move, you are doing so to preserve your baby’s life. You will undoubtedly find new friends, especially since you will probably be informed of an active support group for transplant recipients in that area. The people in this group and you will share a bond stronger than that making up most friendships.
The last option to be discussed is that of doing nothing. Because of advanced surgical techniques, this is becoming much less of an option. It must still be considered an option, though, especially for certain infants. Usually HLHS occurs in otherwise normal babies. Occasionally, however, a baby may have HLHS and some other debilitating defect. The baby may have a chromosomal disorder, may have been without oxygen too long resulting in severe brain injury or may have some other problem with other organs in the body. Because of the complexity of problems, the baby may not be a viable candidate for surgery of any kind.
You may decide, too, that even if the heart were “fixed” the baby may have very little chance of ever leading a normal life. This might be your only option because some disorders would ruin even a normal heart were it to be transplanted into your baby. Or your own religious beliefs might dictate this option. Whatever the case, if you do choose to do nothing, please do not make the decision before completely understanding the consequences. Make sure you thoroughly understand what the doctors have explained to you. You have the right and the responsibility to ask questions and have things explained to you over and over until you understand. You might also want to wait to make this decision until you have had a chance to talk to somebody from your church or religious affiliation, your immediate family (especially the baby’s grandparents) and, perhaps, the hospital social worker. These people will help you feel better about the decision you make and will be a source of support to you in the future.
Some people refer to the option of doing nothing as “The Compassionate Route.” For a parent who has survived the ordeal of having their infant go through open heart surgeries this expression can cause anger and resentment. To some the phrase may seem unusually ironic because doing nothing means certain death and it is hard to imagine how that can be considered compassionate.
I struggled myself to determine how anyone could consider this compassionate. And yet when facing a series of operations which will never cure a disease, or a radical transplantation of another being’s organ (still not a cure), perhaps this could be a compassionate route. No, argues a voice in my head and my heart. No. Regardless of the struggle, regardless of the pain, this person deserves a chance to live. And so the agony continued. How to make sense of this decision. How to accept loving parents choosing to end their child’s life.
Then I happened upon a poem and a light appeared. Perhaps this could be a compassionate route. If this decision absolved one family’s pain and benefited another, maybe even many people, perhaps it could be considered compassionate. And thus my plea is this: if this is the route you feel must be followed then think of how your child can still live. Your child’s eyes might help another see a rainbow; your child’s skin might allow a burned child to look in the mirror and smile; your child’s organs might let another live a normal life; and your child’s heart might give scientists clues for a real cure to this disease. Thus your child’s life would not be in vain. Your child could live in countless others. Your child’s life could make history brighter for future generations. Thus your child will live forever.
Here is the poem that inspired this philosophy:
“To Remember Me”
The day will come when my body will lie upon a white sheet neatly tucked under four corners of a mattress located in a hospital busily occupied with the living and the dying. At a certain moment a doctor will determine that my brain has ceased to function and that, for all intents and purposes, my life has stopped.
When that happens, do not attempt to instill artificial life into my body by the use of a machine. And don’t call this my deathbed. Let it be called the Bed of Life, and let my body be taken from it to help others lead fuller lives. Give my sight to the man who has never seen a sunrise, a baby’s face or love in the eyes of a woman. Give my heart to a person whose own heart has pain. Give my blood to the teenager who was pulled from the wreckage of his car, so that he might live to see his grandchildren play. Give my kidneys to one who depends on a machine to exist from week to week. Take my bones, every muscle, every fiber and nerve in my body and find a way to make a crippled child walk. Explore every corner of my brain. Take my cells, if necessary, and let them grow so that, someday, a speechless boy will shout at the crack of a bat and a deaf girl will hear the sound of rain against her window. Burn what is left of me and scatter the ashes to the winds to help the flowers grow. If you must bury something, let it be my faults, my weaknesses and all prejudice against my fellow man.
Give my sins to the devil. Give my soul to God. If, by chance, you wish to remember me, do it with a kind deed or word to someone who needs you. If you do all I have asked, I will live forever.
Organ donation is a compassionate act. It is a selfless act of kindness unparalleled by any other a human can do. Donating of oneself completely to assist the lives of others is the pinnacle of “loving one another.” Some people believe that God’s only Son died so that we could be reborn. Perhaps some of our littlest angels die so that others might live. Let us hope that these angels are given to parents who snatch the opportunity to share this blessed life with others so that they might love and live a happier life.
What is the prognosis for a child with HLHS?
We really don’t know the answer to this question. The oldest child who has successfully undergone the three-stage Norwood/Fontan procedure is only 12 years old. This procedure has only been in existence since 1983.
Heart transplantation for HLHS has only been done since 1985. Loma Linda University in California has done much to further the research in this area. “Baby Fae” (a baby girl with HLHS) received a lot of publicity from the media when she received the heart of a baboon at Loma Linda. There is still much research being conducted with regard to human and baboon transplantation for newborns.
Prognosis depends on the complexity of your child’s problem, the option you select to deal with HLHS (transplant or Norwood procedure), other problems your child might have (such as feeding problems or other congenital defects) and your dedication as parents. Although you’ll never see this in any of the professional literature, I feel that a child’s own will to survive plays a key role in his or her prognosis.
Each doctor will give a somewhat different prognosis. This is a controversial area. Each year doctors are revising surgical techniques and have raised more answers and more questions. This is a complex problem without an easy answer. We are very lucky, however, that we do have some options. More and more children with HLHS are being saved every day.
One thing is a certainty: without surgical intervention an HLHS baby will die. With surgical intervention your baby has a chance to live. You may have a chance to see your baby smile, hear your baby’s laughter and share your baby’s love. The number of days we are given is not important. What is important is for us to cherish each and every day we do have. There are no guarantees in life for any of our children whether they have this defect or not. Many children die every day of unforeseen problems, car accidents, choking, poisoning, etc. We have the advantage of knowing that every day is special and that we are blessed for whatever time we have these children sharing our life and love.
*The definition of HLHS at the beginning of this booklet is rather general. Your child’s pediatric cardiologist and surgeon can help you understand the complexity of your child’s condition by marking each of the conditions your child has on this page. In the Anatomy section of this booklet you will find definitions of these conditions.
Definition of Functional HLHS:
**This is copied from “Cardiac Transplantation for Infants with Hypoplastic Left Heart Syndrome” by Mario Chiavarelli in the Journal of the American Medical Association (JAMA) Dec. 22, 1993, v270, n24, p. 2944-2948 © 1993 American Medical Association. I added the Other section because my son had some additional conditions not listed in the JAMA article. I also added the space for checks to make this easier to use.