Thursday, May 14, 2009

The News

Yesterday we got the news that we really didn't want to hear. Hope has Hypoplastic Left Heart Syndrome. It was totally unexpected and we are devasted and overwhelmed by the information. This is my first attempt at a blog, but I really wanted to create something that has my thoughts about this diagnosis and how I am feeling. I know my thoughts and feelings will change everyday...they seem to change every hour of the day.

When Jerry and I were told that there was a problem with Hope's heart, we kind of thought 'no big can be fixed'. His sister and a few others have heart murmurs and we fully expected that is what the doctor would tell us after the fetal echocardiogram. It really shocked us and the news is not something either of us were prepared to hear.

Hypoplastic Left Heart Syndrome (HLHS) is one of the most complex cardiac defects seen in the newborn and remains probably the most challenging to manage of all congenital heart defects. It is one of a group of cardiac anomalies that can be grouped together under the description "single ventricle" defects.

Example of a normal heart

Example of Hypoplastic Left Heart Syndrome

1. Hypoplastic ascending aorta and aortic arch.
2. Hypoplastic left ventricle.
3. Large patent ductus arteriosus supplying the only source of blood flow to the body.
4. Atrial septal defect allowing blood returning from lungs to reach the single ventricle.

In a child with Hypoplastic Left Heart Syndrome, all of the structures on the left side of the heart (the side which receives oxygen-rich blood from the lungs and pumps it out to the body) are severely underdeveloped.

The mitral and aortic valves are either completely "atretic" (closed), or they are very small. The left ventricle itself is tiny, and the first part of the aorta is very small, often only a few millimeters in diameter.

This results in a situation where the left side of the heart is completely unable to support the circulation needed by the body's organs, though the right side of the heart (the side that delivers blood to the lungs) is typically normally developed.

Blood returning from the lungs to the left atrium must pass through an atrial septal defect (ASD) to the right side of the heart.

The right ventricle must then do a "double duty" of pumping blood both to the lungs (via the pulmonary artery) and out to the body (via a patent ductus arteriosus (PDA)). The patent ductus arteriosus, a normal structure in the fetus, is often the only pathway through which blood can reach the body from the heart. When the ductus arteriosus begins to close, as it typically does in the first days of life, the blood flow to the body will severely diminish resulting in dangerously low blood flow to vital organs and leading to shock. Without treatment, Hypoplastic Left Heart Syndrome is uniformly fatal, often within the first hours or days of life.

Equipped with the above information we were given the following options:

1. Terminate the pregnancy
2. Comfort care after birth - no medical intervention given and the baby will pass within a week
3. Heart transplant
4. Three open heart surgeries that are typically done in the 1st three years of life

The three-stage procedure is a palliative procedure (not a cure), as the child's circulation is made to work with only two of the heart's four chambers.

The first step is the Norwood procedure. In this procedure, the right ventricle is used to pump blood into the systemic circulation. Since the right ventricle is no longer directly pumping blood to the lungs, a shunt is required in order to pass deoxygenated blood through the lungs. Either the subclavian artery can be connected to the pulmonary circulation (Blalock-Taussig shunt), or a shunt is made directly from the right ventricle to the pulmonary circulation (Sano shunt). The narrow aorta is enlarged using a patch to improve bloodflow to the body.

During this time the baby may be medically fragile and have feeding problems because the heart is working very hard. There is a considerable degree of venous mixing in the right ventricle, leading to lower oxygenation saturations. In addition, the Blalock-Taussig shunt and the Sano shunt both expose the lungs to systemic arterial pressures, leading in the long term to pulmonary hypertension and eventually to heart failure.

The second stage, the bi-directional Glenn procedure or Hemi-Fontan (see also Kawashima procedure) relieves some of the above problems. In this operation, the superior vena cava is ligated from the heart and connected to the pulmonary circulation. At this time, the Blalock-Taussig or Sano shunt is taken down. At this point, the lungs are no longer exposed to systemic arterial pressures, but much lower venous pressures. Although venous blood from the upper half of the body is no longer mixing with oxygenated blood in the right ventricle, there is still venous mixing from the lower half of the body, leading to some degree of oxygen desaturation.

During this time the child may have improved quality of life as the heart does not have to work as hard.

The final procedure, the Fontan (Fontan procedure) completes the repair of the hypoplastic left heart. Although there are several variations, the functional effect is to redirect venous blood from the lower body (through the inferior vena cava) away from the right atrium to the pulmonary artery. Now, there should not be any mixing of oxygenated and deoxygenated blood in the right ventricle. The right ventricle performs the traditional job of the left, supplying the body with oxygenated blood, while the passive systemic venous pressure performs the traditional job of the right, passing deoxygenated blood to the lungs.

The Norwood Procedure is generally performed within a week of birth, the second stage at 3–6 months of age, and the Fontan at 18 months to four years of age. There are two types of Fontan: the Lateral Tunnel Fontan, and the Extracardiac Fontan. When the Fontan Procedure was first being done for children with HLHS, the only Fontan was the Lateral Tunnel Fontan. This requires actual cutting in the heart itself to create a "tunnel" by which the blood can travel passively to the lungs. Within the last decade, doctors have created an Extracardiac Fontan. This operation creates a tunnel outside the heart itself which reduces the chances of Fontan patients developing scar tissue on the heart which might later cause arrythmias.

So you can see that the decision we have to make is very scary and one that we will be contemplating for as long as possible. The major issue we are facing is the time we need to make a decision if terminating the pregnancy is something we are considering...and we are, so we have 2 weeks to decide.

I know there are a lot of people that would not be able to contemplate, let alone make a decision to terminate a pregnancy, but we have to talk to the cardiac surgeon and see if Hope is even stable enough to make it through the 1st surgery. She is also missing her left kidney and that makes it much harder for her recovery with open heart surgery.

I really hate thinking about our little girl and how much she may have to go through in her life and I spend the day wondering if it is 'fair' to her to put her through the pain and lifelong medications and more than likely the heart transplant she will need if she survives. I also have to consider Jerry and Paul and how their lives will be changed by having a sister that is in and our of the hospital, or they would feel if she passed away. It is a heart-breaking time for our family and we are devasted by the decision and the impact that decision will have on all of our lives. I cry all day and all night. I find myself not eating and definitely not able to sleep. I know it has only been one day since we found out, but a day can change your life forever.

1 comment:

  1. I just read your post from Hope's 20 month bday (sorry im so late) and I decided to read this one. I just wanted you to know that I dont think it makes you a bad person at all for debating whether or not to terminate the pregnancy. Before having David we terminated a pregnancy. We found out she had Anencephaly. A neural tube defect where her skull and parts of her brain did not form properly. There was 0% chance of her surviving. We decided to terminate because we did not want to put her through any pain after she was born, and we did not know what kind of toll it would take on our family. I thought, "how could i possibly take care of my son if I was mourning the loss of another child?". I regretted that decision almost immediately after making it. Having David made me regret it even more. Anyhow, I just wanted you to know that it does not make you a bad person because you thought about termination. You're a wonderful mother who puts her children before herself. We all have to make decisions that we feel are best for our family. It's very easy for people to say what they would do in that situation, but truth is, you never know what you will do until you're faced with it yourself.