Sunday, September 30, 2007

The Hybrid Procedure

Here is a bit of information about a new procedure that we may be elegible for. I'm currently weighting the pros and cons, but am leaning towards having this procedure first!

What are some of the more complex conditions requiring surgery?
The patients we're concerned with here are called "single ventricle patients," and that includes those with hypoplastic left heart syndrome (HLHS). Normally, you're born with two circulations: the right heart supplies the pulmonary circulation and the left heart supplies the systemic, or bodily, circulation. Some kids are born, unfortunately, with a deficient ventricle. In HLHS, it's that all-important left one. It can be so small that it's not even visible, and there's no evidence that it's there. Those are children who, 20 years ago, would have uniformly died.

Now, they often live because we perform palliative surgery on them. That may sound like a misnomer, but we call it palliation because it's not a cure. These babies live with their cardiac defect for the rest of their lives, in one way or another. Even under the best circumstances, they will need three surgeries very early on: one at birth (the Norwood), one at 6 months (the bi-directional Glenn Shunt) and one at 2 years of age (the Fontan). On the positive end of this spectrum, a patient can undergo these palliative surgeries and be asymptomatic. On the other end, there are those children who struggle with the one ventricle and who will need a heart transplant or, unfortunately, don't make it through the palliation.

Are there alternatives to the three-stage surgical approach for HLHS?
Yes, there's a hybrid procedure. The traditional way to treat HLHS begins with a Stage I Norwood Procedure, which is major reconstructive surgery laying out the connections in such a way that the right ventricle outflow of blood is directed into the body and pulmonary inflow is enabled passively. That's a lot of surgery and has to be done in the first two weeks of life.
By using the hybrid procedure, you basically establish a stable outflow by implanting a stent in the ductus arteriosus, which connects the pulmonary artery to the aorta. You then band the branch pulmonary artery to restrict some of the pulmonary blood flow. That can all be done without cardiopulmonary bypass. It typically takes between an hour and 90 minutes, as opposed to a six-hour, much more invasive Norwood. Most importantly, you're delaying the major reconstruction until the second stage. The reasoning behind this is that, at that point, the baby is older and stronger and has built up some immune defenses. A further advantage is reducing the number of "pump runs," those stretches of operating time involving the bypass machine, from three to two. Saving that pump run could result in a better outcome.

Why is it called a hybrid procedure?
It's called a hybrid because we're combining techniques that the interventional cardiologist typically uses in the cath lab, such as stenting, with surgical techniques. There are two operators—the interventional cardiologist and the cardiovascular surgeon—working together.

Tuesday, September 25, 2007

A Medical Explanation

Here is some info I found on i village. (we're really learning about this now along with everyone else.)

The Norwood procedure is the first in a series of three open-heart surgeries that gradually improve certain heart defects that are present at birth (congenital heart disease). It is most often used to treat congenital defects in which one or both of the lower chambers of the heart (ventricles) are defective. The Norwood procedure cannot cure the underlying heart defects, but may enable young patients to regain their health. The term "Norwood procedure" is sometimes used to refer to all three stages of the surgeries together.

Each of the three surgeries is done at a different age, beginning from infancy and continuing into the toddler years. The first two surgeries (Stages I and II) are used to temporarily relieve blood flow problems to and from the lungs. The third surgery (Stage III) is used to further improve circulation. The Norwood procedure re-routes the blood flow around some of the defective areas of the heart by creating new pathways for blood circulation to and from the lungs. Despite the complexity of the procedure, many children go on to live a relatively normal lifestyle after completing all three stages. The most common heart defects that are treated by the Norwood procedure are:
Hypoplastic left heart syndrome.

About the Norwood procedure
The Norwood procedure is the first in a series of three open-heart surgeries that gradually improve specific life-threatening forms of congenital disease heart in which one or both of the lower chambers of the heart (ventricles) are defective. The three surgeries are done at three different ages, which are as follows:
Stage I (the Norwood operation) is done soon after birth.
Stage II (the bidirectional Glenn procedure, partial Fontan, semi-Fontan or a staging procedure before the Fontan) is done at three to nine months of age.
Stage III (the Fontan procedure) is done between 18 months and 4 years of age.
The term "Norwood procedure" is sometimes used to refer to all three stages of the surgeries together. The complete three-stage procedure can almost always be performed, and it offers great hope to the vast majority of infants who would probably not survive a month without it.

Stage I for left ventricle defect
If the left ventricle is defective and the right ventricle is relatively healthy, the Norwood procedure turns the right ventricle into the main pumping chamber of the heart. This is accomplished by connecting the aorta and the pulmonary artery with a vein graft from elsewhere in the body or synthetic materials such as Dacron. This new pathway between the aorta and the pulmonary artery is called a Blalock-Taussig shunt. Blood is pumped from the right ventricle and through the pulmonary artery to the lungs and to the rest of the body (via the aorta). The flow of blood bypasses the faulty left ventricle altogether.
Another technique involves making a small hole in the right ventricle and inserting a small synthetic material tube to connect the right ventricle to the pulmonary artery. This may be termed a Sanno procedure.
A component of the stage I Norwood procedure is reconstructing the aorta by sewing the base of the pulmonary artery (main pulmonary artery) to the underdeveloped base of the aorta. Other segments of the aorta may require synthetic material to enlarge the vessel. The pulmonary arteries that go the lungs (right and left pulmonary arteries) are completely separated from their base and receive all of their blood flow from either the Blalock-Taussig shunt or the Sanno procedure. The valve of the pulmonary artery will function as the aortic valve for the rest of the patient's life.
Under normal circumstances, the blood in the right ventricle is oxygen-poor blood returning from the body. However, in addition to creating a Blalock-Taussig shunt during the first stage of the procedure, the physician may also cut away the septum between the heart’s two upper chambers (atria). This allows oxygen-rich blood returning from the lungs to the left atrium to mix with the oxygen-poor blood in the right atrium. In turn, this oxygen-rich blood passes into the right ventricle and is pumped out to both the lungs and the rest of the body via the pulmonary artery and the newly established Blalock-Taussig shunt. After the first stage of the procedure, the right ventricle becomes the main or only chamber responsible for pumping blood to the lungs and to the tissues and organs of the body. A newer procedure has also been developed that avoids use of the heart-lung machine in patients with a left ventricle defect. During this less-invasive procedure, physicians open the chest briefly to gain access to the pulmonary arteries and aorta. They then place restrictive bands around the pulmonary arteries to narrow them and raise the blood pressure in the right side of the heart. Next, they connect the aorta to the right side of the heart with a stent. This creates a right-to-left shunt in which the high blood pressure in the right side of the heart forces blood through the stent and into the aorta. This procedure can replace the first stage of the three-stage process in some patients, allowing them to strengthen before the second and third stages are performed. This procedure is still being developed and is still not widely undertaken
Stage II of the Norwood procedure
Stage II of the Norwood procedure, also known as the Glenn procedure, is typically performed when the infant is between three and nine months of age. It connects the superior vena cava (the large vein that collects oxygen-poor blood from the upper part of the body) directly to the pulmonary artery. This new pathway allows oxygen-poor blood from the head and upper body to flow directly to the lungs, bypassing the heart and defective ventricle. Once the blood receives fresh oxygen in the lungs, it goes back to the heart and is pumped out to the body. At this stage, the Blalock-Taussig shunt or Sanno procedure is removed because oxygen-rich blood can now circulate throughout the body. This is a crucial step in avoiding the mixing of red (oxygen-rich) and blue (oxygen-poor) blood. However, the complete operation is only half-finished because the oxygen-poor blood returning from the lower part of the body has not yet been re-routed. After this stage, the baby will still have a bluish tinge, but will be better able to handle infection and other problems.
Stage III of the Norwood procedure
Stage III of the Norwood procedure is also known as the Fontan procedure. It is performed between 18 months and four years of age. In this stage, both the right atrium and the inferior vena cava (the major vein that collects oxygen-poor blood from the lower part of the body) are connected to the pulmonary artery. Now the oxygen-poor blood from the upper and lower body flows directly to the lungs bypassing the heart completely. The resulting oxygen-rich blood circulating to the entire body will result in a normal, healthy skin tone. Depending upon the nature and severity of the heart defects, some children may be healthy enough after stage II that stage III is not necessary. One variation of Stage III is known as the lateral tunnel Fontan, or a total cavopulmonary connection (TCPC). In this procedure, a patch (made of either synthetic materials or the patient's own tissue) is used to create a tunnel within the right atrium. The tunnel links two major veins, the superior vena cava and the inferior vena cava, and is then connected to the pulmonary artery. A slight variation of the lateral tunnel Fontan is the fenestrated Fontan. In this procedure, a hole is made in the tunnel. This hole allows for decompression of the blood into the right atrium when the pressure within the tunnel gets too high. Later, if the patient has stabilized, the hole can be closed with either a stitch or a cardiac catheterization procedure.
Benefits, risks and survival rates
When the Norwood procedure is complete, oxygen-poor blood can move directly from the veins to the lungs without having to be pumped by a defective ventricle, and oxygen-poor and oxygen-rich blood are no longer mixed.
Similarly, oxygen-rich blood will be pumped to the body by the healthy ventricle. In the case of total cavopulmonary connection (TCPC), the creation of this tunnel lessens the amount of turbulence and inefficient blood flow that occurs.
The recovery from the Norwood procedure can be complicated due to the complexity of the surgery. Most children remain in the hospital for three to four weeks. A smaller percentage may continue to experience problems in the first few months of life. This procedure has a number of side effects. For example, there is increased pressure in the veins because only one ventricle is pumping in the heart. As a result, the body may retain fluid (edema), and there may be some facial puffiness. Fluid may also build up in the stomach or chest. To relieve the extra pressure, a hole is sometimes left in the lateral tunnel to the right atrium.The Norwood operation is considered a complex procedure but survival after each of the three stages of surgery is 80 percent or higher. Long-term survival at five years of age is 70 percent. Once they have gone through all three stages, most children go on to live fairly normal lives, although they must continue with close medical management. However, there are problems that may cause trouble for some patients in both the short and long terms. Up to 40 percent of patients who have had the Fontan operation experience significant arrhythmias over the long term. Those patients require careful follow-up therapy and medical management for this potentially dangerous problem. Another 13 percent develop a syndrome called protein-losing enteropathy in which excessive protein is lost through the gastrointestinal tract, along with symptoms of heart failure. These patients require close medical management throughout their lives.

Monday, September 24, 2007

a description of HLHS from a mom.

After having done research, I understand a lot of the terms used in dealing with this defect, but its much easier when explained in this manner. Taken from a mother who has gone through two o the three surgeries with her daughter...
In HLHS, the left ventricle, which is responsible for pumping oxygenated blood to the body, is underdeveloped. Sometimes it's so small it's basically not even there, other times it is there but not able to perform its function adequately. Normally the right ventricle pumps deoxygenated (blue) blood to the lungs, then it goes back to the left ventricle to be pumped to the body. Since the LV can't do this, in an HLHS patient the heart must be modified. The three-stage surgical procedure reroutes the flow of blood so that blue blood goes straight to the lungs, is oxygenated, and then goes to the heart where the right ventricle pumps it out to the body. Basically the heart is converted to a two-chamber heart. It's a lot to wrap your mind around, but the more you hear it and see drawings of it the more it begins to make sense.The medical field has come a long way in treating HLHS, although it has only been successfully treated for about 20 years. The oldest living HLHS patients are now in their early 20s. I know lots of people with HLHS babies/children, and the positive outcomes outweigh the negative. Here are some links to sites I've found to be helpful:


Welcome to our baby boy's Blog... We've decided to start this blog because our unborn son has been diagnosed with Hypoplastic Left Heart Syndrome. This is one of the most severe congenital heart defects that can happen.
I was 23 weeks pregnant when we were diagnosed with this defect. I'll explain later more of what the defect really is. Before I really had my head wrapped about what was happening to my baby, the doctors were telling me. "This was a once fatal condition, you have a few options... 1. terminate the pregnancy right now. 2. continue with the pregnancy and do nothing, take baby home and let it die within a few days. 3. Staged open heart surgery that will require a minimum of three operations to change the heart from its current conditions to one that can sustain your baby's life 4. Heart Transplant. As I was sent home from the meeting with doctors to be told this news my mind was reeling.. I was going over my choices really unsure as to what really was wrong. I knew deep in my heart, this baby was going to be born unless there was absolutely no chance of survival.
My doctors had scheduled me to meet with the cardiolgists at The hospital for sick Children in Toronto (SickKids) Two days after first hearing about HLHS I was once again letting doctors look at my baby's heart through a fetalecho (ultrasound for the heart) I met with Dr Jaeggi head of cardiology, and he gave me hope. He'll forever be a hero to me for that hope, for without hope, we have nothing. After hearing the same options, Tyler and I have decided we are going to try for surgery. (more to come in upcoming blogs about this) "baby boy" is going to be born in Toronto at Mount Sinai where he will immediately be taken to SickKids where he will be in critical care until his first surgery (a few days of life)

This blog is being created, not only as a journal to remember everything that we are about to undertake, but as well to allow all those who support us and care about this baby a way to get the most up to date information. I'll be doing my best to keep this page up to date (even when not wanting to leave my baby's bedside). I'm sure there will be even more benefits from this blog. but right now its a place to organize my thoughts and prepare... This is one of the most scariest things I will ever do.