Archive for the ‘Hypoplastic Left Heart Syndrome’ Category

Joshua

October 20, 2010

When Joshua Haskins passed away earlier this month there was a great deal of controversy. This post is not about Circumcision safety issues, but about a related and equally important topic.

Joshua’s mother is heartbroken – she’s supposed to be her child’s advocate, his defender, but now he’s gone and she blames herself. But I don’t – she had been a Heart Mom for seven weeks. There was a lot that Jill Haskins didn’t know, including one critical fact that I didn’t mention.

As regular readers of this blog know, I have a hernia. A hernia can be fixed with a simple operation;  when my dad had his hernia repaired it required an overnight hospital stay, but even that may not be required anymore. The average hernia can be repaired pretty easily, but mine can’t.

My Cardiologist has vetoed a hernia repair more than once, because my blood oxygenation level (the familiar PulseOx reading) is low. A Heart Healthy person will register a PulseOx of nearly 100%; but mine is normally 80 to 82%. “I don’t have any doubt we can put you to sleep,” my Cardiologist says. “The real challenge is waking you back up!”

Not only is anesthesia an issue, there’s more. “I could spend all day describing your cardiac anatomy to the operating team,” my heart doctor continues. “And I don’t know if they would understand it any better when I finished.” That’s quite a statement, considering my doctor has won several teaching awards in his career. I’m 44, and one of the open heart repairs I have had has changed greatly over the years. Another one just isn’t done anymore – there are only three places that I know you can find it: 1) in a musty old surgical textbook; 2) in the brain of a very old cardiac surgeon; and 3) in my chest.

If my hernia were to cause enough trouble that it had to be repaired, I couldn’t just have it done anywhere. I can’t run the risk of having a surgeon who has done this operation 12,759 times before and considers me just another patient, because I’m not. Because of my heart, the plan is to go to Emory University Hospital and have my hernia repaired with a Congenital Heart Surgeon in the room, just in case of trouble. Afterwards they would keep me in the Intensive Care Unit for at least 24 hours – again just in case.

I know this… and I never mentioned it to Joshua’s parents. I don’t recall them mentioning the circumcision to me before it occurred, but that doesn’t matter. I should have said “Look, with Joshua’s heart, there is never going to be a ‘simple’ medical procedure. More than likely he’ll even need antibiotics before and after he sees the dentist, so you really need to think about any kind of operation or procedure that can be avoided.”

But I didn’t say that.

We are each put on this Earth for a reason, to accomplish something. Perhaps Joshua’s task was to remind us all that when you have a Congenital Heart Defect, no medical procedure can be considered “routine.”

Miracles

September 27, 2010

If only you believe in miracles, baby (like I do)
We’d get by

Miracles, Jefferson Starship (1975)

I was concerned about traffic (traffic in downtown Charleston,  South Carolina can be a nightmare; almost every street is One Way) so I intentionally planned to arrive at the Palmetto Hearts Pediatric Cardiology Reunion early. A little too early, as I was the first CHDer on the scene… oops! But the wait wasn’t long, and before I knew it the entire museum was full of Cardiac Kids and Heart Parents. The oldest CHDer I met was a 16-year-old with HLHS (I never got your Facebook Friend request, Johnathan! Try it again, please!) but most of them were under 10 years old.

When you are that young, your heart is roughly the size of your fist. Imagine operating on an organ no bigger than an infant’s fist, cutting it open to find and repair a hole that isn’t supposed to be there. Imagine finding two blood vessels that are measured in millimeters – these blood vessels are connected to the tiny heart but they are in the wrong place, and it is your job to cut them, switch them, and sew them into the tiny hole where the other vessel was.

Imagine looking into the chest of an infant with part of his heart missing. It’s supposed to be the size of a walnut, but part of it is missing, so there isn’t even that much. As my grandma used to say, “There’s not enough space in there to change your mind.” No, there isn’t enough space to change your mind – but there is plenty of room to screw up, to shatter an already broken heart, to ruin a life and a family forever.

I didn’t talk to many of the children – If you were a child, which would you rather do: talk to the adult you don’t know or play with all the cool stuff in a Children’s Museum – but I did talk to a lot of their parents. I heard tales of living in the waiting room.

Staying by the bedside.

Waiting for a miracle.

That’s what I saw at the Pediatric Cardiology Reunion I attended Saturday night… miracles. A lot of them. Walking, talking, miracles.

What you need in a Surgeon

September 15, 2010

It had to happen. Consumer Reports is now rating Surgical groups that perform Bypass Surgery. Just pick up an issue; you’ll be able to find out who makes the best toasters, washing machines, and who can re-route your arteries.

If it were only that easy for Congenital Heart Surgery. There are 35 different heart defects – this is the “base” number, that doesn’t count any defect that may have elements of two or more defects – and a multitude of different surgical options. Consumer Reports only looks at Bypass Surgery, a Congenital Surgeon may repair an Atrial Septal Defect (ASD) in the morning and perform a Bidirectional Glenn Shunt in the afternoon. If he’s on call he may come to the hospital in the middle of the night not knowing what problem he’s facing.

So assuming you have time to pick a Congenital Heart Surgeon, what do you need to look for? Experience and skill are the two basic factors, with a little understanding of what the numbers mean.

Experience is easy – Doc, how many years have you been operating? How many heart surgeries have you done, total? The Cardiologist says my son needs to have the *** Procedure, how many of those have you done? Answers to these questions will help you decide.

Now comes the not so easy part – try to determine your surgeons skill level. Let’s invent a situation: You ask the surgeon how his patients do after the operation, and he tells you that 80% of them go on to lead full lives. Now we need to know something else – what kind of operations is he doing? If our fictitious surgeon is fixing ASDs and 80% his patients “go on to lead full lives”… RUN! That’s a pretty low number. But if he is an Hypoplastic Left Heart Syndrome specialist and he usually performs the Norwood Procedure, 80% is very successful.

This is where it helps to be familiar with the Aristotle Complexity Score, and like so much about heart defects, the general public doesn’t know it exists. Most of the CHD community is even unaware of its existence. The Aristotle Score is a “rating system” of congenital heart surgical procedures, based on the three factors. Each factor is assigned a score of 1 to 5 points, then the points are totaled to find the Aristotle Complexity Score.

The first factor considered is Mortality: Historically, what percentage of patients having this surgery perish during or immediately after the procedure? An operation with a low Mortality receives one point; the highest Mortality receives 5. The next concern is Morbidity. Morbidity is defined as “a diseased state or symptom”, but in this context it is a measure of how long the average patient spends in the ICU after the operation. Again, short stays earn one point and the longest earn a five. The third consideration is the perceived difficulty of the operation. All heart surgery is difficult and requires years of practice and training – if it wasn’t, there would be a surgical center on every corner. But closing an ASD is easier than performing the Arterial Switch.

So every heart surgery is rated, with the “easiest” (a straightforward ASD repair) being rated at 3.0 and the most difficult (A Biventricular repair) receiving a 15.0. For example, my 1967 Glenn Shunt would be scored a 7.0. The Blalock-Taussig Shunt is a 6.8, while the Modified Blalock-Taussig (which uses an artificial tube to make the Subclavian Artery to Pulmonary Artery connection) is considered not quite as difficult at 6.3. The Score does not exist as a “ranking system” of surgeons (almost like Consumer Reports)  but as information. If the OR calls Recovery and tells them that we’re sending down a patient with an Aristotle Score of 10, they know a very ill person is on the way.

So knowing the surgeon’s experience and skill level is really only half the question. If you were to be able to determine how difficult his work is, that could increase (or decrease) your confidence level.

Joshua needs you!

August 20, 2010

Just read that Joshua is having a difficult night. His heart rate, Blood Oxygen level, and Blood pressure all fell. Keep him in your thoughts tonight, please.

Update at 6:51 AM (10: 51 AM GMT): Joshua’s mom posted a message to her facebook page that he had “a stable night.”

You’ve come a long way, baby

August 17, 2010

Jill and Shane continue to report on Joshua. Diagnosed prenatally with Hypoplastic Left Heart Syndrome (HLHS), Joshua was delivered at a major medical center and was immediately sent to a Neonatal Intensive Care Unit. By contrast, I was born in a community hospital of less than 100 beds. My grandfather – only an occasional smoker – was so nervous that when I didn’t show up on time, he unwrapped the celebratory box of cigars that he planned to give away and lit up. He was chased outside (smoking was accepted almost everywhere in the mid 1960’s, but a nurse wasn’t comfortable with anyone smoking around the newborns) so he stood under a dogwood tree and smoked until he just had to find out how things were going. Then he jammed his cigar into a fork of the tree and rushed back inside. He never said anything about retrieving his cigar, so for all I know, it could still be there.

Joshua’s parents knew they would be on a roller coaster ride from the moment he arrived. I looked normal. There were no prenatal tests in the 60’s, not even a way to tell if the unborn child was a boy or a girl. Only my mother’s intuition led her to notice that something wasn’t exactly right, and she consulted a pediatrician. The pediatrician that they had used for my older brother was out of town so they talked to the New Guy.

The New Guy realized he was looking at a heart problem but wasn’t sure of the details, so he sent my parents and me to the BIG HOSPITAL 200 miles away. The Doctors at the BIG HOSPITAL figured out that I had Tricuspid Atresia… and stopped. They had never successfully treated a Tricuspid before, so they gave my folks the diagnosis and told them to take me home and enjoy the time I had. I was not long for this world.

New Guy hit the roof. BIG HOSPITAL may not be able to do anything, but he knew of places that would at least try. He found one, and to make a long story short, I was a patient of New Guy until I was 20 years old. The “new” had worn off by then, but he’s one of only two doctors to really understand what is going on inside of me – him and my current Cardiologist. I still see him around town every so often!

By contrast, Joshua was pegged as a Cardiac Kid long ago, while he was still being knit together in his mother’s womb. The moment he was born he was handed off to a skilled care unit. Their only job is to keep him stable until it is time for his repair. And while we thought 200 miles was quite a way to travel to a hospital, that’s no longer true. 200 miles is a short trip.

We’ve literally come a long way.

We’ll meet Joshua soon!

August 8, 2010

We have a new Cardiac Kid in our Blogroll, Joshua.The link is to his mom’s blog, as officially Joshua hasn’t made his appearance yet. He’s scheduled to be here on Monday, August 16.

Joshua will be born with Hypoplastic Left Heart Syndrome (HLHS) and his Mom and Dad will be glad to finally meet him… but understandably, they are worried. Any good parent would be! As of right now his first surgery is scheduled for Thursday, August 19.

I am asking each of you to consider passing our story along to your friends, family, and churches. Either just pass our names, a little snippet of what we will be facing soon, or my blog address, his mom writes.

We can do that! In fact, we can do better than that… Everyone take a moment to head over to Joshua’s Mom’s blog and let her know that we’ll all be pulling for them.


Haunted Dreams

June 23, 2010

Here’s a blog post from a friend and a Heart Mom that you need to read:

Last night as I watched Mary Clare zoom by me in her mini airplane laughing so hard tears were streaming, I couldn’t help but think of what is ahead of us.  The mother in me prayed a silent prayer and pleaded to God that this will somehow make it up to her.  Maybe if she has the most fun summer, she’ll not hate me for what I know must be done to her.  I’ve noticed Mel has also been thinking this way.  He has promised to paint her toenails everyday while in the hospital this fall.  He told her she could pick out 40 different colors, or however many days we are there, so they can be a different color everyday.  He also promised her a trip to Disney World to see all of her beloved Princesses afterward.

How can I do this again?  How can she do this again?  Yet again.  How am I going to be able to hand her over this time, not just for a heart cath, a simple procedure by comparison, but a surgery which rips her tiny body apart?  How am I going to be able to watch her on a vent again?  Lines, leads, monitors.  All over again.  Having her torn away from me at birth and sit by and watch all that she went through, then again to have the same thing happen merely 6 months later was horrific.  She has to do it all over again, but this time she will ask.  Why is this happening?  Why can’t we go home?  Why do I hurt?  Why can’t you put a band-aid on it?  These are the questions which haunt my dreams.

Wow. What a burden to carry around. How could anyone make the decision to send another person – especially a child – to heart surgery? And especially their child?

This Heart Mom obviously knows the answer to those questions: To save her life. To give her the best chance to have a reasonably normal existence.  And to perhaps one day, tell her own children that her mom stood up for her when she was a child; tell how Grandma and Grandpa never gave up on her. Even when it looked like their lives were heading right over the edge of the cliff, they dug their nails in and they hung on.

Because that’s what Heart Parents do. They ask one more question. They explore one more option. They go one more mile.

And please keep this young family in your thoughts. Drop by their blog and wish them the best!

HLHS Advice

June 22, 2010

Hypoplastic Left Heart Syndrome (HLHS) is a known killer. It is 100% fatal if left untreated, and before 1981 there was no treatment. For several years after that there were very few survivors as doctors learned that a surgical correction could not be done in one step, but rather took two (and eventually three) separate operations. Infant Heart Transplantation was a dream until 1984.

So doctors have only been able to combat HLHS for 30 years, and there is still a lot that is unknown. The hows and the whys of the defect are still only being guessed at. The operations are delicate: not only are they demanding on a newborn’s Circulatory System, but so much depends on blood flow and pressures in the heart. All of the blood now flows through the Tricuspid Valve, which wasn’t designed to move that much blood. The proper “balance” often depends on the patient and there is no “standard” series of operations for HLHS. Each one must be customized to the individual.

Which leads to this interesting report from the March 2010 issue of the  medical journal Pediatrics. 749 Pediatric Cardiologists were surveyed concerning their management recommendations when giving a diagnosis of Hypoplastic Left Heart Syndrome. 99.7% of all Pediatric Cardiologists recommended the three surgery approach. 67% of the doctors discussed a heart transplant with the new parents, and 62.2% discussed Compassionate Care without a surgical intervention. (The high numbers in each category shows that the doctors were not limited to one response.) However, only a small percentage – 14.9% discussed all three options.

Also, the doctors were asked what they would recommend when confronted with a prenatal diagnosis of HLHS. Almost all of them (98.8%) recommended continuation of the pregnancy with surgery after birth. 53.5% argued for continuation of the pregnancy with Compassionate Care/no surgery; and a very high 74.3% discussed termination of the pregnancy. Only 36.5% discussed all three options.

Interesting numbers, and much to think about. The bottom line is that most Cardiologists would tell you to fight for your child. But why would so many of them recommend ending the pregnancy when HLHS is diagnosed prenatally?

UPDATE: A reader has brought to my attention (See comments) that the research report states that the Cardiologists/Surgeons discuss these options rather than recommend them. A discussion, of course, is not a recommendation. Some patients may treat it as such (“The doctor will probably know best…”) but it does answer my closing question much better – and reflect the experiences of my readers who have chosen to comment.

I have stated before that I am not immune to mistakes, that is why I try to include a link to anything that is not in my personal experience – so you can check my work for yourself.

And I would like to publicly thank the commenter for bringing this error to my attention.

Cut ’em off at the pass!

May 30, 2010

It’s a common scene in old Western movies: The good guys are in hot pursuit, but the bank robbers are getting away. “Quick, let’s cut ’em off at the pass!” someone shouts, and all the good guys gallop off through a shortcut. A few moments later, they’ve managed to get ahead of the evildoers!

For ten years now, Children’s Hospital of Boston has been working on cutting Hypoplastic Left Heart Syndrome (HLHS) off at the pass – by doing heart surgery while the baby is still in the mother’s womb! One of the early signs of HLHS is Fetal Aortic Stenosis; a small Aorta present before birth. (This can be detected through a Fetal Echocardiogram.) Boston Children’s has developed a procedure in which a probe is inserted into the mother and passes through the womb, into the unborn child, and enlarges the Aortic Valve. This is known as Fetal Aortic Valve Dilation.

An article in the June 2010 issue of Congenital Cardiology Today details the procedure and reports on an analysis of 70 attempts to repair the defect. 52 of the procedures (70%) were “technically successful.” If the Left Ventricle is small (and it almost always is) when the operation is done, the Ventricle will not grow. However, it will support growth of other Left Ventricle structures and HLHS can usually be avoided. Aortic regurgitation (backward blood flow through the Aortic Valve) happened in about 40% of the cases. And unfortunately, death occurred 13% of the time.

But the good news is we are starting to be able to fight a severe Congenital Heart Defect before the child is even born – and stopping a severe Congenital Heart Defect almost before it starts!

The Hybrid Procedure

May 24, 2010

Until the 1980’s, most forms of Hypoplastic Left Heart Syndrome (HLHS) were fatal. That group of defects involve the left side of the heart, the side that pumps blood to the body, and surgeons had no procedure that could offer any hope. More often than not, all Pediatric Cardiologists could do was to offer Compassionate Care. This country has put men on the moon, many doctors would ask themselves. So why in the hell can’t we save these kids?

That changed with the development of the Norwood Procedure (a three operation sequence developed in 1981) and the first Neonatal Heart Transplant in the United States (1986) . But even though it was an answer, the Norwood Stage I is a difficult procedure.  It is a six-hour operation, a major re-plumbing of the heart and surrounding blood vessels performed when the child is less than one week old. Imagine, if you can, operating on a damaged heart the size of a walnut. And the delicate part isn’t the repair, it is balancing the blood flow correctly. Everything flows through the Tricuspid Valve, and you can increase the flow rate (which increases the pressure on the valve) some, but not a lot. Get the pressure too high and you damage the Tricuspid Valve, and we can’t have that. For a HLHS patient, the Norwood Stage I is the most important, the most difficult, and the most dangerous operation of the three.

What if it were possible for the Stage I to be an easier operation? Or better yet, what if you were able to eliminate it completely?

Well you can’t just disregard the Stage I – major HLHS requires some type of surgical intervention – but it can be an easier operation. Doctors in major heart hospitals around the US are refining what is known as the “Hybrid approach” and the early results are promising.

The Hybrid was developed at Nemours Cardiac Center in Orlando, Florida in 1999 and takes place not in the Operating Room, but in the Catheterization Lab. The chest is opened and both Pulmonary arteries are banded to restrict blood flow. Next, a stent is implanted in the Ductus Arteriosus to keep it open (creating a Patent Ductus Arteriosus, or PDA). And while the stent is placed via Catheter, it is not inserted through the groin. The Catheter is inserted directly into the Pulmonary Artery via the incision in the chest. At times, an Atrial Septal Defect (ASD) will also need to be created.

Once the stent is in place, the operation is over. Total time: 60 to 90 minutes, and the Heart/Lung bypass machine was not used. After the Hybrid, the rest of the Norwood is carried out as before. But the Hybrid Procedure allows the major surgery to be delayed, until the child is older and stronger.

A 2008 study showed that the results of this new operation were favorable. The University of Chicago started using the Hybrid for their high risk HLHS patients, but found that the results were so good that it is offered to all Norwood Procedure candidates. (Observant readers will notice that the “60 to 90 minutes” link and the “results were so good” link quote the same doctor, who worked at Chicago but later moved to Boston.)

Very few hospitals perform the Hybrid Procedure for HLHS; so if you get a prenatal diagnosis of Hypoplastic Left Heart Syndrome, be sure to ask about all treatment options – including (and especially) the Hybrid.