Behind Those Doors

Karen Thurston Chavez is Co-Founder of the support group Broken Hearts of the Big Bend (BHBB) and a good friend. We met when I spoke at BHBB’s Congenital Heart Defect Symposium last year and we trade e-mails at least once a week, keeping up with each other and discussing the latest issues affecting the Heart Defect community.

Karen’s blog has a most unusual post you need to read – in September, she was able to shadow her son’s heart surgeon for a day at Shands Children’s Hospital in Gainesville, Florida. But then something she didn’t expect happened:

I figured I would sit in on conferences and consultations he had with families whose children were having, would be having, or just had open-heart surgery to repair their congenital heart defects.  I guessed that I would sit and watch while he handled administrative work. I was right. I did all those things.

I did not think I would step into his operating room.

So I’m sending you over to her blog for today’s post. Karen will help you get scrubbed in and then walk you into the Operating Room as Dr. Mark Bleiweis performs open heart surgery.

SHADOWING MY SON’S HEART SURGEON

Miracles

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

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.

He earned it!

A mechanic is making small talk with a customer as he adds up the bill, and discovers that the customer is a heart surgeon.

“Doc, I just don’t get it,” the mechanic says. “I can take out a piston and grind it down to fit better. I clean dirty valves, I fill the engine with oil, and I can work on the electrical system. You bring me an engine that won’t run correctly and I can fix that thing so that it runs perfectly. We do the same job, but you make four times the money I do! I can’t understand that.”

“Well,” the heart surgeon said. “I normally do my work with the engine running.”

The other side of the OR door

I’ve often written of how surgery day is excruciatingly long. They may tell you that it will take four hours, but that is rarely true. And every minute seems to drag as you wait for news of your loved one. You just want this to be over – but nothing good comes of a short operation. The longer, the better – the doctors are still working, still fighting for you. A short operation could mean that the fight is over and the good guys lost.

It’s the same on the other side of the Operating Room door. When you are waiting, just standing there twiddling your thumbs, time drags. When surgery begins and everyone has a job to do, time flies. “Five people working as one unit,” Gene Hackman said in the movie Hoosiers, and a Surgical Unit is a team in every sense of the word. Everyone has a job to do, and when you work together long enough, you even begin to think together. In this article appearing on KevinMD.com, Dr. Bruce Campbell explains that time distorts in operating rooms, too. But it seems to act in reverse:

I look up at the clock. It seems like only a few minutes have passed since I had anxiously waited to begin the case. Five hours have disappeared like an instant.

Go and read Dr. Campbell’s work.

Cut ’em off at the pass!

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!

Isaac

Isaac is having surgery today at Duke Children’s Hospital! I met Isaac and his mom at the Saving Little Hearts CHD Symposium I attended last year, he’s a pretty cool kid. He has to be at the hospital at 8:30 AM Eastern Time (1230 GMT) and he’s the second scheduled operation of the day. I’ll be updating when I find out what’s going on, so keep refreshing this page!

11:04 AM: No update as of yet. Surgical schedules do run late, I’ll keep checking!

12:48 PM: First update! Things were going really slow – Isaac was called back to the Prep Room at 11:30 AM! He’s finally in the Operating Room.

1:14 PM: Surgery has begun!

3:23 PM: Surgery still going on, everything seems to be under control. Isaac’s mom is beside herself waiting! Keep thinking about both of them, please!

6:21 PM: THE SURGERY IS OVER! The Surgeon said everything went exactly as planned. Isaac is on a ventilator and is in Recovery; his mom is on her way to see him right now!

She’ll probably report once she sees him; if nothing unusual is going on I’ll end this post after that update.

11:48 PM: A final update from Isaac’s mom: HE’S PINK!

Change of Heart

“You can  settle for less – an ordinary life – or do you feel that you were meant for something better? Something special?”

I used to have a T-shirt that read “Change of Heart at UAB”. UAB is the University of Alabama at Birmingham, and in the late 1970’s it was THE place for Cardiac surgery in the Southeast. After my second heart surgery the staff expected you to get up and get around, and one day my “travels” took me into the hospital gift shop, where I bought my shirt. My dad contended that had to be the most expensive shirt I had ever bought (Buy a T-shirt, get a FREE Heart Operation!) but I was proud of it.

But right now, I’m going to send you on a trip. You’ll be visiting my friend Sharon, who is going to tell you the story of an event that happened last year – one year ago exactly, March 26, 2009.

“Pack a suitcase for 3 days and get here as soon as possible,” he said. I started to cry and scream all at the same time. “You’re kidding? Thank G-d!!! We have it! You’re getting the heart!!!” I still get tears when I think of this moment.

You’ve guessed it – you are going to ride along with Sharon as her husband gets a desperately needed heart transplant. You won’t find the entire story – Sharon says she was in tears as she wrote it and had to take a break. But you will see the next part when she posts it.

And I bet you’ll be in tears, also.

But for right now, GO! I told Sharon that she’s have some company today!

Click here to read Sharon and Anthony’s story!

UPDATE: Radcliffe Hospital Investigation

The inquiry into Oxford’s John Radcliffe Hospital continued today, as the British Press found a “suspect” and news came that the investigation is a lot bigger than initially thought.

All four of the children who died had Congenital Heart Defects and all were operated on by the same surgeon, Mr. Caner Salih. (British surgeons are traditionally referred to as “Mister”). Not only was Salih the doctor in all four cases, he has since left the hospital… so obviously, he must be the guilty party.

No so fast – early reports indicate that Salih may have been the person who sounded the alarm and that all aspects of patient care will be examined, not just the surgeons.

Radcliffe has a history of difficulties in cardiac surgery, dating back to the year 2000. Internal problems and surgeons failing to work together were highlighted as the main cause of the difficulties then.

The hospital also performs a low number of procedures (Estimated to normally be “between 30 and 40” in a four-month period, which would be approximately 100 per year.) The National Specialist Commissioning Group is expected to report in July that the proper thing to do is consolidate eleven Cardiac Surgery Centers down to five or six – Centres of Expertise that should have a minimum of four surgeons who perform 400 operations per year in each Center.

That’s the proper thing to do – the only way to get really good at something is to continually practice doing it. The problem at Radcliffe Hospital may prove not to lie with the surgeon, but rather with the entire Cardiac Care Unit.

Devil in the Slot

BONUS: 100 Karma Points to the first person who can leave a comment telling me where the title of this post originated!

You have a heart defect.

No, I’m not talking about the 1 in 125 of us who live with a Congenital Heart Defect, I’m talking about YOU. Mr. or Mrs. Average. You were born with a heart defect. Two of them, actually.

Obviously, you don’t need to breathe while you are still in your mother’s womb. You can’t breathe, unless you can somehow magically breathe fluid. So your lungs are “turned off” and you have two small defects that allow the heart to function but blood to bypass the lungs and pick up its oxygen from your mother. When you are born and you take your first breath, the body sends a signal to those two defects to shut down and for the lungs to take over.

(Pretty cool, huh?)

One of these small defects is called the foramen ovale and it is really a very small Atrial Septal Defect. In fact, it isn’t really a hole in the septum, it is two overlapping flaps. When you begin to breathe and blood begins to flow properly, these two flaps will eventually seal closed and everything will be great. In about 30% of people, however, it doesn’t close. When that happens, it is called a patent foramen ovale, sometimes called a PFO (“patent” means “stuck open”).

Many times this doesn’t cause much of a problem; sometimes it is even undetectable unless the patient coughs while having an ultrasound.

Now here is the problem: when a person has a PFO, or when it is slow to close, occasionally a small “pouch” forms in the wall of the Left Atrium. Doctors at the University of California at Irvine recently found that pouch while doing autopsy research, and it could be the source of several problems.

That little pouch is nice and quiet; any blood that gets in there settles down – it’s no longer part of the normal blood flow, and it is pretty calm in there. While the heart is pumping and blood is churning all around it, it’s a nice quiet little neighborhood… almost a gated community. (This Link has a good explanation and has a nice drawing of the pouch about halfway through the article. )

That’s not good. Blood can settle in the pouch and form a clot. And since it is on the left side of the heart, the clot skips the lungs (which not only add oxygen to the blood, but they also act as scrub brushes, too!) and then it is off on a tour of the body. Round and round she goes, where she stops nobody knows. But if it stops in the wrong place….

STROKE!

So if the doc tells us we have a PFO, we should get it closed, right? Perhaps, and perhaps not. The medical evidence isn’t in yet. We know what might happen, what could happen – but how many times is the pouch really the cause of a stroke? We don’t know yet. A clinical study (the RESPECT trial) is being conducted right now to determine if a PFO causes more strokes. At least one doctor isn’t waiting to find out – she’s full steam ahead. The Food and Drug Administration is saying wait a minute, slow down, catch your breath… let’s see what the evidence says. Then we can decide. (You really need to read what Isis has to say about PFOs, she gets the point across quite well, in simple English and with a lot of humor, too!)

So, is there really a devil hiding in that “slot” that could be in your heart? And if there is, what are the chances of him coming out? No one knows… yet.