Posts Tagged ‘Fontan’

A disturbing report

September 23, 2010

Funky Heart reader Cindy forwarded THIS .PDF FILE that you need to read. Titled Forty Years of the Fontan Operation: A Failed Strategy, this report contains information that you should consider. Download it, print it, and read it carefully. But I’ll warn you: this report will make your hair stand up.

Forty years ago a heart defect that eliminated the Right Ventricle was “uniformly lethal” but today that same patient is “not only likely – but expected – to survive.” One of the major weapons in our arsenal is the Fontan Operation, the the Fontan comes with its own set of difficulties. The author, Dr. Jack Rychik of the Children’s Hospital of Philadelphia, considers it “a failed strategy.”

Dr. Rychik lists detailed statistics concerning the decline of Fontan patients, then explains why they tend to deteriorate: elevated central venous pressure coupled with a reduction in cardiac output. He then explains three important issues for a Fontan patient: liver damage; Plastic Bronchitis, and Protein Losing Enteropathy (PLE).

This report may be disturbing to many readers, but please remember: outcomes aren’t pre-determined. Dr. Rychik states that 40 years ago single ventricle defects were “uniformly lethal” – but I just turned 44. Heart defects aren’t like math, the answers are not so cut and dried. 2 +2 =4; 4 x 4 = 16. That’s a constant. But each defect affects the patient slightly differently, and the “standard answers” may not apply. When you are talking about a defective heart, 2+2 may equal 66.4.

Just making a guess, I believe that I know 12 to 18 people who have had the Fontan. That includes close friends, people I have met just once or twice, and some I only know through email and blogs. These people are in all stages of health – a few are doing wonderfully, most of them are doing good with occasional Bad Days, and a few can barely go. When you rank test subjects based on general health, Fontan survivors won’t be grouped around one point. They’ll be all over the place. To quote almost every Cardiologist who has ever discussed future options with a patient: It’s certainly not perfect, but it is what we have to work with.

Rychik concludes that in the past, giving a single ventricle patient the ability to live 30+ years was a noble goal. Today, it is unacceptable. He contends that Cardiologists and surgeons need to think outside of the box, and come up with new options – either redesign the Fontan (again); scrap it in favor of something else; or develop a Right Ventricle Assist Device.

Either way, every heart deserves to live a lifetime.

The Fontan

August 10, 2010

In late 1987, my cardiologist recommended that I have the Fontan Procedure. Now I was doing pretty good, he said, but having the Fontan would bring me to as close to “normal” as I would ever get. I thought about it, and decided to have the operation – after I graduated college. I was one semester (three months) from graduation and thought that waiting long enough to officially finish school would be a good idea. So that is what I did, and my surgery was scheduled for May 1988.

If you are a regular Funky Heart! reader you know what happened. After heart surgery the heart develops a layer of scar tissue, and in my case there was a lot more than anyone had anticipated, and it had stuck to the back of my rib cage. When my ribs were cut open that scar tissue tore. I required 20 units of blood and nearly bled out on the operating room table. My surgeon, Dr. Albert Pacifico, managed to get the bleeding under control and then backed out – the risk of restarting the bleeding was just too great. And if it started again, this time Pacifico and his team may not have been able to stop it. (“I tried every trick I knew to stop the bleeding,” he said later. “And I even had to make up a few new tricks on the spot.”)

The difficult thing about the Fontan procedure for me is that while I am somewhat familiar with the history of the procedure, I can’t understand it or explain it. I read the books, I study diagrams, (The Illustrated Field Guide to Congenital Heart Disease and Repair has several good illustrations. They don’t seem to help me understand what I am looking at, though!) but I just can’t get into my head how it works. And apparently that is a common problem – the “standard” cardiac treatments don’t seem to work as well, or work differently. The heart itself doesn’t control cardiac output; that really depends on the lungs. Patients who live at higher altitudes can show marked improvement by relocating to a lower altitude – in some cases an operation that has never worked right can suddenly find a happy balance as the altitude decreases. But before we put all of our Fontan Survivors who live in the Rocky Mountains on a train bound for the coast, the altitude adjustment doesn’t always work.

The Fontan Procedure is named after Dr. Francios Fontan, a French surgeon, and was first described in 1971. (CLICK HERE to see the article that describes the Fontan in its original form.) Even the professionals seem to have difficulty understanding exactly why and how the Fontan works: A study of 476 Fontan operations were analyzed and a “Fontan Score” assigned to each individual procedure. Variables that could affect the Score included “surgical center, age, weight, fenestration, length of hospital stay at time of Fontan procedures, and post-Fontan surgeries or interventions.” When the Fontan Score was analyzed, only 18% of the variables could be explained; the rest (82%) of the factors were unknown. And any long-term study of the Fontan is going to be confusing, as the operation has been modified over the years. (Had my operation been successful I would have the second version of the Fontan, and it has been modified since then)

Fontan Survivors are also subject to arrhythmia, and people with stable Fontan circulations can and probably should have a mild to moderate exercise program. It is important that the patient is stable, so don’t just drive down to the gym one day and sign up for Jazzercise classes. Consult your Cardiologist first!

Another problem – a big problem – for Fontan Survivors is the possibility of developing Protein-Losing Enteropathy, or PLE. PLE is an unexplained loss of protein from the body, usually through the intestinal tract. PLE is not a side effect of Congenital Heart Disease, it only affects people who have had the Fontan. One large study done in 1996 found that ten years after having the Fontan, the cumulative chances of having PLE are 13.4%. A 2003 study concludes PLE could be triggered by an infection. This leads to the disturbing thought that maybe all single ventricle patients are predisposed to PLE, and the Fontan Procedure coupled with an infection is the “trigger” that sets it off.

Someone, somewhere, had the Fontan Procedure today… and despite the drawbacks of the operation, they will probably have a better life because of it. As a 2008 report concludes, “this imperfect circulation would still be the only surgical option for this difficult patient population.”

When the test doesn’t work

August 3, 2010

A recent clinical trial showed disappointing results: Enalapril, used to treat high blood pressure and heart failure, was being tested in infants born with a single ventricle heart. The basis of the trial was that since the drug works in adults, perhaps it will also work in children.

It didn’t work at all.¬† When the results were analyzed, the outcomes for Enalapril group and the Control group were¬† practically identical. The results of this randomized trial do not support the routine use of enalapril in this population, the researchers wrote.

Did the drug fail? Certainly, it did not produce the desired result. But the clinical trial shouldn’t be considered a failure – after all, now doctors know what not to do. They may not have any better answers, but now they know that prescribing Enalapril in these circumstances won’t work. And sometimes you have to learn what not to do before you figure out what you should do.

Cardiology is littered with the relics of operations, drugs, and procedures that were abandoned in favor of something better. As I mentioned in my presentation in Houston, I had the Blalock-Hanlon procedure during my first surgery in 1967. At that time it was done regularly – the only way to enlarge or create an Atrial Septal Defect was to open the Right Atrium and use a scalpel or a probe to literally poke a hole. Today the operation has practically been retired, since so much can be done with Catheters. Today’s Heart Parents have never heard of the Waterston Shunt or the Potts Shunt – both operations were dropped when someone thought of a better way.

When someone tells you they have had the Fontan, you should ask them when they had it… it has evolved over the years, and each version has good points and bad points. Protein-Losing Enteropathy (PLE) doesn’t seem to affect anyone except Fontan Survivors, and not all of them. And one day scientists will figure out PLE, or the Fontan will be altered again…. or a better procedure will replace it.

And who knows? Perhaps at a Congenital Heart Defect (CHD) Conference in the year 2041, someone will ask “You had the Fontan? What’s that?”

How I became the Funky Heart

June 8, 2009

It’s a pretty interesting story. You see, I was born on another planet. Right before the planet self destructed, my father placed me in a spaceship and sent me towards Earth –

No, wait a minute, that’s someone else’s story. Sorry about that! This is more of a Dragnet type of story… the names have been changed to protect the innocent.

I had gone to The University of Alabama at Birmingham (UAB) Hospital to have the Fontan procedure, and it had gone wrong. Scar tissue normally forms over your heart after you have open heart surgery, and surgical teams know that and expect it. I had had two previous heart surgeries, so I am sure they felt like there was a good chance that I could have more than usual. But what they didn’t know was that there was a lot more scar tissue than normal, and that it had formed adhesions with the back of my breastbone.

So when they split my ribcage that scar tissue tore, and blood went everywhere. The surgery was canceled and I needed 20+ units of blood to survive the operation. So even though I never had the Fontan, I got all the pain associated with having heart surgery.

*Sigh* The fun was just beginning. I got better and was sent home. I was recovering but the incision wasn’t healing, and finally began to leak pus. Back to the local hospital, where I was quickly transferred to a larger hospital. There the Cardiological team studied me, and finally said “Yep! It’s infected!”

OK, doc, what do we do?


Wikipedia says that debridement is “the medical removal of a patients dead, damaged, or infected tissue to improve the healing potential of the remaining healthy tissue.” How that works in the real world is they sedate you, haul you down to the Operating Room, and scrape out the infected tissue. It’s not fun.

So I was debrided. They re-opened my incision, scraped the dead tissue out, packed the incision with gauze and covered me with a large bandage, sent me for a short stay in Recovery and then back to my room. And not knowing any better, I went along with this.

Important Safety Tip: For a Debridement to work, you’ve got to get all of the dead and infected tissue out. The best way to do this is to 1) Scrape like a maniac while in the Operating Room; and 2) give the patient antibiotics. The problem is, my crack team of local cardiologists didn’t think the antibiotics were necessary. So what could have taken one trip to the Operating Room turned into three.

I was not happy. They opened up my bandages and peeled back the gauze every day, and when they said “Looks like we have to debride again” I literally cried. What in the world was going on here? Why couldn’t I get any better?

And this time, there was no safety in numbers – I had a Cardiological Service, with several doctors who rotated. I saw a different doctor every day, and every one of them had a different opinion:

“I think we can get you out of here by the end of the week.”

“Two weeks, tops.”

“At least two more weeks.”

“I don’t feel comfortable making a prediction yet.”

After that third debridment we had a late night visitor. A nurse came in, made small talk with Daddy and I, and finally said, “Most of the nurses think you need to seriously think about going somewhere else.”

Now THAT is a sign – saying such a thing can get a nurse fired, so when they get to the point that they are advising the patient to grab his stuff and go, listen carefully. So Daddy called the Cardiologists at UAB the next day. They took care of all the arrangements to return and have me admitted.

The first UAB doctor I saw was not a Cardiologist, but a Plastic Surgeon. “Here’s the plan,” he said after he examined me. “We’re going to start you some antibiotics and run them around the clock. I want you to drink a serving of Ensure at each meal and before bed to promote tissue growth. And then I am going to take a long weekend.”


“My daughter is graduating college, so I’ll be out of town. And it’ll take some time to see if the antibiotics and the Ensure are working. I’ll examine you when I return and we’ll plan what to do then.”

So the time passed, and Tuesday morning, as promised, he was back and examined me again. “It’s going to take a while to heal, but we’re on our way. I don’t see the need to do any more debridements.”

In two days I was on the way home. I spent six days in Alabama, while my “adventure” at the other hospital had cost me seven weeks. And I came out knowing that the only one looking out for me all the time was – me! So I needed to stop being passive and start taking an active part of my health care.

And that’s where the Funky Heart was born.

Better Surgery!

June 1, 2009

Here’s an interesting report from the March 2006 issue of Heart: A look at the outcomes of surgical correction for Hypoplastic Left Heart Syndrome, or HLHS. What makes this report so unusual is that it covers twelve years at the same hospital: While surgeons, doctors, and nurses come and go, the hospital policies and standards of care stay in place and hopefully improve. So this can be considered an unbiased study. When results from more than one facility is used, the results can be affected by a hospital that has more (or less) successful results than average.

Beginning in 1992, 333 patients underwent the Stage I surgical correction for HLHS, also known as the Norwood procedure. 203 patients underwent the Stage II operation (the Bidirectional Glenn), and 81 had the Fontan Procedure (Stage III). While HLHS is difficult to overcome, the report does not elaborate on the difference in numbers. It is quite possible that patients had their Stage II or Stage III operations at different facilities.

Over the course of the study, mortality after the Stage I operation dropped from 46% during the first year (1992) to just 16% in the last year (2004). The survival rate jumped dramatically in 2002, when the Stage I was altered: the Modified Blalock-Taussig Shunt that was part of the operation was replaced by a Right Ventricle to Pulmonary Artery Conduit.

Medical research is a long, tedious assignment – there are very few “bolt of lightning” moments and it often requires gathering massive amounts of data. But it works, and is often the only way to learn if a new theory is both effective and safe.

Advanced Cardiology: Knowing the Enemy

December 15, 2008

So after yesterday’s review, we have a good idea how a healthy heart works. But you or your child has a heart defect… what do we do? There are thirty five different, distinct Heart Defects. And if that isn’t bad enough, some of us have a “blend” – a defect that has traits or two (or more!) single defects.

That’s why I’m going to recommend a book to you. Before I do, I’ll state this: I don’t work for this company, nor do I have any type of commission deal with them. What I do have is a copy of their book, and I use it a LOT.

The name of this book is the Illustrated Field Guide to Congenital Heart Disease and Repair.

There are two versions. Both have a spiral binding, so it can lie flat on a desk. The large format version is 7 inches by 10.5 inches, about the side of a larger paperback book. The pocket sized version is 4 inches by 7.5 inches, small enough to slip into a purse or even tuck into your pants at the small of your back comfortably. Even in the smaller size, the print is very readable.

The book features an explanation and a diagram of all the identified heart defects. If that isn’t enough, most of the recognized Congenital Heart Operations are described and diagrammed, along with the major variations. For example, you can find descriptions of the Blalock-Taussig Shunt, the Mustard repair (which is rarely used today), two versions of the Fontan, and five different ways to complete the Norwoord Procedure.

And if that isn’t enough, there is also a section concerning Cardiac ICU units, Electrophysiology, and information on some of the drugs that are available for Congenital Heart patients. This is probably the most useful book I have ever seen. I have the Second Edition, but I recently ordered the Revised 2nd Edition, which has been expanded to over 400 pages! The only thing that I don’t like about the book is that it has a few more “doctor words” than a newcomer to CHD’s may understand. Usually if you don’t understand the words, the diagrams will show you.

You can buy one using this form. Don’t let the form scare you – this company sells the majority of its items to doctors offices and hospitals, that’s why you see the words “Purchase Order” and fairly high prices. The Field Guides cost $50 for the smaller version and $125 for the larger edition, and they can be worth every penny.

No one can make a heart defect any less scary, but this book can make it easier to understand. And as a 1980’s public service announcement stated, “Knowing is half the battle.”

Links for ToF, Glenn, and Fontan Patients

November 8, 2008

There are some very important links for you to read tonight, important enough that I held them back yesterday so I could feature them as a group. If you’ve had a surgical repair, these links could come in handy for you.

The good news is that they are readable online, printable, and you can download them as a PDF file. (The PDF file may not be complete, that’s explained on the download page. But you can print the entire article.) The bad news is that they appear in medical journals and are written for cardiologists and cardiac surgeons, so they are 100% “Doctor Talk.” Bring an interpreter.

But they are important and useful, especially the second and fourth link.

A classic case of Tetralogy of Fallot (ToF).

Repairing a Tetralogy of Fallot (ToF) Heart: Current surgical thoughts and procedures.

The Bi-Directional Glenn Shunt… without using the Heart/Lung Bypass Machine

The Fontan Pathway: What’s down the road?

Don’t let the Water get in the Boat

September 11, 2008

I was back at the University of Alabama at Birmingham (UAB) in May of 1988, getting ready for one last heart operation. My Cardiologist had sat down with my parents and I at an earlier appointment and discussed possibly performing a version of the Fontan Procedure (See “A Final Note:” at the end of this post!) on me. It was really a two stage operation, but my first two surgeries roughly equaled the first part of the Fontan, so I would only need the second part of the operation. I would never be normal, my Cardiologist told me, but this final operation would bring me as close to being heart healthy as I would ever get.

This was the first time that everything hinged on my decision. I had been underage for my previous surgeries, so the responsibility had fallen to my parents. Since I had just turned 21, this time the Go/No Go was my decision. After steeling myself to the thought of all the pain and recovery time that would be involved, I decided to have it. All of the testing and X-rays had given my surgeon the information he needed to devise a plan he was confident in, and he and his team met with me the night before.

“Any questions?” he asked.

“Just don’t start without me,” I joked.

Dr. Albert Pacifico had been my heart surgeon the first time I had been a patient at UAB in 1977. Pacifico is known for two things: being good, and working fast. He averages eight heart operations a day, when the typical surgeon performs two a week. As far as Cardiac Surgery is concerned, he’s the superstar.

The next day, the crap hit the fan. As soon as the surgical team opened my chest, things began to spin out of control. Opening my rib cage tore some scar tissue that had became attached to the back of my rib cage, and I began to bleed profusely. Dr. Pacifico was world renowned and had probably performed more heart operations than anyone alive, and he needed every bit of that skill to get the bleeding under control. “Imagine your chest cavity is a boat, and your blood is water,” he later said as an explanation. “You never want to get too much water in the boat.”

“Shoot, doc,” I groaned. Talking after surgery isn’t the easiest thing to do. “You should have woken me up. I would have bailed out the boat while you fixed the leak.”

The leak was starting to get ahead of him; every time he sealed one bleeder another would pop open. I was bleeding out faster than the surgical team could put it back in.

As he always did, the surgeon came down to a Conference Room where the families of his patients had been summoned. He normally called out a name, and the family members would group around him as he consulted an index card and summarized the operation. (This was a long time before Health Record Privacy became such an important issue.)

“I will speak to you two last,” he told my parents. My mother gasped and began to cry.

The Patient Representative stepped forward. “That’s not the way we handle that,” she said, placing enough emphasis on the word so there was no doubt what THAT was.

The news still wasn’t good. The bleeding had been stopped, but I had required 20+ units of blood. A lot of water had gotten into the boat. I was in recovery, but I wasn’t recovering. I wasn’t going downhill, either; so you could say I was just holding my own. And then he said the phrase that has come out of every surgeon’s mouth:

“We’ll know more in a few hours.”

The waiting room was huge. UAB had designed a building that contained all of its surgical suites and all of the recovery areas, which meant the entire ground floor was a unit’s waiting room. The surgical unit waiting area was over there, while ICU was here, and surgical recovery over there, but if you took out the walls you would have one large room.

So the families sat and waited, talked, and followed each other’s progress. A good report cheered everyone, while bad news made the room grow somber. A young lady – my father thought she was a teenager, but he later learned she was an adult who ran a hairdressing shop in a nearby suburb – heard about me and gathered her friends. “We need to pray for this young man,” she said.

At about the same moment, my surgeon sat by my bedside in Recovery and studied my chart. He was by himself, which was quite unusual. Pacifico was usually at the head of a five to ten person entourage. And he had been reading for an hour, which was also quite unusual for him. Finally he snapped the chart shut and ordered a change in my medications, and from that point on I began to improve.

A miracle? The skill of an experienced, trained surgeon? “Yes” is the answer to both questions. I believe God still works miracles, and I also believe that He does a lot of them without any fanfare. Heart Surgery is a modern miracle, but what about the guy who invented the Band-Aid? His little idea has probably saved just as many lives, and possibly more.

This story doesn’t end here. A few years later, my cardiologist commented, “It’s probably a good thing that there was so much trouble when we tried to perform that Fontan on you. We’re learning that it’s not working as well as we hoped, and some of those who have had it are actually worse off than they were before.”

The hero you find may not be the hero you are looking for.

A Final Note: As I mentioned, these events occurred in 1988. The Fontan Procedure that was to be performed on me (And that eventually showed poor outcomes) is not the Fontan that is being performed today. The operation has undergone several revisions and is now much more effective.