Posts Tagged ‘Glenn Shunt’

The Glenn Shunt

July 17, 2009

One of the more familiar operations used by Congenital Cardiac surgeons is the Glenn Shunt (Also known as the Glenn Procedure, the word “Shunt” means “detour”.) Despite being revised from its original form and falling out of favor for a time, the Glenn is often used in the repair of defective hearts.

Developed by Dr. William Glenn in the 1950’s, the original operation may not be recognized  by today’s doctors. In what is now known as the Classic Glenn or the Unidirectional Glenn, the Superior Vena Cava (SVC) would be sewn closed near its junction with the Right Atrium. The Right Pulmonary Artery (RPA) is then cut and sewn into the SVC, and the open end of the Pulmonary Artery would be sewn closed. In this configuration, the Glenn Shunt only sends blood to the right lung. Here’s a good diagram of the Classic Glenn Shunt and here’s what I think is an even better drawing. The second link contains links to important information about both versions of the Glenn, worth your time to read. For the record, my first heart operation in 1967 was the Classic Glenn Shunt.

The Glenn fell out of favor after the Fontan Procedure was introduced. After years of neglect (I was told in 1977 by a surgical assistant that “we rarely do the Glenn any more”) it was looked at again when the early versions of the Fontan tended to not deliver the expected results. By then the operation had evolved into the Bidirectional Glenn Shunt. In the Bidirectional Glenn, the Superior Vena Cava is cut, and then re-sewn into the Right Pulmonary Artery. This is makes it bidirectional, as blood now flows to both lungs. Here’s a good photo of the Bidirectional Glenn (.pdf file) and here is a .pdf report on modeling a Bidirectional Glenn to study it’s affects on the individual patient. This report may appeal more to readers with a mathematical background, as the first part of the article is a complex discussion of the formulas needed to create the model.

Currently, the Bidirectional Glenn Shunt can be used as an option to repair most of the right-sided heart defects. It is also the second operation of the Norwood Procedure to repair Hypoplastic Left Heart Syndrome. (HLHS) It was first used in the Norwood in 1989; until then, the Norwood repair was a two surgery procedure.

And finally, here is a visual reminder to learn the anatomy of the heart yourself and not trust everything you find on the Internet: The text on this page correctly describes the Bidirectional Glenn Shunt, but the illustration is of a Blalock-Taussig Shunt!

8/2/2009 Update: I listed the same link twice when referring to two drawings of the Glenn Shunt! That has been corrected!

Everything old is new again

April 16, 2009

We had been at the University of Alabama at Birmingham (UAB) Hospital for only a few hours, just long enough to be admitted and assigned a room. A nurse and one of the Cardiac Surgical staff were asking us the “usual” admissions questions.

“Do you happen to know what operation you had at Johns Hopkins?” we were asked.

“Yes,” Daddy replied. “It was called the Glenn Shunt.” (We didn’t say the “Classic Glenn Shunt”; the Bidirectional Glenn Shunt had been invented at that time but wasn’t being used much. It wouldn’t become popular until the early 1980’s, and wouldn’t be part of the HLHS repair until the late 1980’s. I had never even heard of the Bidirectional Glenn!)

The Cardiac staffer put down his pen. “The Glenn Shunt… wow. They don’t do that very much any more.”

My eyebrows shot up. The operation that had saved my life was now in the trashbin of history.

“I guess that’s our…” he paused as he thought. “That’s probably the fourth or fifth option. They are pretty hard to take down.”

When you “take down” an operation, you basically undo it. There are times when you have to restore the heart to it’s original configuration (or as close as you can get it) before you attempt another correction. Because the Pulmonary Artery is cut, it’s pretty difficult to unhook it and reconnect it where it is supposed to be.

The true irony of this story wouldn’t be revealed until a week later, when  surgeon Dr. Albert Pacifico cut my Left Subclavian Artery and connected it to the intact branch of the Pulmonary Artery. That’s right – my second operation in 1977 was a repeat of the very first Congenital Heart Surgery:  the Blalock-Taussig Shunt, first done in 1944!

Everything old is new again!

More resources!

January 12, 2009

Did you know that the Centers for Disease Control (CDC) publishes an online assessment of Flu activity in the United States, updated weekly? They do, click here to read it. It is usually a week behind… it takes a few days for all the information to come into to CDC Headquarters in Atlanta and be compiled. The CDC Flu Report features lots of numbers, charts, and graphs, so if you tend to be a bit geeky, this is for you. (If your are female, single, and a bit geeky, feel free to contact me…!)

Google also has a Flu website, and they claim that their information is current. Their thinking is that when people begin to feel bad, they will go to their computer and enter their symptoms into a search engine, trying to determine if they really are ill. Google FluTrends is programmed to detect words used to describe flu symptoms, and it will report them both on a chart and visually. TIP: When you switch to a state view, the chart will be blank. It takes a few seconds to load the information.

Google is full of wonderful information, if you just know where to look. Typing the words “Congenital, Heart” into Google Book Search brings you a list of books about the subject, and clicking one of the links usually provides you with images of the cover and pages! Because of Copyright issues, some of the books are “limited preview”, and some are not available at all. This tool is useful for seeing what books are available on a subject.

Google Scholar is a compilation of technical works about a subject. This is where you enter can enter more elaborate terms into the search box, like Bidirectional Glenn. There are plenty of papers to study here, and the word is STUDY – these are by doctors for other doctors, with lots of medical words to slog through!

Just remember that not everything you read on the Internet is true… and it may not apply to you! If you look up information of Congestive Heart Failure (CHF), for example, most of the literature states that the average CHF patient survives for five years after the initial diagnosis. That accounts for all patients… including the ones who may already be suffering a debilitating illness and the ones who say “Oh my goodness, my heart is failing!” and just roll over and let it happen. If you change your diet, exercise, and take care of yourself, you can live well beyond the 5 year “limit”. I’m in my 7th year of CHF and doing very well!

Google Blogs will take you to a familiar website! It will also show you other blogs dealing with Congenital Heart Defects, and this may be the most useful tool that Google offers… the comfort of knowing that you aren’t alone!

The Secret Origin of the Funky Heart!

January 1, 2009

I’ll be in Washington DC for the Adult Congenital Heart Association‘s (ACHA) Lobby Day on February 10, 2009. The 10th is fine, but in a way I wish it was scheduled for a week later. February 17th, you see, will be the 42nd anniversary of my first heart operation.

I was born in the fall of 1966 and it wasn’t long before my parents realized that there was something wrong with me. My Pediatrician picked up on it fairly quickly, realizing that I had a heart problem, but he wasn’t sure exactly what the problem was. After seeing several doctors who were also stumped, my folks  took me to the Medical University of South Carolina (MUSC).

The doctors at MUSC figured out the problem – I had been born with a Congenital Heart Defect known as  Tricuspid Atresia. In Tricuspid Atresia, the Tricuspid Valve, located inside the heart between the Right Atrium and the Right Ventricle, never formed. But instead of there just being a hole with a missing valve, I have a wall there. Blood flow is completely blocked.

Blood normally flows into the Right Atrium and down into the Right Ventricle; then is pumped out to the lungs. It return to the heart through the Left Atrium, then to the Left Ventricle, and is pumped out to the body. Block the blood flow through the heart and you kill the owner.

What keeps me alive is the fact that there are holes in the wall that seperates the left and right sides of my heart. My blood literally takes the senic route to get through my heart, and on its journey oxygenated blood and deoxygenated blood are allowed to mix. That normally doesn’t happen, and because of it I am Cyanotic – the amount of oxygen in my blood is lower than normal.  I gasp for breath and tire easily;  and my fingernails and lips have a slight bluish tinge. The blueness really becomes pronounced in cold weather.

The doctors at MUSC told my parents that I had Tricuspid Atresia, and that they should take me home and love me, for I was not long for this world. That didn’t sit well with my parents, who went back to my local Pediatrician and said “We need to try something,” even though they had no idea what “something” was. The Pediatrician gave them four places that could attempt a surgical repair: Houston, Texas; Mayo Clinic in Rochester, Minnesota; the University of Virgina; or Johns Hopkins Hospital in Baltimore, Maryland.

Houston and Mayo were eliminated because of the travel distance. The doc threw out Virgina, the medical school he had graduated from, because he felt they didn’t have enough experience with the operation. That really left one choice, and the doc said that he’d get the wheels in motion with the doctors at Hopkins.

That night all hell broke loose. I went into heart failure, with a pulse of 200 and a temperature of 103. It took them five days, but they were able to get things under control at my local community hospital. Meanwhile the doctors at Hopkins were taking the information from my local doctor over the phone and telling him they wanted to see me as soon as someone could get me there.

The folks hit the road, driving into one of the worst snowstorms to hit the East Coast in memory. Things were OK for a while, they finally stopped somewhere in Virgina to rest (this was long before Interstate 95 was completed). During the night, my mother got up to use the bathroom, glanced out the window… and saw it snowing. She woke up my father; we were in the car and moving within 15 minutes.

Every few miles Daddy would pull off the road long enough to wipe off the windshield with an old rag he had found in the trunk. The snow was getting deeper and more and more cars were stalled along the highway, and at one point my father thought about putting Momma and me on a train for Baltimore and catching up to us later. It never came to that, thankfully.

The Hopkins Admissions Office expected three families from out of town to arrive that day, we were the only ones who made it. The doctors at Johns Hopkins examined me not long after I arrived and Cardiologist Richard Rowe gave my parents the news. “He’s down to hours. We have to operate tonight.”

Dr Alex Haller, the Pediatric Cardiac Surgeon, should have done my operation. He was in Washington DC, trapped there because of the snow. Dr. Vincent Gott, Chief of Surgery, stepped in for him. The surgery was known as the Glenn Shunt – a variation of the operation, known as the Bidirectional Glenn Shunt, is performed every day in operating rooms around the world. But at that time it was new, so new that I was the 5th person to survive the operation.

And sometime over the next few days, my father picked up a copy of The Baltimore Sun and gasped. The snow had been so heavy, it reported, that train service into Baltimore had been halted. If he had put us on that train, I would have died.

When I went to Baltimore for the ACHA a few months ago, I had hoped that the schedule would allow me to go back to Hopkins and look around. Not that I expected to know anyone – doctors retire or move on, especially after 42 years. In fact, I don’t remember a single person. I vaguely remember a statue. I’d like to see Him again.

Ten and a half feet tall, standing on a podium, a statue of Christ greets visitors to Johns Hopkins Hospital. His name is Christus Consolator, or The Divine Healer.

I think we’d have a lot to talk about.

Know the score

December 16, 2008

WARNING: You may find this post to be somewhat disturbing. I stumbled across it quite unintentionally, looking for something else.

Have you ever heard of the Aristotle Complexity Score?

Since 1999, Congenital Heart Procedures have been assigned a score based on how complex the operation is. To calculate the Basic Score, you assign points to three different factors, then add those factors together. Since the low number on each scale as a 1 and the high is a 5, the Basic Score is a range between 3.0 and 15.0.

The reason for the Aristotle system is not to claim “bragging rights” over other surgeons (and I certainly hope it is not used for that!) but rather to improve patient care. If the Operating Room calls the Cardiac Critical Care Unit and tells them that a patient is coming down who just had surgery, and the Aristotle Score is a 10, then CCCU knows to keep their eyes open. This patient just had a major operation and they are very ill. And just by quoting that one number, every Cardiac caregiver down the line will know exactly how fragile their patient’s health is.

The first factor used in the Basic Score is mortality. Based on previous research, this takes into account the chances that the patient will die during the operation or shortly afterward. 1 point is given for an expected mortality of less than 1%, while 20% or greater earns a maximum of 5 points.

The next factor is morbidity. A simple definition for morbidity is “a diseased state or symptom.” The Aristotle system bases the morbidity score on the lengh of time spent in the ICU. Less than 24 hours is one point, longer than 2 weeks is five points.

The third factor is the perceived difficulty of the operation. No heart surgery is simple, of course, but fixing an ASD is less complicated than completing a Bi-directional Glenn Shunt. So an operation of the least perceived complexity is again worth one point, and the most difficult is worth five points.

There are other factors that can influence the Basic Score higher or lower – patient’s age, gender, overall health, etc. – but just looking at the Basic Scores can be informative…. and scary!

The “simplest” operation is an ASD repair, rated 3.0.

My Classic Glenn Shunt (a “unidirectional cavopulmonary anastomosis”) is rated a 7.0.

The Modified Blalock-Taussig Shunt (using an artificial conduit) is a 6.3, while a Blalock-Taussig done with a rerouted artery is a 6.8.

The Norwood Procedure (Which is actually three separate operations, but considered one corrective procedure) is the 2nd most complex operation with a 14.5 rating. The only thing more difficult is an HLHS Biventricular Repair, which rates the highest possible Basic Score, 15.0.

We all know Heart Surgery is serious, and knowledge of the Aristotle System only gives us a glimpse of just how serious it is. A surgeon is literally walking into a minefield, and how many of us would consent to an operation (for ourselves or our child) if we knew the true amount of skill that is required?

You can’t just be good and be a Congenital Heart Surgeon… you need to be among the best!

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?

On the Street Where You Live

November 6, 2008

This past week I’ve spoken a lot about Katie, sometimes I wonder if I’ve said a little too much. I’ve been asked, both online and in real life, if maybe I’m paying just a little too much attention? It’s not really a surprise, as I thought about it myself.

I’ve never met Katie or her parents. All I have learned about them, I’ve read on their website. It’s been a hard thing to try to follow the recent news about her, because it hits so close to home. Katie is five months old – the same age I was when I had my first heart surgery.  She’s had the Bi-Directional Glenn; I’ve had the original version of that operation. Katie is at Yale New Haven Hospital, where Dr. William Glenn himself lived and worked. I’ve read that Dr. Glenn, despite being a world famous heart surgeon, would leave his home on Saturday mornings and walk to Yale’s football field to cheer on the home team. He sounds like my kinda guy.

Katie has Hypoplastic Left Heart Syndrome (HLHS). I have Tricuspid Atresia, which is occasionally referred to as one of the defects that make up Hypoplastic RIGHT Heart Syndrome. (HRHS) The term HRHS wasn’t even invented when I was born (I don’t think anyone had thought of HLHS, either) so I’ve always just referred to myself as having Tricuspid Atresia. But when you think about it, Katie’s heart and mine are nearly mirror images of each other.

Spooky. As the title of this post notes, it hits you on the street where you live.

After my surgery at Johns Hopkins in 1967, one of the doctors mentioned in passing that we were one of only two families who had made it in that night. The other child had been born premature, and despite being healthy in every other respect, hadn’t made it. The doctor shook his head. “Some children…they just aren’t fighters.”

There’s no doubt about it, little Katie is a fighter. She’s hanging on for all she’s worth, clawing and spitting and digging her nails in. Keep fighting, Katie! I’m pulling for you!

Sunday Night Links!

September 28, 2008

I think I have forgotten how to read a calendar: I thought for sure that this week, I had a Cardiologist’s appointment in Atlanta. It’s next week, October 8. I’ll be gone Tuesday and Wednesday, but I’m taking my laptop and I should be able to post. The date on your links might look a bit iffy, but they are not stale; and as of 10:22 PM EST 9/28/2008, everything works. It’s just been a busy week around here! Good information never goes stale!

Here’s a study of survival rates for the Bi-directional Glenn Shunt, the results look good. This study suggests the numbers can be even better!

A DNA test could change organ transplant procedures forever!

I’ve written about Electronic Medical Records before. Here are two articles by a Healthcare Chief Information Officer: Electronic prescriptions are going to be easier said than done, and there are just some personal health records that won’t be transmitted. Meanwhile, Beth Israel Deaconess Medical Center in Boston gives you one of these when you are discharged from their Cardiovascular Institute.

Here’s some local news: Clemson University researchers are working on heart valves. Clemson is right here in my home state, and although I support the Gamecocks, it’s great when the “other guys” do good, especially when it is something this important. Despite nasty rumors you might find on the internet, a Clemson heart valve does not make you want to shout “GO TIGERS!” on Saturday afternoons in the fall!

It was a Dark and Snowy Night…

July 27, 2008

The best program on TV recently has been Hopkins, the six part documentary featuring the doctors and patients of Johns Hopkins Hospital in Baltimore, Maryland. Ironically, the show was probably born during the recent writer’s strike… there’s no need for writers when you find a willing subject who will let a film crew follow him (or her) around. Of course, you run the risk of getting either a boring subject or a slow day, or both. The producers claim they shot 1,500 hours of film, but the show consists of six hour long episodes. That’s a lot of rejected footage.

The “star” of the show is the hospital itself, Johns Hopkins. If you want the best medical care available, Hopkins is the place. U.S. News and World Report ranks America’s hospital’s every year, and either Hopkins or The Mayo Clinic (located in Rochester, Minnesota) are almost always ranked number one.

I’ve been very interested in the show because I had my first heart operation at Johns Hopkins. On February 17, 1967 — at the age of five months — I was in the operating room. The problem was, my surgeon wasn’t there.

Dr. Alex Haller was supposed to operate on me, but he was stuck in Washington, DC. The operation had already been discussed and planned for that summer, then I became critical and it had to be done as soon as possible. Most of the East Coast was snowed in that night; how my parents even got me to Hopkins from South Carolina is a miracle. They left home the day before when my local pediatrician said things were starting to go to hell in a handbasket, and stopped for the  night at a hotel in Richmond, Virginia. My mother just happened to get up during the night, glanced out of the window, and saw that it was snowing pretty hard. She woke up my father and within fifteen minutes, we were back on the road. Interstate 95 hadn’t been completed, so most of the travel was on secondary roads. Daddy was stopping every half hour to clear his side of the windshield with an old cloth, but somehow we avoided getting bogged down. After passing the “Welcome to Baltimore” sign they stopped at the first Esso station they saw (Happy Motoring!) And asked directions. The attendant, very familiar with out of towners visiting Johns Hopkins, said “Stay on this road and go through 13 stop lights. At the 14th light, turn right.” Daddy was doubtful but the directions were precise.

After an examination – an examination at 10:30 at night, mind you, outstanding hospitals do those sort of things – my Cardiologist, Dr. Richard Rowe, told my parents “It’s critical. We’re going to surgery tonight.”… and my surgeon was stuck in the snow forty miles away. Dr. Vincent Gott, the adult heart surgeon, stepped in for Dr. Haller. No matter your age or your gender, your heart is roughly the size of your fist. Imagine the size difference between an adult fist and an infant’s, and you can see the challenge that faced Dr. Gott.

My operation was known as the Glenn Procedure, or sometimes called the Glenn shunt. Readers familiar with the operation may nod their head sagely, but they would be completely wrong. The operation was changed in the mid 1980’s, and my 1967 version doesn’t resemble it. The modern operation, sometimes called the Bi-directional Glenn Shunt, disconnects the Superior Vena Cava from the heart and connects it to the Pulmonary artery, sending blood to the lungs. The Classic Glenn Shunt – mine – disconnects the Superior Vena Cava and connects it to the Pulmonary Artery. The left branch of the Pulmonary is also clamped and cut, making the right lung responsible for oxygenating all of the blood. I didn’t even know I had Version 1.0 until a few years ago, when a Cardiologist slapped my X-ray onto the lightboard and studied it. “I don’t know what the hell this is, but it ain’t a Glenn Shunt,” he said.

Dr. Gott did a great job; I was the fifth person at Hopkins to survive the Glenn Procedure, and the first one to be physically active. And 41 years later I’m still going.

As I am watching Hopkins, I’m keeping my eyes open for the Jesus statue. Really it is named Christus Consolator, or The Divine Healer, and is located in the main administration building. I had to make the trip to Baltimore yearly for several years for follow-up appointments, and seeing that statue as we walked into the hospital is my only memory of the hospital. I can’t remember doctors, nurses, or buildings, but I remember seeing that statue. This statue of Jesus Christ is ten and a half feet tall, and the podium He is standing on is probably four feet high. And when you are five years old, He looks really big!