Posts Tagged ‘Tetralogy of Fallot’

24 hours at Johns Hopkins

November 28, 2010

“Because your whole world can change in 24 hours.” – The Paper (1994)

Tuesday, November 28, 1944: Sometime during the evening of the 28th, Dr. Alfred Blalock places a telephone call to the Surgical Laboratory at Johns Hopkins Hospital. His Surgical Assistant, Vivien Thomas, has recently developed a surgical correction for the heart defect known as Tetralogy of Fallot, also known as Blue Baby Syndrome. The two men have planned for Thomas to teach Blalock the steps needed to successfully complete the surgery during an operation on a dog. Blalock has done the operation only once and many more teaching sessions are needed.

Blalock is calling with grim news: Earlier he had asked Thomas about the possibility of operating on 19 month old infant Eileen Saxon. Weighing only nine pounds and often cyanotic, the dusky blue color that gives this malady its name, she is deteriorating rapidly. At the moment she is so cyanotic that she is purple and is struggling for every breath. Dr. Blalock tells Thomas to meet with Elizabeth Sherwood, the operating room supervisor, first thing in the morning. Thomas has invented several surgical tools specifically for this operation and he is to make sure that they are available.

Thomas is stunned and reminds Blalock that he doesn’t know the operation very well. “But if you don’t get ahead of yourself, break it down into smaller and smaller steps as you work, it can be done.” It is one of the familiar sayings Thomas uses when he is teaching proper surgical procedure and for a moment, Blalock feels as if he is the assistant.

After Blalock hangs up, Dr. Helen Taussig orders him home. Blalock protests, but she reminds him that he plans to operate in the morning – an operation that could very well be emergency surgery. The hospital has his telephone number should he be needed during the night. At roughly the same time, Thomas and Blalock leave for their respective homes. Segregation is still prevalent at the time and Thomas leaves by a back entrance; neither man knows the other one has left.

Dr. Taussig spends the night on the ward; Eileen’s parents are also there. Although they don’t know it, this is an ominous sign: in the 1940’s, visiting hours rules were strictly enforced unless a patient was seriously ill.

Wednesday, November 29, 1944: Too nervous to drive, Blalock asks his wife Mary to take him to the hospital. She lets him out of the car in front of the towering Johns Hopkins dome. Dr. Blalock enters the building, walks through the rotunda (rubbing the toe of the Statue of Christ for luck, an old Hopkins tradition) and turns left. From here he exits the building through a side door, walks approximately 50 yards, and into the Harriet Lane Home for Invalid Children. Vivien Thomas enters the Hopkins complex from a side entrance and goes immediately to Elizabeth Sherwood’s office. Miss Sherwood knows nothing about Dr. Blalock’s plan to operate but immediately shows Thomas the selection of items that will be available to Dr. Blalock. Thomas adds custom-made clamps and needles to the collection. These needles are no more than 1/2 inch long. Thomas insists that the clamps and needles not become part of the general operating room supplies – they have been custom made for this operation only.

Blalock and Taussig examine the child and confer. Eileen has not improved during the night, and Taussig concedes that there is nothing else that she can do. She leaves the meeting as Thomas arrives, perhaps to return to Eileen’s bedside or for a quick trip to the Cafeteria. Blalock and Thomas discuss the upcoming operation. They go over some of the more critical steps, and also discuss “routine” points such as where the incision should be made. Thomas informs him that Miss Sherwood has promised that the large operative theatre will be available but needs to know when the operation will begin. Blalock decides that the operation will take place after the morning rounds, unless events dictate otherwise. He leaves to confer with Eileen’s parents and to conduct Rounds. Thomas did not normally participate in Rounds so he would have gone to the Surgical Lab, although he may have gone to his office. He calls Miss Sherwood and informs her of Blalock’s decision.

The operative team convenes in the Scrub Room annex connected to Room 706. Although first chosen at random, the majority of Hopkins’ early heart surgeries will take place here and the room will come to be known as “The Heart Room.” Dr. William Longmire and Dr. Denton Cooley will assist. An unknown person sets up a movie camera pointed at the operating room table; this film still exists in the Johns Hopkins Hospital Archives.

Blalock continues to discuss the upcoming operation with Thomas as he prepares for surgery. Thomas is not scrubbed in and has no intention to – he is not allowed on the Operating Room floor. He will be seated in the raised seats of the theatre, however. Helen Taussig will be in the Operating Room, even though she is not a surgeon. She’ll spend most of her time at the head of the table, monitoring the patient.

A few minutes before Eileen arrives, Blalock quietly asks his scrub nurse to find Thomas and help him get scrubbed in. As expected, Thomas is seated in the bleachers above the OR. Blalock also orders a milk crate and has it placed behind him. Thomas stands on the crate, peering over Blalock’s shoulder at the operative field.

The operation begins with a curving incision near the 4th rib on the child’s left side. With Thomas guiding him, Blalock gently works past the lung and cuts a path to the heart. The heart is small, dark, and obviously struggling. William Longmire later said “I remember watching him open the patient and just thinking it was impossible.”

Blalock works patiently, finding the Left Subclavian Artery and the left branch of the Pulmonary Artery. He places a clamp on the Subclavian to cut off blood flow – using one of the clamps designed by Vivien Thomas for this procedure – and cuts it. He then places two similar clamps on the left branch of the Pulmonary Artery. Making a small opening in the Pulmonary Artery, Blalock uses the tiny needles Thomas has prepared to sew the Subclavian Artery into the Pulmonary Artery. After double checking his work, Blalock removes the clamps. He is unable to feel blood flowing through the new connection.

Legend has it that Helen Taussig said “Al, the child’s lips are a lovely pink color!” The Johns Hopkins online exhibit about the operation states that the anesthesiologist said “The boy’s a lovely color now!” at a later date, during the third operation. Blalock’s operative notes comment that the circulation in the nail beds of Eileen’s left hand “appeared to be fairly good.”

The difficult segments are complete but the operation is far from over. Sulfanilamide (an antibiotic) is introduced into the incision and Blalock begins to close. He sews the soft tissue closed with silk sutures and is finally done. The operation has taken about ninety minutes. (CLICK HERE to perform the Blalock-Taussig Procedure yourself. Read Blalock’s operative notes here:  PAGE 1 PAGE 2)

Eileen is moved to the Recovery Room, where Dr. Henry Bahnson is responsible for her care. As one might expect, Blalock and Taussig look in on her often.  Bahnson’s opinion is that the little girl is still very blue but improves over time. Eileen’s mother comments “When I saw Eileen for the first time, it was like a miracle… I was beside myself with happiness.” Very little is known of Thomas’ movements after the operation. He is seen in Recovery and also in his Lab.

As the sun sets on the city of Baltimore, Eileen remains in critical condition but she is stable. The operation is a success, but in a few months it will fail and she will need another Blalock-Taussig Procedure, this time on her right side. She will die just before her 3rd  birthday.  The doctors determine that the surgery is more suited to an older child whose blood vessels have had a chance to grow. In early 1945 Blalock and Taussig co-authored a medical journal article about the first three procedures. Hundreds of patients would flock to Johns Hopkins Hospital to receive the life-saving surgery, even though the odds were long: an article in the February 17, 1947 issue of The American Weekly noted that 14 of the first 70 patients had died.

But parents noted that 56 of them had lived and were growing up, something that had never happened before. The era of Congenital Cardiac Surgery has begun.

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The Gross-Taussig Shunt

March 4, 2010

There was once a heart surgeon who was the acknowledged expert in Pediatric Heart Surgery. Dr. Helen Taussig once approached him asking for advice about re-routing some blood vessels to relieve a Congenital Malformation of the Heart known as Tetralogy of Fallot (ToF).

This surgeon was not Dr. Alfred Blalock.

As I mentioned yesterday, everyone is born with two heart defects: The foramen ovale and the  ductus arteriosus, which is a direct connection between the Pulmonary Artery and the Aortic Arch. It allows blood coming from the Right Ventricle to bypass the lungs, and it closes shortly after birth. If it fails to close it is refered to as a “patent ductus arteriosus” (PDA), and can lead to Congestive Heart Failure.

Robert Gross was born in 1905 and by 1938 was the Chief Resident at Children’s Hospital in Boston.  Children’s Hospital had been experimenting with closing PDAs in animals and was having success, and Gross was eager to try the repair in a human. Being a good Resident, he asked his boss, highly respected Surgeon-in-Chief  Dr. William E. Ladd. Ladd said no – we do not touch the heart.

Not long after that, Dr. Ladd went on vacation. Dr. Gross went to his substitute, Dr. Thomas Lanman, and again asked permission. Lanman gave him the OK, and on August 26, 1938, Dr. Gross closed the PDA of  a little girl named Lorraine Sweeney. Everything went perfectly.

Dr. Ladd came back from vacation, learned what had happened, and fired Dr. Gross.

Gross wasn’t out of work for long – the higher-ups at Boston Children’s thought the surgery was a great thing, and sent Dr. Ladd to rehire Dr. Gross! Rather than holding a grudge against Gross, Ladd came to realize just how good he was and the two teamed up. Working together, the two devised several new heart operations over the years and experimented with valve replacements and a heart lung machine. When Dr. Ladd retired, Dr. Gross was selected to replace him.

In the early 1940’s a young female doctor from Baltimore visited Dr. Gross. Since he was one of the leading Cardiac Surgeons of the time, she discussed with him a theory that she had: Although it was impossible (at the time) to do surgery on the heart, she felt that Tetarology of Fallot (ToF) could be relieved by redirecting some of the major blood vessels.

Gross was intrigued by the idea. When this doctor offered him the chance to actually help develop such an operation, Gross hesitated. She seem to be smart enough, and she did work at Johns Hopkins. They were as prestigious as Boston, and wouldn’t just hire anyone. But there were so few female doctors around no one would take her seriously…. and he turned her down.

So Helen Taussig, M.D., got back on the train and returned to Baltimore. A few years later Alfred Blalock was named Chief of Surgery at Johns Hopkins. Had Gross accepted Taussig’s offer, the first Congenital Heart Surgery could have been named the Gross-Taussig Shunt.

If it had worked. Without Vivian Thomas – who came to Hopkins with Dr. Balock – the operation could have failed and wrecked both of their careers.

Hero in the Halfpipe

March 1, 2010

With the Olympics drawing to a close, the Congenital Heart Defect (CHD) community has been abuzz about the exploits of a talented young man you might have heard of. Unless you have been completely out of touch for the past two weeks, you’ve probably heard about Shaun White.

We love the guy; after all, he’s one of us. Shaun was born with a heart defect known as Tetraology of Fallot (ToF) and he’s out there living his life and not letting anything slow him down. A lot of CHDers have (almost) normal lives, too, but we aren’t Double Gold Medal winning Olympic Champions. And if he has any say in the matter, he’s aiming for the next Winter Olympics, too.

Homeboy is living LARGE. (Yeah, I’m a little jealous! Who wouldn’t be?)

There is a small problem here, though: Right now, Shaun is in the perfect position to be a great spokesman for those of us living with a Heart Defect. The public loves him. Heart parents, Cardiac Kids, and even tired ol’ Funky Hearts look up to him. But he just doesn’t seem to be interested!

“I had heart problems,” he said on The Oprah Winfrey Show – the closest she has come to discussing CHDs in 25 years. HAD? I thought. What happened? Where did those “heart problems” go?

But when you think about it, his comment starts to make sense. Every heart defect is a little bit different, and affects each one of us a little bit differently. Shaun is obviously pretty healthy (two Olympics, two Golds, remember?), one of the more fortunate CHDers that I know of. And he’s only 23 years old. He’s young, he’s healthy… and he may not realize that a Heart Defect is a lifetime thing. And that is when it hits you:

Shaun White may be one of the many CHDers who has been lost to good Congenital Heart Defect care.

I am hopeful that as time passes and this young man grows a little older, two things will happen: One, that he realize that he isn’t invincible after all, and will find a good Cardiologist who can help him keep that wonderful level of health that he has. Way too often, CHDers “crash” as they grow older – not just the normal effects of age, but age coupled with an old, tired, damaged heart bring us down more than usual. I certainly hope that he can avoid that.

And second, perhaps Shaun White, with a little age and maturity, will become not only the Hero in the Halfpipe but a true Heart Warrior, fighting for all the CHDers who cheer from the sidelines.

No greater love

February 26, 2010

UPDATE: Zeb’s surgery got pushed back – the new date is March 2, 2010.

Here’s a bittersweet report that I read earlier today: The newest generation of Cardiac CT scanners reduce radiation exposure by up to 91%.

My friend Jim could have used one of those new scanners. Jim was 54 years old; he was from an older generation of survivors. Back in those days when CHD kids were few and far between and hardly anything was known about heart defects, doctors would X-ray and scan a CHD heart from every possible angle.

But no one realized the amount of radiation those children were absorbing. So fast forward to today, and our first and second generation of Heart Warriors are passing on. A fair percentage of them are dying not of their heart problems, but rather of various cancers.

There’s no real medical evidence (yet) to back this theory up, just stories from friends and relatives and survivors about all the testing they went through, coupled with the knowledge that an unusual number of us seem to pass on because of cancer.

Greater love hath no man than this, that a man lay down his life for his friends, reads the scriptures. Jim was a true Heart Warrior, in every sense of the word – he stood up for all of us when there was hardly anyone around to stand up.

Even when he was a little kid.

Partners of the Heart

November 24, 2009

In honor of Red and Blue Day, what follows is a reprint of an article I wrote for the November 2009 issue of The Right Heart Times, the newsletter of the CHD support group Hypoplastic Right Hearts:

The Blalock-Taussig Shunt (Shunt means “detour”) was the brainchild of one of the most unusual people in medicine: Dr. Helen Taussig. Despite being Dyslexic and slowly losing her hearing after becoming a doctor, Taussig had overcome both disabilities to become the head of the Cardiac unit at the Harriet Lane Home for Invalid Children, located at Johns Hopkins Hospital in Baltimore, Maryland.

While at Harriet Lane she began to study Congenital Heart Defects, especially Tetralogy of Fallot (ToF). ToF children suffered from a combination of four heart defects which led to the mixing of oxygenated blood with unoxygenated blood inside their damaged hearts. This caused them to have Cyanosis (have a bluish tinge to their skin due to poor blood oxygenation), have poor stamina, difficulty feeding and usually die before they reached ten years old.  Despite the fact that these children were breathing hard and deep, they were suffocating – and there was nothing that Dr. Taussig could do about it.

Taussig’s frustration would continue until 1943, when Hopkins hired Dr. Alfred Blalock as the new Chief of Surgery. It wasn’t long after his arrival that Taussig and Blalock had a conversation that would change the world.

Hopkins legend states that Dr. Taussig literally broke into a conversation between Dr. Blalock and her boss, Dr. Edwards Park, and convinced him to attempt a surgical repair of the defect. Blalock reminded her that it was impossible to operate on the heart (at that time it was impossible) but Taussig contended that what she had in mind was not an operation on the heart itself, but moving the blood vessels around to send more blood to the lungs. She had the idea, but since she was not a surgeon she could not act on it.

Little did she know that he already had a partial answer. While studying the effects of shock on the human body, Blalock and his assistant Vivien Thomas had sewn a smaller artery onto the Pulmonary Artery in an attempt to increase blood pressure. Blood pressure had not been affected, but blood flow increased. The challenge now was to recreate the effects of ToF in a dog, perform the arterial connection, and evaluate the results. Swamped with his teaching duties and surgical schedule, Blalock turned the assignment almost completely over to Thomas. An African American with a high school education, Thomas had gotten a job in Blalock’s lab after dropping out of college and had become Blalock’s most able assistant.

Re-creating either the heart defect or the planned repair often proved fatal for the dog, but finally Thomas found the perfect combination and a mutt named Anna survived. The next step was to teach Blalock the procedure. The surgeon had observed the operation several times but had never done it himself; Thomas had done the procedure several hundred times – all on dogs.

On November 29, 1944, the trio tried the new surgery. They may have operated sooner than they wanted to, but young Eileen Saxon’s condition was deteriorating. Although she was 15 months of age, Eileen weighed only nine pounds and was badly cyanotic.

As they were preparing for surgery, Blalock turned to his scrub nurse and quietly asked her to summon Mr. Thomas. Although Thomas had taught him the procedure, he wanted his assistant close by in case there was a problem. Thomas entered the surgical suite and stood behind Blalock, guiding him through the operation and giving advice.

Making a five-inch incision on Eileen’s left side, Blalock clamped and cut her Left Subclavian Artery. The Left Subclavian branches off of the Aorta, travels along the shoulder blade (the Clavicle) and down the left arm. For a visual reference, the Left Subclavian Artery is located almost directly behind a police officer’s badge.
Blalock then placed clamps on the left branch of the Pulmonary Artery and made a small hole in the artery. Gently pulling the Subclavian downward, Blalock sewed the vessel onto the Pulmonary Artery, took a deep breath, and disconnected the clamps.

Eileen’s cyanosis almost instantly faded. “She’s a lovely color now!” Taussig exclaimed. Blalock’s surgical notes are a bit more understated, reporting that “the circulation in the nail beds of the left hand appeared to be fairly good at the completion of the operation.”

Originally known as a “subclavian to pulmonary anastomosis,”the operation was soon renamed the Blalock-Taussig Shunt, after the surgeon who performed it and the doctor who conceived it. Thomas received almost no credit for his part of the procedure during his lifetime.

Survivors of the Blalock-Taussig Shunt often have difficulty getting a pulse or a blood pressure reading in the arm on the shunt side (because of the disconnected Subclavian Artery) and should avoid having injections into that arm. In the late 1970’s the Modified Blalock-Taussig Shunt (MBTS) became popular. The MBTS leaves the Subclavian intact and makes the Subclavian-Pulmonary connection by inserting an artificial tube and avoids the arm problems created by the original Blalock-Taussig.
Eileen Saxon did well for a few months but again became Cyanotic as her shunt failed. She underwent another Blalock-Taussig Shunt (on her right side this time) but passed away just before her third birthday.

Blalock’s surgical team performed almost 200 Shunts in the space of a single calendar year and the operation opened the door for Congenital Cardiac Surgery. He continued to operate until just before his retirement in 1964, and died six months later.

Dr. Helen Taussig became known as “the Mother of Pediatric Cardiology” and had a part in averting the Thalidomide crisis in the early 1960’s. She retired in 1963 but often returned to Hopkins, staying current on the latest Cardiac research and contributing  much of it herself. She was killed in an automobile accident in May of 1986.

Vivien Thomas continued to stand at Blalock’s shoulder and eventually became Director of the Johns Hopkins Hospital Surgical Research Laboratories. He trained many of the surgeons who would become famous for their heart surgery accomplishments and invented many of the procedures that they would use. He received an honorary doctorate in 1976 and retired in 1979. Thomas wrote his autobiography, Partners of the Heart: Vivien Thomas and his work with Alfred Blalock and died in 1985, just before the book was published.

Anna the Dog served as the mascot of the Johns Hopkins Surgical Labs until her death in 1957.

The Dirty Little Secret of HLHS

October 1, 2009

She didn’t even have a name for it.

When Dr. Helen Taussig wrote Congenital Malformations of the Heart in 1947, she described one malformation as “Atresia or marked hypoplasia of the aortic orifice prevents the expulsion of blood from the left ventricle in the normal manner.” Taussig described several variations of the defect – actually different defects, later grouped together under the same deadly name – but could offer no treatment suggestions. Her Tetralogy of Fallot (ToF) patients could at least squat and get some temporary relief; children cursed with this malady died in less than one week. The left side of the heart wasn’t damaged as much as it just wasn’t there.

It wasn’t until 1958 that Dr. Jacqueline Noonan and Dr. Alexander Nadas named the group of defects Hypoplastic Left Heart Syndrome (HLHS). Hypoplastic comes from the root word hypoplasia, which means “small”.

But having a name for it didn’t make it any less deadly as HLHS continued to claim 100% of its victims. This sad story continued until 1985, when the first successful infant heart transplant took place.

At about the same time the Norwood Procedure was developed. Originally designed as one operation (which consistently failed) the procedure was soon split into two heart operations – and eventually three – which seemed to work. HLHS children now had a chance. Obviously long term survival rates aren’t known yet, but approximately 70% survive the three surgery protocol.

And thats when the dirty little secret of HLHS showed itself: While survival rates for the three operation procedure may be in the 70% range, getting from Stage I to Stage II is the hardest step.

The goal of the Stage I operation is to make the Right Ventricle do the job of the defective Left Ventricle – getting the blood to the body. With all of the blood flowing through the right side of the heart, that side is subject to higher flow pressures than it usually receives. A lot higher. In a defect in which the entire left side of the heart is damaged, a successful outcome usually depends on the Tricuspid Valve – located on the right side of the heart.

The inital results were confusing. The Norwood worked – then it didn’t. There didn’t seem to be any rhyme or reason to it, no way to predict outcomes. The surgery itself worked, but too often the results  seemed to be as random as a roll of the dice. The answer was the Sano Shunt, invented by Japanese surgeon Shunji Sano – a direct connection from the Right Ventricle to the Pulmonary Artery through a Gortex conduit. (this sounds redundant, but the Pulmonary Artery is normally disconnected from the ventricle in the Stage I operation). Studies showed that survival chances with the Modified Norwood was 11 times greater than with the Classic Norwood.

And now it is time to turn the tables – we’ve got a nasty little trick of our own. Heart defects have to start somewhere… at some point in fetal development, there must be one isolated problem that seems to “snowball” into something bigger as time passes. If we could find that one little problem and repair it then, maybe we can stop the snowball before it gets too big. A pretty cool idea, and maybe one day…

Someday…

A study released Monday (September 28, 2009) revealed that for seven years, doctors at several hospitals in Boston and Harvard Medical School have been detecting Aortic Stenosis in fetuses. Fetal Aortic Stenosis is a snowball; it usually becomes HLHS as the fetus develops.

70 future HLHS patients underwent surgery while still in the womb – a needle was inserted into the mother’s abdomen, passed into the fetus, and into the heart of the unborn child. A small balloon was used to enlarge the abnormally small Aortic Valve. 51 of the procedures were considered successful… and 30% (17 children of the 51) were born with two functional ventricles. That’s 17 children who won’t have HLHS.

Mark September 28  on your calendar… that was the day that the CHD world changed forever.

New studies of ToF

July 30, 2009

There is new research into the causes of Tetralogy of Fallot (ToF), focusing on the genetic makeup of ToF hearts.

“Tetralogy” means “four parts,”  and ToF is a combination of four separate problems : a Ventricular Septal Defect (VSD);  Pulmonary Stenosis; an Overriding Aorta; and an extremely muscular Right Ventricle. (Here’s a diagram)

ToF accounts for about 10% of all heart defects, and was the defect that the Blalock-Taussig Shunt was designed to relieve. The B-T Shunt was a palliative measure, meaning that instead of curing the defect, it was meant to relieve it. Even today there is no cure for Tetralogy of Fallot, although it can be surgically repaired.

ToF has always been a puzzling defect because the parents of a Tet child usually have no cardiac issues. Recent research only deepened the mystery as it seems that there is no genetic predisposition, either. ToF seems to “pop up” when it wants to.

Scientists at the Howard Hughes Medical Institute have recently found some clues into the genetic makeup of  the defect. After scouring the DNA of 114 Fallot patients, they have found 11 segments that might lead to the occurrence of Tetralogy of Fallot. They are so small that if you put all these DNA segments together, you can’t see them.

Technically these segments are known as copy number variations. The segments control how much protein is produced by a cell, and the variations can cause too much of one protein, not enough of another – and alter your health forever. So now the work shifts to identifying and controlling individual genes. One has already been found: change that gene, and the risk of having ToF multiplies by nine.

Perhaps this research will one day lead to a pill or injection for expectant mothers  that will stabilize the child’s DNA and prevent the occurance of Tetralogy of Fallot!

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?