Archive for the ‘Blalock-Taussig shunt’ Category

Bing

April 2, 2010

He was born in Germany and composed many works of classical music. He graduated from medical school twice. He worked with Charles Lindburgh. And he started a Cardiac Catheterization program at the hospital where heart surgery was born. Not only did Dr. Richard Bing see history being made, he helped make it.

Hired in 1942 as an instructor in the Johns Hopkins University Department of Medicine, Bing traveled from New York to his new job in Baltimore. Crossing the Chesapeake Bay on a ferry, Bing noted no less than six people with Cyanosis. Their destination, he surmised, was probably the hospital. It was two years before Blalock and Taussig would attempt the first Congenital Heart Surgery, but Johns Hopkins already had a reputation. If your child has a bad heart, it was said, get them to Hopkins.

There wasn’t much at all that could be done, but Dr. Helen Taussig knew almost everything there was to know about the human heart. Taussig was a huge believer in research, and would spend hours examining defective hearts. Despite her best efforts – she often asked the parents of her deceased patients for permission to dissect the heart, and spent hours studying them in her lab – there was very little progress being made. Even after the first heart operation, very little was known. There was ONE operation, and it was designed to help relive only ONE defect. Everyone else was still out in the cold.

By 1945  Dr. Alfred Blalock asked Bing to set up a Cardiac Catheterization unit at the hospital. Dr. Helen Taussig, who had already been eyeing him suspiciously, hit the roof. Dr. Bing explained their constant disagreements:

She was very jealous and guarded her territory; she considered the sick children as her own, having no family herself. She was also deaf, which increased her suspicion of the world beyond.

(That link contains not only a good description of Taussig-Bing Anomaly, but an in-depth look at Johns Hopkins Hospital during the Blalock-Bing-Taussig years. I highly recommend reading it!)

Dr. Bing eventually left Johns Hopkins, continuing to do major research in the Cath Lab. While at Wayne State University he did some of the early work involving PET scans, using computers belonging to Ford Motor Company to run the scanner.

Dr. Bing retired and now lives comfortably in California, and last year celebrated his 100th birthday!

Experience

January 14, 2010

All the families – including mine – were gathered in a small conference room just down the hall from Surgical Waiting. Surgeon Albert Pacifico flipped through a handful of index cards; consulting them to refresh him memory. Pacifico was fast and he had a system; he often completed six heart operations or more in a single day.

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

“That’s not the way we handle that,” the surgeon’s assistant said, but there was no reassurance. Something was up, they could tell.

Finally it was their turn. There was scar tissue in my chest, a lot of scarring. More than had been anticipated. They had unintentionally torn it making the incision and suddenly blood was everywhere. Pacifico had used every trick he knew – and even made up a few new ones on the spot – and I had needed twenty units of blood, but things were stable at the moment. If we chose to go ahead, he would continue the operation.

It was what he didn’t say that hung in the room. If we go ahead, we could easily tear something else and it could happen again. And we may not be able to stop the bleeding this time.

Just because Daddy told him to call it off didn’t mean it would be any easier. Everything in my chest was being held together with baling wire and bubble gum; he had to make the repairs more permanent and leave the operative field in some type of order for the next surgeon, if anyone ever tried again. Although it would be a good 24 hours before I turned the corner and started getting better, I made it through.

The selection of a surgeon is the most important thing when going into heart surgery. Every surgeon is good, he or she wouldn’t be holding the knife if they weren’t. But when you are talking about operating on an infant with a heart the size of a plum and blood vessels measured in millimeters, “good” just doesn’t exist. This is working at a level that is well beyond the capacities of the average person.

So how do you pick the right surgeon? There’s a good rule of thumb that I often quote: The doctor you need does not practice in a town of 5,000 people.

That’s right – if you live in a smaller town (my hometown has less than 500 people!) you aren’t going to find the doctor that you need. You can make a quick determination just by observing the doctor on the first examination. If they are thrilled to see your child because “They are just so unusual!”… grab your kid’s arm and RUN. You’re in the wrong place. What you need is a doctor who has seen enough heart defects that they are almost boring. And those doctors work in prominent medical centers that see a lot of patients. Ask how many operations they do every year on children like yours, and the higher the number, the better. Post-operative care is just as important as the surgery itself, maybe more so.

(There’s always an exception: If you are at a hospital connected with a medical school, your physician may bring several of his students around to examine your child and learn about their bad heart. Medical students are a good thing; they may remember your child and use what they learned to save a life later in their career!)

Not only do you want a large hospital with lots of surgical experience and a surgeon who is experienced, you want the right type of surgeon. The doctor who did granny’s triple bypass is not who you are looking for. Rarely does a bypass specialist go digging around in the heart, you want a Congenital Heart Surgeon. Adults who need surgery for a Congenital Heart Defect may be surprised (and worried!) to learn that the best person to do their operation is a pediatric surgeon, but most Congenital Cardiac Surgery is performed on children.

You can’t control everything, and you may have to make some snap decisions, but following these simple rules (Major Medical Center, lots of operative experience, surgeon with lots of Congenital Heart Surgery experience) can tilt the odds in your favor.

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.

BATTLE WOUNDS (NSFW)

October 24, 2009

October 25, 2009: Saint Crispin’s Day

“Then will he strip his sleeve and show his scars,

And say`These wounds I had on Crispin’s Day.’ “

Henry V, William Shakespeare

scars

The List

September 14, 2009

Here’s a needed resource for parents of Cardiac Kids: a 36 page booklet listing Pediatric Cardiologists, Congenital Heart Surgeons, and hospitals that provide Open Heart Surgery for children. The listings cover both the United States and Canada and most of the information was updated in October of 2008. (Some was updated later than that)

It’s a downloadable .pdf file published by Congenital Cardiology Today.

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!

What good is a deaf cardiologist?

November 30, 2008

Not much.

I’ve written about Vivien Thomas and Alfred Blaock; so it is time to write a few lines about the third person involved in the Blalock-Taussig Shunt: Pediatric Cardiologist Helen Brooke Taussig.

For those of you who may not know, Taussig’s my personal hero. When I was a patient at Johns Hopkins in 1967, Taussig herself examined me. From what my parents tell me, she was officially retired, but still lived in the Baltimore area and “stopped by the office” occasionally. I was five months old at the time, so I don’t remember it.

Her father was Frank Taussig, a prominent economist who taught at Harvard. Imagine his frustration when his daughter tried her best in school but still seemed to just not get it. I think every parent has shared that frustration at one time or another. It wasn’t her fault – it turns out that Helen Taussig had Dyslexia. Finally after a lot of patient work with her father everything just “clicked” and she was able to graduate. She went to the University of California at Berkley and then applied to Harvard Medical School.

The problem was, Harvard didn’t accept women into its medical program, so Helen tried Johns Hopkins Medical School instead. After she graduated, she took the job as head of the Cardiac Clinic at Hopkins’ children’s hospital, the Harriet Lane Home for Invalid Children.

Ironically, after becoming a doctor, Helen Taussig began to lose her hearing. And what good is a doctor – especially a cardiologist – who loses their hearing?

Thankfully, it wasn’t a sudden event. Helen Taussig lost her hearing slowly, and was able to compensate. Obviously a hearing aid would help, and she took full advantage of them. The movie Something the Lord Made is inaccurate in one respect: Mary Stuart Masterson (the actress who played the role of Helen Taussig) chose to use a more advanced hearing aid than was available at the time. The earpiece available in the early 1940’s was the size of an earmuff, and Masterson thought that such a large, bulky prop would distract from the character. (Look at this 1940’s era photo of Dr. Taussig examining a child in a wheelchair. The black box balanced on the edge of the chair is not Taussig’s medical bag, it is the amplifier for her hearing aid.)

As her hearing faded, she lost the ability to hear some of the chest sounds that a Cardiologist needs to hear to make a diagnosis. So she taught herself how to feel heartbeats by placing her fingertips lightly on the patient’s chest, and for years her hands were her “second stethoscope.” (Click here for a close-up photo of Taussig’s fingertip examination.)

Known mainly for her work in Pediatric Cardiology, few remember that Helen Taussig also played a critical role in averting the Thalidomide crisis in the United States. While Frances Kelsey receives credit (deservedly so) for not authorizing the drug for sale here, Taussig got involved when one of her German students commented that there had been a marked increase in the occurrence of Phocomelia in her home country. Taussig began to study the problem, and before long had determined that the morning sickness drug Thalidomide was causing birth defects. Because of Kelsey’s and Taussig’s actions, only 17 American children were affected by the drug.

So, what good is a deaf cardiologist? It all depends on who that deaf cardiologist is!