Archive for the ‘Pulmonary Artery’ Category

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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I Could have Danced all Night

October 27, 2010

TEDMED is a yearly medical conference in San Diego, so attendees must have wondered what was going on when Charity Tillemann-Dick (Who performs under the name Charity Sunshine) walked out and sang for the audience.  TEDMED has an unusual format – every speaker, from the most well-known to the guy you never heard of, gets 18 minutes to make their presentation. This woman could really sing, but her clock was running. Whatever point she was trying to make, she had better get to it.

And that’s when Charity dropped the bomb: “One year ago today, I awoke from a thirty-day coma after receiving a double lung transplant.”

Charity had been diagnosed with idiopathic pulmonary hypertension, a disease that causes the blood vessels in the lung to lose their elasticity and the right side of the heart to enlarge. Doctors told her she would have to give up singing. Charity wasn’t pleased with that option, so she changed doctors. The drug she chose to slow the hypertension down has to be infused 24 hours a day and requires a four and a half pound pump. Charity learned how to hide it under her opera costumes and just kept on going. As expected, her health deteriorated to the point that she needed a transplant.

There was a good chance that a lung transplant would kill her voice, so she asked the doctors to do a special procedure to try to save her vocal cords. The transplant was a hard thing, throwing her into a coma for a full month. But one year ago, Charity woke up, and began to piece her life and her singing career back together.

By now her 18 minutes were drawing to a close. “We need to stop letting disease divorce us from our dreams. We will find that patients don’t just survive, we thrive,” Charity said, and with that she ended her presentation with a very fitting song.

She sang I Could have Danced all Night.

My Glenn Shunt is worth more on eBay!

October 13, 2008

Yeah, you read that right. My Glenn Shunt would bring a higher price on eBay! Yours? Not so much. I have a classic vintage model, so the price would be higher!

I’m kidding with you, obviously. If you happen to need a Glenn Shunt (or any other heart operation) then the true cost is out of your reach; it’s priceless.

The Glenn Shunt is one of the oldest heart operations around. It was first described in 1951, and Dr. William Glenn of Yale University first reported performing the procedure successfully in 1958. Since he was the first person to routinely have success, the operation bears his name. (If you or someone you know has a Glenn shunt, please click THIS LINK and download and read the PDF file. There is a lot of important information here that you need to know!)

When I tell people I have a Glenn Shunt, the ones who know what I’m talking about will nod their heads knowingly. Most of the time, though, they are still wrong. My Glenn was done in 1967, and I am a proud owner of a Classic Glenn Shunt. Most of the Glenns done today are the Bidirectional Glenn Shunt.

So what’s the difference? Before you describe the Glenn, it helps to have a diagram to help you visualize it. Click HERE for a useful diagram of the heart.

In the Classic Glenn, the Superior Vena Cava (The large vessel that leads into the Right Atrium) is closed near the Right Atrium (usually, it is not cut, but rather sewn closed.) The Pulmonary Artery (the “T” shaped blood vessel that runs under the “loop” formed by the Aorta) is also cut… the right branch of the Pulmonary Artery is disconnected. The hole left by cutting the right branch of the Artery is sewn closed, and then the right branch is connected to the side of the Superior Vena Cava.  By doing this, the Right Atrium is completely removed from the blood flow. Blood coming to the heart through the Superior Vena Cava now goes directly to the Right Lung, and flows back to the Left Atrium normally. Then it goes through the Left Ventricle and back out to the body.

The Bidirectional Glenn was invented, surprisingly, in 1966. While it was around when I had my Classic Glenn in 1967, my operation was the fifth Glenn Shunt (of any kind) that had been performed at Johns Hopkins; so it is a safe assumption that the surgeons weren’t prepared to try the new version just yet. In fact, the Bidirectional Glenn really came into its own in the 1980’s, when it became the second step in the three operation Norwood Procedure used to combat Hypoplastic Left Heart Syndrome (HLHS).  It’s also part of the Fontan Procedure, sometimes performed as a seperate operation as part of a Staged Fontan.  The biggest difference in the two operations is that in the Classic Glenn, the Superior Vena Cava is completely cut and sewn into the right branch of the Pulmonary Artery. In the Bidirectional Glenn the Pulmonary Artery is not cut, which allows blood flow to both lungs.

It’s important for someone with a Congenital Heart Defect (CHD) to know what “version” of an operation they have had. For years, I told doctors “I have a Glenn Shunt,” not knowing that the operation had been changed. After I had told a new doctor that I had a Glenn Shunt, he slapped my X-Ray on the lightboard, took a long pause, and finally said “I don’t know what the hell this is, but it ain’t no Glenn Shunt.” Only after the head of the Cardiology Department came in and said “I haven’t seen one of those in a while!” did I realize that simply saying “Glenn Shunt” wasn’t good enough. Thankfully that snafu occured during a routine office visit and not a crisis visit to an Emergency Department.