Tetralogy of Fallot

50 years ago, on April 30, 1969, I underwent successful open-heart surgery at Boston Children’s Hospital to repair a heart birth defect known as Tetralogy of Fallot. For my 50th anniversary, this article provides insight into the history of medical advancements with congenital heart defects, my experiences during the early days of hospitalization, and looking back over the past half-century.

Early Years: 1800s – 1955

Given the wide variety and effectiveness of cardiovascular surgical techniques that are now routinely used, it is remarkable how, during the late 19th and early 20th centuries, the surgical treatment of heart disease was considered to be outside the limits of propriety and acceptability. The consensus in the medical field at the time was not to even think about touching the heart for repairs. The heart was beyond limits for attempting any type of surgery for reparations.

In 1891, Henry C. Dalton in St. Louis was the first surgeon to repair a pericardial wound in a human being. A similar success was achieved by Daniel Hale Williams in Chicago, in 1893. In 1906, Ludwig Rehn of Frankfurt Germany compiled a summary of 124 cases of cardiac-wound repair that had been performed in Europe during the 1890s and thereafter. The survival rate of 40% was remarkable for that period.1

Dr. Etienne-Louis Fallot (1850–1911) from the University of Marseille in France, showed that affected babies had an abnormal heart, meaning that not all of their blood was directed to the lungs for oxygenation. He described the condition in the his paper “l’anatomie pathologique
de la maladie bleue” in 1888. The condition includes four distinct developmental errors, and is known as tetralogy of Fallot. 2

Dr. Fallot

1930’s – Boston Children’s Hospital – Dr. Robert Gross

Dr. Robert Gross founded the cardiology department at Children’s Hospital in Boston MA in the 1930s, and performed the first surgical correction of a congenital malformation that causes abnormal blood flow between two of the major arteries connected to the heart. 7-year old Lorraine Sweeney suffered from “…a congenital heart condition known as patent ductus arteriosus, a passageway between the pulmonary artery and the aorta that’s supposed to close after birth — but doesn’t.” 3

“By today’s standards, Dr. Gross performed a relatively simple type of surgery,” said Dr. Alexander Nadas, former Children’s cardiologist-in-chief, in an interview in 1988 marking the 50th anniversary of Lorraine’s operation. “He didn’t have to go inside the chambers of the heart to do the job; he just tied off a little tube – simple. Yet, this was the clear-cut beginning of modern heart surgery.” 3

Patient Lorraine Sweeney with Dr. Robert Gross

1962 – Pittsburgh Children’s Hospital – Dr. Robert Pontious

Raised in Monongahela PA south of Pittsburgh, I entered the world born a “Blue Baby”, where my skin color was not of normal pink, but of an abnormal blueish tone. Tetralogy of Fallot (ToF) is the reason for the “Blue Baby”, a congenital heart defect, with an array of these conditions:

  • Pulmonary stenosis, narrowing of the exit from the right ventricle
  • A ventricular septal defect, a hole between the two ventricles
  • Right ventricular hypertrophy, thickening of the right ventricular muscle
  • An overriding aorta, which allows blood from both ventricles to enter the aorta
Just prior to the Blalock-Taussig-Thomas shunt surgery January 1963
Post-surgery, showing off my new scar. Notice the color of my lips to the previous picture. With normal blood flow to the heart, my circulation dramatically improved.

At six-months of age, I underwent the first of two heart surgeries to repair ToF. At Children’s Hospital in Pittsburgh PA, Dr. Robert Pontius, a Navy veteran of WWII and Korean War, performed the Blalock-Taussig-Thomas shunt in January 1963. At Harvard Medical School, Dr. Pontius trained under his uncle, Dr. Robert Gross of Children’s Hospital in Boston.

“That operation brought Pittsburgh into the era of open-heart surgery, and it took a lot of nerve to do it, a special kind of person. These babies were clearly really sick, and we knew what we should do for them, but the mortality rate was very high.” said Dr. Frederic Sherman, pediatric cardiologist at Pittsburgh Children’s Hospital.4

Dr. Robert Pontious

This surgical repair is known as the Blalock – Taussig – Thomas Shunt. At Johns Hopkins in Baltimore, Dr. Alfred Blalock (1899-1964) teamed with Dr. Helen Taussig (1898 – 1986), and Dr. Vivien Thomas (1910-1985) in the 1940s to develop a procedure still used today.

“Dr. Taussig came to Dr. Gross, with her idea for the surgical procedure, but he didn’t find it worth pursuing.  So it was up to Dr. Blalock at Johns Hopkins and his assistant Vivien Thomas to give her idea a try.” – Dr. Joan Pontius, daughter of Dr. Robert Pontius.

Blalcok-Taussig-Thomas Shunt

At Johns Hopkins Hospital in Baltimore, MD., Dr. Alfred Blalock (1899-1964) teamed with Dr. Helen Taussig (1898 – 1986), and Dr. Vivien Thomas (1910-1985) in the 1940s to develop a procedure still used today. In 2004, HBO produced the movie Something The Lord Made, based upon these events. Starring Mary Stuart Masterson as Dr. Helen Taussig, Mos Def as lab technician Vivien Thomas, and Alan Rickman as Dr. Alfred Blalock, the movie depicts the research methods used for the groundbreaking surgical operation, along the backdrop of deep racial divides of the Jim Crow era during the 1930s and 1940s.

In the 1940s, however, ToF patients did not have a good rate of long-term survival. Over the next decade, further research determined infant’s blood vessels needed to grow in order to fulfill the proper blood circulation in the body. The medical community determined that a more invasive open-heart, second surgery would be required for Blalock-Taussig-Thomas shunt procedure patients when they grew older, and their arteries were more stable.

HBO Movie Something the Lord Made
Trailer for HBO’s Something the Lord Made 2003

1969 – Boston Children’s Hospital – Dr. Alexander Nadas

In 1969 at Boston Children’s Hospital, my cardiologist was Dr. Alexander Nadas. Born in Hungary, he emigrated to the United States in 1938, just prior to Hitler’s murderous rampage and extermination of over 400,000 Hungarian Jews during WWII. Dr. Nadas was interviewed in 1993 by David Dyer and remarked:

“I credit Franklin Roosevelt and the New Deal with raising the level of economics in this country. Rheumatic fever is really a social disease, which is a terminology usually reserved for venereal disease. Nobody had time for the unfortunate babies who were born with deformed hearts. They were lost in this big pool of rheumatic fever, nobody paid any attention to them.

There was nothing you could do for them until Dr. Robert E.Gross started operating on patients in 1938. So, with the disappearance of rheumatic fever and rheumatic heart disease, came the emergence of cardiac surgery for children. Not infants — infants came much later. By the time I came to Children’s in 1949 to start the cardiology department we were dealing almost exclusively with congenital disease.” 5

Dr Alexander S. Nadas

In Pittsburgh, doctors told my parents in 1963, when I entered my teenage years, I would need a second and more dramatic, open-heart surgery to close the opening between the two bottom chambers of the heart. It turns out, a second surgery is a requirement for infants with Tetralogy of Fallot.

US Steel, where my Dad worked in the accounting/data processing division, was transferred from the Donora PA plant, as it was closing due to major shifts in steel production from the United States to China. The US Steel office in Millbury MA would be his new place of employment. Incidentally, this plant ceased operations in 1978 – after continued steel manufacturing being transferred overseas forced the closure of many plants in the U.S.

After moving to Massachusetts from Pennsylvania in 1967, I visited to Boston Children’s Hospital to be acquainted with cardiologist Dr. Alexander Nadas. In my first meeting with Dr. Nadas, I had trouble understanding his words. Dr. Nadas had a thick Hungarian accent, it took me awhile to adjust, but I was able to get used to his mannerisms and care for me as his patient.

Fun Fact:

Dr. Nadas’ daughter, Betsy Nadas-Seamans, played the role of Mrs. McFeely in the children’s PBS program Mr. Rogers Neighborhood, and also was a writer for many of the shows.

Source: mentalfloss.com/article/76158/24-rare-photos-mister-rogers-neighborhood(opens in a new tab)

April 1969 – Boston Children’s Hospital

The late 1960s were massively stressful times in our country. At the end of the decade, the Vietnam War raged, and assassinations of civil-rights leader Dr. Martin Luther King and presidential candidate Robert F. Kennedy in 1968, marked a distinct contrast with the triumph of NASA astronauts Neil Armstrong and Edwin “Buzz” Aldrin to have walked on the moon the following year in July 1969.

My parents and I made another trek to Boston Children’s in January 1969, a checkup with Dr. Nadas, as I had started to have displaying difficulties walking across the room or climbing stairs would leave me short of breath. We also noticed how my fingertips, toenails, and lips, would turn a blueish hue during exertion. More visits to Dr. Nadas were accompanied with more tests. After a visit in early April 1969, a letter came to the house from Children’s Hospital. It was a notice that I was to be scheduled for open-heart surgery on April 30th.

Me and my parents leaving home for Boston Children’s Hospital April 1969

Most things I remember of this experience are not fuzzy. However, I do remember specific things to this day. Once example was when I was told I couldn’t have milk, something about having too much sugar in milk was not good for my condition. For breakfast, I was given a bowl of Frosted Flakes (um, what about that sugar doc?), with not milk, but plain old water. That was an interesting experience!

I was placed in an oxygen tent, and this device covered my hospital bed, creating a pure 100% oxygen environment to aid in oxygenating my blood. Mom was always successful at diverting attention away from the obvious. “This oxygen text was special” my Mom said. Now I could go camping now with my very special tent here in the hospital. Another time, she brought me my regular clothes, to be able to spend some time not wearing the johnny all the time and feel a little sense of normalcy.

Advertisement for Oxygen Tent device. (No, that is not me poking around)
Me in the patient’s break room. The nurses at Boston Children’s were outstanding.
I liked the hospital’s fish tank!
The Archie’s “Sugar Sugar” is playing again?!?!?!
I’m ready to go home and be with my friends now.

I remember my hospital bed was across the way adjacent to the nurse’s break room, and I could see and hear the commotion through the confines of the plastic of the oxygen tent placed as a canopy surrounding my hospital bed. There was a small black-and-white TV that all of the nurse’s were huddled around. The nurses were giddy, “It’s Tom Jones!”, and I remember their excitement over the entertainer. I could faintly see the images of a couple of guys singing. Turns out, it was Tom Jones crooning “It’s Not Unusual” from his ABC TV show. I didn’t know the other singer at that time, but it was Stevie Wonder. This was the performance, airing on the ABC broadcast of the Tom Jones Show May 22, 1969:

Stevie Wonder with Tom Jones, The Tom Jones Show May 22, 1969 ABC

Being in the hospital for a lengthy period in isolation, away from home, family and friends, the radio became my close companion. It was always on during my waking hours. The recreation room for kids had a phonograph record. The hit 45 released that year was ‘Sugar, Sugar’ by The Archies, and this was played endlessly by staff. Fortunately, I had my bedside radio tuned to the other top 40 hits of 1969: Tommy James and The Shondell’s Crimson and Clover, Sly and the Family Stone’s Hot Fun in the Summertime, Henry Mancini’s Love Theme from Rome and Juliet, and the Edwin Hawkins Singers Oh Happy Day were often cycled through the AM station on boring days.

One song that I couldn’t give enough of, included a positive, optimistic message, I learned later is two separate songs combined as one. The Fifth Dimension’s Aquarius, has the passage “When You Open Up Your Heart – You Got To Let The Sun Coming In”. The message sent to me fifty years ago resonates to this day.

Aquarius – The 5th Dimension 1969’s Top Ten Hit

I don’t remember meeting the cardiac surgeon, Dr. William Bernhard, who performed my open-heart surgery in Boston. I do remember getting his name mixed up with Dr. Christian Barnard, a cardiac surgeon from South Africa, who pioneered the first human heart transplant operation in 1967. Dr. Bernhard, a WWII veteran from Framingham MA, passed away at age 93 in 2018. He left behind a lasting legacy of innovative cardiac surgery techniques, including the use of the hyperbaric chamber, revolutionary for its time. 6

The first temporary shunt operation (Blalock-Taussig-Thomas) was performed in Pittsburgh in January 1963.

The second complete repair open-heart surgery completed in Boston in April 1969.

Source:https://www.rch.org.au/cardiology/heart_defects/fallots_tetralogy/

Technology Advancements – Heart Catheterization

The fate of babies born with congenital heart disease (CHD) has dramatically changed in the last 4–5 decades, going from a universally
fatal condition in the vast majority of patients in the absence of diagnosis or intervention, to an entity whose outcome, at least in terms
of peri-operative/hospital stay, has improved to an expected survival of about 96%. 7

Technology – Then and Now

The first catheterization performed was in the year 1928. Dr. Warner Forssman of Germany performed the procedure, on himself. He tricked his nurse Gerta Ditzen to assist him, and she brought Forssmann to the X-Ray lab and obtained an X-ray picture of the catheter entering the heart chamber. He put himself under local anesthesia and inserted a catheter into a vein in his arm. Not knowing if the catheter might pierce a vein, he put his life at great risk. Forssmann was nevertheless successful; he safely passed the catheter into his heart.  Dr. Forssman joined the Nazi party, and was later captured by Americans and placed in a prisoner of war camp. After the war ended in 1945, he settled back to his home country of Germany. In 1956, he received the Nobel Prize in Medicine for this achievement. 8

Dr. Werner Forssman
X-Ray Image of catheter from left arm entering the heart chamber.

My catheterization in 1978 was preventative; a standard procedure to measure oxygen levels in each chamber of the heart to ensure the open-heart surgery repair from 1969 was successful. For the procedure, one is not fully put under local anesthesia. A lighter-dose anesthesia is used, in order to be kept awake. I had to hold my breath at certain times, as well as to be able to converse with the doctor. This allowed the cardiologist to measure oxygen levels and pressures inside the heart chambers. X-Ray cameras are used to visualize the heart, and a dye is injected towards the end of the procedure, to show the blood vessels surrounding the heart. This is to see if there are any blockages or other abnormalities.

When the dye is injected, it is a bit surreal experience. The doctor says “We’re about to inject the dye, you will feel heat coming through.”  This was similar to opening an oven door in the kitchen – a series of waves of heat hit my body. With each heart pump, heat pulsated through my body. First pump, the heat first went to my head. The second beat, the heat hit my torso, and by the third beat, my legs were hot. I recall breaking out in a sweat, thankfully the waves of heat quickly dissipated.

Heart catheterizations continue to be used as an effective to to examine the heart. In addition to examining congenital heart defects, the procedure is also effective to replace heart valves, placing stents in blocked arteries, or performing biopsies. Instead of a more intensive open-heart procedure, cardiologist cause catheterization as a less invasive method to perform this critical tasks.

Heart Catheterization Procedure – courtesy Mayo Clinic

2019 – 50 Years On

In the fifty years since the open-heart surgery, I continue to see cardiologists on an annual basis as part of a preventive maintenance program for Tetralogy of Fallot individuals. It is critical to work to keep the heart healthy, and I am fortunate to be able to utilize the fitness professionals and resources in my hometown fitness center, at No Alibi Fitness (www.noalibifitness.com). The facility has a cadre of fitness sessions, my favorite is the spin-cycle class. My heart thanks me every day for getting out there, and keeping the blood pumping!

No Alibi Fitness

A favorite hobby of mine is playing piano, here is a link to some of my projects: http://writingreal.com/musicworks I enjoy working on various pieces, here is one I put together a few years ago:

Prologue: Beauty and the Beast A. Menken arr. B. Podesta

My current team of cardiology professionals include my longtime cardiologist Dr. Joseph Kirkpatrick, and  more recent addition to the team, Dr. Michelle Hadley, who specializes in patients with congenital heart defects (CHD). A good strategy moving forward is to have a strong, positive team of medical professionals and I am fortunate to be blessed with these outstanding physicians. Let me introduce you to these heroes!

“I enjoy sitting down with patients and explaining their situation. I feel everyone should be able to understand exactly what’s wrong with them and what their treatment options are.”
Dr. Joseph Kirkpatrick (Hero)
Dr. Michelle Hadley (Hero)

Advancements in medicine and technology continue to astound us. Over the past 50 years, huge strides have been made to help people. The challenge continues to find cures for cancer, Alzheimer’s, and to further improve our livelihoods. I remain hopeful the best of our angels will prevail to solve these issues.

I remember something said to me by Dr. Nadas at Boston Children’s Hospital. I had a cardiac catheterization procedure in 1978, a planned event for preventative maintenance to view my progress over the previous nine years. I was curious about my longevity, as there didn’t seem to be long-term statistics for ToF patients. Dr. Nadas said “You are doing remarkably well. Each day is a day of building your history.”

50 Years Later – 2019

References:

1 Weiss, Allen B. MD Cardiac Surgery, A Century of Progress 2011. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3231540/

2 Zampieri F, Thiene G. Encyclopedia of Pathology 2017. https://link.springer.com/referenceworkentry/10.1007/978-3-319-28845-1_4080-1

3 Fliesler N Boston Children’s Hospital 2012. https://vector.childrenshospital.org/2012/02/defying-orders-to-make-heart-surgery-history/

4 Carpenter, M. Pittsburgh Post-Gazette 2012. https://www.post-gazette.com/news/obituaries/2014/04/20/Robert-Pontius-pioneering-heart-surgeon-at-Children-s-Hospital/stories/201404200178

5 Dyer, David childrens.harvard.edu Nadas – Interview 2 by David Dyer 1993, Harvard Medical School Archives

6 Schwan, Henry Metrowest Daily News 2018. https://www.metrowestdailynews.com/news/20181101/framingham-innovative-surgeon-dr-william-bernhard-of-boston-childrens-hospital-dead-at-93

7 Weiss, Allen B. MD Cardiac Surgery, A Century of Progress 2011. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5035758/

8 Radeska, T., Vintage News 2016. https://www.thevintagenews.com/2016/09/12/werner-forssmann-nobel-winner-performed-first-human-cardiac-catheterization/

Special thanks and acknowledgement to the family of Dr. Robert Pontius, who provided insight into their father and the Blalock-Taussig-Thomas procedure.

By Brian J. Podesta

Administrator and writer for WritingReal, a place for stories about people and places. Included are my musical projects and my book reviews.

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