Patient stories

Pediatric Cardiology: The Wonder Years

by Sheila Hogan

On October 27, 1969, at the age of 11, I was initiated into a select club at Yale-New Haven Hospital. It was a club with a lifetime membership, or perhaps more succinctly, a club whose membership provided me with a life. On that date I underwent open heart surgery to correct a congenital heart defect which had, for the first decade of my life, made me a skinny kid who tended to tire at play.

For me, the surgery was an inconvenience which would cause me to miss out on Halloween the following week. To my parents, my surgeon and the many caregivers who guided me through my ordeal, I was a little part of history in the making. For if I--and the dozens of other kids who were among the first to undergo successful open heart surgery--were children in the late 60's, pediatric cardiology was barely striding adolescence.

A little over a decade earlier, Yale-New Haven surgeon Dr. William Glenn had performed the medical center's first pediatric open heart surgery on a 10-year-old with a hole between the auricles of her heart. Pediatric cardiology was just coming into its own as a medical specialty.

Now, decades after Yale-New Haven surgeon Dr. Horace Stansel closed a hole between the ventricles of my heart (a ventricular septal defect) in a four-hour operation, I was given the opportunity to seek out some of the individuals and revisit the technology which made the procedure possible. And while I felt in 1969 that I was the only kid on the planet who had a hole in her heart, I have come to discover that I had plenty of company. In seeking out some of my early pediatric pioneers, I would learn that most of us received what medical science strives to attain--a cure, or at least a much-improved quality of life. Perhaps even richer than our experience was the technological explosion which would follow.

The formative years
For those of us who came to Yale-New Haven in the formative years of pediatric cardiology, there was a sense of wonder, if not awe. From the moment it opened its doors over four decades ago as the region's first pediatric cardiology referral center, Yale-New Haven became a mecca for children with congenital heart disease. It was not uncommon for us to arrive at the medical center as babies and then be followed through childhood, adolescence and into early adulthood. The practitioners we saw became a kind of extended family who tracked our progress with a patience and precision that we, as children, would take for granted and our parents would consider an indispensable comfort.

Lisa Ferretti Foley underwent open heart surgery at Yale-New Haven in 1959 at age seven to repair a hole between the upper chambers of her heart, a congenital defect known as an interatrial septal defect, or ASD. Yale-New Haven, "was a tremendously exciting environment, even to a kid," Foley recalled. "There were doctors of every race and color. To be exposed to so many women doctors at that time was truly unique," added the former patient, now a teacher who now lives in upstate New York with her husband and children.

As an outpatient at Yale-New Haven in pediatric cardiology's early days--from the late 1940s through the 50s and 60s--you were most likely followed by a soft-spoken physician whose name has become synonymous with the development of pediatric cardiology at Yale. Dr. Ruth Whittemore, clinical professor of pediatrics at the Yale School of Medicine, would see several thousand children in her long tenure at the medical center. In the beginning, she recalled, happy endings were at a premium.

Dr. Whittemore's 400 children
When she first arrived at Yale-New Haven in 1947 to run a state-funded program called the Rheumatic Fever and Cardiac Clinic, the first such center in New England, Dr. Whittemore remembers feeling slightly overwhelmed. "There were 400 children, many of whom had come from great distances, waiting to be seen; roughly 10 percent of them could be helped."

"We had some children coming in squatting, or lying in the knee-chest position in the back of the family car," Dr. Whittemore continued. "They were in terrible shape, any exertion made it difficult for them." Often, Dr. Whittemore noted, helping these children meant simply making them as comfortable as possible.

The young physician had entered into a scenario where even properly diagnosing her patients was a challenge. Unable to find blood pressure cuffs small enough to fit an infant, Dr. Whittemore managed to convince the manufacturer to produce cuffs a third the normal size. And because ingenuity was a necessity during that early era, she found that an adult ureteral catheter was small enough to accommodate her patient's tiny heart vessels in delicate cardiac catheterization procedures. "People wouldn't believe what we had to make do with back then," she added.

Though presented with formidable obstacles, Dr. Whittemore brought with her to Yale the training and foresight to believe that perhaps even the impossible might one day be probable. The young physician had been trained at Johns Hopkins under Drs. Helen Tausig and Alfred Blalock, the team most noted for developing the "blue baby" operation in which blood was shunted to the lungs to help increase the flow of oxygenated blood. The closed heart procedure brought a degree of palliative relief--including much improved color--to dozens of cyanotic, or blue, children. "We had a sense of what could be done," Dr. Whittemore now says, "but we weren't sure whether the technology to actually get inside the heart would ever exist."

Opening the heart with an Erector Set
It would not be long, however, before Dr. Whittemore began to think differently. One day in 1948, while waiting for an elevator at Yale-New Haven, she was approached by a third-year medical student named William Sewell. The boyish, sandy-haired Sewell had an question. Did she think a machine could be built that would divert blood flow away from the heart and keep it oxygenated long enough to allow the heart to be worked on and then safely reactivated? Taken aback and a little bemused, Dr. Whittemore looked at the wide-eyed student, dismissed him with a patronizing "Well now, that would take some doing," and then disappeared behind the doors of the closing elevator.

Before Dr. Whittemore could begin to understand the immense import of her brief hallway encounter, Sewell had acted on his impulse. Working in the surgical laboratory at Yale, Sewell constructed a mechanical pump capable of rerouting blood flow to and from the heart using $24 worth of dime store parts, including a $6 motor and other parts from a child's toy Erector Set built by the A.C. Gilbert Company of New Haven. On June 24, 1949, Sewell and Dr. William L. Glenn, Yale's young chief of cardiovascular surgery, made medical history when they used their pump to successfully divert blood flow from the heart of a dog for a total of 63 minutes. Today the pump, recognized as the forerunner of the artificial heart and is on permanent display at the Smithsonian Institution in Washington, D.C.

Dr. Glenn, now professor emeritus of cardiothoracic surgery at the Yale School of Medicine, remembers the early experiments with the pump as a necessary part of the evolution of pediatric cardiac surgery. "It was extremely important that these early procedures be carried out first in animal laboratories," Dr. Glenn said. "There's no question that cardiac surgery, more than any other branch of surgery, owes its rapid progress and great success to laboratory experiments."

Three years after Drs. Sewell's and Glenn's historic operation, the first human open heart procedure would be performed in Detroit using a heart by-pass pump based upon on the Yale doctors' concept. And just four years after that, Dr. Glenn would once again make history by performing Connecticut's first open heart surgery at Yale-New Haven Hospital in 1956. Her wildest dreams now a reality, Dr. Whittemore was present in the operating room. "I'll never forget it," said Dr. Whittemore. "It was as though we'd come full circle."

The Glenn operation
Drs. Whittemore and Glenn became a team at Yale-New Haven, she taking the referrals and following the young patients to the point of surgery, and he correcting the congenital defects in the operating room. In 1958, Dr. Glenn would perfect a surgical technique which allowed unoxygenated blood from the vena cava to flow directly to the lungs without the pumping action of the right side of the heart. The Glenn operation, or the Glenn shunt, as it would come to be known worldwide, solidified Dr. Glenn's position as "one of the early pioneers in the world of heart surgery, in particular, pediatric heart surgery," commented Dr. Charles Kleinman, chief of pediatric cardiology at the Yale School of Medicine and YNHH.

Dr. Glenn has high praise for the spirit of cooperation between the medical school and the hospital that "so greatly facilitated the evolution of cardiac surgery at Yale-New Haven. There was an enormously conducive atmosphere between the two institutions at a time when research in this area was expanding in all directions. Together they took a leap of faith at a time when the benefits of heart surgery were yet unproven." For example, he said, the hospital agreed to provide a room for the facility's first intensive care unit to accommodate Dr. Glenn's first open heart youngsters. Located on the fourth floor of the Farnam Unit, it began with just one bed in 1958 and quickly grew to four in just a few years.

Dr. Browne's favorites
In 1958, Dr. Whittemore was joined by a new colleague, a woman who would have a tremendous impact on my life and the lives of innumerable other pediatric cardiology patients and their families. I caught up with Dr. Marie Browne, retired professor of pediatric cardiology at the Yale School of Medicine, one morning in the hospital's Primary Care Center. Though she officially retired in 1988, Dr. Browne continues to volunteer her time one morning a week to teach medical students how to listen for and diagnose heart murmurs. "I've been listening to murmurs for 35 years," she said with a smile. "If you know murmurs, said this pediatric cardiology trailblazer, you know heart disease in children."

The image of a dark, slender woman bent over a young patient, stethoscope perched on her ears listening intently, glasses slid half way down her nose, is etched forever in the minds of my parents and countless others who brought their children to Yale-New Haven over the decades. "I used to say to parents, 'You're not children. I will tell you exactly what's going on.' My job was to educate and support," Dr. Browne recalled, adding that she had two sets of students--the house staff and parents.

Dr. Browne's no-nonsense approach endeared her to families. As her patients grew, she found herself writing their letters of college recommendation, attending their weddings and sharing their joy as new parents. "One day on rounds I got a call from the father of one tetralogy patient to say she had just delivered a perfectly beautiful baby boy," she recalled proudly. One letter from a former patient, a college student at the time, noted that she was afraid of Dr. Browne as a child. "Now I realize there was no reason to be afraid, that you were just a little old lady." That letter is one of Dr. Browne's favorites.

Kathy Baker is typical of Dr. Browne's extended family of patients. Born with two holes between the chambers of her heart, Baker first became a patient of Dr. Browne's at eight months. After they were patched by Dr. Stansel in 1969, when Baker was five, Dr. Browne continue to follow her throughout her childhood and teen-age years. At age 21, it was Dr. Browne who told Baker she could safely have children. "It was as though she was there for all the important moments in my life," Baker, now the mother of a 17-month-old son, recalled.

In those early years it was Dr. Browne, along with Dr. Whittemore and a growing number of physicians who would choose to follow in the footsteps of these gentle women doctors, who became our medical interceptors in a world that was simultaneously terrifying and miraculous. It was often they who would announce that the time for surgery had finally arrived. "We were always weighing the benefits of doing surgery. If the child was doing well, you delayed operating as long as possible." Well into the 1970s, Dr. Browne noted, it was not unusual to delay surgery on children until school age or even later.

Pediatric surgery today
Today, in sharp contrast, open heart surgery is performed at Yale-New Haven on infants just hours old with even the most serious defects usually repaired by age two. Much of that progress is owed to the visionary efforts of Dr. Norman Talner, newly retired chairman of pediatric cardiology at Yale-New Haven, who was the driving force at Yale behind the development of diagnostic and therapeutic procedures for infants with congenital heart disease. Under Dr. Talner's direction, Yale became the first center to do blood gases on infants, a test to measure the amount of oxygen in the blood and a key tool in determining heart function. "The Yale catheterization lab and the pediatric cardiology group became known for the diagnosis of heart disease in infants," he added.

Within the last decade, fetal echocardiography, a non-invasive procedure that uses sound waves to produce pictures of the fetal heart, has come into its own at the medical center, allowing doctors to diagnose as many as 95 percent of major congenital heart defects in-utero, according to pediatric cardiology chief Dr. Kleinman.

Dr. Kleinman played a key role in opening the country's first fetal cardiovascular center at Yale-New Haven in 1984. "While parents are never pleased to receive information about the presence of heart disease in their fetus, it allows them to make advance decisions about where to deliver and to prepare themselves for the surgery or treatment their baby may require at birth," Dr. Kleinman said.

Both of Long Island resident Judy Greene's children were diagnosed with congenital birth defects at Yale-New Haven while still in the womb. Green and her husband, Edward Vigneau, knew that because Judy was diabetic, that there was an increased risk of a cardiac problems in their offspring. Greene was completing her master's degree in epidemiology at the Yale School of Medicine's Department of Epidemiology and Public Health, when she became pregnant with the couple's first child, Ted. Under the care of Dr. Kleinman, Greene underwent a fetal echocardiography which showed the fetus had a ventricular septal defect (VSD). The hole was closed soon after birth and today Ted is an active young man.

The couple's second child, Kate, was born with multiple and extensive heart defects. When she came into the world without a pulmonary valve and the key vessels of her heart transposed in a condition known as situs inversus, Yale-New Haven surgeons were ready to respond just hours after birth.

"We thought we had been through a lot with Ted and going through it again with Kate was an enormous strain," recalled Green. "But knowing beforehand helped us prepare. Everyone was on alert that a very sick baby was coming into the world."

Extensive corrective surgery by Dr. Gary Kopf, professor of cardiothoracic surgery at the Yale School of Medicine and director of pediatric cardiac surgery, during the first few weeks of Kate Vigneau's life have put the now lively three-year-old on the track toward a normal life. Although her pulmonary valve and cardiac pacemaker will need to be replaced as she grows, "she's completely active," noted her mother. "It's difficult to think back on those tense times but what's important is that my husband and I were always informed; everything was always spelled out for us--the risks as well as the pluses," Greene added.

Dramatic technological advances in pediatric cardiology are vastly improving the longevity and quality of life for patients like Kate, Ted and the estimated 150 children who have cardiac surgery annually at Yale-New Haven. Now, due to the pioneering efforts of Dr. William Hellenbrand, professor of pediatric cardiology at the Yale School of Medicine and director of Yale-New Haven Hospital's Pediatric Cardiac Catheterization Laboratory, a growing number of corrective procedures are no longer performed in the operating room. Once seen strictly as a tool for diagnosing cardiac malformations, catheterization at Yale-New Haven is now exploring a new frontier: the repair of congenital heart defects without open heart surgery.

Currently, Yale-New Haven is one of only four centers in North America being allowed by the FDA to close atrial septal defects (ASDs) in the catheterization lab. Another common defect, patent ductus arteriosus, an opening between the pulmonary artery and the aorta which normally closes after birth but remains open in some newborns, is also being corrected in the cath lab at Yale-New Haven with excellent results. Both procedures are accomplished via a technique in which a tiny umbrella-like device is guided into the heart by way of a small catheter and then released and opened, blocking the hole. Although many complex defects will continue to require surgery, the future looks promising for interventional cardiac catheterization.

At the present time, Dr. Kleinman pointed out, nearly half of all pediatric cardiac patients at Yale-New Haven benefit from some form of therapeutic catheterization. "All one needs to do is see a patient come in for this type of procedure and go home either later the same day or the next day with a bandaid on their groin compared to an open heart surgery scar, and the difference becomes remarkable," said Kleinman. "This really is a means of being kind to patients and keeping medical costs down at the same time," he added.

Yale-New Haven is pioneering a number of other procedures. The medical center is on the leading edge in the development of the use of cardiac homografts, in particular the transplantation of human pulmonary and aortic valves, in children with congenital defects.

Heart transplantation in babies will come into its own over the next several years, as more organs for this age group become available, according to Dr. Kopf. The answer to a shortage of human hearts may one day be the perfection of xenograft transplantation, or the use of animal hearts in humans, Dr. Kopf added. "The heart of the baboon and the pig are remarkably similar to the human heart. They hold great promise for the future of cardiac transplantation," he said.

If we were the pediatric pioneers of the this century, then certainly these new pediatric patients are blazing trails in their own right. And while it will be years before a long term prognosis is available for the hundreds of children who are now undergoing the newest and boldest of today's cardiac procedures, some answers are now beginning to emerge for those of us treated early. According to Dr. Norman Talner, former chief of pediatric cardiology and newly retired professor of pediatric cardiology at the Yale School of Medicine, data to be released later this year by Yale-New Haven and a consortium of several other large centers across the country, is expected to show that the quality of life, as well as life expectancy, of the pediatric cardiology pioneers has well surpassed medical expectations.

"We now have a 20- to 30-year natural history of procedures like atrial septal defects. Someone who had such a repair back then and who has had no residual problem is a normal person. These kids go to college; their intellectual performance is normal; they have families," Talner said. "The major problem," he added, is insurability. "It's affecting kids now entering their 20s, 30s and 40s. The insurance industry continues to label these preexisting conditions, even though the defects are considered completely repaired." The issue is one that Dr. Talner, the American Heart Association and the American Academy of Pediatrics, are continuing to confront at the national level.

Yet overall, we early pioneers have much to be thankful for. In three short decades, Dr. Talner noted, medical science has come as close to a cure for congenital heart disease as we might dare to expect. "Today, upwards of 90 to 95 percent of kids can be helped and more and more repair is being done sooner than ever before. It's been a lucky time," Dr. Talner added.

The history of pediatric cardiology at Yale-New Haven

1947 Dr. Ruth Whittemore sets up the state's first pediatric cardiology referral center at Yale-New Haven Hospital.

1949 Yale medical student William Sewell builds the forerunner of the artificial heart using $24 worth of dime store parts and a child's toy erector set.

1956 Dr. Glenn performs Connecticut's first open heart surgery.

1958 Dr. Glenn develops a surgical technique which greatly improves the survival rate of youngsters with congenital cardiac malformations. The "Glenn Shunt" allowed the lungs to be supplied with oxygen-rich blood, by-passing the weakened right chamber.

1959-64 Dr. Glenn and associates pioneer the world's first radio frequency-powered cardiac and diaphragm pacemakers for use in children.

1970s Yale's Dr. Michael Berman develops a special "balloon tip" catheter which allowed physicians to more safely explore the inner chambers of the heart. The Berman catheter remains the standard for angiographic studies in heart disease throughout the world.

1970s Yale is among the first medical centers worldwide to use sound waves to achieve a clear image of the heart. Non-invasive echocardiography would dramatically transform the diagnosis of heart disease in both children and adults.

1980s Yale-New Haven develops the transplantation human heart valves in children with congenital defects.

1984 Yale-New Haven opens the nation's first fetal cardiovascular center, making possible the diagnosis of 95 percent of major heart defects in utero.

1990s Yale's Dr. William Hellenbrand pioneers the use of therapeutic cardiac catheterization to repair some congenital heart defects without open heart surgery.

Next story: Harriet Gelderman's story

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Last revised: February 19, 2004 (vv)

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