Helen Brooke Taussig (1898–1986) is recognized as the mother of Pediatric Cardiology. She became my mentor and my friend. She overcame many obstacles in her path to success. The first was dyslexia. Her father, Professor of Economics at Harvard, patiently taught her to read so that she could enter school. Each summer he took the family to their home on Cape Cod. There he spent the mornings on his work and the rest of the day with his children, enjoying the pleasures of the beach, the water, and the woodlands where the beach plums grew. Years later, Dr. Taussig built her home on family property on the beach, and she adopted the same habits of work and play. There over a period of 10 years she wrote her book, Congenital Malformations of the Heart (1). This was the right book at the right time. Published in 1947, just two years after the success of the “blue baby” operation, the book was written in a clear and readable style, with each malformation documented as to clinical as well as postmortem details. It immediately became the bible for doctors around the world who were becoming excited by the promise of not only medical but also surgical treatment for congenital heart disease.
Long before that achievement she encountered other obstacles which she overcame by virtue of her intellect and persistence. A continuing roadblock to her desire to enter medicine was that at the time it was a man's game. After study at Radcliffe and then at the University of California at Berkeley, she graduated with a Phi Beta Kappa key and with an AB degree in 1921. She applied to study Medicine at Harvard. Despite her father's prominence on the faculty, Harvard was adamant against women in medical school and even against awarding her a degree should she study in the School of Public Health. She was, however, allowed to study Histology while seated in a remote corner of the lecture hall, “so that she would not contaminate the students”, she said. Then she studied Anatomy at Boston University in a room by herself. For dissection she was given a beef heart to learn about muscle bundles, her introduction to Cardiology.
She applied to enter the Johns Hopkins School of Medicine, which since its inception had admitted women on the same basis as men. Thus began her long and distinguished career there. Even at Hopkins her progress was not without problems. Despite her being an excellent student, elected to Alpha Omega Alpha, she did not upon graduation obtain the internship in Internal Medicine that she sought. Throughout medical school she had spent time each year in the Heart Station; so that is where she worked for the year following graduation.
She entered Pediatrics as an intern and came under the influence of the chairman, Edwards A. Park, who became her mentor. He recognized that although hospitalized children received good acute care, those with chronic illness often did not when they went home. In 1930 he established specialty clinics in the outpatient department and he put Dr. Taussig in charge of the Cardiac Clinic. He encouraged her to concentrate not only on rheumatic fever, the big problem, but also to learn about congenital heart disease.
She visited Dr. Maude Abbott, whose Atlas of 1,000 specimens of congenital cardiac abnormalities was published in 1936 (2). This important treatise clarified some of the symptomatology and classified malformations according to the presence or absence of cyanosis. Dr. Taussig learned to identify by barium swallow a right aortic arch, information that became practical later when the subclavian-to-pulmonary artery anastomosis was developed (3), for it utilized the subclavian artery on the side opposite that to which the aorta arched.
Dr. Taussig recorded her findings in each patient. She realized that certain malformations repeated with characteristic and recognizable patterns. She correlated her observations with the anatomy at postmortem examination. Soon she was able to make clinically an accurate anatomic and physiologic diagnosis, using the tools of history taking, physical examination, electrocardiogram, fluoroscopy and X-ray. She paid particular attention to pulmonary vascular markings, whether increased or decreased.
Just as she was gaining recognition for her clinical acumen came the next handicap. She began to lose her hearing. She obtained an amplifying stethoscope and also a hearing aid, which hung from a cord around her neck. Years later, when miniaturized hearing aids were placed in the temple pieces of glasses she rejoiced in “having her ears back up where they belonged”. Much later, just before her retirement, she successfully underwent surgery for otosclerosis and happily could use a regular Littman stethoscope. Dr. Taussig believed she had a moral responsibility to share knowledge. She gave lectures, conducted symposia, published papers and her book that covered not only medical aspects but also the new modalities of cardiac catheterization and angiocardiography as well as cardiac surgery.
It was in 1939 that surgical treatment for a congenital anomaly was first accomplished. Dr. Robert Gross at Boston Children's Hospital (4) successfully ligated a patent ductus arteriosus in a child. His pediatric colleague, Dr. Hubbard, had made the diagnosis by recognizing the continuous murmur in the pulmonary area, typical of that malformation. This accomplishment gave Dr. Taussig an idea. She had noted that children with tetralogy of Fallot who had a continuous systolic and diastolic murmur over the lungfields were less cyanotic and impaired than those without. She reasoned that this murmur was due to the continuous flow of blood from vessels that left the aorta and anastomosed with the pulmonary artery or its branches, thereby increasing pulmonary blood flow. If a surgeon could tie off a patent ductus, could he also create one and achieve this beneficial effect?
She went to Boston to ask Dr. Gross this question. He firmly told her that he was in the business of closing a patent ductus, not in creating one. She had to wait until 1942 when Dr. Alfred Blalock returned to Hopkins as Chairman of Surgery, bringing with him Vivien Thomas, his fine assistant and technician at Vanderbilt. Dr. Taussig and Dr. Blalock met with Dr. Park, the Chairman of Pediatrics, who encouraged their collaboration. With Vivien Thomas in the laboratory Dr. Blalock perfected a technique already familiar to him from his research in trying to produce pulmonary hypertension by anastomosing an artery off the aorta to the pulmonary artery. When he was ready to do the operation on a child, he insisted that the first patient be severely handicapped so as to justify the risk of being the first subject of a new surgical procedure.
Eileen Saxon qualified. She was an undersized, deeply cyanotic infant with many hypercyanotic attacks daily due to her severe form of tetralogy of Fallot. On November 24, 1944 history was made when the first subclavian-to-pulmonary artery anastomosis was performed. In the operating room that day Vivien Thomas stood behind Dr. Blalock, and Dr. Taussig positioned herself at the head of the table by Dr. Merrel Harmel, the anesthesiologist. When the anastomosis was completed and the clamps released, Dr. Taussig exclaimed with delight that Eileen had a “lovely pink color”. I was a substitute intern on Pediatric Surgery at the time working alongside of Denton Cooley. I shall never forget the excitement and awe that accompanied this accomplishment.
The following spring at a meeting of the Johns Hopkins Medical and Surgical Association, the auditorium was packed with faculty and students. Dr. Blalock described the operation and Dr. Taussig brought in the first five postoperative children with their smiling faces and normal pink color. She told how before the operation they could not walk across a room without stopping to squat down to rest and catch their breath. When the first cases were reported in 1945 (3), cyanotic patients from all over the world were referred to the team at Hopkins. The influx of patients needing attention called for assistants and thus to the appointment by Dr. Taussig of fellows and the establishment of the first training program. She asked me to be one of her first fellows.
Drs. Blalock and Taussig divided the responsibilities, she for the diagnosis and recommendation for surgery, they for the decision at a joint conference to advise the operation on each patient, he for the operation, they for postoperative care in the hospital, and she for the longterm followup. They willingly gave their time for talks at medical and surgical meetings and they accommodated the many doctors who came to observe the coordination of pediatric cardiac and surgical care. Dr. Taussig analyzed not only the many successes of the operation but also the failures. My assignment as Dr. Taussig's fellow was to review the cyanotic patients who did not benefit from the surgery. This led to publication of the clinical syndromes of Ebstein's anomaly of the tricuspid valve (5) and of valvular pulmonic stenosis with patent foramen ovale (6).
Throughout her distinguished career Helen Taussig modestly accepted many honors and awards. A major honor was becoming the first woman president of the American Heart Association. Another was receiving from President Lyndon Johnson the Medal of Freedom, the nation's highest civilian award. This was for her work on thalidomide. That research took place after Dr. Alois Beuren, a former fellow, returned from Germany in 1962 for a visit. He told her about a birth defect of epidemic proportions there: phocomelia. Babies were born with flippers rather than normal arms or legs. A link was suspected to a drug taken by many pregnant women because of morning sickness. Dr. Taussig announced that she would go to Germany to investigate. Her thorough inquiries established the connection between thalidomide taken even as a single dose before the 42nd day of pregnancy, when limb buds were forming, and phocomelia. Her report halted the epidemic and prevented its occurrence elsewhere.
Rheumatic fever with its sequel, rheumatic heart disease, had for years been the major cardiologic problem around the world. Treatment with salicylates, bed rest, and long periods of recovery at home or in convalescent homes gradually became replaced as knowledge grew that related the illness to antecedent Group A beta hemolytic Streptococcus infection. Although sulfonamides, available since the late 1930s, were effective in healing a Strep throat, resistance to these bacteriostatic rather than bacteriocidal drugs developed. Penicillin became available in time to be the answer to prevention of rheumatic fever, either as an initial illness or as a recurrence.
Congenital heart disease was of little interest until some kind of treatment became available. In medical school, our lecturer on this topic began by saying that the subject was not very important because it was a rare condition, that no one could make the diagnosis with accuracy during life, and even if that should happen, nothing could be done about it. Within two years that concept was disproven by Dr. Taussig and Dr. Blalock. Furthermore, the condition is not so rare; it occurs once in 100 live births. In a study of the longterm followup for the first five years of life in a cohort of 19,502 births, Hoffman and Christensen confirmed the incidence to be 10.4 per 1,000 live births (7).
Key to progress in congenital heart disease was the collaboration of cardiologists and cardiac surgeons as well as the development of increasingly sophisticated diagnostic techniques such as cardiac catheterization, angiocardiography, miniaturization of oxygen analyses of blood samples so that even the smallest and sickest infant could undergo a diagnostic study that could be lifesaving, and the development of color echocardiography as well as magnetic resonance imaging.
The concept of therapy at cardiac catheterization was introduced by William Rashkind at Philadelphia Children's Hospital in 1966 (8). In infants with complete transposition of the great arteries it had been established in 1950 by surgeons Blalock and Hanlon at Hopkins that survival could be enhanced by improving mixing of arterial and venous blood at atrial level (9). Based on this principle Dr. William Mustard in Toronto devised a two-stage correction in 1964 for this common cyanotic anomaly (10). In newborns, Dr. Rashkind accomplished this mixing by using a balloon-tipped catheter, passed through a patent foramen ovale, inflated, and forcefully withdrawn into the right atrium, tearing the fossa ovalis tissue and creating an atrial septal defect. Now many abnormalities are treated via the cardiac catheter.
After my fellowship with Dr. Taussig, I went to the New York Hospital-Cornell University Medical Center in New York City. In my free time when I arrived at Cornell, I reviewed the charts and the postmortem specimens of all patients with congenital heart disease. Since my experience at Hopkins was in patients who had survived long enough to be referred from afar, I was surprised to find that most of the deaths from this birth defect occurred in early infancy in the first weeks or months of life. Cardiac failure was the chief cause of death, and a large ventricular septal defect was the principal cause of the heart failure (11). Until then a ventricular septal defect had been considered benign. Recognition of the signs and symptoms of cardiac failure in infancy led to improvements in treatment and the saving of many lives (12).
Surgical treatment of people of all ages with congenital heart disease was advanced when intracardiac surgery under direct vision and with the patient on cardiopulmonary bypass was introduced in 1955 by C. Walton Lillehei of Minnesota (13). Patch closure of a large ventricular septal defect became a reality even for an infant.
The incision that permits open repair of cardiac anomalies deserves mention. Typically it is a midline sternotomy extending from just below the suprasternal notch to the xiphoid process. Often a keloid forms in the scar. While this may be acceptable in boys, it is not appreciated by little girls as they mature. In New York I suggested, and my surgical partners, Drs. Paul Ebert and Bill Gay, agreed, that they would use instead a bilateral inframammary incision which would not be visible later.
While benefit from surgery was often immediate and dramatic, it took time to determine longterm effects and to analyze residua and sequelae (14). I cite as an example a followup over a period of 10 to more than 20 years of 85 patients operated upon using the Mustard procedure at New York Hospital by Paul Ebert and William Gay, my fine cardiac surgical partners. Of these patients, 63 had simple and 22 complex transposition of the great arteries. The youngest was 2 days of age at surgery. Event-free survival was 77% for those with simple but only 46% for those with complex transposition (15).
Tachyrhythmias or heart block occasionally followed surgery. Until the anatomy of the cardiac conduction system was appreciated, complete heart block sometimes followed patch closure of a large ventricular septal defect. Development of cardiac pacemakers provided relief if heart block should occur. Relevant to the prognosis for patients with acquired complete heart block is a study reported in 1972 by Dr. Magnus Michelsson from Sweden, representing the Association of European Paediatric Cardiologists, and me. We conducted an international cooperative study on the natural history of congenital complete heart block (16). As might be expected, the death rate was highest in the period of early infancy and for those with another associated anomaly.
The adult with congenital heart disease deserves special attention (17). Those with functionally insignificant lesions, such as mild pulmonic stenosis or small ventricular septal defect, can expect a normal life span. Their risk is that of bacterial endocarditis, and that risk has been minimized by the American Heart Association's widely publicized recommendations for prophylactic antibiotics in therapeutic doses at the time of dental cleaning or oral surgery.
A great many patients reach adulthood by virtue of medical and/or surgical treatment. What professional specialty should take care of them? Most physicians caring for adults have had little experience or training concerning congenital heart disease. Their lack of confidence is often perceived by the patient, who “votes with his feet” and returns to his old friend, the pediatric cardiologist. I believe that training programs in Internal Medicine and in Cardiology should incorporate some “hands on” time with these patients in cooperation with pediatric cardiologists at the medical center.
A question of significance for the adult with congenital heart disease is insurability. As a member of a committee of the American Heart Association I participated in a series of conferences with medical representatives of major insurance companies (18). We gained a better understanding of their position, and we hoped that they learned about the differences between mild, moderate or severe forms of congenital heart disease and the impact of cardiac surgery on the expectation of a long and healthy life.
Another question important to adults with congenital heart disease is the chance of having an offspring similarly affected. From her experience at Yale Dr. Ruth Whittemore reported on a second generation study of 427 male and female probands with congenital heart disease. Of their 837 children who were followed through childhood, 118 (14.1%) had congenital heart disease. The rate was about the same for mothers and for fathers (19). Genetic counseling for these people is important. So too is combined obstetric and cardiologic care for the mother. If the potential mother has pulmonary vascular obstructive disease, it is unwise for her to either undertake or to continue a pregnancy.
Risk factors for having a baby with congenital heart disease were addressed by Dr. Charlotte Ferencz and colleagues in the Baltimore-Washington Infant Study: 1981–1989 (20). This large, collaborative, population-based etiologic study involved six pediatric cardiac centers and provided 4390 cases and 3572 controls for the investigation. One fourth of the cases had other congenital anomalies. In addition to a positive family history of congenital heart disease, they found the following risk factors relating to the mother: age over 30 years, diabetes, influenza and fever, history of previous abnormal reproductive outcomes, and use of therapeutic or non-therapeutic pharmaceutical. In addition, certain lifestyle factors played a role, especially smoking cigarettes, use of recreational drugs, and hair dyes. They noted an association between cardiac defects as a group and maternal ingestion of more than eight mg/Kg of coffee, tea, or cola daily.
Is congenital heart disease preventable? Not enough is known about causes to accomplish that goal. However, maternal ultrasound studies that include analysis of the fetal heart may detect abnormalities which, if very severe, might warrant discussion by family and physician of the possibility of terminating that pregnancy.
While rheumatic fever and congenital heart disease are the most prevalent conditions in Pediatric Cardiology, others also require accurate diagnosis and appropriate treatment. Among these are cardiomyopathy and mitral valve prolapse. A third condition, Kawasaki disease, is rare except in early childhood, but potentially significant in adult life since it can affect the coronary arteries. In 1967 Dr. Kawasaki, a Japanese pediatrician, reported a new febrile illness in young children aged 5 years or less (21): the mucocutaneous lymph node syndrome. The remarkable cardiological feature is aneurysmal dilatation and sometimes occlusion of the origin of the coronary arteries. Echocardiography identifies this event. Our epidemiologic studies (22) confirmed a suggestive association with the handling of rugs, such as taking them up or beating them. We studied and reported an effective treatment: the use of single, high dose gamma globulin in the first week of this disease. Signs and symptoms of illness terminated and coronary arterial complications did not occur (23). During international conferences on this disease, Dr. Kawasaki and I became friends. He lectured at our hospital and stayed with us at our home. His greatest delight there was walking barefoot on the grass.
Many children of Mediterranean origin in New York City were affected by this hemolytic form of anemia. Most required transfusions about every two weeks. Since the body could not eliminate iron derived from their own hemolyzed cells or from the transfusions, the children acquired hemosiderosis and later in adolescence, hemachromatosis. Cardiac manifestations included gradually progressive cardiac enlargement, followed by cardiac failure and death in early adulthood (24). Beginning in the 1980s chelation therapy improved their outlook (25).
Pediatricians have little experience with diseases found often in adults, such as coronary artery disease and hypertension, but can hope to help prevent them. A family history of either condition alerts the practitioner to advise for that child a prudent diet, exercise and regular medical followup. Unfortunately, obesity is now an epidemic worldwide. Drs. Berenson and Srinivasan conducted the Bogalusa Heart Study (26). They identified an elevated cholesterol as a risk factor of early atherosclerosis.
During the period of recognition of conditions needing pediatric cardiologic expertise this subspecialty was organized. Upon completion of training programs the fellows fanned out to medical centers in this country and abroad to continue to learn, to teach, and to care for patients. As an early Taussig trainee I was appointed to several committees. In the American Academy of Pediatrics, in 1957 we established the first subspecialty section, that of Pediatric Cardiology with 47 members. Four years later came board examination for certification and the establishment of training programs with high standards.
International development of the specialty began in 1964 when the Association of European Paediatric Cardiologists was established, with 57 members from Europe and 17 of us from the United States. The first World Congress was held in London in 1980 with Dr. Jane Somerville in charge of the planning committee. I headed the committee for the second World Congress in 1985 in New York City (Figure 1). Both meetings were successful, drawing cardiologists and surgeons from all over the world, for the science, socializing, and sightseeing. Interest in pediatric cardiology became truly worldwide.
 | Fig. 1 The Second World Congress of Pediatric Cardiology in New York City in June, 1985. From left to right—Dr. Jane Somerville, Chairperson, Organizing Committee of the First World Congress of Pediatric Cardiology in London, 1980; Dr. Mary Allen Engle, (more ...) |
Travel by invitation to foreign places became one of the benefits of being a pediatric cardiologist. An early and memorable trip was to China in 1978. Chairman Mao had recently died. As an anti-intellectual he had removed medical leaders from hospitals and sent them to work in fields and factories. The Chinese Medical Association reunited to help doctors and medical facilities catch up for the wasted years during which modern medicine had continued to progress elsewhere. Recognizing that heart disease was the leading cause of death, they invited a team of cardiologists to visit the major cities, lecture, and make rounds. I was fortunate to be included along with Dr. Tom James, another member of ACCA. Chinese hosts were most gracious as they listened to us, dined with us, and showed us some of the beauties of their ancient land. I was a lucky lady not only to be a pediatric cardiologist helping children and teaching physicians but also to be a world traveler. Over the years, my husband and I were invited as lecturers to over 83 foreign countries including Sudan, the Philippines, Malaysia, Nepal, and Tibet.
On the international level, I was able to collaborate with a former fellow of Dr. Taussig, Dr. John Nihoyanopoulos of Athens, Greece. When Ralph and I visited him there, we recognized that there was no coordinated program for those infants with critically severe congenital heart disease who, without treatment, would likely die early. On return to New York, I secured the cooperation of several important groups: doctors at Cornell Medical center, the Greek Consulate, and the Greek Orthodox Church. The plan was that we at Cornell would carry out the needed diagnostic studies and treatment until facilities were developed in Greece. His imminence the Archbishop Iakovos introduced me to the philanthropic ladies of the church. Many acted as interpreters and also helped me to raise funds needed for the families' expenses. I gave so many talks at their luncheons held by the Archdiocese at the Plaza Hotel that one of the waiters sent in a contribution with a note saying he had been impressed by the worthiness of the program. Onassis, who owned Olympic Airlines, provided air transport for the families free of charge. Stavros Niarchos contributed funds to Cornell University to endow the Professorship of Pediatric Cardiology, named for him. I was the first holder of that chair. More than three hundred critically ill Greek children benefited from this program. Years later, in Athens, Mrs. Nihoyanopoulos invited many healthy young people who had benefited from this program to come to see me. They were so appreciative. The happy ending to this Greek experience is that Christina Onassis contributed a hospital in Athens for the comprehensive diagnosis and treatment for children with heart disease.
The result of the international sharing of knowledge and exchange of creative ideas was the development and continuing improvement of comprehensive cardiac/surgical programs in medical centers worldwide, all for the benefit of children with heart disease.