Taussig-Bing Anomaly

Abstract

Taussig-Bing anomaly is a rare congenital heart malformation that was first described in 1949 by Helen B. Taussig (1898–1986) and Richard J. Bing (1909–). Although substantial improvement has since been achieved in surgical results of the repair of the anomaly, management of the Taussig-Bing anomaly remains challenging. A history of the original description of the anomaly, the life stories of the individuals who first described it, and the current outcomes of its surgical management are reviewed herein.

How is one to judge one's past? Many would rate it by honors received, societies elected to, or fortune amassed. I judge my life by the degree of happiness I have received from my work ….

— Dr. Richard J. Bing¹

A heart malformation known as Taussig-Bing anomaly (also called Taussig-Bing syndrome, heart, or malformation) consists of transposition of the aorta to the right ventricle and malposition of the pulmonary artery with subpulmonary ventricular septal defect (VSD) (Fig. 1). Although this cardiac malformation was first described 60 years ago,¹ its management still presents a challenge to cardiologists and cardiac surgeons. A history of the original description of the anomaly, life stories of the individuals behind the description of the anomaly, and the current outcomes of its surgical management are reviewed herein.

Fig. 1. Original drawing of the heart shows the size and position of the aorta and pulmonary artery and their relation to the septal defect. Reprinted from Taussig HB, Bing RJ. Complete transposition of the aorta and a levoposition of the pulmonary artery. Am Heart J 1949;37(4):551–9, with permission from Elsevier.

The anomaly bears the names of 2 outstanding physicians, Dr. Helen Taussig and Dr. Richard Bing, who worked together at The Johns Hopkins Hospital in Baltimore.

Helen B. Taussig (1898–1986)

Helen Brooke Taussig (Fig. 2) was born on 4 May 1898 in Cambridge, Massachusetts. She was the youngest of 4 children of Frank and Edith Taussig. Her father was a professor of economics at Harvard University and financial advisor to President Woodrow Wilson (1856–1924). Edith died of tuberculosis when Helen was 11 years old. As a child, Helen was afflicted with tuberculosis, dyslexia, and progressive deafness,² but she excelled in school because of her diligent work and extensive tutoring from her father.

Fig. 2. Helen Brooke Taussig (1898–1986). Courtesy of Andrew N. Redington, Hospital for Sick Children, Toronto, Canada.

After earning her first degree in 1921 from the University of California, Berkeley, Helen tried to enter Harvard, but the university was not willing to admit women. Instead, in the words of Dr. Mary Allen Engle (1922–2008), “she was offered the opportunity to audit courses at Harvard, and she said that she would have been seated up in the back so that she wouldn't ‘contaminate’ the male medical students. When she received an honorary degree from Harvard many years later, in her acceptance speech she reminded them of this. She was a survivor, and she prevailed. She continued to learn, and grow and accomplish, and teach.”³ Taussig instead took a course in anatomy at Boston University and went to study medicine at The Johns Hopkins Medical School in Baltimore—one of the first American medical schools to accept women—in 1923. She qualified as a medical doctor in 1927 and became an intern in pediatrics at Johns Hopkins under the guidance of Dr. Edwards A. Park (1877–1969). In 1930, Park appointed Taussig as a director of the Children's Cardiac Clinic at the Harriet Lane Home.^4–6 In 1940, Dr. Alfred Blalock (1899–1964) was appointed chief of surgery at Johns Hopkins. Inspired by Taussig, Blalock developed the first systemic-to-pulmonary shunt in collaboration with Vivien Thomas (1910–1985), and performed the procedure in an 11-month-old girl on 29 November 1944—an operation known today as Blalock-Taussig shunt. After 2 similarly successful operations, Blalock and Taussig published their findings in 1945.⁷

Although writing did not come easily to Taussig because of her dyslexia, she insisted that the publication of findings was an obligation of academic life, and her writings spanned from 1926 to 1988.² Taussig's book, Congenital Malformations of the Heart,⁸ took almost 10 years to prepare. It was published in 1947, and the second edition followed in 1960.

In 1959, Taussig was appointed Professor of Pediatrics at Johns Hopkins. In 1962, she published the long-term results of the Blalock-Taussig operation.⁹ She spent her entire career at Johns Hopkins until her retirement in 1963. In 1964, Taussig received the Medal of Freedom from President Lyndon B. Johnson. In 1965, she became the first woman and first pediatrician to be elected president of the American Heart Association. Looking back, Taussig commented, “Over the years I have gotten recognition for what I did, but I didn't at the time. It hurt for a while. It hurt when Dr. Blalock was elected to the National Academy of Sciences and I didn't even get promoted from assistant to associate professor.”² In the words of P.M. Dunn, “Taussig was a handsome, compassionate, and forceful woman who inspired respect and admiration among her colleagues and trainees. She had a love of new knowledge and a desire to share it with others. She also had a sense of wonder and a joy in life. Doctors came from many parts of the world to learn from her; she accorded the doctors, their wives and families a warm welcome and gave them loyalty and friendship. ‘Family’ reunions for her ex-fellows were held on her lawn every 2 to 3 years.”²

Helen Taussig died on 21 May 1986 in a car accident in Kennett Square, Pennsylvania, just days before her 88th birthday, while driving to cast her vote in an election.

Richard J. Bing (1909–)

Richard John Bing (Fig. 3) was born on 12 October 1909 in Nürnberg, Bavaria, Germany. His father was an amateur botanist and art historian and was responsible for furnishing one of the local breweries in Munich with hops. Richard's mother was a professional singer.¹⁰ While attending a medical school in Munich, Bing was inspired by his physiology teacher, Dr. Otto Frank (1865–1944). Using isolated frog heart, Frank formulated a law of myocardial contraction that was subsequently confirmed in warm-blooded animals by Ernest Starling (1866–1927); the law is known today as the Frank-Starling law of the heart. Frank retired in 1934, the same year in which Bing earned his medical degree and started on his path in medicine.

Fig. 3. Richard John Bing (1909–). Courtesy of Richard J. Bing.

After spending a year working in the department of surgery of a university hospital in Bern, Switzerland, Bing moved to the Carlsberg Biological Institute in Copenhagen, Denmark, where he studied cell cultures. In 1936, the institute was visited by Charles A. Lindbergh (1902–1974), the famous flyer and inventor, and Dr. Alexis Carrel (1873–1944), a surgeon, both from the Rockefeller Institute in New York. They had designed a machine for the perfusion of whole organs. The Carlsberg Institute wanted to use this machine and needed someone to install it. His ability to speak Danish, German, and English made Bing an ideal candidate to go to New York to learn the method and then return to Copenhagen to teach its use.¹¹

In 1936, Bing began his apprenticeship at the Rockefeller Institute. In his own words, “Youth needs heroes, and there were plenty of them at the Rockefeller Institute.”¹¹ He was indeed among elite company. Carrel had earned the Nobel Prize in 1912 for his pioneering work in organ transplantation and vascular surgery. Karl Landsteiner (1868–1943) had received the Nobel Prize in 1930 for the discovery of blood groups. Philip Levine (1900–1987) was a prominent immunohematologist. Payton Rous (1879–1970) was awarded the Nobel Prize in 1966 for discovering the role of viruses in the transmission of certain types of cancer. Working next to such prominent scientists fueled Bing's enthusiasm. In his letter to me on 7 October 2008, he wrote,

I was received by the people of the Institute in New York with great warmth; they found me a small apartment at Irving Place, near Gramercy Park. In the evening I could sit at my desk at the Rockefeller Institute and watch the tugboats on the East River. New York was exhilarating! Lindbergh visited us at the Institute many times to help us with our work. After about 11 months, I had to return to Denmark, a commitment I had made to the Rockefeller Foundation, and I needed a new job to return to America. Carrel gave me wonderful letters of recommendation. He remained my friend and sponsor, as did Lindbergh. Although I never discussed personal or political matters with either of them, I was not happy to return to Denmark, because the Nazis were threatening. So, after 2 months to fulfill my obligations, I returned to America. Then I heard that the Presbyterian Hospital of New York was choosing new residents in surgery. I never had an interest in surgery, but I had nothing to lose. So I took the examination to become a surgical resident, being grilled by 6 forgiving surgeons as well as the head of surgery, Dr. Allen O. Whipple. Miraculously, I passed, so that I had a job when I returned from Denmark. During the first year, I set up the Lindbergh-and-Carrel method, modifying the perfusion system to accept larger organs. After a year, I told Dr. Whipple that I would prefer to become a physiologist. He was understanding and helpful. I then joined the Department of Physiology at Columbia University.

In 1938, 2 important events happened in Bing's life—first, his publication in Science¹² that opened the way to a brilliant academic career; and second, his marriage to Allen O. Whipple's daughter, Mary. They were married for 53 years and had 4 children.¹⁰

In the late 1930s, André Cournand (1895–1988) and Dickinson Richards (1895–1973) developed the technique for cardiac catheterization; in 1940, they established perhaps the world's first cardiac catheterization laboratory (in Bellevue Hospital, New York) and received the Nobel Prize for this work in 1956. In 1942, Bing was appointed as an instructor in the Department of Medicine at Johns Hopkins under Dr. Warfield Longcope. In 1945, Alfred Blalock asked Bing to set up a cardiac catheterization laboratory at Johns Hopkins for the diagnostic study of congenital heart disease. Recalling his move to Baltimore, Bing wrote, “I had a premonition of what was to come when, on the drive from New York to Baltimore, we crossed the Chesapeake Bay on the ferry. I encountered at least 6 cyanotic subjects, not just children but also adults, on the short boat ride. Obviously, Blalock and Taussig's work was well enough known to attract patients to a pilgrimage to Baltimore.”¹³ Later, Bing admitted that, until that moment, he had little interest in congenital heart disease.¹³ He wrote, “I had until then never used an intracardiac catheter, but I had spent a few weeks in New York with André Cournand at Bellevue Hospital, although I never saw him perform a catheterization himself. My first catheterization at Hopkins in 1945 is described by Vivien Thomas in his book Pioneering Research in Surgical Shock and Cardiovascular Surgery.”¹³

Cardiac catheterization was in its early stages. Bing recalled an event that he observed in Cournand's laboratory, “There was one episode I watched that I remember with horror. A catheterized man was riding a bicycle, and they wanted to see how the right ventricular and pulmonary artery pressures went up with exercise. All of a sudden, the pressure tracings and everything disappeared. They began to fiddle around with the machine until someone had the good sense to look at the patient, who had slumped over and was unconscious!”³ With support from Blalock, Bing started a new catheterization laboratory at Johns Hopkins. In Bing's words, “Blalock was a superbly trained physiologist and surgeon who already had made outstanding contributions to the understanding of circulatory failure. He was extremely polite and pleasant, but a tough surgeon and, like many great people, had an iron will to succeed. His outstanding attribute was simplicity and directness of approach, which was also one of Taussig's characteristics. Taussig's greatness was not in the field of science or research, but in the clinical approach. She could remember a child's clinical findings for years and associate them with the personality of the child and the child's family. Being deaf, she relied primarily on visual and fluoroscopic examination. Thus, she formulated disease patterns by simple clinical observation. It became clear from the beginning that right-heart catheterization was to be my main investigative tool.”¹¹

Recalling his time at Johns Hopkins, Bing wrote the following to me on 7 October 2008: “Our group with Cooley, Bigelow, Mustard, Vandam, Spencer, Hanlon, and many others had a wonderful time working in a new and exciting field. But my main interest changed to the field of cardiac metabolism and coronary heart disease. My gratitude goes to Alfred Blalock and his associates. We had a great time!” After 8 years at Johns Hopkins, Bing served as Professor of Medicine at the University of Alabama (1951–1955), Wayne State University (1956–1969), and the University of Southern California (1969–). He currently lives in California. He loves music and has composed more than 250 musical pieces.¹¹

Description of Taussig-Bing Anomaly

In 1949, Taussig and Bing described the case of a 5.5-year-old girl who had cyanosis since birth, developmental delay, systolic murmur, and dyspnea at rest. Her hemoglobin concentration was 23.5 g/dL and her oxygen saturation was 57%. Her electrocardiogram showed sinus rhythm, high P waves in the second lead, and signs of right ventricular hypertrophy. Although her cyanosis, clubbing, and erythrocytosis were consistent with an Eisenmenger complex, the cyanosis since birth made Taussig and Bing suspect “some totally different malformation.”¹

Angiocardiography was performed. After a third intravenous injection of contrast, “the child sat bolt upright and the heart stopped. All efforts at resuscitation failed.”¹ The cardiac anatomy was defined at autopsy (Fig. 1). Further in the case report, Taussig and Bing wrote, “The relatively small volume of blood entering the aorta from the left ventricle furnishes the only means by which oxygenated blood reaches the body. This explains the low oxygen saturation in the peripheral arterial blood and the severe cyanosis. Consequently, any diminution of this volume may have dangerous consequences. This may explain the fatal outcome of angiocardiography. In this instance, the rapid injection of the Diodrast into the superior vena cava raised the pressure in the right side of the heart and, consequently, decreased the left-to-right shunt. For this reason, angiocardiography was exceptionally dangerous for this patient. Furthermore, angiocardiography did not clarify the nature of the malformation and therefore is not necessary to establish the diagnosis.”¹ Taussig and Bing concluded, “A new clinical syndrome is described. The malformation consists of a transposed aorta, a large pulmonary artery which arises primarily from the right ventricle and partially overrides the ventricular septum, a high ventricular septal defect, and right ventricular hypertrophy.”¹

Maurice Lev (1908–1994) was particularly interested in this anomaly and apparently was one of the first to call it “Taussig-Bing syndrome.”¹³ The important differences between Taussig-Bing anomaly and transposition of the great arteries were described by Richard Van Praagh in his editorial review¹⁴: “The distinctive features of the Taussig-Bing malformation are true double-outlet right ventricle, semilunar valves side-by-side and approximately at the same height, a bilateral conus with approximately equally well-developed muscular subaortic and subpulmonary conal free walls, and a large subpulmonary VSD.” He stressed that “If this eponym is used, it would seem appropriate to use it accurately, that is, for cases closely similar to the original one of Taussig and Bing, with a bilateral conus and hence without pulmonary–mitral continuity. The presence or absence of such continuity is not a trivial anatomic detail. Indeed, this is the essential difference between transposition with VSD and overriding pulmonary artery, in which pulmonary–mitral continuity is present, and the Taussig-Bing malformation, in which pulmonary–mitral continuity is absent.”

A Difficult Working Relationship

Great minds and strong personalities can clash during collaboration. An example of this was the strained professional relationship of the extraordinary Taussig and Bing. On 7 October 2008, Bing wrote to me, “Helen Taussig was a great physician because of her interest in the personality of the patient, and his or her family and background. She was no scientist, and complicated scientific matters disturbed her. She had an aversion to our determinations of flow and pressures and adaptation to anoxia, subjects which made the study of congenital heart disease a science. 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. One day, she informed Blalock that she needed no catheterization studies to make a correct diagnosis, so I stopped studying these children. After 3 weeks, she recanted! She was not easy to work with, and one of the reasons I left Johns Hopkins was Taussig's opposition. It is a wonder that she consented to a joint publication!”

Others also recalled this strained professional relationship between Taussig and Bing. For instance, I remember talking with Dr. Wilfred G. Bigelow (1913–2005) in 2000, who recalled that Taussig had a “very difficult and tense working relationship” with both Bing and Blalock. During 1946 and 1947, while working as a research fellow at Johns Hopkins under the tutelage of Bing and Blalock, Bigelow recalled “almost daily arguments” that they had with Taussig on various subjects. Similarly, Dr. Denton A. Cooley (1920–) wrote to me on 12 November 2008, “I recall that Dr. Helen Taussig seemed resistant to the new laboratory, and in general, was not very cooperative with Dr. Bing. He later described her as ‘a tough nut.’ It is interesting that the anomaly bears both the name of Taussig and Bing, since they were not close in their relationship. In fact, Dr. Blalock had certain problems with Dr. Taussig, who was sometimes intrusive and overbearing in the management of children after surgery.” It appears that Taussig's fierce dedication to her patients made for a difficult relationship with some of her colleagues.

Current Results of Surgical Management of Taussig-Bing Anomaly

The original description of the Taussig-Bing anomaly has been broadened to include all kinds of double-outlet right ventricles with subpulmonary VSD.^14,15 Anatomic repair of the Taussig-Bing anomaly is achieved with connection of the morphologically “left” ventricle to the aorta and of the morphologically “right” ventricle to the pulmonary artery.^16–18 In selected patients, such anatomic correction can be achieved by interventricular repair, also known as the Kawashima operation.^19,20 The arterial switch operation, however, appears to be preferred.^21–28 Excellent early and intermediate survival has been reported recently.^26–29 Historically, the arterial switch operation has been associated with higher rates of morbidity and death when it is performed in patients with the Taussig-Bing anomaly, compared with patients who undergo arterial switch for d-transposition of the great arteries.^30–32 In children with Taussig-Bing anomaly, subaortic right ventricular outflow tract obstruction is found in 50% to 60%,^21,29 and arch obstruction is common and present in 39% to 52%.^21,29 One of the earliest studies to show that a Taussig-Bing anomaly, with or without arch obstruction, can be repaired via the arterial switch operation during the neonatal period with good outcome was reported in 1996 by investigators at the Royal Children's Hospital in Melbourne, Australia.²¹ This report described 28 consecutive patients who underwent arterial switch operations with baffling of the left ventricle to the neo-aorta between 1983 and 1995. The overall operative mortality rate was 7% (2 of 28 patients). In 11 children with Taussig-Bing anomaly associated with aortic arch obstruction, the actuarial survival rate was 100%, and the freedom-from-reoperation rate was 73% at 6 years. The corresponding rates for 17 children with isolated Taussig-Bing anomaly were 81% and 100%.²¹ A recent report from the Hospital for Sick Children in Toronto described 33 consecutive neonates with Taussig-Bing anomaly who underwent arterial switch operations between 1979 and 2005.²⁸ The mean 1-year survival rate of 17 patients who were operated upon from 1979 through 1999 was only 47% ± 5%, but it was 100% for 16 who were operated upon from 1999 through 2005. The 5-year event-free survival rates for those groups were 35% ± 6% and 87% ± 1%, respectively.²⁹

Epilogue

The detailed description of the Taussig-Bing anomaly paved the way toward better understanding of the malformation and toward improvement of surgical results in affected patients. Sixty years have passed since the original description of the syndrome. The life stories, achievements, and work ethics of Drs. Taussig and Bing will remain a source of inspiration for subsequent generations of young doctors who are interested in the mysteries of the heart. One could summarize Taussig and Bing's work in the words of Johann Wolfgang von Goethe (1749–1832): “It is not doing the things we like to do, but liking the things we have to do, that makes life blessed.”

Acknowledgments

I am grateful to Richard J. Bing, Denton A. Cooley, and the late Wilfred G. Bigelow for their recollections of the events leading to description of the Taussig-Bing anomaly that made this article historically accurate, and to Dr. Andrew N. Redington for providing me with a copy of an original photograph of Helen Taussig. This photograph was personally presented to the late Dr. Robert M. Freedom by Dr. Taussig. I am also grateful to Mrs. Geraldine Cullen-Dean for her kind help with the illustration.