Charles F. Wooley, MD, Heart Lung Research Institute, 473 West 12th Avenue, The Ohio State University, Columbus, OH 43210; e-mail: firstname.lastname@example.org
In 1965, Helen Taussig traced the evolution of knowledge of congenital heart disease (CHD) during the 20th century, beginning with the William Osler-Maude Abbott lineage at McGill University in Montreal, Canada. Osler encouraged Abbott in her CHD pathologic observations. Abbott's London Exhibit (1934) preceded her classic text Atlas of Congenital Cardiac Disease (1936). Taussig's friendship with Abbott (1935) began in Boston; Abbott brought Taussig to meet Paul Dudley White whose text Heart Disease (1931) featured Abbott's work. Taussig visited Abbott (Montreal 1938). Abbott's statistical approach was based on post-mortem malformations; Taussig's concern was why CHD babies died. Abbott (1927) suggested surgery for a patent ductus arteriosus; Taussig conceived of creating a patent ductus arteriosus shunt to improve lung blood flow in cyanotic “blue babies”. Surgeon Alfred Blalock and Taussig collaborated with the blue baby shunt operations (1944–1945), opening the field of cardiac surgery in cyanotic babies. Taussig's Congenital Malformations of the Heart text came 2 years later. Sequential contributions by Osler, Abbott, White, and Taussig were landmarks in the evolution of knowledge of CHD in North America.
In 1965 Dr Helen Taussig, consummate pediatric cardiologist from Baltimore, Maryland, traced the evolution of knowledge about congenital heart disease (CHD) during the 20th century.1 Acknowledging the origins of CHD from antiquity through the autopsy-based 19th-century literature with a focus on Peacock, Ebstein, and Rokitansky, her narrative shifted to North America and Montreal, Canada, in the early 20th century. She assigned primacy to Dr Maude Abbott's accomplishments at McGill University, set within the William Osler-Maude Abbott lineage in the McGill Medical Museum in Montreal.
Following the McGill-Montreal beginnings, a series of interactions were pivotal between Abbott and Osler, Abbott and Dr Paul Dudley White of Boston, Massachusetts, and Abbott and Taussig; these form the essence of this narrative (Figure 1).
William Osler, Pathologist
William Osler (1849–1919) obtained his medical degree at McGill University in 1872 and completed his postgraduate studies abroad. While in Berlin, Germany, he was exposed to the precise methodology of the pathologist Rudolph Virchow, the leading medical scientist of the day. Osler returned to McGill as a faculty member and physician during the decade of 1874 to 1884. During this period he also served 7 years (1876–1884) as pathologist at Montreal General Hospital where he conducted about 750 autopsies (Figure 2).
So much emphasis has been placed on Osler's later multifaceted career in the United States and England that his days in Montreal as a pathologist receive less attention. Osler's primary biographers, however, have described certain facets of his time as a pathologist. Cushing2 described the impact of Virchow's precise methodology on Osler's apprenticeship as a pathologist, while Bliss3 observed that during Osler's time in Montreal, he became the pathologist for the entire Montreal medical community. In addition, Osler's skills as a microscopist contributed to his development as a qualified morbid anatomist. In retrospect, Osler's early career as a pathologist was a powerful influence on his subsequent development as a clinician and contributed to his remarkable clinical persona.
Osler's earliest publications were based on his pathology studies. “Cases of cardiac abnormalities” was published in 18804 and the more specific “Congenital affections of the heart” in 1889.5 He described his findings in patients with congenital bicuspid aortic valve, a subject of long-term inter-est; pulmonic valve stenosis; pulmonary atresia with ventricular septal defect; patent foramen ovale; and idiopathic ventricular hypertrophy. When Osler moved to Philadelphia, Pennsylvania, in 1884, he left a collection of autopsy specimens that in time became part of the newly organized Medical Museum under Maude Abbott's direction.6,7
Maude Abbott and the Classification of CHD
Maude Elizabeth Seymour Babin (1869–1940) was made motherless as an infant by her mother's death from tuberculosis and was abandoned by her father. Maude was brought up by her maternal grandmother, Mrs. William Abbott, who adopted Maude and her sister, Alice.
The Maude Abbott story is one of remarkable personal achievement despite the obstacles faced by women in medicine at the turn of the 20th century.8 She was graduated with a Bachelor of Science degree from McGill University in 1890, but she was refused admission by McGill's Medical Faculty because she was a woman. She then attended Bishop's Medical School in Quebec, graduating with a medical degree in 1894. Following postgraduate study in Europe in pathology and internal medicine, she returned to Montreal in 1897. She was encouraged by Dr Charles Martin and supported by Dr George Adami, chair of pathology at McGill, who appointed her assistant curator of the McGill Pathology Museum in 1898 and curator in 1899. She became the first woman faculty member at McGill in 1900.8
Abbott's first personal encounter with Osler took place in Baltimore in 1898. Osler's encouragement led her to devote her energies to develop the medical museum and to extend Osler's earlier CHD pathologic observations. In 1906 and again in 1927, her extensive CHD experience prompted Osler to ask her to contribute to Osler and McCrae Systems of Medicine, the only woman among the 104 contributing authors.9,10 The published articles made her reputation in the field of cardiac disease.
Paul Dudley White: Recognition of Abbott's Research
Boston cardiologist Paul Dudley White (1886–1973) occupied a unique place in the genesis and recognition of US cardiology as a discipline during the 20th century. White was an early participant in the Anglo-American cardiology experience before and after World War I. From 1911 to 1913, he was a Sheldon traveling fellow, working primarily with Sir Thomas Lewis and for a time with Sir James Mackenzie. He renewed his English acquaintances during and after his World War I military service in France and Macedonia.11
White's 1931 textbook, Heart Disease,12 was a masterful, one-author, clinically oriented work that revealed the clinical knowledge at a time when application of the technology of the electrocardiograph, x-ray, and cardiac fluoroscopy was augmenting the traditional approaches of the cardiac physical examination, cardiac auscultation and pulse recordings. Chapter XII, “Congenital heart disease,” incorporated Abbott's work from 1923 and 1928 and included a large foldout section with her original descriptive, statistical, and classification data. The chapter was augmented by an extensive bibliography, characteristic of White's text. The inclusion of Abbott's research and contributions in White's text was an important recognition of her work because the book was revised and reissued during the next 20 years and gained worldwide acceptance.
White became ambassador-at-large, cardiologist to the world, and a tireless early advocate of diet, exercise, and moderation. White's role as consultant physician to US President Dwight D. Eisenhower when the president experienced a heart attack in 1955 featured frank, open, almost blunt medical reports that changed the way physicians related to the press in such circumstances. Today, among his many contributions, White is recalled as the second “W” in the Wolff-Parkinson-White syndrome, a touchstone entity in cardiac electrophysiology.
Abbott's London Exhibit and “The Atlas”
Abbott's 1934 London exhibit of her CHD material at the British Medical Association was a milestone in her career. The exhibit incorporated her work of 35 years, including a compilation of the existing knowledge of CHD and her personal identification and classification schemes. Her innovative concepts and clinical correlations, as compiled in the exhibit, were futuristic and certainly stand the test of time.
The exhibit served as prototype for her classic text, Atlas of Congenital Cardiac Disease, published in 1936.13 In his foreword, White credited “Maude Abbott, fired by a spark from Osler” for making the subject of such general and widespread interest, having removed the topic from the mystery of the autopsy table and into a living part of clinical medicine.
The atlas was a major contribution to the understanding of CHD at a time when the spectrum of disorders was poorly understood, clinical CHD diagnosis was rudimentary, and there was no medical or surgical treatment for the disease. What was distinctive about the atlas? Part I presented the developmental stages and comparative anatomy of the heart, a compilation of observations and illustrations dealing with the reptilian and mammalian heart. Part II featured a clinical classification of CHD with illustrative specimens; Abbott's exhibits and atlas incorporated clinical classifications and correlations including chest x-rays, electrocardiograms, and graphic presentations of cardiac murmurs by Dr Harold N. Segall. Segall earned his medical degree from McGill in 1920 and was appointed demonstrator in pathology and assistant curator of the Medical Museum under the direction of Abbott in 1921. It was Abbott who introduced him to cardiology, medical history, and the writings of William Osler. Segall went on to a distinguished, long-lived career in cardiology, medical history, and Osleriana.
All of the atlas presentations were based on Abbott's large personal series augmented by pertinent observations from the medical literature:
Group I, acyanotic group - cardiac lesions that deformed cardiovascular structures without accompanying communications or shunts between the arterial and venous systems
Group II, arterial-venous shunts - cardiac lesions that resulted in communications between the arterial circulation and the venous system
Group III, veno-arterial shunts - cardiac lesions that resulted in communications between the venous system and the arterial circulation with venous blue blood being shunted into arterial red blood, producing cyanosis. Thus, this group comprised the cyanotic group of lesions.
The last section of the atlas presented a statistical analysis of CHD based on Abbott's analysis of 1000 cases.
In 2006, a new edition of the atlas was published and included a perspective on Abbott's life (Figure 3). The color plates of her pathologic specimens were created using contemporary digital photography, reinforcing the landmark nature of her work from a perspective not possible in the 1930s.8
Helen Brooke Taussig
The pathway of Helen Taussig (1898–1986) to worldwide recognition as a leader in pediatric cardiology was marked by a series of early obstacles—childhood dyslexia, the death of her mother when Helen was 11 years old, progressive hearing loss in young adulthood, and discrimination against women in medicine. Her individual characteristics, her academic father's personal guidance, and the wisdom of Dr Edwards A. Park, professor of pediatrics at the Johns Hopkins University, contributed to her development, maturation, and success.
Born in Cambridge, Massachusetts, she attended Radcliffe College and the University of California at Berkley, was Phi Beta Kappa, and received a Bachelor of Arts degree in 1921. Refused admission to Harvard Medical School, she subsequently received her medical degree from Johns Hopkins in 1927. After the Department of Medicine refused her an internship in medicine, she was offered a fellowship under Dr Edward P. Carter, then in charge of the Heart Station.
When Park came to Johns Hopkins in 1927, he recognized Taussig's promise and in 1930 put her in charge of the children's cardiac clinic. “He gave me a social worker, a technician, and an electrocardiograph machine,” Taussig wrote. Initially, the emphasis was to be placed on rheumatic fever and rheumatic heart disease. When the fluoroscope was installed, Taussig went to New York City to learn fluoroscopy from Dr May Wilson (1889–1971), professor of clinical pediatrics at Cornell University and an authority on rheumatic fever and rheumatic heart disease.14
It was not until 1935 when Taussig carried out a fluoroscopic examination of a cyanotic baby with no enlargement of the right ventricle and made a diagnosis of an absent right ventricle and then saw another cyanotic baby with the same condition that the dawn of her interest in CHD came. She began to realize that malformations of the heart repeated themselves, similar malformations caused similar changes in the size and shape of the heart, and the clinical diagnosis of congenital malformations was feasible.
Taussig spoke of the true arousal of general interest in CHD that accompanied Maude Abbott's contributions. “My friendship with Dr. Abbott started in 1935 when we met in Boston and I told her of the two cases of nonfunctioning right ventricle. Quite characteristically she then and there arranged to take me to Dr. Paul Dudley White's home for dinner that night!”1
Taussig, no stranger to professional bias herself, described Abbott as “a delightful, friendly, warmhearted person, who throughout her life was miserably treated by most of her medical colleagues.” The great exceptions were Drs Martin, Adami, Segall, and White, who more than anyone else made Abbott's work appreciated by her colleagues. Osler certainly should be added to Taussig's list of individuals who championed Abbott's cause.
Taussig, Abbott, and the Correspondence
Taussig and Abbott exchanged queries, observations, and information in a series of letters during 1935 and 1936. In one early exchange, Abbott suggested that Taussig should get in touch with White.15
Taussig, puzzled by a recent case of a patient with an enormous left auricle, queried Abbott as to whether the left auricle enlarges in cases of truncus arteriosus without a patent ductus in which the lungs are supplied through the bronchial arteries. A long letter from Abbott to Taussig goes into great detail on the subject of interauricular septal defect, with concerns about the nature of the mechanisms of left-to-right shunting and cardiac chamber enlargement. Abbott then discussed chamber enlargement and valvular deformities in persistent ostium primum, a complex atrial defect with mitral and tricuspid valvular involvement.
Abbott ended one letter with a request for “x-rays of hearts with auricular septal defect confirmed by autopsy.” She was scheduled to address the New York Heart Association at the New York Academy of Medicine in mid-January 1937 and her need was somewhat acute. Taussig's prompt reply referred to the photographs she was sending along with short clinical descriptions of the children with these defects who died from progressive heart failure.
In 1938 Taussig's growing CHD interest prompted her to visit Abbott in Montreal where “she [Abbott] taught me the diagnosis of a right aortic arch . . . Maudie, as we called her, introduced me to everyone she knew in Montreal and while there gave a dinner for me.”1
After studying the CHD collections in Montreal, Taussig returned to her research at Johns Hopkins. While Abbott took a statistical approach to the postmortem analysis of congenital malformations, Taussig's interest was in the rationale for specific findings in particular patients and the clinical diagnosis of malformations of the heart on a broad functional basis. She was most concerned as to why the cyanotic babies died. Both investigators were working their way through the pathophysiologic changes resulting from specific cardiac malformations. As early as 1927, Abbott had suggested surgery might be possible for certain congenital lesions such as valvular pulmonary stenosis.
Taussig speaks of an encounter with a 4-year-old child with tetralogy of Fallot who died without going into heart failure but just became bluer and bluer. She realized the child died from anoxemia, ie, he could not get enough blood to his lungs to live. She came to understand that circulation to the lungs was essential to extrauterine life and that these babies died as a result of the closure of the previously patent ductus arteriosus (PDA) and loss of the oxygenated blood flow to the lungs. Thus, she realized that cardiac malformations were not static. In these circumstances, closure of the ductus arteriosus was usually fatal to infants with pulmonary atresia and made patients with a tetralogy of Fallot and pulmonary stenosis worse.
CHD: Surgical Considerations
At this stage, the narrative accelerates as the surgeons enter the CHD arena. The initial surgical approaches to congenital cardiovascular malformations involved repair of coarctation of the aorta, a constriction or narrowing of the thoracic aorta, and closure of a persistent PDA. Both of these structural defects were located outside the heart itself and did not require the surgeon to enter into the cardiac chambers per se. The rapid progress in the surgical therapy of CHD within the heart is a story for another time.
Dr Robert Gross at Harvard University in Boston, Massachusetts, published his report of surgical ligation of a PDA in February 1939,16 confirming the feasibility of a surgical approach to this persistence of the fetal connection between the aorta and pulmonary artery. Taussig conceived of creating a PDA-type shunt (arterial to venous) to relieve lack of blood flow to the lungs and improve oxygenation in a cyanotic “blue baby.” Realizing that it should be possible to build a ductus-type shunt in a cyanotic child, she approached Gross, but he was not interested in her suggestion.
Later, Taussig said she was grateful to Harvard and Dr Gross for the refusal, as she returned to Baltimore to bide her time until Dr Alfred Blalock became surgeon-in-chief. The Blalock-Taussig shunt operation came about after 2 years of arduous laboratory research by Blalock and his associate, Dr Vivian Thomas, which opened up the field of cardiac surgery in cyanotic babies and children.1 The relief of cyanosis and its consequences as a result of the heralded “blue baby” surgery in the mid-1940s captured the imagination of the medical community and the lay press. It was a signal event in the history of cardiac surgery and brought the next generation of cardiac surgeons to Johns Hopkins in Baltimore for training.1
Two years after the first Taussig-Blalock shunt was performed,17 Taussig published her renowned text, Congenital Malformations of the Heart18 (Figure 4). The foreword by Park described Taussig's maturation as an investigator of cardiac malformations, relying on skillful clinical appraisal complemented by cardiac fluoroscopy. The next generation of cardiovascular techniques, cardiac catheterization and cineangiography, which would transform diagnostic methodology and precision, were at the gates, but in 1947 neither Park nor Taussig could foresee the extent of the coming diagnostic revolution.
The path from yesteryear to the present frequently runs through unexplored territories. The sequential interactions, contributions, and publications by Drs William Osler, Maude Abbott, Paul Dudley White, and Helen Taussig were landmarks in the evolution of knowledge of CHD in North America.
The sequence of major steps in this narrative began with Osler as pathologist with his descriptive CHD observations early in his career. Abbott, meeting with Osler in Baltimore in 1898, was stimulated to expand her role at the medical museum in Montreal and to perform her statistical studies of specimens and create new classification schemes. White's early recognition of Abbott's seminal work was an integral contribution to the furtherance of Abbott's medical career. The friendship that developed between Abbott and Taussig facilitated the exchange of information between these two investigators. Taussig's emphasis on the physiologic principles marked the transition between the basic pathologic, morphologic, and descriptive period into the cardiovascular surgical era that continues to resonate to the present.
Graphic presentations by Dennis Mathias, graphic artist, Division of Cardiology, The Ohio State University, Columbus, OH.