Sixty Years After Tetralogy of Fallot Correction.

This report describes one of the early cases of open surgical correction of tetralogy of Fallot performed by C. Walton Lillehei and colleagues at the University of Minnesota and discusses findings from the patient's follow-up 60 years later.

Tetralogy Surgery - Back To Baltimore 70 Years Later: Melbourne Heritage and Group Tribute to Juan Comas.

Surgery for Tetralogy of Fallot progressed rapidly from the palliative arterio-pulmonary Blalock-Taussig shunt, introduced in Baltimore 70 years ago, to the "classic" complete transventricular repair technique, with which excellent early results were achieved soon thereafter. However, as duration of follow-up increased, so did the awareness of development of troubling late complications, including severe pulmonary insufficiency, right ventricular dilatation and dysfunction, and tricuspid valve insufficiency, all contributing to increasing incidence of late reoperations, as well as to arrhythmias and sudden death. This realization fueled the initial introduction of the transatrial-transpulmonary repair technique by Kawashima, as well as the subsequent firm establishment of this technique within the framework of an integrated surgical approach by Roger Mee in Melbourne. In turn, Mee's numerous trainees and associates led the dissemination of this approach and provided the impetus for the current wide adoption of a variety of right ventricular and pulmonary valve preservation techniques. In addition to the outstanding surgical results reported by individual centers adopting this surgical strategy, encouraging multi-institutional data are emerging regarding the benefits of these approaches for more favorable early and, most importantly, late outcome. One student and strong proponent of the Melbourne approach was our late colleague and friend Juan Comas, to whose memory this article can serve as tribute.

Tetralogy of fallot: yesterday and today.

Tetralogy of Fallot (TOF) is a cyanotic congenital cardiac defect that was first described by Stenson in 1672 and later named for Fallot, who in 1888 described it as a single pathological process responsible for (1) pulmonary outflow tract obstruction, (2) ventricular septal defect (VSD), (3) overriding aortic root, and (4) right ventricular hypertrophy. The surgical history of TOF began with the development of the systemic to pulmonary artery shunt (BT shunt) by Blalock, Taussig, and Thomas in 1944. Ten years later complete repair of TOF was performed by Lillehei using cross-circulation and by Kirklin with a primitive cardiopulmonary bypass circuit. Notable contributions by several other surgeons including Bahnson, Ebert, Malm, Trusler, Barratt-Boyes, and Castaneda would lead us into the modern era of surgery. Today, complete repair of TOF is performed before six months of age with low mortality (<2%). In select cases a modified version of the BT shunt is still performed as the initial procedure. Long-term survival rates are excellent (85%-90%). Adult survivors with TOF are an ever-increasing population and may require reintervention, surgically or catheter based. Promising future innovations include percutaneous pulmonary valve replacement, tissue-engineered autologous valves and conduits, and genetic manipulation. This article presents a review of TOF, including the history of surgical treatment, present-day approaches, and long-term outcomes.

The first Stella van Praagh memorial lecture: the history and anatomy of tetralogy of Fallot.

Stella Van Praagh, MD (1927-2006) of Children's Hospital Boston was one of the greatest pediatric cardiologists and pediatric cardiac pathologists of the 20th and early 21st centuries. Née Stella Zacharioudaki from Crete, Greece, in addition to her stellar professional attainments, she was also an outstanding cuisinière, hostess, linguist, philosopher, and philanthropist. In 1962, she married Richard Van Praagh, MD, beginning a life-long collaboration that was in every sense an affaire de coeur. They had three children and seven grandchildren. Dr Stella was the author of more than 110 scientific publications which helped to clarify the pathologic anatomy, the clinical and laboratory diagnosis, and often the surgical management of many different forms of congenital heart disease, including dextrocardia, single ventricle, truncus arteriosus, tetralogy of Fallot (TOF), transposition of the great arteries, double-outlet right ventricle, sinus venosus defect, anomalous pulmonary venous drainage, the heterotaxy syndromes with asplenia or polysplenia, juxtaposition of the atrial appendages, and apical muscular ventricular septal defect. In 1999, Dr Stella Van Praagh received the Distinguished Achievement Award of the Society for Cardiovascular Pathology, and in 2004, she was honored with the Paul Dudley White Award of the American Heart Association. Dr. Stella Van Praagh was that vanishingly rare combination of brilliant clinician, internationally renowned medical scientist, and deeply cultivated humanist. The anomaly now known as the TOF was first described by Niels Stensen in 1671, with other early reports by Edouard Sandifort (1777), William Hunter (1784), and many others. In 1888, Etienne-Louis Arthur Fallot published five serialized contributions in Marseille Médical concerning what he called the "blue malady," in which he described the now classical tetralogy of pulmonary outflow tract obstruction, ventricular septal defect, aortic overriding, and right ventricular hypertrophy. The other outstanding feature of Fallot's report was its emphasis on clinicopathologic correlation. In 1924, Maude Abbott coined the term "tetralogy of Fallot." In 1970, Van Praagh and colleagues presented the concept that the TOF is basically just one anomaly, a failure of normal expansile growth of the subpulmonary infundibulum and its sequelae. The anatomy of TOF is presented angiocardiographically, diagrammatically, and anatomically. A morphometric study of typical neonatal TOF is presented, based on 16 autopsied heart specimens with age-matched normal controls. The morphometric study documents that TOF is characterized by a low-volume subpulmonary infundibulum. The diagnostic and surgical significance of these findings is highlighted. Two rare and recently discovered forms of TOF are presented: tetralogy {S,D,I}, and tetralogy {I,D,S}. Because tetralogy {I,D,S} has atrioventricular discordance, in addition to a standard TOF repair, such patients also need an inverted (mirror-image) atrial switch operation (inverted Senning or inverted Mustard procedure). Because associated malformations can be very important to the surgical outcome of patients with tetralogy, the associated anomalies found in 100 randomly selected autopsied cases are presented.

Something the Lord Made.

When Alfred Blalock (1899-1965), the pioneer Hopkins surgical professor, saw the pulmonary artery suturing technique that his laboratory technician, Vivien Thomas (1910-1985), had just performed in a dog, his level of astonishment was so high that he expressed, "Vivien, this looks like Something the Lord Made." In all his years of practice, Dr. Blalock had never seen anything like it before. The level of perfection was so superior to other works that it could not be considered to be of human descent. The surgical technician far exceeded anyone else in the surgical department. In due time, he taught surgical residents and faculty the exquisite details of the laborious vascular suturing techniques. Thomas and Blalock learned to respect and appreciate each other's work. Thomas, the advanced student, and Blalock, the noted teacher, learned to understand one another and recognize their unique abilities. With the support and theoretical advice of his mentor, Thomas proceeded in the old Hunterian Laboratory to solve the problem of the Blue Baby heart disease, also known as Tetralogy of Fallot. The fruits of Thomas's work were put to a test on November 29, 1944, when surgeon-in-chief Blalock took baby Eileen to room 706 to perform the first pulmonary artery to subclavian artery shunt in humans. The results were especially good, and baby Eileen recovered satisfactorily. It was obvious that history had been made, and Thomas and Blalock were the creators, the heroes of this extraordinary feat. They had participated together in Something the Lord Made.

Alfred Blalock. Surgeon, educator, and pioneer in shock and cardiac research.

Alfred Blalock (1899-1964) was a pioneer American surgeon who made significant advances in the knowledge and treatment of hemorrhagic and traumatic shock as well as in the palliative treatment of congenital heart disease, particularly tetralogy of Fallot. This historical note reviews highlights in the life and accomplishments of Alfred Blalock, noted academic surgeon and researcher. Blalock originated from Culloden, Georgia and attended Georgia Military College, the University of Georgia, and Johns Hopkins Medical School. He finished his surgical training at Vanderbilt University where he remained ultimately as professor of surgery until 1941 when he became the Chairman and Professor of Surgery at Johns Hopkins Medical School. His research in shock saved many soldiers' lives in World War II. In 1944 he performed the first subclavian-pulmonary artery shunt for tetralogy of Fallot with the support of the superb pediatric cardiologist Helen Taussig and the great technical help of Viven Thomas. Hundreds of operations followed. He educated and trained an incredible cadre of cardiovascular surgeons. He passed away in Baltimore, Maryland, on September 15, 1964.

A black technician and blue babies.

In this paper, I discuss the many contributions of a versatile black technician, Vivien Thomas, to surgical animal research between 1930 and 1979 at Vanderbilt University and Johns Hopkins University. Thomas' experimental work led to a surgical solution for a life-threatening heart defect, called tetralogy of Fallot. Children with this condition lack sufficient oxygenation and were referred to as 'blue babies'. Following Thomas' research trajectory and his relationship with surgeon Dr Alfred Blalock, I review the conditions under which differing expectations towards race and occupation clashed, creating a status dilemma for Thomas. While the torsion originated in the laboratory where technical skills are valued, the research locale also facilitated a temporary, fragile solution for the status dilemma, because it separated Thomas from public view. Yet, Thomas' dexterity as a laboratory researcher enhanced his dilemma, because credit kept eluding him. In order to track the dynamics of race and occupational subordination as lived experience in the laboratory, I argue for an analysis of the process of crediting people for their scientific accomplishments.

Tetralogy of Fallot. The first 300 years.

The chronicle of tetralogy of Fallot is part of a dramatic evolution in cardiology, cardiac surgery, and understanding of the developing heart. Many new tools and concepts have evolved since Steno of Denmark first described the defect in 1673, and since Fallot of Marseilles coined the term tetralogy in 1888. Four major eras of progress can be recognized. The 1st, the era of pathologic anatomy, culminated in the publication of Maude Abbott's Atlas of Congenital Cardiac Disease in 1936. The next, the era of clinicophysiology and surgery, was highlighted by the 1st Blalock-Taussig anastomosis in 1944, by open-heart surgery 10 years later, and by a new team approach to cardiology. The 3rd, or infant era, began in the mid 1970s with successful intracardiac repair in infants, the rise of echocardiography, and the introduction of prostaglandin therapy. The current era of cardiac development (beginning in the 1990s) gives hope for early understanding of the molecular basis of tetralogy. Tribute is due to the surgical and medical pioneers, and to the pioneer patients and their families, for revolutionary changes in diagnosis and treatment. The challenge of the next 100 years lies in increased understanding of the molecular biology of the defect and in preserving the blend of humanism, scholarship, and skill that have graced the advances of the past 3 centuries.

Etienne Louis Arthur Fallot: una descripción más que centenaria / Etienne Louis Arthur Fallot: a description of over 100 years old

La perspectiva histórica ha sido siempre importante para una mejor comprensión de la Medicina. Este artículo revive la descripción original de un ade las cardiopatías más interesantes e ilustra la gran intuición de quien le dio el nombre. Se incluyen algunos comentarios sobre la actitud de Fallot frente a aspectos polémicos de esta cardiopatía, que históricamente resultan de interés

Etienne-Louis Arthur Fallot and his tetralogy: a new translation of Fallot's summary and a modern reassessment of this anomaly.

As this translation of Fallot's conclusions makes clear, he emphasized the clinical diagnostic usefulness of his findings because of the high frequency of this malformation in cyanotic congenital heart disease. Terming this anomaly "tetralogy," he clearly and simply delineated its four cardinal features. He stated that cyanosis was not due to a patent foramen ovale. He attributed the morphogenesis of the tetralogy to an intrauterine pathologic process at the level of the pulmonary valve leaflets and the subpulmonary infundibulum. The tetralogy and Fallot's contribution are reassessed in the light of present understanding.

Tetralogy of Fallot: the centenary of the name. A new translation of the first of Fallot's papers.

1988 marks the centenary of the cognomen "tetralogy of Fallot". Fallot's works are often quoted and translated without mentioning that it was he who applied the noun "tetralogy" to the malformation. This new translation shows that his contribution to the morbid anatomy was not great, but he was unknowningly responsible for providing a unifying concept and a name for the commonest of all cyanotic congenital heart defects.