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. Nee Stella Zacharioudaki from Crete, Greece, in addition to her stellar professional attainments, she was also an outstanding cuisiniere, 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 Medical 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.