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Before operating on a congenital heart defect, cardiac surgeons need the most precise diagnosis possible. For more than 40 years, the Cardiac Registry at Children's Hospital Boston has been a place where pathologists, cardiologists and cardiac surgeons have gone to learn more about the anatomy of heart defects. Its collection of over 3,600 specimens, some dating back as far as 1944, is irreplaceable, since large, intact examples of heart defects are no longer seen at autopsy in this country. Today, under the direction of Amy Juraszek, MD, the Registry has been broadened to include developmental cardiology and molecular biology projects.
The Registry was founded in 1965 by Richard Van Praagh, MD, with his wife Stella Van Praagh, MD, who served as its directors until 2002. The Van Praaghs and their colleagues generated more than 280 publications detailing the anatomy of heart defects. They are known for devising the segmental approach to congenital heart disease, which allows cardiologists and cardiac surgeons the world over to communicate in a common language. Here, director emeritus Richard Van Praagh, MD, reminisces about his life's work and his longtime collaboration with Stella.
In 1961, I was a young pediatric cardiologist-in-training at the Hospital for Sick Children in Toronto when my chief, John Keith, MD, called me into his office and told me to shut the door, never a good sign. "Dick, why don't you figure out dextrocardia?" he said. "How many kinds are there? How can we diagnose them? What can we do about them surgically? And oh, by the way, you've got 10 days. We're having an international meeting, and I've already put you on the program." I swallowed hard and said, "Yes, sir."
Dextrocardia is a congenital heart defect in which the heart is abnormally positioned on the right side of the chest. It wasn't well understood, except for its devastating effects. If sufficiently malformed, right-sided hearts can be fatal.
I didn't know then that my research would lead me to my future wife and lifelong collaborator. I didn't know our nascent method of diagnosing complex congenital heart disease would be accepted worldwide.
After quickly examining all the dextrocardia heart specimens at Toronto, I travelled to Buffalo Children's Hospital to study their cases as well. There, another young pediatric cardiologist, Stella Zacharioudaki, assisted me, and we labored around the clock to meet our deadline. We'd met earlier at a cardiology conference—I remembered a girl with enormous brown eyes who asked very good questions.
I'd been developing a new way of looking at congenital heart disease based on fundamental principles of anatomy and embryonic development. I taught this approach to Stella, who shared my fascination with cardiac pathology and embryology. She was my first student—but she spent the rest of her life teaching me.
We knew there had to be a better way of classifying heart defects. All hearts have multiple components: three main segments (the right and left atrial chambers, the right and left ventricular chambers and the great arteries) and two connecting segments (the atrioventricular canal, consisting of the mitral and tricuspid valves and the atrioventricular septum; and the infundibulum, separating the valves and ventricles from the great arteries). All these components can be abnormally developed, joined or positioned in myriad ways, leading to many, often conflicting, classifications that cardiologists and cardiac surgeons had to memorize.
An example is transposition of the great arteries (TGA), in which the arteries that take blood away from the heart to the lungs, or to the body, are improperly connected. Under the existing classification system, TGA had at least eight subtypes—A1, A2, A3, A4, B1, B2, B3 and/or B4. It was hopelessly impractical trying to remember these alphanumeric systems without a cheat sheet. Sometimes individual classifications disagreed, creating bedlam.
Stella and I developed a method that greatly simplified and clarified diagnosis by considering each heart's different segments simply by their anatomic pattern and developmental characteristics. The great arteries, for example, can be normally related, with the aorta arising from the anatomically left ventricle, and the pulmonary artery originating from the anatomically right ventricle. Or the great arteries can be transposed—placed across the ventricular septum and arising above the anatomically wrong ventricles. Or both great arteries can originate above the right ventricle—called double-outlet right ventricle. Or they can arise above the left ventricle—called double-outlet left ventricle. No more numbers and letters to try to remember.
Just six months after I presented my work on dextrocardia, Stella and I were engaged, and we married in 1962. Three years and three children later, at the request of then-cardiology chief Alexander Nadas, MD, surgery chief Robert Gross, MD and pathology chief Sidney Farber, MD, we joined the Children's Hospital Boston staff. There we founded Children's cardiac pathology lab, applying the segmental method to describe each case in what became known as the Cardiac Registry.
Stella called it a "library of hearts, hearts that Mother Nature deprived of a normal structure, but that teach us what we need to know to save the children we operate on." Some cases predated open-heart surgery and are irreplaceable examples of uncorrected congenital heart disease.
The Registry became an invaluable research and teaching facility. Surgical residents, cardiology fellows, radiologists, neonatologists, technicians in the catheterization lab—everyone felt at home there. We trained people from all over the U.S. and Canada, and from Japan, France, Germany, England, Greece and Italy.
Stella and I tested and refined the segmental approach for years, and investigated and clarified the anatomy, morphogenesis, diagnosis and management of many specific forms of congenital heart disease. In 1972, I published perhaps my most important paper, "The Segmental Approach to Diagnosis in Congenital Heart Disease." Over the years, we discovered 13 new types of congenital heart disease—including a new type of tetralogy of Fallot—and developed five new surgical operations.
We retired in 2002, and Stella passed away after a brief illness in June 2006. We shared a lifelong affaire de coeur—and also the adventure of growing up with and contributing to the pediatric cardiology and cardiac surgery fields. I miss Stella in so many ways, but I'm making steady progress on our life's work—a clinico-pathological study of more than 3,400 cardiac cases, some never described before. Each chapter of the book is an original study from the Cardiac Registry. Some of the material is more than 60 years old, but it's amazing how many new insights I'm discovering. How I wish I could share them with Stella.
Visit the Cardiac Registry at www.cardiacregistry.org.
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