Interview with Dr. Richard Van Praagh, author of Congenital Heart Disease: A Clinical, Pathological, Embryological, and Segmental Analysis
Why did you feel that it was important to write a book on this topic?
To this day, congenital heart disease (CHD) is the commonest anomaly in live-born children. CHD is much more complex than is acquired (adult) heart disease. As such, it was my hope in writing this book to add to the development of the vitally important clinical fields of pediatric cardiology and congenital heart surgery.
What does Congenital Heart Disease, 1st Edition add to the field?
The exciting new contribution of this publication to the field of CHO is the development and presentation of set of new simple mathematical equations that define many of the known, morphological forms CHD (see Appendix I). Now, it is important for the reading audience to understand that this publication is, in fact, more than a latest effort to contribute to the field of CHD. It is a summary of the new understandings in CHO that I have been fortunate enough to contribute to, along with many colleagues, over the span of my entire career. This new publication is, indeed, beyond its new contributions, a distillation of the understanding presented in 300 plus publications over the span of 50 years. For example, concerning the correct morphologic method of cardiac chamber identification, I describe how it should include both the septal and the free wall surfaces of the right ventricle (RV) and of the left ventricle (LV), not just the septal surfaces only. This made it possible to understand what single ventricles are (see Chapter 17).
Furthermore, regarding Tetrology of Fallot, I discuss in this publication that it is basically just one anomaly, not four different, unrelated malformations (see Chapter 20). And furthermore, in this publication, I have attempted to present my understanding that most anomalies of the great arteries are not, in fact, anomalies of the great arteries at all. They are, instead, malformations of the muscular infundibulum under the great arteries. In normal embryonic development of the heart, genetically programmed cell death (apoptosis) occurs beneath the right-sided aorta (Ao), making possible the normal embryonic aortic switch from above the RV to above the LV. Normally, infundibular apoptosis does not occur beneath the left-sided pulmonary artery (PA). Consequently, the PA is not switched to the LV.
Ectopic infundibular apoptosis results in many different anomalies of the great arteries, including: (1) Transposition of the Great Arteries (TGA, see Chapter 22). In terms of the new mathematical equations, mentioned above, this understanding of TGA may be expressed as follows: TGA {S,0,D} = 4R+ 0L, where no apoptosis occurs under the Ao, while extensive apoptosis occurs under the PA. (2) Double outlet Right Ventricle (0ORV), i.e., the Taussig-Bing malformation has no sub-arterial apoptosis. Consequently, neither great artery gets switched, resulting in DORV. And, again, in term of the new equations, DORV can be described as follows: DORV {S,0,D} = 4R+ 4L. And analogously, and briefly, (3) Double Outlet Left Ventricle (DOLV) can be described as DOLV {S,0,0} =OR+ 0L. The apoptosis is bilateral, and this is why both arteries, the Ao and the PA, get switched in DOLV. Please see Chapter 24 for further details. And for a summary of all the equations, please see Appendix 1.
What is the most exciting aspect of Congenital Heart Disease, 1st Edition?
The most exciting part of this book? In my opinion, it is the equations (Chapter 26 and 27).
Why? Math is the language of science. These equations express a general theory of normally and abnormally related great arteries (again, please see Chapter 26 and 27 and Appendix I).
Who will find the greatest value from this book and why?
Everybody! That is, everybody who is seriously interested in congenital heart disease: pediatric cardiologists, pediatric cardiac radiologists, pediatric cardiac surgeons, pathologists, embryologists, and geneticists. I really do not know whose needs are greatest. All such medical professionals may benefit greatly by reading this book.
What new ideas, practices, or procedures do you hope your readers take away from Congenital Heart Disease, 1st Edition?
There is a lot that is new in this book. And this is clearly inferred by the book’s subtitle-“A Clinical, Pathological, Embryological, and Segmental Analysis.” I was fortunate during my medical career to acquire an understanding of CHD from multiple vantage points: from that of a pathologist, embryologist, and clinician. With this understanding, I have attempted, with the assistance of many colleagues, to construct a morphologically-based understanding of CHD that could be of practical use to the pathologist, the pediatric cardiologist and the pediatric cardiac surgeon. Hopefully, the multidisciplinary approach presented in this book can serve as an important “step forward,” as a considerable advance, in the field of CHD.
What problem do you hope the future generations of your specialty will be able to solve?
It is my hope that ensuing generations of investigators will be able to derive a comprehensive genetic understanding of the etiology of congenital heart diseases, and that this new understanding will facilitate the treatment and eradication of such cardiac-based diseases in future patient populations.
Is there anything else about the book you’d like to say?
Yes! May the readers be inspired by this book to continue and to advance their own clinical practices and/or research in the field of congenital heart disease. This book is the work of my professional lifetime, and it is my final contribution to the field of congenital heart disease. I have put my heart into it. Enjoy!
About Dr. Richard Van Praagh
I am Richard Van Praagh, MD, Harvard Professor of Pathology Emeritus. I founded the Cardiac Registry-the cardiac pathology and embryology lab of Boston Children’s Hospital-in 1965. In 1972, I proposed the segmental approach to the diagnosis of congenital heart disease. This segment-by-segment method of diagnosis is now used worldwide. My colleagues and I have published more than 300 scientific papers and 3 books. In all of this work, I have been assisted by pediatric cardiology fellows, cardiac surgery residents, and many wonderful friends and colleagues from all over the world. I would like to make special mention of the invaluable assistance that I have had from my first wife, Dr. Stella Van Praagh, MD, who died in 2006, and from second wife, Marcella (Marty) V. Holmes.
I would like to note here that I have also previously published a work of non-fiction, that has no bearing on congenital heart disease. Instead, it is my summary understanding of the current societal factors that are at play in shaping the future of mankind. And how a clear understanding of these forces is a vital ingredient in our survival as a species, and that of Nature as we know it. In this book, entitled Survival, I have addressed this broad, societal topic from the perspective of a scientist and a humanist, as one who is a practitioner of the scientific method, and ultimately, as one who is an advocate for the truths made self-evident by this approach.
This book, with the full title of, Survival: A New Approach from the Life Sciences to the Major Problem of Our Time, can be found on amazon.com. It was originally published in 1985 by Falcon Press, Phoenix, Arizona, 85012, USA.
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