Pediheart: Pediatric Cardiology Today

This week we enter into the world of cardiac morphology and speak with Professor Leo Lopez of Stanford University about a recent work he wrote with the International Society for Nomenclature of Pediatric and Congenital Heart Disease regarding proper nomenclature for VSD's. Is it defect geography or borders that are most important in naming these structures? How was agreement forged between such disparate super-hero experts in cardiovascular medicine and congenital heart disease? Is Dr. Lopez using these designations at the present time? What's next for the nomenclature group? Dr. Lopez shares his insights this week and, as a special treat, also sings as the musical coda to today's program. A very special treat indeed! doi: 10.1016/j.athoracsur.2018.06.020

This week we enter into the world of cardiac morphology and speak with Professor Leo Lopez of Stanford University about a recent work he wrote with the International Society for Nomenclature of Pediatric and Congenital Heart Disease regarding proper nomenclature for VSD's. Is it defect geography or borders that are most important in naming these structures? How was agreement forged between such disparate super-hero experts in cardiovascular medicine and congenital heart disease? Is Dr. Lopez using these designations at the present time? What's next for the nomenclature group? Dr. Lopez shares his insights this week and, as a special treat, also sings as the musical coda to today's program. A very special treat indeed! doi: 10.1016/j.athoracsur.2018.06.020

This week we review a landmark paper reviewing the very large experience of Dr. Hanley and his team at Stanford University in managing VSD/PA with MAPCA's. What advantages are seen with the 'midline unifocalization' approach popularized by this team over the past 20+ years? Is this an approach that can be broadly applied or are the technical operative skills too challenging for most? Why are these operations so difficult? How does the Stanford team decide who is a candidate for a single operation and who is not? We explore many of these topics and others with Surgery Professor Richard Mainwaring of Stanford University who is the first author of this important paper published recently in JTCVS. You will not want to miss Dr. Mainwaring's insightful commentary on the challenges and potential benefits of this very complex and painstaking approach. doi: 10.1016/j.jtcvs.2018.03.153.

This week we review a landmark paper reviewing the very large experience of Dr. Hanley and his team at Stanford University in managing VSD/PA with MAPCA's. What advantages are seen with the 'midline unifocalization' approach popularized by this team over the past 20+ years? Is this an approach that can be broadly applied or are the technical operative skills too challenging for most? Why are these operations so difficult? How does the Stanford team decide who is a candidate for a single operation and who is not? We explore many of these topics and others with Surgery Professor Richard Mainwaring of Stanford University who is the first author of this important paper published recently in JTCVS. You will not want to miss Dr. Mainwaring's insightful commentary on the challenges and potential benefits of this very complex and painstaking approach. doi: 10.1016/j.jtcvs.2018.03.153.

This week we return to the world of adult congenital heart disease to review 2 related works on the topic of late cardiovascular risks in patients who have undergone prior coarctation repair. Are such patients at elevated risk for early coronary artery disease and stroke? What are the causes for these late complications? What role does hypertension play in the development of these late sequelae? Are there other factors that may explain these risks and how should patients be screened for these issues? These are amongst the questions we review with Dr. Sarah Pickard, Instructor in Pediatrics, Harvard Medical School and first author of 2 important new works from the team at Boston Children's Hospital and Brigham and Women's Hospital. doi: 10.1016/j.amjcard.2018.08.051 and doi: 10.1161/JAHA.118.009072

This week we return to the world of adult congenital heart disease to review 2 related works on the topic of late cardiovascular risks in patients who have undergone prior coarctation repair. Are such patients at elevated risk for early coronary artery disease and stroke? What are the causes for these late complications? What role does hypertension play in the development of these late sequelae? Are there other factors that may explain these risks and how should patients be screened for these issues? These are amongst the questions we review with Dr. Sarah Pickard, Instructor in Pediatrics, Harvard Medical School and first author of 2 important new works from the team at Boston Children's Hospital and Brigham and Women's Hospital. doi: 10.1016/j.amjcard.2018.08.051 and doi: 10.1161/JAHA.118.009072

This week we explore the topics of cardiomyopathy, electrophysiology and sudden death and delve into the world of arrhythmogenic right ventricular cardiomyopathy (ARVC). What role might autoimmune disease play in this disorder? Can a biomarker potentially allow us to identify the vast majority of individuals with this disease with high sensitivity and specificity? Can this biomarker identify 'pre-clinical' disease? Can immunological therapies possibly be used to change the course of this disorder? We explore all of these issues and more with Professor Robert Hamilton, University of Toronto, this week on the podcast. This is groundbreaking research that represents a paradigm shift in our understanding of this potentially lethal disorder. doi: 10.1093/eurheartj/ehy567

This week we explore the topics of cardiomyopathy, electrophysiology and sudden death and delve into the world of arrhythmogenic right ventricular cardiomyopathy (ARVC). What role might autoimmune disease play in this disorder? Can a biomarker potentially allow us to identify the vast majority of individuals with this disease with high sensitivity and specificity? Can this biomarker identify 'pre-clinical' disease? Can immunological therapies possibly be used to change the course of this disorder? We explore all of these issues and more with Professor Robert Hamilton, University of Toronto, this week on the podcast. This is groundbreaking research that represents a paradigm shift in our understanding of this potentially lethal disorder. doi: 10.1093/eurheartj/ehy567

This week we review a recent paper from Circulation on the topic of preoperative factors that may impact outcomes of the arterial switch operation. Is there an optimal time to perform an arterial switch operation? Are there other modifiable factors that may auger a better outcome with shorter hospital stay and lower cost? We discuss these questions and others with the first author of this work, Assistant Professor of Pediatrics at The Perelman School of Medicine, University of Pennsylvania, Dr. Michael O'Byrne. Circulation. 2018;138:2119–2129.

This week we review a recent paper from Circulation on the topic of preoperative factors that may impact outcomes of the arterial switch operation. Is there an optimal time to perform an arterial switch operation? Are there other modifiable factors that may auger a better outcome with shorter hospital stay and lower cost? We discuss these questions and others with the first author of this work, Assistant Professor of Pediatrics at The Perelman School of Medicine, University of Pennsylvania, Dr. Michael O'Byrne. Circulation. 2018;138:2119–2129.

This week we enter the world of fetal cardiology to ask the question, 'Can an inexpensive heart rate monitor for fetuses accurately identify in a timely manner fetal patients with impending heart block and can this allow for more timely and potentially effective therapies for AV conduction disease associated with maternal antibodies?' Is there an optimal time to intercede? We discuss a recent multi center work on a home monitoring program with its first author and world authority on fetal cardiology, Professor Bettina Cuneo of the University of Colorado. This is another possible 'game changer' article. doi: 10.1016/j.jacc.2018.07.076

This week we enter the world of fetal cardiology to ask the question, 'Can an inexpensive heart rate monitor for fetuses accurately identify in a timely manner fetal patients with impending heart block and can this allow for more timely and potentially effective therapies for AV conduction disease associated with maternal antibodies?' Is there an optimal time to intercede? We discuss a recent multi center work on a home monitoring program with its first author and world authority on fetal cardiology, Professor Bettina Cuneo of the University of Colorado. This is another possible 'game changer' article. doi: 10.1016/j.jacc.2018.07.076

This week we review a paper from the Amrita Institute in Kerala, India on outcomes of infant surgery in that region and discuss the results with its senior author, Dr. Raman Krishna Kumar. Professor Kumar explains how this large center in India has been able to develop a program that would be the envy of any city in the world, all on a 'shoestring' budget. He reviews with us the path he took from the time he left the US with cardiology training and how he and his associates were able to build one of the finest cardiovascular centers for children in the world. This is a fascinating and inspiring discussion with one of finest cardiologists and human beings in our field. Dr. Kumar's stirring accomplishments for the children of India are noteworthy and his discussion is one of the true highlights of this podcast thus far. For donations to Dr. Kumar's center, please reach out to him at kumar_rk@yahoo.com. doi: 10.1053/j.semtcvs.2015.09.008.

This week we review a paper from the Amrita Institute in Kerala, India on outcomes of infant surgery in that region and discuss the results with its senior author, Dr. Raman Krishna Kumar. Professor Kumar explains how this large center in India has been able to develop a program that would be the envy of any city in the world, all on a 'shoestring' budget. He reviews with us the path he took from the time he left the US with cardiology training and how he and his associates were able to build one of the finest cardiovascular centers for children in the world. This is a fascinating and inspiring discussion with one of finest cardiologists and human beings in our field. Dr. Kumar's stirring accomplishments for the children of India are noteworthy and his discussion is one of the true highlights of this podcast thus far. For donations to Dr. Kumar's center, please reach out to him at kumar_rk@yahoo.com. doi: 10.1053/j.semtcvs.2015.09.008.

This week we speak with Dr. Douglas Mah, Assistant Professor of Pediatrics - Harvard Medical School and Director of the Pacemaker and ICD Service at Children's Hospital Boston about a recent paper he published with his colleagues on coronary artery compression from epicardial pacemaker leads. Who is at risk for this rare complication? How can patients be screened? Once identified, what is the treatment? We review all this and more with Dr. Mah. doi: 10.1016/j.hrthm.2018.06.038