Pediheart: Pediatric Cardiology Today

This week we review the Fontan and long term issues of surveillance and Fontan failure with 2 articles. The first is a large survey study reviewing practice patterns amongst large Fontan clinics in the US. We review this interesting topic of optimal assessment of the Fontan with Dr. Mike DiMaria who is first author of this recent work and also Associate Professor University of Colorado. We then review a case report on biphasic curaiss ventilation for the treatment of Fontan associated PLE and plastic bronchitis with first author Professor Seigo Okada from Kyushu Hospital in Japan and also with Dr. Pradeep Charla who is the director of the adult congenital heart program at University Hospitals and Rainbow Babies Hospital. Both Dr. Okada and Dr. Charla have extensive experience with this form of 'iron lung' therapy in the setting of a Fontan and share this experience with us all. doi: 10.1007/s00246-018-1966-9 and doi: 10.1536/ihj.17-292.

This week we turn to the world of cardiovascular nursing and review the notion of individualized family centered care for infants in the CICU. What is meant by the term "Individualized Family-Centered Developmental Care" and what are its components? What is the evidence that use of this approach can result in improved neuro-developmental outcomes? We review these important concepts with the first author of this work, Amy Jo Lisanti PhD, RN who is a clinical nursing specialist and researcher at The Children's Hospital of Philadelphia. Dr. Lisanti explains this novel multidisciplinary approach to care in the CICU and why she believes it may hold hope in improving outcomes following complex neonatal surgery. doi: 10.1097/JCN.0000000000000546

This week we review the notion of sports participation in athletes who have ICD's. Is this a safe approach? How do the athletes fare? How do the devices stand up to sport? What are the subtleties of discussing this important topic with a family and how does shared decision making work in this process? We review this serious and complex topic with Dr. Rachel Lampert, Professor of Medicine at Yale University and Dr. Elizabeth Saarel, Professor of Pediatrics - The Cleveland Clinic. Both experts have much to share about their experience in this important work on how to potentially improve the quality of life for our ICD patients. doi: 10.1161/CIRCEP.118.006305

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 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 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 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

This week we explore the world of 'fast tracking' patients following cardiac surgery and focus on how to best provide analgesia and sedation to such patients. Are there advantages to intermittent boluses of such agents rather than a low dose basal infusion? What are the implications of such a strategy for nursing and medical care? Does 'waiting' for discomfort prior to administration of medication work better than 'preventing' such? Are there patients who would not be good candidates for such an approach? We explore these issues with Assistant Professor of Pediatrics at Mount Sinai Medical Center - NYC, Dr. Scott Aydin who is also associate director of the pediatric critical care unit at that same institution.

This week we explore the question of optimal timing of the Fontan operation. What factors are associated with good or bad outcome after Fontan in regards to age at surgery? Can Fontan palliation have a positive impact on cardiac function? Is there a 'sweet spot' regarding timing of this single ventricle palliation? Who deserves a fenestration? These are amongst the many questions we review this week in our review of a recent large German experience in Fontan in patients with advanced age at Fontan. We discuss the paper and aspects of it with noted surgeon Dr. Ram Kumar Subramanyan, Assistant Professor of Surgery and Pediatrics of USC - Children's LA. DOI: 10.1016/j.jtcvs.2018.03.155

Interventional cardiology is on tap this week as we explore the world of transcatheter management of the dysfunctional RVOT with 2 papers on this topic. In the first, Dr. Mary Hunt Martin, Assistant Professor of Pediatrics - University of Utah is the first author on a recent PHN paper on outcomes of Melody implantation in the native RVOT. Dr. Martin joins us to answer a number of questions about this important work. The second work is a recent multi center French study on use of the Sapien valve in the RVOT/conduit. How do the acute results with this valve compare with the Melody? What are the indications for Melody vs. Sapien? These are amongst the questions we tackle this week on the podcast. DOI: 10.1016/j.jcin.2018.05.051 and DOI: 10.1016/j.jcin.2018.05.050

This week we explore the interpretation of T wave inversions in the athlete with Professor Sanjay Sharma, Professor of Cardiology University of London. Which T wave inversions are "normal variants" and which are worrisome for disease? What is the role of genetic testing in determining who has disease? We review Prof. Sharma's group's recent paper on this topic and discuss different aspects of this work with this world-renowned figure in this field. DOI: 10.1161/CIRCULATIONAHA.118.034208

This week we delve into the world of Marfan Syndrome and review a recent report from the Pediatric Heart Network with the first author of this work, Dr. Seda Selamet Tierney - Associate Professor of Pediatrics, Stanford University. Why does stiffness of the aortic wall predict dilation and need for surgery? Does the use of atenolol or losartan affect aortic growth or dilation? Which agent is preferable? Dr. Selamet Tierney shares her insights this week on the podcast. DOI: 10.1016/j.amjcard.2018.01.016

This week we review a novel paper on MRI changes observed in TOF adults. Are these signs for advanced brain 'aging' in tetralogy adults? We also review a prospective trial of different mechanical ventilation strategies during SVT ablations in children. Can tidal volume or PEEP affect catheter stability while mapping or ablating SVT? We discuss this question with the first author of this work, Dr. Christopher Janson, Assistant Professor of Pediatrics at The Children's Hospital of Philadelphia. Articles: DOI: 10.1371/journal.pone.0202496; DOI: 10.1007/s00246-018-1968-7

This week we explore the world of cardiac transplantation with particular emphasis on the Fontan patient and transplantation. Outcomes are improving rapidly. Why is this? What about the liver and how are decisions made regarding cardiac versus cardiac+hepatic transplantation in this patient population? We review all of these questions with world authority on cardiac transplantation Dr. Daphne Hsu, Professor of Pediatrics, Chief of Pediatric Cardiology and Interim Chair of Pediatrics at Montefiore - Albert Einstein College of Medicine. Her insights into this complex patient group are valuable and novel. Article reviewed: DOI: 10.1016/j.athoracsur.2016.08.110

This week we explore both fetal cardiology and cardiovascular surgery. The first work reviews a fascinating case report of fetal treatment of cardiac rhabdomyomas in the setting of hydrops fetalis. The second reviews a recent report on LV retraining prior to attempted double-switch operations in LTGA and DTGA. Can one accurately predict 'retrainability' of the LV? Can life threatening rhabdomyomas be treated in fetal life? These are amongst the questions answered this week. Articles: DOI: 10.1056/NEJMc1800352; DOI: 10.1016/j.athoracsur.2017.11.047

This week we explore the notion of pulmonary valve function preservation at the time of TOF/PS repair with Dr. Ram Emani, Associate Professor of Surgery at Harvard Medical School/Boston Children's Hospital. Dr. Emani shares his insights from recent work he and colleagues have performed using a novel intraoperative balloon dilation approach. Can TOF/PS repair be performed without creating significant degrees of pulmonary valve regurgitation? We learn the answers this week.