Pediheart: Pediatric Cardiology Today

This week we review a recent report from Duke University regarding the incidence of femoral arterial thrombosis after femoral arterial cardiac catheterization in small infants. How did the use of routine ultrasonography improve identification of this post-cath complication? What are the outcomes of therapy with heparin or enoxaparin for this problem? Are there factors that are associated with femoral arterial thrombosis? We discuss these questions and more with Associate Professor of Pediatrics, Kevin Hill. doi: 10.1002/ccd.28009

This week we explore the utility of exercise stress echocardiography in pediatric patients. How often were clinical decisions made based upon findings at stress echo? What sort of extra training is needed to perform these studies? How safe was this form of testing? How are stress echo results incorporated into the decision making process for pediatric patients at Emory University? Dr. Soham Dasgupta, 2nd year fellow in cardiology and first author of this work, shares some of the insights of this large Emory experience. doi: 10.1111/echo.14326

This week we review 2 surgical reports. The first is on the Ozaki Procedure for aortic valve repair and we speak with valvular expert David Kalfa, MD PhD of Columbia University. Who is a good candidate for this approach and what evidence exists of its efficacy in children? Dr. Kalfa shares his insights. We also review a recent report from Taormina, Italy on outcomes of repair of anomalous origin of the PA's from the ascending aorta. We discuss this work with its author, Professor Salvatore Agati. doi: 10.1016/j.jtcvs.2018.01.087

This week we review a recent randomized and controlled trial from the NEJM on the use of the novel Medtronic TYRX envelope for the prevention of pacemaker or ICD infections in adult high risk patients. Did the use of this antibiotic envelope result in reduced incidences of infection? Were there complications associated with its use? Should this be used in only in high risk patients? Is this a cost effective solution? These are amongst the many questions we tackle with co-author of this important trial, Dr. Suneet Mittal who is the Director of Cardiovascular Research at Valley Hospital in Ridgewood, NJ. Dr. Mittal offers great insights into this trial and possible implications of these important findings. DOI: 10.1056/NEJMoa1901111

This week we review a recent report from the team at the Kravis Children's Hospital at Mount Sinai - Icahn School of Medicine at Mount Sinai on the use of pentobarbital for oral sedation for transthoracic echocardiography. Associate Professor of Pediatrics Rajesh Shenoy shares with us insights into sedation for this indication, the general approach of the echo lab at Sinai and how decisions are made regarding sedation for this reason. doi: 10.1111/echo.14301

This week we review a recent report on transcatheter pulmonary valve replacement with the Sapien S3 prosthesis. What are the circumstances in which this valve might be preferable versus other valve types? What are the disadvantages of this device? How can patients be effectively screened as candidates for different valves? How can decisions be made between self expanding systems like Harmony or Venus P versus this sort of device for large RVOT's? We review all of these issues and more with Dr. Daniel Levi of UCLA who offers a wonderful overview of transcatheter PVR in 2019. You will not want to miss his many insights. doi: 10.1002/ccd.27973

This week we review an impressive review of the large Australian/New Zealand registry of Fontan patients assessing the impact of AV valve regurgitation and failure on Fontan outcomes. We discuss this work with the senior author Yves D'udekem, Associate Professor of Surgery at The Murdoch Children's Research Institute and also speak with Professor Frank Cetta of the Mayo Clinic who authored a wonderful editorial on this work. Who is at most risk for AV valve failure at Fontan? How well do patients do with AV valve replacement or repair? We discuss all these issues this week on this exciting episode. doi: 10.1016/j.jacc.2018.12.025

This week we review a recent publication on modern day outcomes for children with dilated cardiomyopathy. It seems as if they are doing better than patients did 10 years earlier - why is this so? What factors may account for the improvements in outcomes? We discuss these questions and more with the first author of this multi center work, Dr. Rakesh Singh, Associate Professor of Pediatrics at UC SanDiego and also the Director of the Pediatric Heart Failure and Transplantation Program at Rady Children's Hospital. doi: 10.1016/j.jacc.2017.09.1089

This week we discuss the rationale for intervening for unilateral proximal PA stenosis with a stent or angioplasty. In the setting of normal RV function or pressure, are there exercise data to support intervening and if so, at what degree of maldistribution should such an intervention be considered? How should this decision be made? Is this safe? We review these and other questions with the first author of this work, Gurumurthy Hiremath MD who is the director of the pediatric catheterization laboratory at the University of Minnesota. doi: 10.1016/j.jcin.2018.11.042

This week we review a recent paper on the topic of extended ambulatory ECG monitoring in the ACHD patient. Does this form of monitoring provide benefit versus a more traditional 24 or 48 hour "Holter" monitor? How commonly were arrhythmias in ACHD patients found after 48 hours and how commonly were changes made to management based upon these findings? We discuss these issues and more with the senior author of this work, Dr. Scott R. Ceresnak, Associate Professor of Pediatrics - Stanford University. doi: 10.1111/chd.12736

This week we review the topic of pulmonary hypertension and specifically the use of sildenafil for treatment of this difficult problem. Is this agent safe in children? How commonly does it need to be discontinued for side effects? Is it effective? Do patients ever improve to a degree that it can be discontinued? We review all of these questions and more with Dr. Jennifer Cohen who wrote this work while a general cardiology fellow at Columbia University but is now the Advanced Non-Invasive Imaging Fellow at The Icahn School of Medicine at Mount Sinai - NYC. doi: 10.1016/j.jpeds.2018.09.067

This week we speak with Associate Professor of Medical Psychology at UNSW, Nadine Kasparian about a recent publication regarding evidence for efficacy for various forms of psychological therapy in the setting of childhood-onset heart disease. What do we know about the efficacy of interventions for this fragile population of congenital heart disease patients? How prevalent are psychological problems amongst patients and patient families with childhood onset heart disease? What approaches work best? Dr. Kasparian points to the future in regards to what is needed going forward to advance our knowledge of this critically important problem affecting pediatric and adult congenital heart disease patients. doi: 10.1037/hea0000704

This week we go back to the world of artificial intelligence and review a recent work from Stanford University on the use of deep neural networks to interpret ECG rhythms. How good did a novel algorithm do in comparison to cardiologists in characterization of rhythm abnormalities? What are some novel uses of AI in medicine today and how can AI potentially identify signs of disease that even human scientists are unable to discern? We discuss these interesting questions and many others with Pranav Rajpurkar who is a PhD candidate in computer science at Stanford University and a co-author of this week's important work that we review. doi: 10.1038/s41591-018-0268-3.

This week we delve into the world of fetal cardiology to review a recent work comparing fetal MRI to fetal echo. How did this relatively new technology application fair in comparison to more traditional echocardiography? What role might this technology have in the assessment of congenital heart disease in the fetus? We explore these questions and others with fetal cardiology expert, Professor Lisa Hornberger of the University of Alberta - Stollery Children's Hospital. Dr. Hornberger also discusses how she personally uses CMR in her own fetal practice. doi: 10.1161/CIRCIMAGING.118.007745

This week we review a recent work on contrast induced acute kidney injury in the ACHD patient undergoing catheterization. Are there data to suggest what a safe volume of dye might be in this population of patients? Does the renal response to contrast suggest something about the general health of the patient? How does the team in Boston plan catheterizations in the adult congenital population to minimize contrast usage? We review these questions and others with the first author of this work, Dr. Laura Gellis, who is a senior clinical fellow in cardiology in the Department of Cardiology, Boston Children's Hospital - Harvard Medical School. doi: 10.1002/ccd.27798.

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.