Pediheart: Pediatric Cardiology Today

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-...

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, As...

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 mult...

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...

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 trea...

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...

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 we...

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 t...

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...

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 prefe...

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....

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,...

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...

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 significa...

This week we discuss 2 papers on different areas of congenital cardiology. The first is a recent Brazilian report on Zika Virus Syndrome and associations with congenital heart disease. The second is a recent report by the team at Children's Hospital of Philadelphia on exposure to plastic neurotoxicants found in many common medical devices. Do these plastic substances have an impact on neurodevelop...

This week we review a very recent work on long term (14 years!) outcomes of children with hypertrophic cardiomyopathy in Australia and speak with its author, Professor Robert Weintraub of Royal Children's Hospital in Melbourne. Dr. Weintraub explains the data in his work for us and what he considers to be important risk factors for poor outcomes. He also shares some thoughts on how he thinks about...

This week we explore 2 unrelated but possibly 'game changing' papers. First we delve into a very recent report on a novel intranasal therapy for acute termination of SVT. Then we review a recent report from the Boston Adult Congenital Heart Program (BACH) on a potential role for inflammation as a possible cause for poor outcomes in ACHD patients. Can inflammatory markers predict poor outcomes in A...

This week we delve into the world of novel means for epicardial pacing and defibrillation in small children or those with congenital heart disease. Dr. Bradley Clark of The Children's Hospital at Montefiore and Dr. Yaniv Bar-Cohen of Children's Hospital of LA discuss their recent works on developing minimally invasive epicardial pacing and defibrillation in infants and those with congenital heart...

This week we replay the 2nd podcast in the series. Dr. George Ofori of Montefiore reviews a recent paper on a project in Angola to provide outstanding congenital cardiovascular care in that nation and the challenges associated with these sorts of projects and we also review an extraordinary report of non-invasive ablation of severe ventricular arrhythmias using radiation. Can arrhythmias be ablate...

In this landmark 25th episode of the podcast we delve into the world of echocardiography with one of the masters of this field, Dr. Shubhika Srivastava of Mt. Sinai Medical Center in NYC to discuss a recent paper by her team on 'twisting' and 'untwisting' abnormalities in repaired tetralogy patients. Who is at risk for these problems? What do these findings mean? Can they help inform decisions reg...

This week we explore the role of transcatheter closure of the PDA in the infant, particularly those < 2 kg and even < 1 kg. We review data from the IMPACT trial on closure of the PDA in small infants and also discuss an important paper by Dr. Evan Zahn on transcatheter closure of PDA's in the premature or very small infant. What are the outcomes when closing the PDA in a small infant? What are the...

This week we review a recent paper on follow-up of the novel approach of percutaneous carotid artery vascular access in infants requiring neonatal or early infancy catheterizations with Dr. Christopher Petit of Children's Healthcare Atlanta - Emory University. Dr. Petit reviews how the team in Baylor and Atlanta came to use this type of vascular approach and also some important 'pearls' of perform...

This week we explore fetal cardiology with 2 fetal cardiology experts - Mary Donofrio, MD from Children's National Medical Center and Maciej Slodki of Polish Memorial Mother's Research Institute. Both authors/experts discuss the notion of predicting the initial minutes and hours after birth with complex congenital heart disease. Are there new ways to predict the need for atrial septal intervention...

This week we delve into the world of postoperative CICU management of complex congenital heart surgical patients with one of the pioneers of this field, Dr. Anthony Rossi of Nicklaus Children's Hospital. Dr. Rossi has been at the forefront of research into the optimal management of children following complex congenital heart surgery. Are there ways to predict problems before they become problems i...

This week's 20th episode tackles the topic of pulmonary hypertension both in the newborn premature infant and the adult with primary pulmonary hypertension. We have 2 experts this week - Dr. Nicole Sutton of Albert Einstein College of Medicine and Erika Berman Rosenzweig of Columbia University College of Physicians and Surgeons speak about managing pulmonary hypertension in infants with BPD and hi...