Pediheart: Pediatric Cardiology Today

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.

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 neurodevelopmental outcomes? Dr. William Gaynor of CHOP speaks with us about this very important and somewhat troubling work.

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 who might be a candidate for primary ICD prophylaxis in the young HCM population.

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 ACHD patients as they do in non ACHD adult patients? Dr. Alexander Opotowsky, Associate Professor of Pediatrics at Harvard Medical School shares his insights into this very unusual and novel concept in the world of ACHD.

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 disease. The ability to provide epicardial pacing or defibrillation without the need for a surgical incision is exciting and both experts speak of the challenges and future of this important work.

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 ablated successfully without even placing a catheter in the heart? This recent article from The New England Journal suggests that this may be an exciting paradigm shift in electrophysiology. We will be back with a new podcast next week!

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 regarding surgery or follow-up? Dr. Srivastava helps us understand this somewhat complex topic on the podcast this week!

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 possible benefits or risks? Dr. Zahn offers many answers and also gives us some insights into the near future of this rapidly developing approach to the transcatheter management of the large duct in a small child.

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 performing these very difficult and dangerous procedures. What happened to the carotid arteries after Dr. Petit and colleagues placed vascular sheaths in them when they performed repeat angiography 3+ months later? Dr. Petit shares his experience with us all this week!

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 in HLHS or DTGA? Drs. Donofrio and Slodki help explain.

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 in these ill infants? Dr. Rossi has 30 years of experience with this question and shares his insights and the evolution of his thoughts on this topic this week...

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 history of prematurity and also the exciting novel approach of using a Pott's shunt to palliate primary pulmonary hypertension in the adult. You will not want to miss these expert's insights into their field.

This week we delve into the topic of pregnancy in the single ventricle Fontan patient with 2 experts in this area - Dr. Melinda Davis of University of Michigan and Dr. Mark Johnson of Imperial College - London, England - to discuss who is a good Fontan candidate for pregnancy and what are the risks. We review the latest data on this area and also the many aspects of this topic that remain unknown at this time.

In this week's episode we learn about the latest data on pulmonary vein stenosis outcomes from a large meta-analysis of prior works on this topic and then review recent provocative data from the group in Boston on the use of adjunctive biological inhibition agents in concert with interventional approaches to combat multi vessel intraluminal pulmonary vein stenosis. The work's first author, Dr. Ryan Callahan of Boston Children's Hospital joins us to review this important trial's results and to review aspects of how his team assesses and manages these complex and heterogeneous patients.

In this episode we review 2 wonderful recent works. The first reviews the use of perioperative EP studies to predict postoperative VT and SCD amongst TOF patients undergoing PVR. The second is an intriguing study on the relatively new concept of HIS BUNDLE pacing. Joining us this week is Dr. Edward Walsh from Boston Children's Hospital to discuss his thoughts on ventricular arrhythmias in the setting of TOF and their prevention. His insights are worth a listen!

This week we learn about the exciting field of 3D printing in congenital heart disease. We discuss an interesting article on this topic that was recently published with one of the true world authorities on this topic, Dr. Kanwal Farooqi who is Assistant Professor of Pediatrics at Columbia University. Dr. Farooqi will take us through the basics of this rapidly evolving field and explain to us how the technology may one day lead to printing biological 'spare parts' like patches, conduits or even valves for the heart. You won't want to miss this peephole into an exciting future with Dr. Farooqi.

This week we delve into the exciting world of congenital interventional cardiology with 2 very different types of interventional cardiology research. In the first highly technical work from the group at Children's Hospital of Michigan in Detroit, we review an innovative approach to transcatheter management of pulmonary regurgitation in tetralogy when the RVOT is just too large for a valved stent. Dr. Tom Forbes, director of the cardiac catheterization laboratory and co-author of this work, discusses the challenges to and indications for bilateral branch pulmonary artery valve implantation in such cases. In the second work, the concept of practice variation and its impact on outcomes is explored by Dr. Andy Glatz and the team from Children's Hospital of Philadelphia reporting data from the IMPACT registry on compliance of interventionalists with established and published guidelines for proceeding with aortic or pulmonary valvuloplasty. Dr. Glatz will join us to discuss some of the interesting aspects of his wo