Pediheart: Pediatric Cardiology Today

This week we review the impact of conduit size on exercise capacity in the single ventricle Fontan patient. Is there an optimal sized conduit for the Fontan patient? What is the role of formal exercise stress testing in the evaluation and monitoring of the Fontan patient? We review these questions and more with Associate Professor of Medicine at UT Southwestern - Dr. Ari Cedars. doi: 10.1007/s00246-019-02190-4

This week's episode is co-branded with SADS.ORG and we review a paper on the clinical presentation of those who have conditions associated with sudden cardiac death. Are there questions about the patient and family history and physical examination signs that can improve the identification of those at risk before an event of cardiac arrest? We review this important question and others with Assistant Professor of Pediatrics at Washington University in St. Louis, Dr. Aarti Dalal. Referenced also in this discussion is the following webpage: https://www.sads.org/Library/School-Materials/Risk-Assessment#.XW25gy2ZOu4.DOI:10.1016/j.jpeds.2016.06.088

This week we discuss the results of a phase 2 trial of autologous stem cell therapy for the treatment of patients with HLHS. How challenging was it to obtain autologous stem cells from a large number of patients in multiple locations throughout North America? What are the theoretical benefits for this form of therapy? What evidence presently exists for its potential efficacy? How is this therapy best delivered to the myocardium. We discuss these and other questions with the senior author of a recent work on this topic, Professor Timothy Nelson of the Mayo Clinic. doi: 10.1016/j.jtcvs.2019.06.001

This week we enter the worlds of fellow education and echocardiography to review a recent report of a QI project performed at Children's Hospital of Philadelphia regarding the performance of echocardiograms by first year fellows in cardiology. Can the implementation of a checklist protocol improve the consistency of echocardiograms performed by fellows? What was the impact of this protocol? What areas were particularly well served by this approach? Dr. Brian White, senior cardiology fellow at CHOP provides important insights into this project that he co-authored. doi: 10.1111/echo.14441

This week we review a recent study from The University of Toronto on the impact of timing of the arterial switch operation for DTGA on brain growth and neurodevelopmental outcomes. Can the timing of this surgery impact these outcomes? What is the reason for this? Is it rational to perform this surgery early to improve neurological outcomes? How important is the conduct in the OR versus the ICU? These are amongst the questions we address with Professor Jane Newburger of Harvard University - Boston Children's Hospital this week! doi: 10.1161/CIRCULATIONAHA.118.037495

This week we review a recent paper on the topic of durable VAD's in congenital heart patients. Who is a candidate for such a device and what are the outcomes with these in this selected patient group? What are the impediments to their use? What role might public reporting have on the use of these devices and transplantation in general on the congenital heart population? We touch on all of these questions and more with Dr. Lauren Glass, Assistant Professor of Pediatrics at The Icahn School of Medicine at Mount Sinai - NYC where she is Associate Director of Heart Failure and Cardiac Transplantation. doi: 10.1097/MAT.0000000000001041

This week's episode is our second 'co-branded' episode with the SADS Foundation (SADS.ORG) and we speak with the founder and director of Crediblemeds.org, Professor of Medicine Ray Woosley of the University of Arizona. Dr. Woosley speaks with us about how he started the first website dedicated to assessing the evidence for drugs' impact on the QT interval establishing a reliable and trusted website for clinicians and patients to visit to learn about possible drug interactions and their impact on LQTS and the QT interval in general. How was the site first conceived? What are the latest changes on the site? What are the plans for this work going forward? Is grapefruit juice really dangerous? Why does the site request that all have to sign up to access the site? Professor Woosley provides all the answers and more this week!

This week we review a recent Swedish paper that assessed the risk for cancer in patients with congenital heart disease and compared this to normal controls in a large administrative database. Why are cancer rates different in the congenital heart patient? What are the most important factors? What actions should be taken by those cardiologists caring for ACHD patients to properly screen for cancer? We discuss all of the above and more with Assistant Professor of Pediatrics - Harvard University - Dr. Michelle Gurvitz. doi: 10.1001/jamanetworkopen.2019.6762

This week we explore a provocative paper on the use of a femoral vein homograft for Sano shunt for Stage I palliation for HLHS. Does the incorporation of this form of shunt improve outcomes? How does the valve in this graft function over time? Are there demonstrable improvements in RV function or PA growth when using this approach? We speak with Dr. TK Susheel Kumar who is Associate Professor of Surgery at NYU Medical Center. Dr. Kumar shares his thoughts and insights into this novel approach. https://doi.org/10.1016/j.jtcvs.2019.03.123

This week we have a replay of an episode from 9 months ago on T wave inversions in athletes and why their presence may represent possible disease in the athlete. We speak with Professor Sanjay Sharma of the UK who is a world authority on this topic. This is an important episode worth hearing once more. Coming this week in the middle of the week is our 75th episode with Professor Sylvia Griffiths! Keep a look out for Professor Griffith's episode coming later this week!

This week we review the topic of transposition of the great arteries and the most common late long term complication of the arterial switch operation - supravalvar pulmonary distortion or stenosis. How effective were interventions for this problem and what complications were encountered? Does balloon dilation alone ever work to help this problem? Are there risk factors for possible catheterization related complications when working on the pulmonary arteries? We discuss these and more questions with the first author of a recent report on this topic - Dr Jesse Lee who is Assistant Professor of Pediatrics at Baylor College of Medicine. Dr. Lee reviews this large series from his former group at Rady Children's Hospital. doi: 10.1111/chd.12726

This week we jump back into the world of ACHD and review a recent registry study assessing outcomes in adults with ASD and elevated PVR who underwent ASD closure. Who was a good candidate for this approach? What were the long term outcomes of this form of therapy? Did ASD closure in this setting confer a survival benefit? We review these and many other questions in a 'virtual roundtable' discussion with Drs. Elisa Bradley of Ohio State and Ali Zaidi of Mount Sinai - NYC. Both are world authorities on ACHD and it is a great treat to have such 2 experts both on the show to discuss the subtleties of this complex topic. doi: 10.1016/j.ijcard.2019.03.056

This week we enter the worlds of cardiovascular surgery and interventional catheterization to review a recent STS dataset analysis of postoperative transcatheter interventions. Who needed these sorts of catheterizations more commonly after surgery and what was the relation between needing such an intervention and overall outcomes? How much variability existed between centers in their use of interventional cath following congenital heart surgery? We speak with Associate Professor of Pediatrics, Kevin Hill of Duke University about his recent study on this subject. doi: 10.1161/CIRCINTERVENTIONS.119.007853

In this special episode # 71 co-presented with the SADS Foundation (SADS.ORG) we speak with the president of the foundation, Professor Michael Ackerman of The Mayo Clinic about a recent work he co-authored on a novel approach to assessing patients with possible CPVT. How should the phenotype help to inform genotype results? Can a novel CPVT scorecard help to improve our understanding of genetic test results? How are genes determined to be 'benign', 'likely disease causing' or a variant of uncertain significance (VUS)? We discuss these issues and more with Dr. Ackerman who provides great insights into this fascinating topic that affects all cardiologists who use genetic testing as part of their practice. doi: 10.1161/CIRCGEN.119.002510

This week we review the role that complications have on outcomes in the ACHD patient undergoing cardiac surgery. How commonly do complications occur and if they do occur, what impact do these have on ultimate outcomes? Are there strategies that can be employed to reduce complications amongst this fragile patient population? We discuss these issues and more with the senior author of this work, Dr. Oscar Benavidez who is the Director of Congenital Cardiology at Massachusetts General Hospital in Boston, MA. doi: 10.1007/s00246-019-02103-5.

This week we speak with Professor Yoav Dori of Children's Hospital of Philadelphia about a recent paper he co-authored on the topic of lymphatic abnormalities in children with Noonan's syndrome. What are the typical findings in severely affected children and who is a candidate for intervention? Are there patients who are good candidates for 'thoracic duct ligation' or pleurodesis? Is there anyone in whom this would be considered appropriate therapy? We discuss these questions and more with the master of the lymphatic system, Dr. Dori. doi: 10.1007/s00247-018-04337-6.

This week we explore a recent CMR study in Fontan patients assessing the impact of pulmonary blood flow maldistribution and exercise capacity. How closely associated are these differences in relative pulmonary blood flow to functional status and overall outcomes? How aggressively should we intervene for these sorts of problems? We discuss these issues and more with Dr. Rahul Rathod who is the senior author of this work and also Assistant Professor of Pediatrics, Harvard Medical School. doi: 10.1186/s12968-018-0505-4

This week we review a fascinating paper that assesses the direct myocardial effects of IV antiarrhythmic agents upon cardiac muscle function performed by the group at Boston Children's Hospital - Harvard University. We speak with one of the principle authors of this work, Dr. Elizabeth DeWitt, who is an attending electrophysiologist at that large institution about this important work. Which agents were substantial negative inotropes and which had no effects? The answers may be surprising. Dr. DeWitt shares the general approach of the team in Boston when treating arrhythmias that require rapid IV therapies. doi: 10.1177/1074248418810811

This week we review a critically important neurodevelopmental issue affecting infants who have single ventricle palliation - namely hearing loss. How common is hearing loss seen and what role does IV furosemide play in this finding amongst single ventricle patients? Are there techniques to avoid this complication and yet still use this commonly used diuretic agent? We discuss this important issue and study with Dr. Ari Joffe, Professor of Pediatrics University of Alberta - Stollery Children's Hospital who is the senior author of this week's work. doi: 10.1097/PCC.0000000000001807

This week it's National Pediatric Transplant Week and we review a recent work on ECMO following cardiac transplant and speak with its first author, Assistant Professor of Pediatrics in Vanderbilt University, Dr. Justin Godown. How does the use of ECMO in the postoperative period influence outcomes in the short and long term? Are there differences in outcomes when it is used for patients with cardiomyopathy versus congenital heart disease? We review all of these and more with Dr. Godown. We speak also with Melissa McQueen of Transplantfamilies.org about her organization which strives to educate and support the families of children undergoing transplantation. Ms. McQueen's story is certainly an inspiring one. doi: 10.1111/petr.1341