Pediheart: Pediatric Cardiology Today

This week we review a recent work from the UK on the causes of death in adolescents who die suddenly. The novelty of this week's work is that all the individuals in this very large study had specialized cardiac pathologists perform careful assessments of the hearts of these subjects. What is the most common cardiac cause of SCD in the adolescent and how do the findings of this work compare with prior works on this topic? What sort of screening should be considered when encountering a patient with SCD? Why would LVH (without HCM) possibly be a cause of SCD in the young? Should all SCD victims have evaluation by a cardiac pathologist? What is the role of molecular autopsy? These are amongst the many topics reviewed with the senior author of this week's work, noted Professor of Pathology, Dr. Mary Sheppard of St. George's, University of London, London UK. https://doi.org/10.1016/j.jacc.2023.01.041

This week we travel back in time 2 years to re-review a work from the team at Boston Children's Hospital on the long term outcomes of truncal valves in the setting of truncus arteriosus. Who amongst truncus patients is at risk for the development of truncal regurgitation or a need for reintervention? Are there predictors that might help forecast patients who should be more closely monitored? How do the data in this work help inform the fetal counseling of the team at Boston Children's Hospital? Then instructor in pediatrics, Dr. Laura Gellis, the first author of this week's work, shares her deep insights into this topic this week!doi: 10.1161/JAHA.120.019104

This week we review a recent fascinating work from the team in Toronto on a novel large mammalian model of HLHS (in fetal lambs). How did the team develop this model and what insights into the development of HLHS has it afforded? How does reduction in inflow to the developing LV result in changes in cellular proliferation in the LV of the fetal lamb? Which is more important in the development of HLHS - genetics or flow patterns? Is this a false dichotomy? Does this novel model provide insights into outcomes thus far observed in fetal interventions for HLHS and aortic stenosis in humans? We speak with the senior author of this week's work, Professor Rajiv Chaturvedi of the University of Toronto about this potentially groundbreaking new model and some of the insights it has provided. https://doi.org/10.1038/s42003-023-05132-2

This week's episode is a replay from 2019 of a '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 delve into the world of ACHD when we review a recent important work from the multicenter INDICATOR study group assessing the impact on overall outcomes in TOF patients who have their pulmonary valve replaced. Are survival rates improved by replacement of the pulmonary valve in the TOF patient? What about the effect on ventricular tachycardia? What are the proposed mechanisms by which PVR can positively affect this critical outcomes? Are there differences between transcatheter PVR and surgical in this regard? Is there an RV volume that is the 'sweet spot' for PVR? These are amongst the many questions posed to Professor of Pediatrics and Medicine at Harvard University, Dr. Anne Marie Valente. https://doi.org/10.1016/j.jacc.2023.02.052

This week we delve into the world of cardiovascular surgery when we check in with the group from Nationwide Children's Hospital and review a novel approach to managing the dreaded LPA stenosis or hypoplasia in the HLHS patient. Why does the LPA seem to be small or stenosed in this condition and how does a preemptive stent address this? Why can't standard PA plasty be used for this particular indication as is used in TOF/MAPCA's? Does one stent size fit all HLHS patients? These are amongst the many questions posed to the first author of this week's work, cardiovascular surgeon Dr. Sergio Carillo. https://doi.org/10.1016/j.xjon.2022.11.007

This week we delve into the world of electrophysiology and also single ventricle physiology when we review a recent work from the Stanford group on outcomes after the development of ventricular arrhythmias in the single ventricle Fontan patient. Just how worried should the clinician be when these are recorded on ambulatory ECG monitors? What associations exist between these arrhythmias and the use of a Sano shunt? How should the clinician respond to a finding of non-sustained VT in the single ventricle patient? Who is a candidate for an ICD in this setting? These are amongst the questions posed to the first author of this week's work, Clinical Assistant Professor of Pediatrics and electrophysiologist at Stanford University, Dr. Heather Giacone. doi: 10.1161/CIRCEP.122.011143

In this week's episode, we delve into the world of pediatric heart transplantation and the impact that race or insurance status may have on outcomes. What are the factors that explain worse outcomes for black children waiting for a heart transplant? How is the PHTS Racial Disparity Taskforce working to reduce inequities in this field? What role does insurance status have on these outcomes? We speak with noted heart failure and transplantation expert, Dr. Neha Bansal who is Assistant Professor of Pediatrics at The Icahn School of Medicine at Mount Sinai about this most recent PHTS multicenter study. DOI: 10.1016/j.healun.2022.12.002

In this week's first of 2 Pediheart podcast releases, we review the nursing and critical care tracks of the upcoming 8th World Congress of Pediatric Cardiology and Cardiac Surgery coming in late August, 2023. In this collaborative podcast between the Pediheart and PCICS podcasts, hosts Robert Pass, Sadie Rodriguez and Deanna Tzanetos speak with Dr. Amir Ashrafi of CHOC, Bruna Cury of Sao Jose do Rio Preto in Brazil and Dr. Sandra Staveski of UCSF about the upcoming meeting. Dr. Ashrafi, Ms. Cury and Dr. Staveski are on the planning committees and share some of the most exciting aspects of their various tracks in the upcoming meeting.For more information about the World Congress, please go to the website:https://www.wcpccs2023.org/event/1da8563e-0f65-486c-88df-70c3db431af5/summaryAnd don't forget to take a listen to Episode #257 of Pediheart also released today on the topic of fetal cardiology!

In this week's episode we speak with Assistant Professor of Pediatrics at Mount Sinai, Dr. Jennifer Cohen about a recent large scale Fetal Heart Society project she conducted on the topic of fetal ccTGA and outcomes. What observations in fetal life are associated with worse clinical outcomes? Why is it valuable to perform serial fetal echocardiograms in this patient group? How common are genetic or extracardiac abnormalities in this patient group? These are amongst the many questions reviewed with Dr. Cohen in this week's extra special 2 episode release week. DOI: 10.1161/JAHA.122.029706

This week's episode is a collaborative episode between the Pediheart and PICS Podcasts and we have 4 guests. Joining us this week are Dr. Carissa Baker-Smith of Nemours Medical Center in Delaware, Dr. Martina Bruekner of Yale University, Dr. Shelby Kutty of Johns Hopkins University and Dr. Jacqueline Sanz of Children's National Hospital. In this week's episode we learn about the general cardiology, imaging, neurodevelopmental and basic science tracks. Each guest representing the planning committee for their respective tracks will share some of the upcoming highlights of this wonderful and comprehensive conference. The list of topics of interest is positively dizzying and you will not want to miss this exciting introduction to some of the more popular tracks coming in late August, 2023!The World Congress of Cardiology and Cardiac Surgery will take place 8/27-9/1 and information can be obtained about it at this website:https://www.wcpccs2023.org/event/1da8563e-0f65-486c-88df-70c3db431af5/summary

This week we re-review a work from the large ACHD team in Sydney, Australia regarding outcomes of ACHD patients at age 50. How are these patients progressing from both a medical and psychosocial perspective? What resources are needed to address this rapidly growing population of middle aged ACHD patients? We speak with Professor Rachael Cordina of the University of Sydney about her work on this topic.doi: 10.1016/j.hlc.2020.05.114

In this week's episode from SCAI 2023, we speak with Professor Kritvikrom Durongpisitikul of Mahidol University, Siriraj Hospital in Bangkok, Thailand about their experience in transcatheter and surgical pulmonary valve replacement (PVR). Who is a candidate for each in the present era? Why was surgery associated with higher mortality rates in this work? Should the criteria for transcatheter PVR differ in comparison with surgery? Dr. Durongpisitikul shares his insights in this live session from SCAI which is co-moderated by Dr. Aimee Armstrong of Children's Nationwide Hospital. https://doi.org/10.1016/j.jscai.2022.100408

In this week's episode from SCAI 2023, Dr. Aimee Armstrong of Nationwide Children's Hospital joins Dr. Pass in a live session from the annual scientific sessions of SCAI. Professor of Pediatrics at UC Davis, Dr. Frank Ing, reviews his experience with recanalization of central veins in a large pediatric cohort. Who is a candidate for this intervention? When are stents preferred vs. angioplasty alone? Should all patients having central lines removed be evaluated to prevent venous occlusion? What sort of radiation dose is associated with this intervention? Dr. Ing shares his deep knowledge in a live "Pediheart" session from Phoenix, Arizona on 5/19/23 at SCAI 2023. https://doi.org/10.1016/j.jscai.2022.100547

In this week’s episode we delve into the evidence for digoxin reducing mortality in the interstage period following Norwood palliation for HLHS. We speak with Assistant Professor of Pediatrics at the University of Pennsylvania, Dr. Michael O’Byrne about a recent analysis of the PHIS database using the relatively novel “preference based instrumental variable” approach to analysis of an observational data set. How does this approach allow for possible proof of causation of effect of a variable? Why might digoxin reduce mortality in the HLHS patient following stage I palliation? Are there patients in which the impact of this agent might be greater than others? These are amongst the many questions reviewed this week with Dr. O'Byrne.https://doi.org/10.1016/j.ahj.2023.05.005

In this week's episode we review the important topic of troponin levels in children. What is an abnormal high sensitivity troponin level in children and do the levels vary based upon the assay? What are the differences between high sensitivity troponin I and T levels? Are there differences between boys and girls? Why would using the 97.5%ile upper reference limit result in a more reliable 'line in the sand' in comparison with the more traditional, high sensitivity troponin level cut off of 99th%ile. These are amongst the questions we review with this week's author, Dr. J. Bill McEvoy, Professor of Preventive Cardiology at University of Galway, Ireland. DOI: 10.1161/CIRCULATIONAHA.122.063281

This week we delve into a critically important topic which is the mental health of our patients with chronic disease. How common are mental health issues amongst patients with sickle cell disease, cystic fibrosis and congenital heart disease? What accounts for differences in diagnosis or treatment of things like anxiety, depression or ADHD in these different chronic disease state patients? What is structural racism and how does this impact these findings? What is the solution to the under-recognition of mental health disorders in these chronic medcial patients? We speak with ACHD fellow at Texas Children's Hospital and the first author of this week's work, Dr. Vincent J. Gonzalez. DOI: 10.1016/j.jpeds.2023.113411Thanks to all who have been listening to the podcast the past 5 years on today's landmark, 250th episode.

This week we delve into the world of adult congenital heart disease when we review a recent work from the ACHD team at UCSF that assesses the impact of BMI on clinical outcomes in the single ventricle adult Fontan patient. What is the cause of elevation in BMI in some Fontan patients? Is obesity the only explanation? Is BMI a modifiable risk factor for our Fontan patients and should exercise be 'prescribed' for these patients? If so prescribed, what type of exercise is best for the Fontan patient? This week's work's senior author, Dr. Anushree Agarwal, Assistant Professor of Medicine at UCSF, shares her insights into this important topic. DOI: 10.1161/JAHA.122.026732Also mentioned in today's episode is episode #222 with Dr. Dan Halpern of NYU medical center (https://www.stitcher.com/show/pediheartpediatric-cardiology-today/episode/pediheart-podcast-222-impact-of-cardiac-rehab-on-exercise-tolerance-in-the-achd-patient-206781483)

This week in a replay of a previous co-branded episode with the SADS Foundation (SADS.ORG) from 2.5 years ago, we speak with Professor Peter Schwartz of University of Pavia about a recent work he authored on the topic of changes in repolarization amongst well trained athletes. Can athletic training masquerade as LQTS causing prolongation and/or abnormalities in the QT interval? How can these patients be properly identified and not 'lumped' into the LQTS 'pile'? This is a very important work by one of the foremost authorities on the planet on LQTS and Professor Schwartz also shares with us how he became interested in this topic 50+ years ago as well as some pearls for clinical investigators at the start of their careers. One of the best and most inspirational interviews of the entire nearly 250 episode series! doi: 10.1161/CIRCULATIONAHA.120.048916

In this episode we speak with Professor of Surgery at the University of Pennsylvania, Dr. Stephanie Fuller about a recent work she co-authored on the impressions of CT surgical trainees and attitudes towards the field of congenital heart surgery. What factors were important amongst those surgeons who chose to pursue the field? What are the obstacles that may make it less appealing to the CT surgical trainee? How can surgeons be effectively trained in this demanding field in an era of public reporting and USNWR rankings? What sort of innovation is happening today to improve the training of congenital heart surgeons? Dr. Fuller shares her deep insights this week. doi: 10.1053/j.semtcvs.2022.03.004