Pediheart: Pediatric Cardiology Today

This week we review a report from Italy regarding the observation of RA disk thrombus formation in some patients following GORE Cardioform ASD closure. How common is this complication seen and are there some patient or device characteristics that would be considered higher risk for this complication? How does this information square with the Assured trial data? Why might there be differences between the two data sources? We speak with Associate Professor of Pediatrics at Kravis Children's Hospital at Mount Sinai, Dr. Barry Love who is also Director of the Congenital Cardiac Catheterization Laboratory at Mount Sinai Hospital. https://doi.org/10.1002/ccd.31002

This week we delve into the world of antibiotic prophylaxis for dental procedures. What are the data to support its use in the congenital heart patient? Why has this been such a difficult topic to study? What do we now know about this topic and what still remains unanswered? Given the low prevalence but dire consequences of this disease, how can investigators consider studying this topic in the future? Is there adequate equipoise to proceed with a randomized controlled trial? We speak with Advanced Imaging Fellow at Boston Children's Hospital, Dr. Francesca Sperotto of Harvard University about her recent multicenter meta-analysis of 30 studies including over one million cases of endocarditis. 10.1001/jamacardio.2024.0873

This week we review a recent work on the use of sodium-glucose cotransporter 2 inhibitors in the ACHD heart failure patient from Europe. Can these agents impact heart failure outcomes in this patient group? What is the mechanism by which they may improve outcomes? Are there data on their effects on the single ventricle patient? What sorts of side effects can be seen and are these more or less commonly observed in the ACHD patient? We speak with the corresponding author of this week's work, Dr. Anastasia Egorova of Leiden University in the Netherlands. https://doi.org/10.1016/j.jacc.2024.02.017

This week we delve into the world of fetal cardiology when we review a recent large, multicenter study on the impact of prental diagnosis of 22.q11 deletion syndrome. How did the prenatally diagnosed patients differ from those diagnosed postnatally in regards to degree of heart disease and postnatal morbidity and mortality? What is the role of cell-free DNA testing in the screening for patients with this deletion syndrome? How do outcomes differ for this syndrome between the US and other countries and why might this be? These are amongst the many questions reviewed with the first author of this week's work, Dr. Lindsay Freud who is the director of fetal cardiology at Toronto Sick Kid's Hospital and also Associate Professor of Pediatrics at the University of Toronto. Also joining the podcast this week are the co-directors of this year's Mount Sinai Imaging Symposium entitled: Conversations in Care - The Single Ventricle. Dr. David Ezon, Dr. Jennifer Cohen, Dr. Kenan Stern and Mount Sinai Technical Director and Echo Lab Manager, Ms. Jen Lie Yau share their thoughts about the upcoming conference. This year's symposium will be taking place on May 4th, 2024 and the faculty are a who's who of important pediatric cardiac specialists from the worlds of imaging, interventional cardiology, sonography, surgery and more. For those interested, you can get more information about this wonderful conference at the following web address: https://mssm.cloud-cme.com/assets/MSSM/data/Single%20Ventricle%20Symposium24G.pdf Today's article link: 10.1016/j.ajog.2023.09.005

This week we delve into the world of cognitive bias when we review a recent review of this topic by the team at the University of Arkansas. What are some of the more important common cognitive biases that plague humans and are particularly deleterious for good decision making in 'case management/surgical' conference? What are some approaches to reducing the impact of these biases? How does the advent of ZOOM enhance or reduce bias for surgical conference decisions? We speak with noted authority on this topic, Dr. Joshua Daily who is Associate Professor of Pediatrics at The University of Arkansas in Little Rock for his deep insights into this topic. https://doi.org/10.1007/s00246-024-03462-4 Other resources mentioned by Dr. Daily include: - Thinking Fast and Slow by Daniel Kahneman - Thinking In Bets by Annie Duke - Predictably Irrational by Dan Ariely - How To Decide by Annie Duke

This week we review a recent survey study on the topic of junctional rhythm and sinus node dysfunction in the Fontan. How important is this finding in our Fontan patients? When should a patient with a Fontan undergo pacemaker insertion? Is the loss of AV synchrony the more important hemodynamic effect of junctional rhythm or is it chronotropic incompetence? Who should be considered for a possible endocardial, transvenous atrial pacemaker in the Fontan patient? These are amongst the questions posed to this week's guest, noted pediatric electrophysiologist and Professor of Pediatrics at OHSU, Dr. Seshadri Balaji. https://doi.org/10.1007/s00246-023-03296-6

This week we delve into the world of interventional cardiology and the Fontan when we review a recent report from the Children's Hospital of Philadelphia on the use of the "MitraClip" for the treatment of AV valve regurgitation in the congenital heart patient. Who would be a good candidate for this novel approach? What are the steps in planning or consideration of this technique in a congenital heart patient? How durable is this form of repair? These are amongst the many questions reviewed this week with this week's senior author, Professor of Pediatrics at the Children's Hospital of Philadelphia, Dr. Matthew J. Gillespie. 10.1002/ccd.30935

This week we review an episode from 2022 in the world of cardiac surgery and pediatric pharmacology when we review a work from Australia on levels of cefazolin during and after surgery. Is there an optimal dose regimen that will achieve proper MIC levels of drug? What should the goal of therapy be in regards to MIC levels? Are there novel means of dosing cefazolin? We speak with pediatric critical care pharmacy coordinator at Mount Sinai Kravis Children's Hospital, Dr. Jessica Frye for the pharmacist's perspective on some of these important questions. https://doi.org/10.1016/j.athoracsur.2022.02.047

This week we review a recent work in the artificial intelligence/deep learning space when we review a recent report from the team at Boston Children's Hospital on a novel model trained to predict LV characteristics in children from the ECG. How does such a program identify LV dilation, dysfunction or hypertrophy from a test (the ECG) not designed to provide most of this information? How well did the model perform when it was tested in a different center entirely? Why is it important to understand how a model of this sort is making its determinations? How might such a model be used clinically? These are amongst the many questions reviewed with the work's first author, Dr. Joshua Mayourian MD, PhD who is a first year cardiology fellow at Boston Children's Hospital. https://doi.org/10.1161/circulationaha.123.067750

This week we enter the world of the adult congenital heart patient when we discuss Fontan associated liver disease with an ACHD expert, Dr. Katia Bravo-Jaimes of the Mayo Clinic. In this episode we discuss the role of intrahepatic transcriptomics and how genetic analysis of liver biopsy tissue may provide new insights into the pathophysiology of this disease process. How can differential gene expression in the liver of Fontan patients help to explain why some patients have mild versus others severe fibrosis or even frank cirrhosis? Can these insights provide hope for improved surveillance or therapy for this problem? Dr. Bravo-Jaimes provides the answers this week. https://doi.org/10.1016/j.jacc.2023.12.005

This week we enter the world of the adult congenital heart patient when we discuss Fontan associated liver disease with an ACHD expert, Dr. Katia Bravo-Jaimes of the Mayo Clinic. In this episode we discuss the role of intrahepatic transcriptomics and how genetic analysis of liver biopsy tissue may provide new insights into the pathophysiology of this disease process. How can differential gene expression in the liver of Fontan patients help to explain why some patients have mild versus others severe fibrosis or even frank cirrhosis? Can these insights provide hope for improved surveillance or therapy for this problem? Dr. Bravo-Jaimes provides the answers this week. https://doi.org/10.1016/j.jacc.2023.12.005

This week we review a recent report from the Boston fetal intervention group about fetal aortic valvuloplasty. What fetal/maternal factors are associated with improved outcomes or technical success of this procedure? What factors are associated with serious adverse events? Who is the 'optimal' candidate for this type of intervention? Why is larger LV size associated with improved technical success? What other types of fetal interventions are being attempted by the Boston team? Drs. Ryan Callahan and Wayne Tworetzky of The Children's Hospital of Philadelphia and Boston Children's Hospital, respectively, share their deep knowledge of fetal intervention this week. https://doi.org/10.1016/j.jacadv.2024.100835

This week we review a recent report on the development of valvular obstruction due to intimal tissue ingrowth in the Harmony valve. How common is this problem? What factors may predispose a Harmony valve patient to develop this rare complication? Does the location of the valve implant matter in regards to this complication? How should Harmony valves be surveilled? There are amongst the questions posed to this week’s guest, Assistant Professor of Medicine at U. Washington, Dr. Zachary Steinberg. doi: 10.1002/ccd.30901.Epub 2023 Nov 5.

This week we go back in time to 2019 to re-review an important episode in the world of ACHD and review a 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, presently of Penn 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 delve into the world of cardiovascular surgery when we review a recent review of STS data on the pulmonary artery band (PAB). The STS assigns a STAT category of 4 to this operation, denoting higher risk for mortality. Is this warranted? Are all PAB candidates equal? What features are associated with higher or lower mortality rates in patients undergoing banding? Should the data in this work drive innovation to avoid the PAB in some settings? These are amongst the questions posed to the senior author of this week's work, cardiovascular surgeon Dr. Tara Karamlou who is Professor of Surgery at the Cleveland Clinic in Cleveland, Ohio. DOI: 10.1016/j.athoracsur.2023.09.020

What is the incidence of pediatric out of hosital cardiac arrest and how does it vary depending upon age? What are the most common causes of sudden cardiac arrest in children and what factors can be potentially affected by intevention? What sorts of intervention might reduce rates of sudden cardiac arrest amongst children? Dr. Elizabeth Paratz of St. Vincent's Hospital in Melbourne, Australia, who is an expert in sudden cardiac death, will share her insights into her work and the topic this week. 10.1016/j.hrthm.2023.06.010

In this week's episode we review a recent Dutch study on 50 year follow-up of patients who had ASD repaired at an average age of 7.5 years. How do these patients compare to their middle aged counterparts who did not have congenital heart surgery? Why might the RA or RV be enlarged in these patients, decades after successful ASD closure? How common are arrhythmias seen in this cohort? How common is pulmonary hypertension seen? These are amongst the questions reviewed this week with the work's first author, Dr. Robert Martijn Kauling. 10.1016/j.ijcard.2023.131616

This week we review a recent report from the team at Boston Children's Hospital on outcomes of patients following Stage I palliation for HLHS. When patients are not doing well following this surgery, how important is it to identify surgical problems early in these patients? What are the most common problems encountered when a problem is observed? When a reintervention is performed in this setting, how do patients fare overall and is the timing of the reintervention a factor in overall outcome? Cardiac surgeon and Assistant Professor of Pediatrics at Harvard University, Dr. Meena Nathan shares her insights this week. doi: 10.1016/j.jtcvs.2022.04.033

Unplanned extubation in the PCICU patient is a serious concern with many untoward associations. Who is at highest risk for this complication? Why is this complication associated with worse outcomes in general? How does race or ethnicity impact the likelihood of this complication? We speak with Assistant Professor of Pediatrics at the University of Cincinnati and pediatric cardiac critical care expert, Dr. Tanya Perry about a recent PC4 registry study on unplanned extubation in the PCICU. doi: https://doi.org/10.1097/pcc.0000000000003235

To conclude 2023 we end with noted national authority on personal finance and investing, Mr. Paul Merriman. Mr. Merriman reviews some basic ideas about asset allocation within a retirement plan such as a 403b, 401k or IRA with the goal of achieving the highest degree of return for level of risk. He reviews the importance of diversification and also shares with us both a '2 fund' and '4 fund' approach to creating a low cost, efficient and diversified portfolio that has, historically, been associated with good long term returns. Mr. Merriman's work can be seen at: paulmerriman.com For those interested learn more about his '2 Fund' solution: https://www.paulmerriman.com/2-funds-for-life-update-2023 For those interested to learn more about his '4 Fund' solution: https://www.paulmerriman.com/4-fund-combo For those interested in target date funds: https://www.paulmerriman.com/target-date-funds Finally, for those who did not previously take advantage of Paul's free book, the following is a link to a free PDF of the book: https://irp.cdn-website.com/6b78c197/files/uploaded/Were-Talking-Millions.pdf Please note: This episode is not intended to provide specific personal finance advice and is for entertainment purposes only. Please consider speaking with a trusted advisor before making any significant changes to your retirement assets.