Pediheart: Pediatric Cardiology Today

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. DOI: 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. DOI: 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.DOI: 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.comFor those interested learn more about his '2 Fund' solution: https://www.paulmerriman.com/2-funds-for-life-update-2023For those interested to learn more about his '4 Fund' solution: https://www.paulmerriman.com/4-fund-comboFor those interested in target date funds: https://www.paulmerriman.com/target-date-fundsFinally, 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

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.comFor those interested learn more about his '2 Fund' solution: https://www.paulmerriman.com/2-funds-for-life-update-2023For those interested to learn more about his '4 Fund' solution: https://www.paulmerriman.com/4-fund-comboFor those interested in target date funds: https://www.paulmerriman.com/target-date-fundsFinally, 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

In this replay episode from 5 years ago in 2018, we enter into the world of cardiac morphology and speak with Professor Leo Lopez of Stanford University about a recent work he wrote with the International Society for Nomenclature of Pediatric and Congenital Heart Disease regarding proper nomenclature for VSD's. Is it defect geography or borders that are most important in naming these structures? How was agreement forged between such disparate super-hero experts in cardiovascular medicine and congenital heart disease? Is Dr. Lopez using these designations at the present time? What's next for the nomenclature group? Dr. Lopez shares his insights this week.As a special treat, Dr. Lopez also sings as the musical coda to today's program. A very special treat indeed and certainly worth replaying! Hoping that all enjoy! doi: 10.1016/j.athoracsur.2018.06.020

This week we return to the world of ACHD when we review a recent German research paper on the prevalence of PTSD symptoms amongst the ACHD patient group. How common is this type of symptom observed in the ACHD patient group? What contributes to the high prevalence (17-20.5%) described in this work? What are the benefits of 'embedding' a mental health care worker in the ACHD clinic/program? What strategies can be employed in pediatric cardiology to potentially mitigate PTSD symptoms in our future ACHD patients? These are amongst the many topics reviewed this week with the Mount Sinai ACHD program clinical social worker, Ms. Meredith Kalbacker who also comments on this week's work. DOI: 10.1016/j.amjcard.2023.06.087For those interested, the book referenced by Ms. Kalbacker, Healing Hearts and Minds, can be purchased on Amazon:https://www.amazon.com/Healing-Hearts-Minds-Holistic-Congenital/dp/0197657281

In this week's episode we delve into the world of cardiac catheterization and speak with Assistant Professor of Pediatrics at USC, Dr. Neil Patel about a recent work he co-authored at Children's LA about continuation of anti-coagulation during catheterization. Does AC have to be stopped to safely perform a catheterization? Are there certain cases or patients in whom the risk may be especially high? What about NOACs or DOACs? When should closure devices be considered? These are amongst the questions posed to Dr. Patel this week.DOI: 10.1007/s00246-023-03097-x

This week we review a recent work on postoperative ectopic atrial tachycardia (EAT) following congenital heart surgery in children. Are there risk factors for this arrhythmia and are any modifiable? What is the 'go to' therapy used by the electrophysiologists at Children's LA for the acute and chronic treatment of this arrhythmia in the postoperative period? Is the presence of EAT in a postoperative congenital heart patient a marker for a worse outcome? These are amongst the questions posed to Children's of Los Angeles pediatric electrophysiologist, Dr. Jonathan Uniat. DOI: 10.1007/s00246-022-03068-8