Pediheart: Pediatric Cardiology Today

This week we review the results of the latest update of the Single Ventricle Reconstruction trial, specifically SVR III. How are HLHS patients are faring post Norwood at age 12? What is the overall transplant-free survival rate in this group? Are there differences in survival seen at 12 years between BTTT shunt patients and RV-PA conduit shunt patients? How do they compare in exercise capabilities? What about rates of PLE or arrhythmias seen? Are there interventions that may improve outcomes going forward? These are amongst the questions reviewed this week with the first author, Dr. Caren Goldberg who is Professor of Pediatrics at the University of Michigan. DOI: https://doi.org/10.1161/circulationaha.123.065192

This week's special episode of Pediheart is a conversation with NYT author James G. Robinson. Mr. Robinson recently released his memoir More Than We Expected: Five Years With A Remarkable Child (Post Hill Press) which reviews the life and times of the Robinson family and their son Nadav who was born with complex single ventricle disease in the setting of heterotaxy syndrome. Nadav lost his life at age 5 but was a blessing to his family and those who knew him. What were some of the characteristics of the better medical communicators that the Robinson's observed? How can parents better advocate for their children during times of critical illness? How can we all bring the 'simple power of human kindness' to our care of children with congenital heart disease? Why is the opinion of the parent so important in delivering optimal care to a child with serious disease? These are amongst the many lessons we take away from Mr. Robinson's book and our conversation this week. More Than We Expected: Five Years With A Remark

This week we delve into the worlds of cardiac critical care and congenital heart surgery when we review a recent report of a randomized controlled trial assessing the impact of nasal intubation on rates of NG or G tube feeds at discharge following neonatal congenital heart surgery. Do nasally intubated patients eat by mouth faster and have shorter lengths of stay? What would be the theoretical reasons that this might be useful? What is an 'ICU Liberation bundle' and how did its application to all postop newborns improve outcomes? Can the route of tracheal intubation truly be viewed as a possible 'modifiable risk factor'? These are amongst the questions posed to cardiology fellow at the Cleveland Clinic, Dr. Melissa Yildirim who conducted this study at the University of Virginia during pediatric critical care fellowship. doi: 10.1007/s00246-023-03322-7

This week's episode is co-branded with SADS.ORG and is a review of a recent multicenter PACES study on the general outcomes of patients with congenital heart disease who have undergone MRI's. Just how worried should the practitioner be to send a pacemaker or ICD patient for an MRI of any body part? Is there a period of time after implantation when it is safer to proceed with an MRI? What is meant by an MRI conditional device or lead? Does the part of the body imaged matter in regards to risk to CIED's? These are just some of the questions posed to the senior author of this week's work, Dr. Thomas Pilcher of Primary Children's Hospital in Salt Lake City, Utah. doi: 10.1016/j.hrthm.2023.08.034For those interested to learn more about the SADS Foundation, please visit their website:SADS.ORG

In this week's episode we sit down with Professor Jane Newburger of Harvard University to learn a little bit about her life and times. Where did she grow up and who was her inspiration for becoming a doctor and cardiologist? Who were some of the mentors in her professional life? What was it like to work with Dr. Ami Rosenthal or Dr. Alexander Nadas? After such a rich and impactful career and life, what does Dr. Newburger count as her most satisfying and important accomplishments? These are amongst the many questions posed to Dr. Newburger during a live interview from the New England Congenital Cardiology Association (NECCA) in Rockport, Maine that took place on 10.21.23. Also joining the podcast to discuss the organization and its goals and accomplishments is its immediate past president, pediatric cardiac electrophysiologist, Dr. Michael Epstein. For those interested to learn more about NECCA, please go to their website: https://necca.wildapricot.org

In this replay episode of 2021, week we review a work on the use of bivalirudin on outcomes of pediatric VAD patients. We speak with Dr. Caroline Carly Heyrend of Primary Children's Hospital in Utah about a recent work she co-authored on time in therapeutic range for this agent in children with VAD's. How does bivalirudin work differently than heparin and what are the theoretical benefits or disadvantages of this agent in comparison with unfractionated heparin? Why are thrombotic complications still common amongst those with pediatric VAD's even when anticoagulation is maintained appropriately? What are the factors at work that make safe anticoagulation so difficult in this complex and fragile group of patients? Dr. Heyrend provides the pharmacist's perspective this week.DOI: 10.1097/MAT.0000000000001261

This week we delve into cardiovascular surgery when we review a recent report from the Congenital Surgeons' Society on the outcomes of the hybrid approach of pulmonary artery bands for treatment of infants with critical left heart obstructive disease. What risk factors are associated with worse outcomes? Does this approach confer an advantage in comparison to the traditional 'stage I/Norwood' approach to this problem? Does the presence of multiple risk factors worsen outcomes and if so, to what degree? How does the heterogeneity of indications for this approach to obstructive left heart lesions stymie efforts to study this intervention? DOI: 10.1016/j.jacc.2023.07.020Editorial Comment :DOI: 10.1016/j.jacc.2023.08.023

This week we discuss the role of the stress echo in the evaluation and follow-up of the patient with Kawasaki disease. What are the present recommendations on the role of this form of imaging in this patient group? What are its benefits and limitations? Can this form of testing provide actionable data beyond imaging such as CT angiography? What is the possible role of CMR in the management and follow-up of the KD patient? Dr. Kirstin Dummer of Rady Children's Hospital shares her deep insights into this endlessly fascinating topic. DOI: 10.1007/s00246-022-03037-1The AHA Scientific Statement On KD:DOI: 10.1161/CIR.0000000000000484

This week we delve into the world of fetal cardiology when review a recent report from Nationwide Children's Hospital on the utility of fetal echocardiography in the setting of a negative obstetrical ultrasound and Down syndrome. Down syndrome is associated with congenital heart disease but can the obstetrical ultrasound effectively rule out 'critical' newborn heart disease, obviating the need for a fetal echo, particularly if a postnatal transthoracic scan is planned? Associate Professor of Pediatrics, Dr. Clifford Cua shares his thoughts on this provocative question and work. doi: 10.1007/s00246-023-03183-0.Epub 2023 Jun 23.

This week we discuss an important 'general cardiology' question - namely, do all exertional chest pain patients need an echocardiogram. In this debate from the World Congress of Pediatric Cardiology and Cardiac Surgery, we hear 2 noted authorities in non-invasive imaging. First up is Dr. Carolyn Altman of Texas Children's Hospital taking the stance that it is indicated in the majority and following Dr. Altman is Dr. Kenan Stern of Mount Sinai Kravis Children's Hospital defending the role of discretion in these decisions and how 'one size may not fit all'. Both authorities provide deep insights into general cardiology and the nuances of evaluating a common cardiology complaint seen in general practice.

This week we delve into the world of interventional cardiology and specifically that of transcatheter valves when we review a recent report on the Harmony valve. How are these valves performing at one year in regards to valvular function? Is frame fracture an issue? What percentage required reintervention? How common was ventricular tachycardia seen? Should patients undergoing these implantations undergo pre-procedural EP studies? We review these questions and more with the first author of this work, noted authority on interventional cardiology, Dr. Matthew J. Gillespie, Professor of Pediatrics at the Perelman School of Medicine at the University of Pennsylvania. https://doi.org/10.1016/j.jcin.2023.03.002

This week we will hear 2 lectures from the World Congress on ablation in children from two world authorities. In the first, Professor Jeff Kim of Texas Children's Hospital speaks about unusual accessory pathway connections and offers some insights into how to ablate them. Following Dr. Kim, Professor John Papagiannis, chief of cardiology at the Onassis Center in Greece offers us some insights into why some AVNRT is challenging to ablate and how to approach these rare but difficult cases. Why is ablation not 100% effective in children for 'simple' things like accessory pathways or AVNRT? Drs. Kim and Papagiannis provide a few insights into why there is no such thing as 'just AVNRT' or 'just a pathway'.

This week we go back in time 2 years ago and we spend the entire episode speaking with Professor Peter Lang of Harvard University about his rich and long career in pediatric cardiology during one of the most exciting times of the field. Dr. Lang's story includes a virtual who's who of important cardiologists and cardiac surgeons for the past 50 years including Dr. Connie Hayes, Dr. Roberta Williams, Dr. Alexander Nadas, Dr. Richard Van Praagh, Dr. Aldo Castaneda, Dr. Bill Norwood and many, many more. This is a rare opportunity to hear about the development of the Norwood procedure from someone who was at the bedside of the first 30-50 ever performed as well as someone who helped develop the entire field of cardiac critical care. Prepare to be inspired by this towering figure this week.

This week we review a recent large scale assessment of survival amongst patients with CHD who were born between 1980 and 1997 in the US. What percentage of those born with CHD actually survived to age 35? What were the factors associated with improved or worse outcomes? How did those with HLHS fair in this time period? What was the impact of genetic syndromes or non-cardiac abnormalities on outcomes of children with congenital heart disease? We speak with this week's work's first author, CDC epidemiologist, Ms. Karri F. Downing. https://doi.org/10.1161/circulationaha.123.064400

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