Pediheart: Pediatric Cardiology Today

This week we delve into global pediatric cardiology and review a massive and important work from the Global Burden of Disease study group assessing the trends in congenital heart disease in the world from lower income nations to higher. How has the prevalence of congenital heart disease changed over the past 30 years? How are countries faring in their treatment of congenital heart disease? What are some strategies to address global inequities in care? We discuss these and other questions with the corresponding author of this work, Professor Craig Sable of George Washington University. doi: 10.1016/S2352-4642(19)30402-X

This week we speak with Professor of Surgery, Mark Rodefeld of Indiana University about the most recent report of his group on a sub pulmonary pump for Fontan patients. What is different about this pump versus other presently available pumps for other cardiovascular indications? What is known about the thrombogenicity of these devices? How close is the team in Indiana to animal studies? Why is a lower pressure type pump preferable for this indication? Dr. Rodefeld provides most of the answers this week. doi: 10.1016/j.jtcvs.2019.06.112.

We review a recent paper on the use of the AED for children who suffer out of hospital arrest. What factors are associated with their use and what barriers exist to their use for children? What should be the next steps to improve AED usage for children? We discuss these and other questions with Professor Peter Aziz of the Cleveland Clinic in our first co-branded episodes with the SADs Foundation in 2020! doi: 10.1542/peds.2017-1903

This week we review a recent large single center study comparing pulmonary homografts to bioprosthetic valves in the TOF patient requiring valve replacement. Is there a superior choice? Who is a better candidate for one versus the other? Which is technically easier to use and why? Who would benefit from an aortic vs. pulmonary homograft in this pulmonary position? We speak with Professor Massimo Caputo of the Bristol Heart Institute who answers these and other questions related to this important surgical paper this week. doi: 10.1161/JAHA.119.013654

Alin Gragossian, DO has an unusual perspective in medicine - she is an emergency room physician but also a cardiac transplant recipient. In this milestone 100th episode Dr. Gragossian shares her experiences of learning that she had dilated cardiomyopathy just one year ago, getting admitted to the ICU, and undergoing a heart transplant - all in < 6 months. She is 'back on the job' and she shares the details of her trials and tribulations, inspiring us all with her courage and insights into the patient experience, from the doctor's viewpoint. A different kind of inspirational, portrait in courage episode for our 100th episode! Happy New Year to all!

This week we review a recent report on the benefit of 3D imaging and printing in preparing for ductal stenting. This intervention is complex and is fraught with risk. The team at Duke review their experience with the use of 3D modeling prior to intervention and what impact this has on outcomes. We speak with the first author, Dr. Reid Chamberlain, research fellow at Duke University Pediatric Cardiology about this recent publication. doi: 10.1002/ccd.28669

This week we review the results of one of the rare placebo controlled double blind trials of an agent in pediatric cardiovascular medicine - specifically, the Fontan Undenafil trial assessing the impact of this agent on parameters of exercise in the single ventricle patient. What measures of exercise were most affected by this agent and why? What side effects were encountered? Who should receive these agents? These are amongst the many questions we tackle with the first author, Dr. David Goldberg of The Children's Hospital of Philadelphia. doi: 10.1161/CIRCULATIONAHA.119.044352

Cardiogenetics is the topic this week as we review a relatively recent report on the topic of LV non-compaction. How often do patients with this finding have positive genetic findings? Who deserves genetic testing with this finding? Should all such patients be seen by a geneticist? How frequently should phenotype positive, genotype negative patients 'check in' with a geneticist? These are amongst the questions we review with famed cardiogeneticist and senior author, Professor Stephanie Ware of Indiana University. doi: 10.1161/CIRCGENETICS.117.001735

This week we review a recent report of the PC4 group assessing the impact of inter hospital open communication and collaboration on surgical outcomes. In what manner did participation in this collaborative CICU registry improve outcomes for different hospital surgical outcomes? Why was the improvement not homogeneous between centers? How long did it take for the impact of collaboration to positively improve outcomes? We discuss these and other questions with first author Dr. Michael Gaies, Associate Professor of Pediatrics - University of Michigan. doi: 10.1016/j.jacc.2019.09.04

This week the topic is cardiovascular surgery and we speak with Dr. Ignacio Berra of Buenos Aires Argentina about a recent work he co-authored with the surgical bioengineering team at Boston Children's Hospital regarding a newly developed tool to assess the aortic valve. Can this device safely allow for visualization of the valve under the usual aortic diastolic pressures to aid in anatomical assessment and post-repair assessment? What are the technical hurdles of using this device? Is it ready for use in humans? We discuss these and other questions with Dr. Berra this week. doi: 10.1016/j.jtcvs.2018.07.113

This week we review a work that aimed to improve the thoughtfulness of echocardiography usage in the postoperative period after congenital heart surgery. Dr. Kelly Cox and colleagues report on the use of a 'checklist' type 'pause' aimed at making certain that the ordering team in the CVICU are making an informed decision regarding the need for a new echocardiogram. How often should postoperative studies be performed? How often did the 'fluid check' actually show fluid? These are amongst the many questions we post to Professor Cox who presently works at Lurie Children's Hospital - Northwestern University. doi: 10.1111/echo.1450

ECMO use in the pediatric cardiac patient can be lifesaving but the long term effects are still not well known. This week we explore the neurodevelopmental outcomes in a cohort of patients from Boston Children's Hospital who underwent ECMO support following cardiac surgery and explore factors associated with their neurodevelopmental outcomes. How do these patients fare in comparison to non-ECMO pediatric cardiac surgical patients? Are there modifiable factors that may improve outcomes? We explore these issues with Dr. Ravi Thiagarajan, Professor of Pediatrics - Harvard University and Director of Cardiac Critical Care at Boston Children's Hospital this week. doi: 10.1007/s00246-019-02115-1

This week we speak with cardiovascular surgeon Dr. Johann Brink about a recent work he co-authored from the Royal Children's Hospital in Melbourne, Australia about the team's experience with shunt malfunctions. Are there factors that are associated with shunt failure in this sometimes fragile patient group? Survival to Fontan in most series is roughly 70% - what, if anything, can 'move the needle' to higher levels of survival in this patient population? Dr. Brink offers some interesting and important insights this week. doi: 10.1016/j.jtcvs.2019.03.126.

This week's episode is co-branded with the SADS Foundation and we review a recent work by the team at U. of Washington on a comparison between the ECG and the AHA 14 point evaluation recommendation for the identification of cardiac diseases that may predispose to sudden death amongst high school athletes. We speak with Dr. Jonathan Drezner of U. of Washington about this important work and he offers many insights into the pluses and minuses of both the history and physical approach as well as the ECG. Referenced in the podcast also is https://uwsportscardiology.org/e-academy/. doi: 10.1161/JAHA.119.012235

This week we review a recent report from the CTOTC-04 study group assessing the impact of novel anti-rejection approaches which generally markedly reduce or remove steroids in the prevention of rejection. How are rejection rates affected by such an approach? Did incidences of diabetes or PTLD differ when using this form of steroid reduced approach in the 'low immunological risk' subset of patients undergoing heart transplantation? We speak with the first author of this work, Professor of Pediatrics, Dr. Jacqueline Lamour of The Albert Einstein College of Medicine. Dr. Lamour provides deep insights this week! doi: 10.1016/j.healun.2019.06.006

Identification of the tetralogy of Fallot patient at risk for ventricular arrhythmias might be the 'holy grail' of ACHD and still today the best predictors are unclear. This week we review a novel approach to this problem - the development of personalized computational models to effectively perform 'virtual EP studies' and predict reentrant ventricular arrhythmia risk in repaired TOF patients. We speak with Dr. Mark Cartoski, Assistant Professor of Pediatrics at Nemours duPont Children's Hospital about a recent work he co-authored on this topic. Can VT be predicted on a computer rather than with catheters in the heart? doi: 10.1016/j.hrthm.2019.10.002

This week we review a recent work from the team at Children's National in DC and the NIH on the use of 3D fusion of MRI images with fluoroscopy to help inform interventional procedures. Does this form of "XFM" fusion improve outcomes and reduce radiation or contrast usage? Are there procedures in which this form of imaging might be best applied? We review these and other questions with the first author of this work, Dr. Elena Amin of UCSF. doi: 10.1002/ccd.28324

This week we review a recent small work on the impact of fetal diagnosis of congenital heart disease on the psychological well being of families. Does the fetal prenatal diagnosis and counseling result in less stress in comparison to the families of those diagnosed in the postnatal period? We review this and other questions regarding prenatal counseling with Dr. Miwa Geiger - Director of Fetal Cardiology at Mount Sinai Kravis Children's Hospital and Ms. Michele Cafone, MSN, RN, CPNP - Clinical Nurse Coordinator of The Fetal Heart Program at Sinai. Two top experts on counseling to address some important and provocative questions. doi: 10.1017/S1047951119001781

This week we explore an important paper from CHOP on the optimal approach to repair for Tetralogy of Fallot (TOF). Is there a benefit to a staged approach versus primary complete repair? Are there factors that might influence this decision of approach? We speak with Assistant Professor of Pediatrics Dr. Laura Mercer Rosa of The University of Pennsylvania about her recent large PHIS database study. doi: 10.1016/j.jacc.2019.05.057.

This week we celebrate Children's Cardiomyopathy Awareness Month by reviewing a recent work from PACES on the impact of obesity on ventricular thickness in hypertrophic cardiomyopathy. Does obesity enhance thickness of HCM patients similarly to those without HCM? How does obesity affect risk for SCD in this condition? We review these and other questions with Dr. Seshadri Balaji, Professor of Pediatrics at OHSU. We speak also with Ms. Lisa Yue of the Children's Cardiomyopathy Foundation. What have been the foundation's greatest achievements? What are its present goals? Ms. Yue shares with us the inspiring story of how she and her husband started this important organization supporting pediatric cardiomyopathy research throughout the US. doi: 10.1007/s00246-019-02145-9