This week we review a recent retrospective review from Phoenix Children’s Hospital about coronary artery fistulae. How common are they and who needed intervention? How should the cardiologist think about the small fistula? Should they all be ‘followed’ or can they be discharged from cardiology follow-up? How small is ‘small enough’? We speak with Dr. Marie Chevenon who is a fetal cardiologist at Phoenix Children’s Hospital about her recent study on the Phoenix experience with coronary artery fistulae.  

https://doi.org/10.1007/s00246-024-03600-y

This week we speak with Ohio State ACHD fellow Andrew Freddo MD, PhD about a recent large single center study he conducted assessing the cardiovascular drugs that adult Fontan patients are taking and whether they might offer us insights into general well-being. Are there agents that are associated with worse outcomes? If so, is this a situation of the agent causing harm or is it a possible marker of illness? Are there agents associated with improved outcomes? These are amongst the questions reviewed with Dr. Freddo this week. 

• DOI: 10.1016/j.jacadv.2025.102070

This week we review the results of the new pivotal trial of the 'Minima' stent by Renata Medical. What is special about this new device that makes it particularly desirable for use in small children? How strong is cobalt chromium and what evidence exists that it can be expanded repeatedly over 1-2 decades? Can this technology supplant surgery for certain diseases such as native coarctation? What are the limitations of this device? We speak with the first author of the pivotal trial, Dr. Patrick Sullivan of CHLA who is Associate Professor of Pediatrics at Keck Medicine at USC. 

• DOI: 10.1161/CIRCINTERVENTIONS.125.015618

This week we review a recent survey study assessing the beliefs of fetal cardiologists and how these may influence the content and conduct of their counseling. Do most fetal cardiologists review all options including intervention, comfort care and pregnancy termination? Should there be a mandate that all do? Is it realistic or appropriate to ask a fetal cardiologist to suggest an option when they do not believe it to be a reasonable or correct choice? We speak with two of the authors of this work and they are Dr. Joanne Chiu of Harvard University and Dr. Caitlin Haxel of The University of Vermont. 

• DOI: 10.1002/pd.6706

This week we review an episode from 3 years back and 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.122.026732

Also 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 we review a recent work from the "Women In Pediatric Cardiology" group which is a 4 year old initiative to help lift up women pediatric cardiologists through the fostering of connections and also via mentorship programs. Why did this program develop and what are the most pressing issues affecting female pediatric cardiologists? What are the best solutions to help address gender inequities in our field? We speak with two co-authors of this week's work, Drs. Sarah Ford of Brown University and Kristin Laraja of University of Massachussetts. For those interested in participating in their work, feel free to email  Pediheart@gmail.com and your information will be passed along to the authors. 

• DOI: 10.1007/s00246-024-03576-9

This week we review a recent multicenter, randomized trial pitting 2 different immunosuppressive therapeutic approaches against each other. How did a novel approach of everolimus + low dose tacrolimus compare to more standard MMF + standard, higher dose tacrolimus in avoidance of major adverse transplant events or complications? How did this first ever prospective trial in the pediatric heart transplantation world start and how difficult was it to perform in the absence of corporate or NIH support? Why can these data help inform FDA labelling for this novel approach and why is this important? Dr. Kevin Daly of Boston Children's Hospital shares his deep insights into this work this week!

• DOI: 10.1001/jama.2025.14338

This week we review a recent report of the use of phenoxybenzamine for the reduction of SVR in children undergoing congenital heart surgery in India. How does this agent work and what might be the advantage versus other agents like nitroprusside or milrinone which are more commonly used? How does the cost of this agent (or phentolamine) compare with newer agents? We speak with Dr. Rohit Loomba of Lurie Children's Hospital. For those interested, in addition to working as a critical care cardiologist, Dr. Loomba is also a noted cardiac morphologist and his wonderful videos can be seen at the following web address:

https://www.youtube.com/@Talking_Hearts

Today's paper:

DOI: 10.4103/jpbs.jpbs_868_25

This week we replay an episode from 2 years ago 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

This week we review a recent report from the Boston Children's team on their 39 year experience managing and caring for children with myocardial infarction following Kawasaki disease (KD). What were the clinical features of KD patients that were most associated with this rare complication? What signs or symptoms should be concerning for the caregiver of patients with KD? What are the more common approaches to the management of MI in this fragile and young patient population? Dr. Sunil Ghelani of Boston Children's Hospital offers the answers this week. 

• DOI: 10.1016/j.jpeds.2025.114638

This week we review a landmark paper that came out this month on the topic of ACHD. How common is neurocognitive dysfunction amongst the ACHD patient population? What factors are associated with worse or better such outcomes? What interventions should be considered to mitigate these issues? What is the relationship between mental illness and neurocognitive dysfunction? Dr. Scott Cohen of The Medical College of Wisconsin and Dr. Ali Zaidi of The Icahn School of Medicine at Mount Sinai share their insights into their recent multicenter study. 

doi: 10.1016/j.jacc.2025.06.051

Also featured after the article is a brief description of the upcoming Mount Sinai ACHD Conference called: Kawasaki Disease Across The Ages: From Childhood Onset To Adult Outcomes - The 4th Mount SInai ACHD Symposium. We speak with Dr. Nadine Choueiter who is the Course Director of this conference about the goals and objectives of the conference. Dr. Zaidi is the course co-director. For those interested in signing up, please go to this webpage:

https://physicians.mountsinai.org/news/register-today-for-the-fourth-annual-mount-sinai-adult-congenital-heart-disease-symposium

This year is the 50th anniversary of the first transcatheter ASD closure in a human. To celebrate this achievement, this week we air a live interview from PICS 2025 in Chicago (conducted 8/26/25) with living interventional cardiology legend Dr. Terry King speaking about the events surrounding his landmark intervention as the first person to ever perform a transcatheter ASD closure with his partner, surgeon Dr. Noel Mills. In this one on one interview, Dr. King discusses how he came up with this idea with Dr. Mills and how he found a patient in which to proceed. He reviews what 'informed consent' was in 1975 and also how he and Dr. Mills accurately estimated ASD size in an era that preceded 2D echocardiography. Dr. King speaks about the role of family in his life and offers advice to the next generation. Finally, he shares with us what he is up to today and why he does not believe in retirement. Prepare to be excited and inspired by this wonderful figure in our field. 

This week we review a recent work addressing the growing mismatch between the numbers of ACHD patients and the numbers of well trained ACHD providers. What are the most important factors influencing trainees' decisions regarding the pursuit of a career in ACHD? How can more young people be influenced to pursue this fascinating and growing field? We speak with Dr. Katia Bravo-Jaimes of The Mayo Clinic in Jacksonville, FLA about a recent survey study she conducted on the factors that influenced these important decisions. Dr. Bravo-Jaimes shares her thoughts on solutions to increase the ACHD workforce.

https://doi.org/10.1161/jaha.125.041276

In this week's replay episode from 3 years ago, 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 Associate Professor of Pediatrics at The Icahn School of Medicine at Mount Sinai about this recent PHTS multicenter study.

• DOI: 10.1016/j.healun.2022.12.002

This week we lost one of the true 'nice people' in pediatric cardiology - Dr. Richard J. Golinko who died at the age of 95. Dr. Golinko led the Mount Sinai Pediatric Cardiology Program for many years. He established many of the programs at Sinai that are now fundamental to the program and the care of children with congenital heart disease in the tri-state region. To honor Dr. Golinko's memory, we stretch back over 40 years to review a paper he co-wrote with Dr. Rubin Cooper on balloon angioplasty of aortic coarctation. We speak with Dr. Cooper, the work's first author about the work as well as his remembrances of the man, Dr. Golinko. Also joining us this week is Dr. Anthony Rossi of Nicklaus Children's Hospital and Dr. Rica Arnon of Mount Sinai to share their individual remembrances of this wonderful and warm leader. 

doi: 10.1161/01.cir.70.5.903

Professor Ziyad Hijazi of SIdra Medical joins Pediheart this week to celebrate Pediheart's 350th milestone and discuss his life and times from his early days in Jordan to the absolute heights of interventional cardiology. How did he meet Dr. Kurt Amplatz and become involved in the development of Amplatzer devices? Who were some of his mentors and what does he think about being a mentor? How did he come up with the idea for the PICS interventional course and how has he managed to do this consistently for over 20 years? What does he believe is important when thinking about a work/life balance? This is a rare opportunity to learn from someone who has seen and done it all in every corner of the world in pediatric cardiology. 

For those interested, this is the link to the PICS course in late August, 2025 in Chicago:

https://www.picsymposium.com/home.html

This week we go back in time to 2022 to re-enter the world of cardiogenetics and electrophysiology when we review the topic of catecholaminergic polymorphic ventricular tachycardia (CPVT). Is there a 'best' beta blocker for the treatment of this condition? Why is one better than others? How should one manage the patient who is intolerant of beta blockade but needs it for prevention of arrhythmia? What is the role of flecainide, sympathectomy or even ICD's for these patients? PhD candidate and physician Dr. Puck Peltenburg and CPVT world authority, Dr. Christian van der Werf (both of University of Amsterdam) share their deep insights this week. doi: 10.1161/CIRCULATIONAHA.121.056018. Epub 2021 Dec 7

This week we review a recent important longitudinal follow up study of patients who have undergone palliation for the treatment of HLHS at The Children's Hospital of Philadelphia over the past 35+ years. What are the 'transplant-free' survival numbers at 35 years follow up following newborn palliation? Are there differences between hybrid and traditional "Norwood/Stage I" palliation? What factors explain the group of HLHS patients who do are 'super-Fontan's'? What accounts for the marked variability in outcomes of these complex patients? Dr. J. William Gaynor shares his deep insights both from his large scale paper and long-term experience caring for patients with HLHS.

doi: 10.1016/j.jacc.2025.04.028

This week we review a recent surgical paper on the 'off-label' use of the Melody valve for replacement of the AV valve in small infants and children. How effective and safe was this procedure? What factors were associated with the need for reintervention and what sorts of reinterventions were most common? Why was catheter based reintervention rarely employed? What sort of anti-coagulation protocol seems best to protect these valves? Assistant Professor of Pediatrics at the University of Nebraska, Dr. Samantha Gilg shares the insights from her work this week. 

• DOI: 10.1007/s00246-024-03538-1

This week Dr. Nadine Choueiter of Mount Sinai hosts a special episode of Pediheart: Pediatric Cardiology Today in which we speak with emeritus Professor of Pediatrics at the University of Toronto, Dr. Brian McCrindle about his career and life. How did he develop a love of pediatric cardiology? Who were some of his early mentors? How did he develop the international Kawasaki Disease Registry and how has he cultivated it despite minimal funding? How did he develop an interest in preventive cardiology? How can a young person make their clinical work also their academic work? Dr. McCrindle also shares some insights into navigating a successful life as well as retirement. This is a rare opportunity to be inspired by one of the great pediatric cardovascular researchers of the past 3 decades.