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 thi ..read more

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 Arkans ..read more

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 ..read more

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 Philadelp ..read more

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 ..read more

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 ..read more

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 t ..read more

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 t ..read more

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, resp ..read more

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 ..read more