Pediheart: Pediatric Cardiology Today

This week we jump back into the world of ACHD and review a recent 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 of Ohio 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 enter the worlds of cardiovascular surgery and interventional catheterization to review a recent STS dataset analysis of postoperative transcatheter interventions. Who needed these sorts of catheterizations more commonly after surgery and what was the relation between needing such an intervention and overall outcomes? How much variability existed between centers in their use of interventional cath following congenital heart surgery? We speak with Associate Professor of Pediatrics, Kevin Hill of Duke University about his recent study on this subject. doi: 10.1161/CIRCINTERVENTIONS.119.007853

In this special episode # 71 co-presented with the SADS Foundation (SADS.ORG) we speak with the president of the foundation, Professor Michael Ackerman of The Mayo Clinic about a recent work he co-authored on a novel approach to assessing patients with possible CPVT. How should the phenotype help to inform genotype results? Can a novel CPVT scorecard help to improve our understanding of genetic test results? How are genes determined to be 'benign', 'likely disease causing' or a variant of uncertain significance (VUS)? We discuss these issues and more with Dr. Ackerman who provides great insights into this fascinating topic that affects all cardiologists who use genetic testing as part of their practice. doi: 10.1161/CIRCGEN.119.002510

This week we review the role that complications have on outcomes in the ACHD patient undergoing cardiac surgery. How commonly do complications occur and if they do occur, what impact do these have on ultimate outcomes? Are there strategies that can be employed to reduce complications amongst this fragile patient population? We discuss these issues and more with the senior author of this work, Dr. Oscar Benavidez who is the Director of Congenital Cardiology at Massachusetts General Hospital in Boston, MA. doi: 10.1007/s00246-019-02103-5.

This week we speak with Professor Yoav Dori of Children's Hospital of Philadelphia about a recent paper he co-authored on the topic of lymphatic abnormalities in children with Noonan's syndrome. What are the typical findings in severely affected children and who is a candidate for intervention? Are there patients who are good candidates for 'thoracic duct ligation' or pleurodesis? Is there anyone in whom this would be considered appropriate therapy? We discuss these questions and more with the master of the lymphatic system, Dr. Dori. doi: 10.1007/s00247-018-04337-6.

This week we explore a recent CMR study in Fontan patients assessing the impact of pulmonary blood flow maldistribution and exercise capacity. How closely associated are these differences in relative pulmonary blood flow to functional status and overall outcomes? How aggressively should we intervene for these sorts of problems? We discuss these issues and more with Dr. Rahul Rathod who is the senior author of this work and also Assistant Professor of Pediatrics, Harvard Medical School. doi: 10.1186/s12968-018-0505-4

This week we review a fascinating paper that assesses the direct myocardial effects of IV antiarrhythmic agents upon cardiac muscle function performed by the group at Boston Children's Hospital - Harvard University. We speak with one of the principle authors of this work, Dr. Elizabeth DeWitt, who is an attending electrophysiologist at that large institution about this important work. Which agents were substantial negative inotropes and which had no effects? The answers may be surprising. Dr. DeWitt shares the general approach of the team in Boston when treating arrhythmias that require rapid IV therapies. doi: 10.1177/1074248418810811

This week we review a critically important neurodevelopmental issue affecting infants who have single ventricle palliation - namely hearing loss. How common is hearing loss seen and what role does IV furosemide play in this finding amongst single ventricle patients? Are there techniques to avoid this complication and yet still use this commonly used diuretic agent? We discuss this important issue and study with Dr. Ari Joffe, Professor of Pediatrics University of Alberta - Stollery Children's Hospital who is the senior author of this week's work. doi: 10.1097/PCC.0000000000001807

This week it's National Pediatric Transplant Week and we review a recent work on ECMO following cardiac transplant and speak with its first author, Assistant Professor of Pediatrics in Vanderbilt University, Dr. Justin Godown. How does the use of ECMO in the postoperative period influence outcomes in the short and long term? Are there differences in outcomes when it is used for patients with cardiomyopathy versus congenital heart disease? We review all of these and more with Dr. Godown. We speak also with Melissa McQueen of Transplantfamilies.org about her organization which strives to educate and support the families of children undergoing transplantation. Ms. McQueen's story is certainly an inspiring one. doi: 10.1111/petr.1341

This week we review a recent report from Duke University regarding the incidence of femoral arterial thrombosis after femoral arterial cardiac catheterization in small infants. How did the use of routine ultrasonography improve identification of this post-cath complication? What are the outcomes of therapy with heparin or enoxaparin for this problem? Are there factors that are associated with femoral arterial thrombosis? We discuss these questions and more with Associate Professor of Pediatrics, Kevin Hill. doi: 10.1002/ccd.28009

This week we explore the utility of exercise stress echocardiography in pediatric patients. How often were clinical decisions made based upon findings at stress echo? What sort of extra training is needed to perform these studies? How safe was this form of testing? How are stress echo results incorporated into the decision making process for pediatric patients at Emory University? Dr. Soham Dasgupta, 2nd year fellow in cardiology and first author of this work, shares some of the insights of this large Emory experience. doi: 10.1111/echo.14326

This week we review 2 surgical reports. The first is on the Ozaki Procedure for aortic valve repair and we speak with valvular expert David Kalfa, MD PhD of Columbia University. Who is a good candidate for this approach and what evidence exists of its efficacy in children? Dr. Kalfa shares his insights. We also review a recent report from Taormina, Italy on outcomes of repair of anomalous origin of the PA's from the ascending aorta. We discuss this work with its author, Professor Salvatore Agati. doi: 10.1016/j.jtcvs.2018.01.087

This week we review a recent randomized and controlled trial from the NEJM on the use of the novel Medtronic TYRX envelope for the prevention of pacemaker or ICD infections in adult high risk patients. Did the use of this antibiotic envelope result in reduced incidences of infection? Were there complications associated with its use? Should this be used in only in high risk patients? Is this a cost effective solution? These are amongst the many questions we tackle with co-author of this important trial, Dr. Suneet Mittal who is the Director of Cardiovascular Research at Valley Hospital in Ridgewood, NJ. Dr. Mittal offers great insights into this trial and possible implications of these important findings. DOI: 10.1056/NEJMoa1901111

This week we review a recent report from the team at the Kravis Children's Hospital at Mount Sinai - Icahn School of Medicine at Mount Sinai on the use of pentobarbital for oral sedation for transthoracic echocardiography. Associate Professor of Pediatrics Rajesh Shenoy shares with us insights into sedation for this indication, the general approach of the echo lab at Sinai and how decisions are made regarding sedation for this reason. doi: 10.1111/echo.14301

This week we review a recent report on transcatheter pulmonary valve replacement with the Sapien S3 prosthesis. What are the circumstances in which this valve might be preferable versus other valve types? What are the disadvantages of this device? How can patients be effectively screened as candidates for different valves? How can decisions be made between self expanding systems like Harmony or Venus P versus this sort of device for large RVOT's? We review all of these issues and more with Dr. Daniel Levi of UCLA who offers a wonderful overview of transcatheter PVR in 2019. You will not want to miss his many insights. doi: 10.1002/ccd.27973

This week we review an impressive review of the large Australian/New Zealand registry of Fontan patients assessing the impact of AV valve regurgitation and failure on Fontan outcomes. We discuss this work with the senior author Yves D'udekem, Associate Professor of Surgery at The Murdoch Children's Research Institute and also speak with Professor Frank Cetta of the Mayo Clinic who authored a wonderful editorial on this work. Who is at most risk for AV valve failure at Fontan? How well do patients do with AV valve replacement or repair? We discuss all these issues this week on this exciting episode. doi: 10.1016/j.jacc.2018.12.025

This week we review a recent publication on modern day outcomes for children with dilated cardiomyopathy. It seems as if they are doing better than patients did 10 years earlier - why is this so? What factors may account for the improvements in outcomes? We discuss these questions and more with the first author of this multi center work, Dr. Rakesh Singh, Associate Professor of Pediatrics at UC SanDiego and also the Director of the Pediatric Heart Failure and Transplantation Program at Rady Children's Hospital. doi: 10.1016/j.jacc.2017.09.1089

This week we discuss the rationale for intervening for unilateral proximal PA stenosis with a stent or angioplasty. In the setting of normal RV function or pressure, are there exercise data to support intervening and if so, at what degree of maldistribution should such an intervention be considered? How should this decision be made? Is this safe? We review these and other questions with the first author of this work, Gurumurthy Hiremath MD who is the director of the pediatric catheterization laboratory at the University of Minnesota. doi: 10.1016/j.jcin.2018.11.042

This week we review a recent paper on the topic of extended ambulatory ECG monitoring in the ACHD patient. Does this form of monitoring provide benefit versus a more traditional 24 or 48 hour "Holter" monitor? How commonly were arrhythmias in ACHD patients found after 48 hours and how commonly were changes made to management based upon these findings? We discuss these issues and more with the senior author of this work, Dr. Scott R. Ceresnak, Associate Professor of Pediatrics - Stanford University. doi: 10.1111/chd.12736

This week we review the topic of pulmonary hypertension and specifically the use of sildenafil for treatment of this difficult problem. Is this agent safe in children? How commonly does it need to be discontinued for side effects? Is it effective? Do patients ever improve to a degree that it can be discontinued? We review all of these questions and more with Dr. Jennifer Cohen who wrote this work while a general cardiology fellow at Columbia University but is now the Advanced Non-Invasive Imaging Fellow at The Icahn School of Medicine at Mount Sinai - NYC. doi: 10.1016/j.jpeds.2018.09.067