Pediheart: Pediatric Cardiology Today

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...

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 seni...

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...

This week we speak with Associate Professor of Medical Psychology at UNSW, Nadine Kasparian about a recent publication regarding evidence for efficacy for various forms of psychological therapy in the setting of childhood-onset heart disease. What do we know about the efficacy of interventions for this fragile population of congenital heart disease patients? How prevalent are psychological problem...

This week we go back to the world of artificial intelligence and review a recent work from Stanford University on the use of deep neural networks to interpret ECG rhythms. How good did a novel algorithm do in comparison to cardiologists in characterization of rhythm abnormalities? What are some novel uses of AI in medicine today and how can AI potentially identify signs of disease that even human...

This week we delve into the world of fetal cardiology to review a recent work comparing fetal MRI to fetal echo. How did this relatively new technology application fair in comparison to more traditional echocardiography? What role might this technology have in the assessment of congenital heart disease in the fetus? We explore these questions and others with fetal cardiology expert, Professor Lisa...

This week we review a recent work on contrast induced acute kidney injury in the ACHD patient undergoing catheterization. Are there data to suggest what a safe volume of dye might be in this population of patients? Does the renal response to contrast suggest something about the general health of the patient? How does the team in Boston plan catheterizations in the adult congenital population to mi...

This week we review the Fontan and long term issues of surveillance and Fontan failure with 2 articles. The first is a large survey study reviewing practice patterns amongst large Fontan clinics in the US. We review this interesting topic of optimal assessment of the Fontan with Dr. Mike DiMaria who is first author of this recent work and also Associate Professor University of Colorado. We then re...

This week we turn to the world of cardiovascular nursing and review the notion of individualized family centered care for infants in the CICU. What is meant by the term "Individualized Family-Centered Developmental Care" and what are its components? What is the evidence that use of this approach can result in improved neuro-developmental outcomes? We review these important concepts with the first...

This week we review the notion of sports participation in athletes who have ICD's. Is this a safe approach? How do the athletes fare? How do the devices stand up to sport? What are the subtleties of discussing this important topic with a family and how does shared decision making work in this process? We review this serious and complex topic with Dr. Rachel Lampert, Professor of Medicine at Yale U...

This week we enter into the world of cardiac morphology and speak with Professor Leo Lopez of Stanford University about a recent work he wrote with the International Society for Nomenclature of Pediatric and Congenital Heart Disease regarding proper nomenclature for VSD's. Is it defect geography or borders that are most important in naming these structures? How was agreement forged between such di...

This week we review a landmark paper reviewing the very large experience of Dr. Hanley and his team at Stanford University in managing VSD/PA with MAPCA's. What advantages are seen with the 'midline unifocalization' approach popularized by this team over the past 20+ years? Is this an approach that can be broadly applied or are the technical operative skills too challenging for most? Why are these...

This week we return to the world of adult congenital heart disease to review 2 related works on the topic of late cardiovascular risks in patients who have undergone prior coarctation repair. Are such patients at elevated risk for early coronary artery disease and stroke? What are the causes for these late complications? What role does hypertension play in the development of these late sequelae? A...

This week we explore the topics of cardiomyopathy, electrophysiology and sudden death and delve into the world of arrhythmogenic right ventricular cardiomyopathy (ARVC). What role might autoimmune disease play in this disorder? Can a biomarker potentially allow us to identify the vast majority of individuals with this disease with high sensitivity and specificity? Can this biomarker identify 'pre-...

This week we review a recent paper from Circulation on the topic of preoperative factors that may impact outcomes of the arterial switch operation. Is there an optimal time to perform an arterial switch operation? Are there other modifiable factors that may auger a better outcome with shorter hospital stay and lower cost? We discuss these questions and others with the first author of this work, As...

This week we enter the world of fetal cardiology to ask the question, 'Can an inexpensive heart rate monitor for fetuses accurately identify in a timely manner fetal patients with impending heart block and can this allow for more timely and potentially effective therapies for AV conduction disease associated with maternal antibodies?' Is there an optimal time to intercede? We discuss a recent mult...

This week we review a paper from the Amrita Institute in Kerala, India on outcomes of infant surgery in that region and discuss the results with its senior author, Dr. Raman Krishna Kumar. Professor Kumar explains how this large center in India has been able to develop a program that would be the envy of any city in the world, all on a 'shoestring' budget. He reviews with us the path he took from...

This week we speak with Dr. Douglas Mah, Assistant Professor of Pediatrics - Harvard Medical School and Director of the Pacemaker and ICD Service at Children's Hospital Boston about a recent paper he published with his colleagues on coronary artery compression from epicardial pacemaker leads. Who is at risk for this rare complication? How can patients be screened? Once identified, what is the trea...

This week we explore the world of 'fast tracking' patients following cardiac surgery and focus on how to best provide analgesia and sedation to such patients. Are there advantages to intermittent boluses of such agents rather than a low dose basal infusion? What are the implications of such a strategy for nursing and medical care? Does 'waiting' for discomfort prior to administration of medication...

This week we explore the question of optimal timing of the Fontan operation. What factors are associated with good or bad outcome after Fontan in regards to age at surgery? Can Fontan palliation have a positive impact on cardiac function? Is there a 'sweet spot' regarding timing of this single ventricle palliation? Who deserves a fenestration? These are amongst the many questions we review this we...

Interventional cardiology is on tap this week as we explore the world of transcatheter management of the dysfunctional RVOT with 2 papers on this topic. In the first, Dr. Mary Hunt Martin, Assistant Professor of Pediatrics - University of Utah is the first author on a recent PHN paper on outcomes of Melody implantation in the native RVOT. Dr. Martin joins us to answer a number of questions about t...

This week we explore the interpretation of T wave inversions in the athlete with Professor Sanjay Sharma, Professor of Cardiology University of London. Which T wave inversions are "normal variants" and which are worrisome for disease? What is the role of genetic testing in determining who has disease? We review Prof. Sharma's group's recent paper on this topic and discuss different aspects of this...

This week we delve into the world of Marfan Syndrome and review a recent report from the Pediatric Heart Network with the first author of this work, Dr. Seda Selamet Tierney - Associate Professor of Pediatrics, Stanford University. Why does stiffness of the aortic wall predict dilation and need for surgery? Does the use of atenolol or losartan affect aortic growth or dilation? Which agent is prefe...

This week we review a novel paper on MRI changes observed in TOF adults. Are these signs for advanced brain 'aging' in tetralogy adults? We also review a prospective trial of different mechanical ventilation strategies during SVT ablations in children. Can tidal volume or PEEP affect catheter stability while mapping or ablating SVT? We discuss this question with the first author of this work, Dr....

This week we explore the world of cardiac transplantation with particular emphasis on the Fontan patient and transplantation. Outcomes are improving rapidly. Why is this? What about the liver and how are decisions made regarding cardiac versus cardiac+hepatic transplantation in this patient population? We review all of these questions with world authority on cardiac transplantation Dr. Daphne Hsu,...