Progress in Pediatric Cardiology RSS feed: Current Issue. Progress in Pediatric Cardiology is an international journal of review presenting information and experienced opinion of importance in the understanding and management of cardiovascular diseases in children. Each issue is prepared by one or more Guest Editors and reviews a single subject, allowing for comprehensive presentations of complex, multifaceted or rapidly changing topics of clinical and investigative interest. Future Topics and Guest Editors •Proceedings of The 21st Annual Course on Congenital Heart Disease in the Adult Gary D. Webb and William G. Williams •Marfan Syndrome and Loeys-Dietz Syndrome Duke E. Cameron, Luca A. Vricella and Hal Dietz •Diagnosis and Management of SVT in Children and Congenital Heart Patients Seshadri Balaji •Pediatric Ventricular Assist Devices Kimberly Gandy Readers interested in being a guest editor or participating in the development of a review for publication should contact the Editor-in-Chief, Gary K. Lofland, MD, Progress in Pediatric Cardiology, The Children's Mercy Hospital, 2401 Gilliam Road, Kansas City, MO 64108, USA; E-mail: glofland@cmh.edu Books and reports of clinical or investigative relevance to pediatric cardiovascular medicine will be reviewed and published. Authors, editors and institutions should send one copy for review to the Editor. Electronic usage: An increasing number of readers access the journal online via ScienceDirect, one of the world's most advanced web delivery systems for scientific, technical and medical information. Average monthly article downloads for this journal: 3,648* * Figure is a monthly average of full-text articles downloaded via ScienceDirect in 2011 http://www.ppc-journal.com/?rss=yesElsevier Inc.en © 2012 Published by Elsevier Inc. All rights reserved. Progress in Pediatric Cardiology1058-9813332May 2012 © 2012 Published by Elsevier Inc. All rights reserved. healthpermissions@elsevier.comhttp://www.ppc-journal.com/article/PIIS1058981312000331/abstract?rss=yesEditorial Board10.1016/S1058-9813(12)00033-1Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1iihttp://www.ppc-journal.com/article/PIIS1058981312000197/abstract?rss=yesPediatric cardiology has always been at the forefront of cardiac device development. Portsmann described the first PDA device closure in 1967 and King and Mills performed the first transcatheter ASD closure in 1975. As a frame of reference, the first intracoronary balloon angioplasty was not performed until 1986. Our field has driven the development of smaller and more innovative devices and technology including pacemakers and ventricular assist devices such as the Berlin Heart. Most recently, Phillip Bonhoeffer, a pediatric cardiologist, was instrumental in bringing transcatheter valve therapy to fruition.IntroductionKevin D. Hill, Jennifer S. Li10.1016/j.ppedcard.2012.02.001Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1103103http://www.ppc-journal.com/article/PIIS1058981312000203/abstract?rss=yesAbstract: Pediatric medical devices play a vital role in the treatment of children with cardiovascular disease. Most cardiac medical devices used in children today are used off-label where the risk–benefit of devices has not been well characterized. Pediatric medical devices face a variety of challenges to FDA approval largely due to the small target population, heterogeneity of the patient population and ethical considerations around device testing in children. While relatively few cardiac devices have received FDA approval in children, the number of devices navigating the approval process successfully appears to be growing. Most pediatric device approvals are being granted through the humanitarian device exemption (HDE) pathway, which is designed for rare diseases and therefore suitable for devices intended to treat most forms of congenital heart disease. This review summarizes the FDA review process for pediatric cardiovascular medical devices as it continues to evolve in response to the unique challenges of evaluating device performance in children with cardiovascular disease.The FDA review process for cardiac medical devices in children: A review for the clinicianChristopher S. Almond10.1016/j.ppedcard.2012.02.002Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1105109http://www.ppc-journal.com/article/PIIS1058981312000215/abstract?rss=yesAbstract: Implanted cardiac devices have improved and prolonged the lives of countless children. Some of these devices have undergone vigorous pre-market assessment and are US Food and Drug Administration (FDA) approved for use in children; however, many devices are used “off label” following approval in adults. The potential for unrecognized and/or under-estimated long term complications is a very real concern, particularly in children where there are unique constraints related to growth, development and need for prolonged device longevity. Long-term oversight is necessary to identify rare and unanticipated device related complications. Current systems include FDA surveillance via adverse event reporting databases and mandated post-approval studies, as well as device related registries. Adverse event reporting databases have provided important information on rare device-related complications but are hampered by a documented very low rate of reporting. Recently several large registries have been developed to track outcomes, however, registries are inherently more effective at tracking short-term safety and efficacy. Looking forward, it is incumbent on physicians, regulatory agencies, and manufacturers to recognize the potential for rare, but serious, device-related complications and to report such complications to established registries and FDA surveillance databases.Safety of cardiac devices in childrenKristin M. Corey, Jennifer S. Li, Sara K. Pasquali, Kevin D. Hill10.1016/j.ppedcard.2012.02.003Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1111114http://www.ppc-journal.com/article/PIIS1058981312000227/abstract?rss=yesAbstract: In 1975 Drs. Terry King and Noel Mills performed the first percutaneous closure of a secundum atrial septal defect. This remarkable innovation sparked over 35years of dramatic advances resulting in a variety of devices designed for the sole purpose of closing secundum atrial septal defects. These devices have various shapes, sizes, and delivery methods. No device has been perfect, and each device increases our knowledge and understanding of percutaneous closure of secundum atrial septal defects. In this manuscript we review the history of percutaneous atrial septal defect closure and will provide a brief discussion of many of the devices from the past, present, and future.Percutaneous closure of secundum atrial septal defectsErin Albers, Dana Janssen, Drew Ammons, Thomas Doyle10.1016/j.ppedcard.2012.02.004Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1115123http://www.ppc-journal.com/article/PIIS1058981312000239/abstract?rss=yesAbstract: Transcatheter closure of persistently patent ductus arteriosus (PDA) has been performed since the early 1990s. Over the past 15years, there have been significant developments in the devices used to close PDA. The currently available devices in the United States that are or will soon be approved for PDA closure are: embolization coils, Amplatzer Duct Occluders I/II, and the Product For Medicine Nit-Occlud devices. Each device has its strengths and limitations. Three criteria will be used to determine which device is superior to close PDA. Safety, including the occurrence of hemolysis, pulmonary artery and/or aortic obstruction, vascular complications, and frequency of device embolization will be evaluated for each device. Efficacy will be determined at 6month echo follow-up regarding residual shunt. Finally, overall cost will be considered. After evaluating each device on these merits, we will conclude which device, if any, is superior for closure of PDA.What is the optimal device for closure of a persistently patent ductus arteriosus?Thomas J. Forbes, Daniel R. Turner10.1016/j.ppedcard.2012.02.005Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1125129http://www.ppc-journal.com/article/PIIS1058981312000240/abstract?rss=yesAbstract: Ventricular septal defects (VSD) are among the most commonly occurring congenital heart lesions. While successful surgical repair of VSDs has been possible for over 60years, peri-operative and late complications still occur and there are inherent risks associated with sternotomy and exposure to cardiopulmonary bypass. A desire to avoid these risks, as well as the successful application of catheter-based device closure for other intracardiac and vascular shunting lesions, has lead to an interest in developing a less invasive means by which to close VSDs. The aim of this article is (a) to understand the important differences in approach to catheter-based closure of congenitally occurring VSDs, based on their location, (b) to discuss the technical aspects of device closure of VSDs, from both a percutaneous and perventricular (hybrid) approach, and (c) to review the outcomes of the experience with these devices. Acquired (post-infarction, post-trauma) VSDs will not be addressed.Transcatheter device closure of congenital ventricular septal defectsDaniel H. Gruenstein, John L. Bass10.1016/j.ppedcard.2012.02.006Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1131141http://www.ppc-journal.com/article/PIIS1058981312000252/abstract?rss=yesAbstract: Each year in the United States more than 4000 newborns are diagnosed with complex congenital heart disease involving right ventricular outflow tract obstruction. Despite advances in surgical techniques with right ventricle to pulmonary artery conduits, progressive conduit failure often occurs within 4–12years. Consequently, the majority of these patients, prior to becoming adults, require 2–4 operations for the right ventricle outflow tract to reverse the adverse ventricular effects of a volume or pressure load. Fortunately, over the past decade, percutaneous pulmonary valve replacement has emerged as a nonsurgical intervention for right ventricle to pulmonary artery conduit failure.Currently, there are two percutaneous pulmonary valves available in the United States with the Edwards SAPIEN™ valve actively enrolling in a Phase II FDA approved clinical trial, and the Medtronic Melody® valve now FDA approved through a Humanitarian Device Exception pathway. In this article, we review indications, technique and outcomes for percutaneous pulmonary valve replacement and compare and contrast the available percutaneous stent valves.Percutaneous pulmonary valve replacementGregory A. Fleming, Kevin D. Hill, Amanda S. Green, John F. Rhodes10.1016/j.ppedcard.2012.02.007Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1143150http://www.ppc-journal.com/article/PIIS1058981312000264/abstract?rss=yesAbstract: Stents can be used to treat many forms of congenital heart disease, however, the majority of use remains in the pulmonary arterial system. Initial experimental work in the 1990's proved that stents were an effective means for treating pulmonary artery stenosis. Subsequent experience demonstrated ways to overcome the limitations of small children, bifurcating stenoses, jailed side branches and the ways to cope with potential complications. We review the use of stents to rehabilitate the pulmonary arterial tree and outline the acute and long-term results. Finally, we discuss the potential improvements and opportunities with bioabsorbable stents and other future directions for bare metal stents as well.Pulmonary artery stents: Past, present and futureMatthew A. Crystal, Frank F. Ing10.1016/j.ppedcard.2012.02.008Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1151159http://www.ppc-journal.com/article/PIIS1058981312000276/abstract?rss=yesAbstract: Coarctation of the aorta is one of the more common congenital cardiac lesions. While transcatheter approaches, such as balloon angioplasty and stent implantation are often considered the primary therapeutic strategies for most patients with recoarctation, there is still considerable controversy with regard to the best therapeutic strategy for older patients with primary coarctation, mainly related to the lack of long-term outcome data. This article will highlight technical and procedural considerations for stent implantation for aortic coarctation.Stenting of aortic coarctation: Technical considerations and procedural outcomesJack R. Stines, Ralf J. Holzer10.1016/j.ppedcard.2012.02.009Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1161167http://www.ppc-journal.com/article/PIIS1058981312000288/abstract?rss=yesAbstract: End stage heart failure is a growing problem in the pediatric population. These patients are conventionally managed with a combination of oral and intravenous medications. When these fail, mechanical circulatory support is indicated. Improvements in technology have made options for mechanical circulatory support available to infants and children. These devices may be indicated as a bridge to recovery of the native heart function, or, more commonly as a bridge to transplant. Particularly in small patients, extracorporeal life support (ECMO) has been historically used for this purpose. In some cases, adult ventricular assist devices have been used in older children. At the current time, there are devices in clinical use or being developed that will further improve the care and outcomes of these patients. In this article, the indications, implementation, and results of pediatric ventricular assist devices are presented.Pediatric ventricular assist devicesAndrew J. Lodge, Alexis G. Antunez, Robert D.B. Jaquiss10.1016/j.ppedcard.2012.02.010Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1169176http://www.ppc-journal.com/article/PIIS105898131200029X/abstract?rss=yesAbstract: Pacemakers and implantable cardioverter defibrillators are important therapies in the pediatric and congenital heart disease population. However, these therapies, while potentially life-saving, have major limitations which are particular to the pediatric age group. Lead malfunction, inappropriate discharges, and venous obstruction all complicate device usage in this group. This review will address issues with pacemaker and defibrillator use in the pediatric and congenital heart disease population and discuss potential advances in the area in the next 5 to 10years.Update on pacing and implantable cardioverter defibrillators in childrenAnne M. Dubin10.1016/j.ppedcard.2012.02.011Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1177181http://www.ppc-journal.com/article/PIIS1058981312000306/abstract?rss=yesThe authors regret that in the original publication of the above mentioned article reference [10] was incorrect. Here is the correct reference: [10] Sykes D., Barach P, Basner H, Belojevic G, Busch-Vishniac I, Cavanaugh W, Edworthy J, West J, Xiang N, Davenny B, Jones D, Mazer S. Clinical alarms and fatalities resulting from “alarm fatigue” in hospitals: perspectives from clinical medicine, acoustical science, signal processing, noise control engineering and human factors. White paper, Copyright ARC 2011, published with permission by the organizers of the national summit on Alarms, Association for the Advancement of Medical Instrumentation.Corrigendum to “Managing alarm fatigue in cardiac care” [Progress in Pediatric Cardiology 33 (2012) 85–90]Jo M. Solet, Paul R. Barach10.1016/j.ppedcard.2012.04.001Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1183183http://www.ppc-journal.com/article/PIIS1058981312000379/abstract?rss=yesFuture Topics and Guest Editors10.1016/S1058-9813(12)00037-9Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1IIhttp://www.ppc-journal.com/article/PIIS1058981312000380/abstract?rss=yesFuture Meetings10.1016/S1058-9813(12)00038-0Progress in Pediatric Cardiology 33, 2 (2012)2012-05-01Progress in Pediatric Cardiology2012-05-01332S1058-9813(12)X0003-1IIIIII