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. Topics of Forthcoming Issues •Pulmonary atresia ( Guest Editor: Gary K. Lofland ) •Marfan syndrome ( Guest Editor: Duke E. Cameron ) •Tetralogy of Fallot ( Guest Editor: Tal Geva ) •Adult congenital heart disease (Highlights of Toronto ACHD) ( Guest Editors: Erwin N. Oechslin and Gary Webb ) •Pulmonary hypertension ( Guest Editor: Jeffrey A. Feinstein ) •Non-invasive cardiac imaging, MRI and CT ( Guest Editors: Andrew Taylor and Vivek Muthurangu ) 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: 3,344* * Figure is an average based on full text articles downloaded monthly via ScienceDirect between August 2008 and March 2009 http://www.ppc-journal.com/?rss=yesElsevier Inc.en © 2010 Published by Elsevier Inc. All rights reserved. Progress in Pediatric Cardiology1058-9813291May 2010 © 2010 Published by Elsevier Inc. All rights reserved. healthpermissions@elsevier.comhttp://www.ppc-journal.com/article/PIIS1058981310000433/abstract?rss=yesEditorial Board10.1016/S1058-9813(10)00043-3Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-0iiiihttp://www.ppc-journal.com/article/PIIS1058981310000342/abstract?rss=yesPulmonary atresia with intact ventricular septum (PAIVS) continues to pose problems in treatment as shown by a continuing high mortality rate and the relatively low percentage of two ventricle repairs (2VR). Further evidence of the challenges these patients provide is the lack of a consistent approach across institutions and also the many papers on this defect. Although the recent mortality figures have improved somewhat, a general opinion is that they fare little, if any, better than those with the hypoplastic left heart syndrome. At the least, the deceptively simple diagnosis of PAIVS does not reveal the complexity of the clinical spectrum and the difficulties encountered in treatment.IntroductionJohn E. Foker10.1016/j.ppedcard.2010.02.006Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-012http://www.ppc-journal.com/article/PIIS1058981310000378/abstract?rss=yesAbstract: Pulmonary atresia with intact ventricular septum is a severe cardiac malformation that is ductus dependent and needs immediate postnatal care. The phenotype is characterized by three levels of pathology that are considered important for diagnosis and the choice of cardiac surgery. The pathology concerns an hypoplasia and hypertrophy of the right ventricle in which there is a variation in the involvement of the inlet, trabecular and outflow part of the right ventricle. There is also a variable degree of tricuspid valve pathology including hypoplasia and stenosis. The pulmonary valve is atretic which may have been preceded, in the fetal stage, by pulmonary stenosis. This latter phenomenon may be triggered by the severe coronary anomalies that are found in about 30% of the patients. This coronary anomaly is characterized by ventriculo-coronary arterial communications which is accompanied by sometimes severe main coronary artery pathology including interruptions of the main branches. In the latter situation the coronary myocardial perfusion becomes right ventricular dependent. Current cardiac developmental data show that the right ventricular myocardium is derived from the second heart field. Its anterior part contributes this myocardium to the outflow tract and is also employed by the neural crest cells to enter the heart. The posterior second heart field provides the epicardium derived cells (EPDC) that are important for myocardial wall compaction and formation of the coronary vasculature. We hypothesize that pulmonary atresia with intact ventricular septum without ventriculo-coronary arterial communications is primarily based on anterior SHF directed outflow tract septation anomalies, while pulmonary atresia with intact ventricular septum with ventriculo-coronary arterial communications has an additional major problem related to epicardium derived contribution. Developmentally they can therefore be considered as two different diseases that might need separate treatment protocols.Pulmonary atresia with intact ventricular septum: Second heart field derived myocardial and epicardial developmental cluesAdriana C. Gittenberger-de Groot, Monique R.M. Jongbloed, Lambertus J. Wisse, Robert E. Poelmann10.1016/j.ppedcard.2010.02.009Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-039http://www.ppc-journal.com/article/PIIS105898131000038X/abstract?rss=yesGrowth of the prenatal heart is predominantly based on adding new cells (hyperplasia), in contrast to the postnatal heart, where further increase of ventricular mass is due almost entirely to increase in myocyte size (hypertrophy) soon after birth. The key epigenetic factors regulating cardiac growth are the hemodynamics (volume/preload and pressure/afterload), whose gradual increase reflects the changing demands of the growing embryo and fetus. While genes may play a significant part in the etiology of congenital heart disease, hemodynamic perturbations also can lead to predictable changes in morphogenesis and produce cardiac lesions. At present, developmental perturbations seem to produce many of the common congenital defects, while genetic abnormalities are clearly linked to the many chromosomal syndromes that have been well described.Factors in ventricular and atrioventricular valve growth: An embryologist's perspectiveDavid Sedmera10.1016/j.ppedcard.2010.02.010Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-01114http://www.ppc-journal.com/article/PIIS1058981310000366/abstract?rss=yesAbstract: Pulmonary atresia with an intact septum (PAIVS) presents a therapeutic challenge and the initial decisions about following a univentricular pathway or creating a biventricular circulation are critical. The data of 1039 neonates reported to the Pediatric Cardiac Care Consortium from 1982 to 2006 were analyzed. As with other reports, the overall mortality has remained high (20% in-hospital deaths). Despite continuing difficulties with these patients, however, the treatment approaches remained variable and have not changed over this period. The variable surgical and interventional approaches presumably reflected the anatomy at the initial evaluation and, as expected, were important in determining whether the patient followed a single or two ventricle repair track. Opening the atrial septum, for example, as an initial procedure resulted in a greater frequency of subsequent Glenn and Fontan procedures than did opening the right ventricular outflow tract. The only procedure change has been the more frequent use of balloon pulmonary valvotomy in the past 15years to open the pulmonary valve when the anatomy was favorable.Operative and interventional procedures in 1039 neonates with pulmonary valvular atresia and intact ventricular septum: A multi-institutional studyJames H. Moller10.1016/j.ppedcard.2010.02.008Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-01518http://www.ppc-journal.com/article/PIIS1058981310000299/abstract?rss=yesAbstract: Severe hypoplasia of the right ventricle leads to the need to offer an alternative strategy beyond two-ventricle repair for the infant with pulmonary atresia and intact ventricular septum. Although the relative short-term results for the Fontan operation have improved considerably over the past two decades, long-term results are worrisome. The Fontan circulation with resultant elevation in systemic venous pressure and low cardiac output leads to end-organ dysfunction and a series of new diseases. In this review, we discuss the physiological consequences of the Fontan circulation, its effect on the liver and gastrointestinal tract, and propose a new clinical care model for the management of these unique patients.Long-term results and consequences of single ventricle palliationJack Rychik, David Goldberg, Kathryn Dodds10.1016/j.ppedcard.2010.02.001Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-01923http://www.ppc-journal.com/article/PIIS1058981310000354/abstract?rss=yesAbstract: Pulmonary atresia and intact ventricular septum (PAIVS), more than most congenital cardiac defects, requires detailed assessment for initial decision making and to guide intraoperative procedures. Among the important questions to answer are the degree of tricuspid valve (TV) and right ventricular (RV) hypoplasia and whether or not significant connections exist between the coronary arteries and the right ventricle (RV-CACs). When the TV and RV hypoplasia are severe and/or significant RV-CACs exist, these lesions are thought to preclude a two-ventricle repair (2VR) at most institutions. Our approach, however, is that these lesions can be defined and successfully treated making an adequate 2VR probable.We have developed and successfully employed a combined echocardiographic and angiographic approach for neonates at the severe end of the PAIVS spectrum and particularly those with RV-CACs that has guided and allowed 2VRs in these infants. Our approach includes preoperative, intraoperative and postoperative echocardiography including transthoracic, transesophageal (TEE) and cardiac surface imaging. The echocardiographic studies are used to define the TV and RV abnormalities and determine if RV-CACs are present. Significant RV-CACs are indicated by bidirectional flow in the RV-CAC with associated coronary flow reversal and dilated major coronary arteries with abrupt caliber changes at the site of the connection. When significant RV-CACs are present, cardiac catheterization with complementary right ventricular and aortic root angiography from similar projections aids localization of the connections as well as the associated coronary stenoses and interruptions which seem to result from hypertensive, hypoxic perfusion. The combined use of these techniques allows preoperative localization of significant RV-CACs while the use of intraoperative transesophageal and epicardial surface echocardiography aids in their ligation. Postoperatively, identification of any regional wall motion abnormality by TEE, should prompt a search for previously undetected or residual coronary artery connections. This detailed diagnostic approach to the evaluation and treatment of PAIVS will even allow infants at the severe end of the spectrum to be on a 2VR track.Initial, intra-operative, and post-operative evaluation of children with pulmonary atresia with intact ventricular septum with emphasis on the coronary connections to the right ventricleLee A. Pyles, James M. Berry, Julia Steinberger, John E. Foker10.1016/j.ppedcard.2010.02.007Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-02534http://www.ppc-journal.com/article/PIIS1058981310000305/abstract?rss=yesAbstract: The outcome for all children born in Sweden between January 1, 1980 and December 31, 2008 with ventriculo-coronary arterial communications (VCACs) and pulmonary atresia with intact ventricular septum (PAIVS) was assessed. Fifty-one subjects, 42%, of all 125 children born with PAIVS had identifiable VCACs. Cumulative survival after surgery was 67%, as compared with 73% for all subjects born with PAIVS (n.s.). Survival improved over time, and there was no late death after 3years of age. Median follow-up time was 12years (range 1–28years). At follow-up, 33 subjects were alive (12 female/21 male) with the following repairs: six had a biventricular repair, one had 1.5 ventricle repair, 25 were palliated with a single ventricle repair (final stage in 20), and one had a heart transplantation. Notably after decompression (n=9 survivors), the disease in the coronary arteries did not progress, although four subjects in this group had large VCACs. In the group of subjects with single ventricle repair and large VCACs, eleven were found to have stenoses in the coronary arteries. The disease in the coronary arteries in this group, however, often progressed with an adverse effect on myocardial perfusion. Four of the survivors had documented myocardial infarctions.The long-term consequences of the coronary artery lesions in pulmonary atresia with intact ventricular septumBritt-Marie Ekman-Joelsson, Katarina Hanséus, Björn Söderberg, Rune R. Sixt, Peeter Jögi, Mats Synnergren, Jan Sunnegårdh10.1016/j.ppedcard.2010.02.002Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-03542http://www.ppc-journal.com/article/PIIS1058981310000329/abstract?rss=yesAbstract: A hypertensive right ventricle adversely affects left ventricular function in patients who are Fontan candidates with pulmonary atresia with intact ventricular septum (PAIVS). Decompression of a hypertensive right ventricle should improve left ventricular performance. Recently, a right ventricular outflow tract reconstruction, the closed Brock procedure, and/or percutaneous balloon pulmonary valvuloplasty were performed to decompress the hypertensive right ventricle before and during the bidirectional Glenn procedure in Fontan-candidate patients with PAIVS in our hospital. This strategy significantly improved left ventricular performance (contractility and ventricular efficiency) after the bidirectional Glenn procedure and a staged total cavopulmonary connection.Consequences of a hypertensive right ventricle on left ventricular performance of patients with pulmonary atresia and intact ventricular septum after right heart bypass surgeryYoshihisa Tanoue, Hideaki Kado, Tomoki Ushijima, Ryuji Tominaga10.1016/j.ppedcard.2010.02.004Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-04348http://www.ppc-journal.com/article/PIIS1058981310000391/abstract?rss=yesAbstract: The pulmonary atresia and intact ventricular septum (PAIVS) spectrum usually includes very hypoplastic right ventricles (RVs) and tricuspid valves (TVs) and often RV-coronary artery connections (RV-CACs). These abnormalities are often thought irreversible and typically only 30% reach a 2-ventricle repair (2VR). Our hypothesis, however, has been PAIVS is a developmental defect and catch-up growth is possible in all. To answer this question, we attempted to obtain up to 10year echo data on RV and TV growth and function in the 24 survivors (of 28 patients) from 1989 to 1999. To maximize the validity of the growth results obtained, we used only paired studies and required biplane echos to accurately estimate RV volumes, TV size, and function. Measurements were indexed to determine the z score (standard deviation of the mean from predicted).All patients had an RV outflow patch and ASD reduction. We have found catch-up growth required complete relief of RV obstruction and a mildly restrictive (3–5mmHg) ASD to encourage TV flow. A central shunt was placed in 38%. RV-CACs (2.7±1.6/pt) were uneventfully ligated off bypass in 9/28 (32%). For the 24 survivors, 19 adequate paired biplane studies were obtained from here and elsewhere and for 12 allowed a 10year follow-up.The RV volume z scores grew from −5.1±2.5 to 0.9±1.9 about 10years later. The TVs, even with increased flow early, grew more slowly than the RVs. Nevertheless, the very small TVs in 10 pts went from z scores of −4.6±0.4 to −0.04±−1.5 within about 5–10years. Valvotomies were done in 3. RV function was high normal (EF=74.5±0.1%) presumably because of pulmonary regurgitation in the 8 pts who had adequate studies.We conclude: (1) Catch-up growth appears reliable in PAIVS pts when RV obstruction is relieved and TV flow is increased (2) RV function was good and resembled well-repaired TOF (3) These results encourage pursuing 2VRs in PAIVS patients.Growth and function of hypoplastic right ventricles and tricuspid valves in infants with pulmonary atresia and intact ventricular septumJohn E. Foker, James Berry, Shaun P. Setty, Brian A. Harvey, Andrew L. Rivard, Adriana C. Gittenberger-de Groot, Lee A. Pyles10.1016/j.ppedcard.2010.02.011Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-04954http://www.ppc-journal.com/article/PIIS1058981310000317/abstract?rss=yesAbstract: Pulmonary Atresia with intact septum can be diagnosed by echocardiography from early pregnancy where a spectrum of morphology can be recognised. Important tricuspid regurgitation is common and the right ventricle develops at supra-systemic pressures leading to progressive ventricular hypertrophy and mineralisation that can be seen on serial echocardiograms. This is likely to compromise later ventricular function and only about a third of children achieve an eventual two-ventricle circulation. Fetal valvuloplasty has been proposed as a potential therapy to open the pulmonary valve, reduce right ventricular pressure and improve the potential for normal myocardial development. There is no evidence yet, however, that fetal pulmonary valvuloplasty alters the growth of the pulmonary or tricuspid valves or influences the ventricular size to change the eventual designation, although theoretical benefits in quality of the myocardium may lead to improved outcomes.The in-utero development and treatment of Pulmonary Atresia with intact septumHelena M. Gardiner10.1016/j.ppedcard.2010.02.003Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-05560http://www.ppc-journal.com/article/PIIS1058981310000330/abstract?rss=yesAbstract: The articles in this issue reveal the complexity of the PAIVS spectrum and the methods of treatment that are currently being used. Despite the complexity, the articles provide greater understanding of the developmental events leading to these lesions and the methods that could be used to reverse them. Taken together, the evidence certainly favors embarking on a 2VR track whenever possible. The number of completed biventricular repairs will be increased and also the adverse later consequences of a hypertensive RV and persisting significant RV to coronary artery connections will be eliminated when they are not. The algorithm, therefore, is heavily weighted to 2VRs.Treatment algorithm for Pulmonary Atresia with Intact Ventricular SeptumJohn E. Foker, James M. Berry, Lee A. Pyles10.1016/j.ppedcard.2010.02.005Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-06163http://www.ppc-journal.com/article/PIIS1058981310000470/abstract?rss=yesFuture Topics and Guest Editors10.1016/S1058-9813(10)00047-0Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-0IIhttp://www.ppc-journal.com/article/PIIS1058981310000482/abstract?rss=yesFuture Meetings10.1016/S1058-9813(10)00048-2Progress in Pediatric Cardiology 29, 1 (2010)2010-05-01Progress in Pediatric Cardiology2010-05-01291S1058-9813(10)X0003-0IIIIII