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Table of ContentsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Source Type: journals

PrefaceEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
It is a pleasure for me to introduce the 2009 Pediatric Cardiac Surgery Annual. In keeping with the tradition of previous years, the Annual contains a number of articles derived from the 2008 postgraduate course in congenital heart surgery from the annual scientific meeting of the AATS. This section of the Annual has been very ably assembled by Dr Frank Hanley from Stanford. I would like to thank Dr Hanley for his considerable efforts as well as the choice of topics.
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Richard A. Jonas Source Type: journals

IntroductionEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In the Preface to the 2008 Seminars in Thoracic and Cardiovascular Surgery Pediatric Cardiac Surgery Annual, Senior Editor Richard A. Jonas made reference to the rationale for, and potential importance of, the creation of an international organization to represent all congenital heart surgeons and to serve the interests of our growing subspecialty and our patients throughout the world. With satisfaction and appropriate pride, he referred to the creation of the World Society for Pediatric and Congenital Heart Surgery (WSPCHS), which held its inaugural meeting in Washington, DC in May 2007. Much of the success of that first ...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Marshall L. Jacobs Source Type: journals

The World Society for Pediatric and Congenital Heart Surgery: Its Mission and HistoryEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) was established in 2006 to assemble pediatric and congenital heart surgeons from all continents and regions of the world and their colleagues from related specialties dealing with pediatric and congenital heart disease. Since its birth, it has held a highly successful inaugural scientific meeting in 2007 in Washington, DC, and a World Summit on Pediatric and Congenital Heart Surgery Services, Education, and Cardiac Care for Children and Adults with Congenital Heart Disease in 2008 in Montreal. It currently has 549 members from 71 countries and in a short...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Christo I. Tchervenkov, Giovanni Stellin, Hiromi Kurosawa, Jeffrey P. Jacobs, Constantine Mavroudis, Pierre-Luc Bernier, Bohdan Maruszewski, Christian Kreutzer, Sertac Cicek, Robin H. Kinsley, Graham R. Nunn, Richard A. Jonas Tags: World Society for Pediatric and Congenital Heart Surgery Source Type: journals

Pediatric Cardiac Surgery: A Challenge and Outcome Analysis of the Guatemala EffortEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The objective of this study is to examine the outcome analysis of the Guatemala effort. The goals of our new and first pediatric cardiac care program were to: 1) provide diagnosis and treatment to all children with a CCM in Guatemala; 2) train of local staff surgeons, 3) established a foundation locally and in the United States in 1997 to serve as a fundraising instrument to acquire equipment and remodeling of the pediatric cardiac unit and also to raise funds to pay the hospital for the almost exclusively poor pediatric cardiac patients. The staff now includes 3 surgeons from Guatemala, trained by the senior surgeon (A.R....
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Juan R. Leon-Wyss, Altin Veshti, Oscar Veras, Guillermo A. Gaitán, Mauricio O'Connell, Ricardo A. Mack, Gonzalo Calvimontes, Flor Garcia, Amilcar Hidalgo, Alfredo Reyes, Aldo R. Castañeda Tags: World Society for Pediatric and Congenital Heart Surgery Source Type: journals

Challenges and Progress of the Pediatric Cardiac Surgery in Shanghai Children's Medical Center: A 25-year Solid Collaboration With Project HOPEEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Congenital heart disease (CHD) is one of major categories of illness that, if treated, can restore health and improve quality of life. Unfortunately, in many developing countries, health professionals may not have the resources to replenish themselves with modern technology and/or surgery skills to treat a child with complex CHD. Until now, CHD remained a health challenge for many children and families. During the past few decades, due to limited medical resources and operative techniques, only a very small proportion of the children with CHD in our region were able to be operated for lesion repair. However, the situation ...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Jinfen Liu Tags: World Society for Pediatric and Congenital Heart Surgery Source Type: journals

The First Stella Van Praagh Memorial Lecture: The History and Anatomy of Tetralogy of FallotEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Stella Van Praagh, MD (1927-2006) of Children's Hospital Boston was one of the greatest pediatric cardiologists and pediatric cardiac pathologists of the 20th and early 21st centuries. Née Stella Zacharioudaki from Crete, Greece, in addition to her stellar professional attainments, she was also an outstanding cuisinière, hostess, linguist, philosopher, and philanthropist. In 1962, she married Richard Van Praagh, MD, beginning a life-long collaboration that was in every sense an affaire de coeur. They had three children and seven grandchildren. Dr Stella was the author of more than 110 scientific publications which helped...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Richard Van Praagh Tags: World Society for Pediatric and Congenital Heart Surgery Source Type: journals

Early Primary Repair of Tetralogy of FallotEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Outcomes for surgical repair of tetralogy of Fallot with pulmonary stenosis are often considered to be a useful benchmark for the assessment of congenital surgical programs. This procedure, including preoperative assessment and postoperative management, falls in the middle of the spectrum of difficulty of all the congenital cardiac procedures. Within this anomaly itself, there is a wide spectrum of severity and difficulty. However, it is important to distinguish tetralogy with pulmonary stenosis or “simple tetralogy” from the much more complex anomaly tetralogy of Fallot with pulmonary atresia. Tetralogy of Fallot with...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Richard A. Jonas Tags: World Society for Pediatric and Congenital Heart Surgery Source Type: journals

Transatrial-Transpulmonary Repair of Tetralogy of FallotEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Routine early transatrial-transpulmonary repair of tetralogy of Fallot (TOF) has been performed at our institution since 1990. A transatrial-transpulmonary approach implies a minimal or no ventriculotomy and therefore it is expected to improve the right ventricular performance in the long term. Early transatrial-transpulmonary repair has been used for avoiding the deleterious effects of preoperative chronic cyanosis and spells, surgery for systemic-to-pulmonary artery shunts and their consequences, chronic right ventricular systolic pressure, and myocardial compensatory hypertrophy, with the aim of achieving better preserv...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Massimo A. Padalino, Vladimiro L. Vida, Giovanni Stellin Tags: World Society for Pediatric and Congenital Heart Surgery Source Type: journals

Tetralogy of Fallot Repair: The Right Ventricle Infundibulum Sparing (RVIS) StrategyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Despite the excellent operative survival for tetralogy of Fallot (TOF) repair, well-documented long-term complications and reduced life expectancy remain challenges for these patients and their clinicians. In an attempt to change the natural history of repaired TOF, we at Texas Children's Hospital (Houston, TX) have developed a management strategy not focused on age, but rather focused on preserving the right ventricular (RV) infundibulum. The RV infundibulum sparing (RVIS) repair of TOF consists of a transatrial and transpulmonary approach to close the ventricular septal defect and resect RV infundibular muscle coupled wi...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: David L. Morales, Farhan Zafar, Charles D. Fraser Tags: World Society for Pediatric and Congenital Heart Surgery Source Type: journals

A Novel Approach to the Repair of Tetralogy of Fallot With Absent Pulmonary Valve and the Reduction of Airway Compression by the Pulmonary ArteryEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Controversy persists regarding the management of patients suffering from tetralogy of Fallot with absent pulmonary valve syndrome. Airway obstruction caused by the dilated pulmonary arteries is the determining factor in the mortality of symptomatic newborns and infants. A number of surgical techniques for the reduction of bronchial obstruction have been proposed, producing variable results. All strategies have focused on plication and reduction of the anterior or posterior wall of the normally positioned pulmonary arteries, with or without pulmonary valve replacement. An alternative approach is to bring the pulmonary arter...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Viktor Hraška, Joachim Photiadis, Ehrenfried Schindler, Nicodeme Sinzobahamvya, Christopher Fink, Christopher Haun, Martin Schneider, Hedwig-Christine Blaschczok, Bulos Asfour Tags: World Society for Pediatric and Congenital Heart Surgery Source Type: journals

How I Manage Neonatal Ebstein's AnomalyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Ebstein's anomaly of the tricuspid valve is characterized by a wide spectrum of severity. The condition involves more than a malformation of the tricuspid valve alone; a substantial portion of the right ventricle may be involved as well. Those patients presenting with symptoms in the first month of life represent a challenging group with a high mortality and uncertainty as to the best treatment options. In this review, the practical decision making process and the outcomes for neonates at one center are discussed.
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Edward L. Bove, Jennifer C. Hirsch, Richard G. Ohye, Eric J. Devaney Tags: Presentations from the 2008 AATS Meeting Source Type: journals

Transverse Aortic Arch Obstruction: When to Go from the FrontEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Transverse aortic arch hypoplasia involving some or all segments of the arch (tubular hypoplasia) may exist in association with intra-cardiac anomalies of varying severity. Surgical repair of the distal transverse aortic arch and isthmus are adequately managed by an extended end-to-end coarctation repair in most infants via a left thoracotomy. The surgical management and timing of proximal aortic arch obstruction is controversial but almost always requires an approach via sternotomy using cardiopulmonary bypass.
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: John W. Brown, Mark D. Rodefeld, Mark Ruzmetov Tags: Presentations from the 2008 AATS Meeting Source Type: journals

Management of Systemic Outlet Obstruction in Patients Undergoing Single Ventricle PalliationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Early or late systemic outlet obstruction in patients undergoing staged palliation for single ventricle variants may preclude a successful Fontan circulation. In assessing the patient presenting with real or impending obstruction, the surgeon is justified in pursuing an aggressive approach in protecting ventricular and semilunar valve function. Options include subaortic resection (transventricular, transatrial, or via a semilunar valve), modifications of the Damus-Kaye-Stansel connection, or a palliative arterial switch operation. In this review, the various options will be discussed and presented in the context of applica...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Charles D. Fraser Tags: Presentations from the 2008 AATS Meeting Source Type: journals

Aortic Stenosis and Aortic Insufficiency in Children: Impact of Valvuloplasty and Modified Ross-Konno ProcedureEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Aortic stenosis and aortic insufficiency in young children present multiple challenges to the patient, family, and surgeon. Mechanical valves require anticoagulation therapy, which is a poor option in active youngsters and noncompliant adolescents. Aortic valvuloplasty and the various forms of the Ross/Ross-Konno operations appear to be good solutions in this patient population due to valve preservation for the former and autograft growth for the latter. However, valvuloplasty failure and autograft dilatation have developed in some patients. In addition, heart block remains a problem in those patients who require an annula...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Constantine Mavroudis, Carl L. Backer, Sunjay Kaushal Tags: Presentations from the 2008 AATS Meeting Source Type: journals

How I Manage Mitral Stenosis in the Neonate and InfantEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Congenital mitral valve stenosis is rare. Intervention is indicated in the most severe cases only and is determined by the site and mechanism of valvar obstruction. Correction of mitral valve stenosis is attempted in order to improve mitral valve function and delay replacement as early valve replacement is associated with significant mortality and morbidity. Balloon valvuloplasty can be used for isolated mitral valve lesions. In cases of associated left heart lesions such as aortic stenosis or left ventricular outflow tract obstruction, early palliation with single ventricle repair is performed. Newborn mitral valve replac...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Stephanie Fuller, Thomas L. Spray Tags: Presentations from the 2008 AATS Meeting Source Type: journals

Fontan or Septation: When I Abandon Septation in Complex Lesions With Two VentriclesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This article will review the complex decision making that is required in the gray area between a clear biventricular pathway and the single ventricle track for patients with two ventricles.
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Richard A. Jonas Tags: Presentations from the 2008 AATS Meeting Source Type: journals

MR and CT Imaging of the Pediatric Patient With Structural Heart DiseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Cardiac magnetic resonance imaging (MRI) and computed tomography (CT) are imaging modalities increasingly used in the diagnosis and management of structural heart disease. They are powerful imaging tools that have individual strengths and weaknesses. Rational choice between MRI and CT should be based on a sound understanding of these issues. Management guidelines that incorporate the use of MRI and CT are currently being developed, and their utilizations are expected to grow rapidly in the future.
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Frandics P. Chan Tags: Presentations from the 2008 AATS Meeting Source Type: journals

Evaluation, Risk Stratification, and Management of Pulmonary Hypertension in Patients With Congenital Heart DiseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Surgical outcomes for patients with congenital heart disease have improved dramatically over the past few decades due, in part, to improvements in preoperative and postoperative management, cardiopulmonary bypass and intraoperative technique, and the development of pediatric cardiology as a subspecialty. Patients with pulmonary hypertension, however, remain a particularly challenging subgroup worldwide. Determination of operability and, when reasonable, timing of surgery, remains at times difficult. An overview of the evaluation process for this patient population is presented along with a review of evidence for risk strat...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Jeffrey A. Feinstein Tags: Presentations from the 2008 AATS Meeting Source Type: journals

Percutaneous Pulmonary Valve ImplantationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Percutaneous pulmonary valve implantation is a new treatment option in patients with dysfunctional conduits. The aim of percutaneous pulmonary valve implantation is to prolong the lifespan of right ventricle to pulmonary artery conduits and thereby postponing open-heart surgery. Early results have shown a significant reduction in right ventricular pressure and right ventricular outflow tract gradient. During a follow-up of a median of 28 months, freedom from re-operation is 93 (2), 86 (3), 84 (4), and 70 (13)% at 10, 30, 50, and 70 months, respectively. The most common complication during follow-up are stent fractures with...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Philipp Lurz, Regis Gaudin, Andrew M. Taylor, Philipp Bonhoeffer Tags: Presentations from the 2008 AATS Meeting Source Type: journals

How We Approach Peripheral Pulmonary Stenosis in Williams-Beuren SyndromeEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This report will address the approach to this lesion with catheter-based approaches, surgical approaches, and their combination.
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Kim L. Gandy, James S. Tweddell, Andrew N. Pelech Tags: Presentations from the 2008 AATS Meeting Source Type: journals

Management of the Aortic Root in Adult Patients With Conotruncal AnomaliesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conotruncal anomalies such as tetralogy of Fallot, double outlet right ventricle, truncus arteriosus, and transposition of the great arteries are a group of congenital heart defects with abnormalities of the outflow tracts and great vessels. It is common for the ascending aorta and aortic root to be significantly dilated following initial repair of the conotruncal anomaly, and little information is available on the management of this increasing problem. Although there are few case reports of aortic dissection and rupture in the literature, it appears to be rare in the setting of a conotruncal anomaly and may be related to ...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Joseph A. Dearani, Harold M. Burkhart, John M. Stulak, Thoralf M. Sundt, Hartzell V. Schaff Tags: Presentations from the 2008 AATS Meeting Source Type: journals

Management of Older Single Functioning Ventricles With Outlet Obstruction due to a Restricted “VSD” in Double Inlet Left Ventricle and in Complex Double Outlet Right VentricleEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The occurrence of a restriction of the bulbo-ventricular foramen (BVF) in older patient with double inlet left ventricle (DILV) or tricuspid atresia (TA) with ventriculo-arterial discordance is a well-known condition. Today, the surgical management is to perform a Damus-type operation at the time of the bi-directional Glenn or at the Fontan completion. The ventricular septal defect (VSD) enlargement, associated with muscular resection and a patch enlargement of the subaortic accessory ventricular chamber, is rarely performed but remains indicated in cases with pulmonary valve atresia or regurgitation. This condition is ess...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Francois Lacour-Gayet Tags: Presentations from the 2008 AATS Meeting Source Type: journals

Repair, Replacement, Ross: How I Approach the Older Child With Mixed Aortic Stenosis/Aortic InsufficiencyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Most children with aortic valve disease present with isolated aortic regurgitation or stenosis, in which case valve repair is often possible, thus delaying or eliminating the need for valve replacement. In the child with mixed aortic stenosis and regurgitation, repair is often more complex and less successful, requiring replacement of the valve and/or root. Several elements require careful consideration in children including growth potential of the child, risk of future reoperations, and the need for anticoagulation. A formal decision tree in this context is difficult because of the high variability between patients and pa...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Ismail El-Hamamsy, Magdi H. Yacoub Tags: Presentations from the 2008 AATS Meeting Source Type: journals

Pulmonary Atresia, VSD and Mapcas: Repair Without UnifocalizationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The last three decades have seen considerable development in the management of infants with pulmonary atresia, ventricular septal defect, and major aorto-pulmonary collaterals. The technical difficulty of surgical treatment lies in the fashioning of a pulmonary vascular bed capable of providing suitably low right ventricular pressure for repair of the intracardiac defect. With the current trend toward early and complete unifocalization of major aortopulmonary collateral arteries (mapcas), we describe an alternative approach - “repair without unifocalization” - and explain our reasoning for pursuing this technique.
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Christian P. Brizard, Matthew Liava'a, Yves d'Udekem Tags: Presentations from the 2008 AATS Meeting Source Type: journals

Surgical Management of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals: A Protocol-Based ApproachEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We describe a management protocol that has been developed to handle the highly variable nature of pulmonary blood flow observed with this lesion.
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: Sunil P. Malhotra, Frank L. Hanley Tags: Presentations from the 2008 AATS Meeting Source Type: journals

How We Manage Patients With Major Aorta Pulmonary CollateralsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Patients with major aortopulmonary collateral arteries usually present in one of three ways: either with marked heart failure because of lung overflow, cyanotic because of reduced lung flow, or fairly well balanced with systemic oxygen saturations in the high 70s to low 80s. All patients require a planned cardiologic surgical approach, with careful investigation to delineate the collateral morphology. A carefully coordinated, combined approach between surgery and cardiology intervention is required throughout the treatment of these patients. The majority of these patients now enter a program of reconstruction of the collat...
Source: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual - June 25, 2009 Category: Cardiovascular & Thoracic Surgery Authors: William J. Brawn, Timothy Jones, Ben Davies, David Barron Tags: Presentations from the 2008 AATS Meeting Source Type: journals