Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual

Preface

Richard A. Jonas

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.

Introduction

Marshall L. Jacobs

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 meeting was due to the efforts of WSPCHS President Christo Tchervenkov and of Dr. Jonas, the local host.

The World Society for Pediatric and Congenital Heart Surgery: Its Mission and History

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 period of time has become the largest organization in the world of pediatric and congenital heart surgeons. Its brief history already seems to be a guarantee of a promising future. Projects in the areas of research, training and education, patient care, and community service will allow the Society to reach its goals. By bringing together professionals from every region of the world, the WSPCHS should play a significant role in the improvement of care for children and adults with congenital heart disease around the world.

Pediatric Cardiac Surgery: A Challenge and Outcome Analysis of the Guatemala Effort

A large underserved population of children with congenital cardiac malformation (CCM) exists in many developing countries. In recent years, several strategies have been implemented to supplement this need. These strategies include transferring children to first-world countries for surgical care or the creation of local pediatric cardiovascular surgical programs. In 1997, an effort was made to create a comprehensive pediatric cardiac care program in Guatemala. 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.C.), seven pediatric cardiologists, 3 intensivists, and 2 anesthesiologists, as well as intensive care and ward nurses, respiratory therapists, echocardiography technicians, and support personnel. The cardiovascular program expanded in 2005 to 2 cardiac operating rooms, 1 cardiac catheterization laboratory, 1 cardiac echo lab, 4 outpatients clinics a 6-bed intensive care unit and a 4-bed stepdown unit, a 20 bed general ward (2 beds/room) and a genetics laboratory. Our center has become a referral center for children from Central America. A total of 2,630 surgical procedures were performed between February 1997 and December 2007, increasing the number of operations each year. Postoperative complication occurred in 523 of 2,630 procedures (20%). A late follow-up study was conducted of all the patients operated from 1997 to 2005. Late mortality was 2.7%. Development of a sustainable pediatric cardiac program in emerging countries presents many difficult challenges. Hard work, perseverance, adaptability, and tolerance are useful aptitudes to develop a viable PCP in an “emerging” country. We are not in favor of Medical-Surgical Safari efforts, unless these efforts include training of a local team and eventual unit independence. It helps if an experienced (± senior/retired!) surgeon leads this effort on a full-time, pro bono basis. Local and international fund raising is essential to complement vastly insufficient government subsidies.

Challenges and Progress of the Pediatric Cardiac Surgery in Shanghai Children's Medical Center: A 25-year Solid Collaboration With Project HOPE

Jinfen Liu

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 in the last 20 years has changed dramatically. Treatment for pediatric CHDs has been greatly improved and developed in China. The collaboration with Project HOPE, an international health professional education organization, has promoted the development of our specialty. Specifically, Project HOPE has played an important role in facilitating the collaboration between Shangahi Xin Hua Hospital, Shanghai Children's Medical Center, and many world-leading pediatric health care institutions. With contributions from Project HOPE in the last 25 years, cardiovascular surgeons at Shanghai Children's Medical Center have achieved tremendous progress. The landmark of the cardiac center and successful clinical data in treating children with cardiac illness symbolizes the successful collaboration among a state-owned hospital, a non-profit organization-Project HOPE, and multiple international corporations, which have provided valuable resources to facilitate the growth of the hospital into a state-of-the-art tertiary hospital to serve children and families.

The First Stella Van Praagh Memorial Lecture: The History and Anatomy of Tetralogy of Fallot

Richard Van Praagh

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 to clarify the pathologic anatomy, the clinical and laboratory diagnosis, and often the surgical management of many different forms of congenital heart disease, including dextrocardia, single ventricle, truncus arteriosus, tetralogy of Fallot (TOF), transposition of the great arteries, double-outlet right ventricle, sinus venosus defect, anomalous pulmonary venous drainage, the heterotaxy syndromes with asplenia or polysplenia, juxtaposition of the atrial appendages, and apical muscular ventricular septal defect. In 1999, Dr Stella Van Praagh received the Distinguished Achievement Award of the Society for Cardiovascular Pathology, and in 2004, she was honored with the Paul Dudley White Award of the American Heart Association. Dr. Stella Van Praagh was that vanishingly rare combination of brilliant clinician, internationally renowned medical scientist, and deeply cultivated humanist. The anomaly now known as the TOF was first described by Niels Stensen in 1671, with other early reports by Edouard Sandifort (1777), William Hunter (1784), and many others. In 1888, Etienne-Louis Arthur Fallot published five serialized contributions in Marseille Médical concerning what he called the “blue malady,” in which he described the now classical tetralogy of pulmonary outflow tract obstruction, ventricular septal defect, aortic overriding, and right ventricular hypertrophy. The other outstanding feature of Fallot's report was its emphasis on clinicopathologic correlation. In 1924, Maude Abbott coined the term “tetralogy of Fallot.” In 1970, Van Praagh and colleagues presented the concept that the TOF is basically just one anomaly, a failure of normal expansile growth of the subpulmonary infundibulum and its sequelae. The anatomy of TOF is presented angiocardiographically, diagrammatically, and anatomically. A morphometric study of typical neonatal TOF is presented, based on 16 autopsied heart specimens with age-matched normal controls. The morphometric study documents that TOF is characterized by a low-volume subpulmonary infundibulum. The diagnostic and surgical significance of these findings is highlighted. Two rare and recently discovered forms of TOF are presented: tetralogy {S,D,I}, and tetralogy {I,D,S}. Because tetralogy {I,D,S} has atrioventricular discordance, in addition to a standard TOF repair, such patients also need an inverted (mirror-image) atrial switch operation (inverted Senning or inverted Mustard procedure). Because associated malformations can be very important to the surgical outcome of patients with tetralogy, the associated anomalies found in 100 randomly selected autopsied cases are presented.

Early Primary Repair of Tetralogy of Fallot

Richard A. Jonas

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 pulmonary stenosis is virtually always operable. It is also a progressive condition that is therefore best repaired in early infancy following echocardiographic diagnosis alone.

Transatrial-Transpulmonary Repair of Tetralogy of Fallot

Massimo A. Padalino, Vladimiro L. Vida, Giovanni Stellin

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 preservation of the right ventricular function in the long term. In our experience, transatrial-transpulmonary repair of TOF has been feasible at any age with immediate results that are comparable to those performed later in infancy.

Tetralogy of Fallot Repair: The Right Ventricle Infundibulum Sparing (RVIS) Strategy

David L. Morales, Farhan Zafar, Charles D. Fraser

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 with a mini (< 5 mm) transannular patch or no ventricular incision. This strategy is applied with the ambition of decreasing the well-documented, long-term complications of TOF repair with large right ventriculotomies such as RV dilation, arrhythmias, need for pulmonary valve replacement, and RV failure. The RVIS strategy is an attempt based on our current knowledge and experience to optimize the time of repair so that we can not only maximize the early operative results but the long-term effects of this approach as these children mature into adolescents and adults. We have uniformly applied the RVIS strategy since 1995, which includes over 320 isolated TOF patients. We are currently reviewing this cohort in hopes that it will strengthen our beliefs and known results as well as give us more insight into whether the RVIS strategy can change the natural history of repaired TOF.

A Novel Approach to the Repair of Tetralogy of Fallot With Absent Pulmonary Valve and the Reduction of Airway Compression by the Pulmonary Artery

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 arteries anterior to the aorta and away from the trachea and bronchial tree. This technique has the potential to reduce or eliminate bronchial compression by the central pulmonary arteries. Failure of the treatment could be expected in symptomatic patients, where the pathology of the airways extends beyond the proximal pulmonary arteries.

How I Manage Neonatal Ebstein's Anomaly

Edward L. Bove, Jennifer C. Hirsch, Richard G. Ohye, Eric J. Devaney

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.

Transverse Aortic Arch Obstruction: When to Go from the Front

John W. Brown, Mark D. Rodefeld, Mark Ruzmetov

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.

Management of Systemic Outlet Obstruction in Patients Undergoing Single Ventricle Palliation

Charles D. Fraser

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 application to specific morphologic elements that make one more favorable than another in an individual patient.

Aortic Stenosis and Aortic Insufficiency in Children: Impact of Valvuloplasty and Modified Ross-Konno Procedure

Constantine Mavroudis, Carl L. Backer, Sunjay Kaushal

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 annular enlarging operation. We review our experience with the various forms of valvuloplasty, Ross operation, Konno operation, Ross-Konno operation, and the modified Ross-Konno operation, which we have used to eliminate heart block in patients who require an annular enlarging operation.

How I Manage Mitral Stenosis in the Neonate and Infant

Stephanie Fuller, Thomas L. Spray

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 replacement in the supraannular position remains the last option and typically requires re-replacement. Below we discuss our preferred management strategies for congenital mitral valve stenosis.

Fontan or Septation: When I Abandon Septation in Complex Lesions With Two Ventricles

Richard A. Jonas

As the long term results of the lateral tunnel Fontan procedure, as well the extracardiac Fontan procedure, continue to improve, the threshold for shifting to a single ventricle track in the setting of a complex and non-ideal biventricular repair alternative has been lowered. Two ventricle options that subject the child and family to multiple surgical procedures and lengthy hospitalizations, as well a limited cardiac output because of ventricular hypoplasia or AV valve hypoplasia, should be avoided. 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.

MR and CT Imaging of the Pediatric Patient With Structural Heart Disease

Frandics P. Chan

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.

Evaluation, Risk Stratification, and Management of Pulmonary Hypertension in Patients With Congenital Heart Disease

Jeffrey A. Feinstein

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 stratification and a generalized account of the current state of medical therapy.

Percutaneous Pulmonary Valve Implantation

Philipp Lurz, Regis Gaudin, Andrew M. Taylor, Philipp Bonhoeffer

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 an incidence around 20%. Although clinically silent in the majority of cases, stent fractures led to re-intervention in the form of implantation of a second device (valve-in-valve). Valvar function during follow-up was well maintained. Significant pulmonary regurgitation was only seen in the context of endocarditis. Pulmonary valve implantation has the potential to become the standard procedure in the treatment of dysfunctional conduits. Bigger challenges will now have to be met in order to extend this technology to patients with native outflow tracts and free pulmonary regurgitation.

How We Approach Peripheral Pulmonary Stenosis in Williams-Beuren Syndrome

Kim L. Gandy, James S. Tweddell, Andrew N. Pelech

Williams-Beuren syndrome is associated with supravalvar aortic stenosis and peripheral pulmonary artery stenosis in the majority of affected individuals. Among patients in whom surgery for supravalvar aortic stenosis is contemplated, stenosis of the branch pulmonary arteries is common. For asymptomatic patients with subsystemic right ventricular pressure, the natural history is favorable and no intervention is necessary. For patients with important proximal branch pulmonary artery, stenoses patch arterioplasty can be accomplished at the time of surgery for supravalvar aortic stenosis. For patients with important peripheral pulmonary stenosis, preoperative catheter-based therapy is indicated. Surgical repair of peripheral pulmonary stenosis is indicated when preoperative intervention is unsuccessful and can include a combination of patch arterioplasty as well as intraoperative application of catheter-based techniques. This report will address the approach to this lesion with catheter-based approaches, surgical approaches, and their combination.

Management of the Aortic Root in Adult Patients With Conotruncal Anomalies

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 the absence of hypertension and smoking in many of these patients. The timing of operation with regard to the size of the aortic root is difficult. In the absence of a family history of aortic dissection or aneurysm, or documented rapid growth of the ascending aorta, we proceed with replacement of the ascending aorta when the size is ≥ 55 mm. When the size of the ascending aorta is 5.0-5.5, treatment is individualized depending on the associated anomalies that need to be addressed, patient comorbidities, and life expectancy. In this group of patients we consider a simple reduction ascending aortoplasty. We generally proceed with root replacement and coronary reimplantation when there is effacement of the sinotubular junction, or when there is severe dilatation of the aortic root with an intact sinotubular junction. If the ascending aorta is ≥ 55 mm with an intact sinotubular junction and the sinuses are ≤ 4 cm, then we use a supracoronary tube graft.

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 Ventricle

Francois Lacour-Gayet

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 essentially prevented by doing an early Norwood-type operation in the presence of DILV/TA with transposition of the great arteries associated with an aortic arch obstruction. The palliative switch operation is an option that was abandoned because of poor control of the pulmonary blood flow. It is only in cases of large unobstructed BVF that pulmonary artery banding could be undertaken in neonates, followed by close echocardiographic follow-up. The occurrence of a restriction or a closure of the VSD in complex DORV following a Fontan operation is a dramatic event and is quite “new business.” It has been recently recognized that the VSD becomes restricted in a number of patients with DORV-nc-VSD treated with a Fontan palliation. This new condition is not surprising knowing that 75% of the VSDs must be enlarged preventively in DORV-nc-VSD repair. In the setting of a Fontan circulation, the supra-systemic left ventricle has severe consequences the right ventricle performance. Attempts at surgical VSD enlargement or catheter-based procedures have resulted in almost constant recurrence. This recently reported complication is in favor of also performing a VSD enlargement at the time of the Fontan completion in complex DORV. It justifies the biventricular repair in complex DORV with two viable ventricles.

Repair, Replacement, Ross: How I Approach the Older Child With Mixed Aortic Stenosis/Aortic Insufficiency

Ismail El-Hamamsy, Magdi H. Yacoub

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 pathologies and the lack of prospective randomized data. Nevertheless, we here present our approach to the child with mixed aortic stenosis and regurgitation, exploring the various options and explaining our favored approach.

Pulmonary Atresia, VSD and Mapcas: Repair Without Unifocalization

Christian P. Brizard, Matthew Liava'a, Yves d'Udekem

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.

Surgical Management of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals: A Protocol-Based Approach

Sunil P. Malhotra, Frank L. Hanley

Historically, outcomes of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals have been quite poor. Over the past 15 years, our group has strived to apply a consistent surgical strategy for this lesion based on two guiding principles: early unifocalization of all important collaterals; and the early establishment of a low-pressure pulmonary arterial bed, preferably using simultaneous intracardiac repair. We describe a management protocol that has been developed to handle the highly variable nature of pulmonary blood flow observed with this lesion.

How We Manage Patients With Major Aorta Pulmonary Collaterals

William J. Brawn, Timothy Jones, Ben Davies, David Barron

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 collaterals to a valved right ventricular pulmonary artery conduit with or without ventricular septal defect closure. Further catheter intervention to stretch and enlarge the pulmonary arteries may be necessary, followed by staged ventricular septal defect closure. Other techniques to enlarge central pulmonary arteries or to recruit collaterals can be used. Outcomes over the last 20 years have been satisfactory, with survival of 80% over 10 years, which is a marked improvement on the natural survival in this group of patients.

Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual

Table of Contents

Preface

Introduction

The World Society for Pediatric and Congenital Heart Surgery: Its Mission and History

Pediatric Cardiac Surgery: A Challenge and Outcome Analysis of the Guatemala Effort

Challenges and Progress of the Pediatric Cardiac Surgery in Shanghai Children's Medical Center: A 25-year Solid Collaboration With Project HOPE

The First Stella Van Praagh Memorial Lecture: The History and Anatomy of Tetralogy of Fallot

Early Primary Repair of Tetralogy of Fallot

Transatrial-Transpulmonary Repair of Tetralogy of Fallot

Tetralogy of Fallot Repair: The Right Ventricle Infundibulum Sparing (RVIS) Strategy

A Novel Approach to the Repair of Tetralogy of Fallot With Absent Pulmonary Valve and the Reduction of Airway Compression by the Pulmonary Artery

How I Manage Neonatal Ebstein's Anomaly

Transverse Aortic Arch Obstruction: When to Go from the Front

Management of Systemic Outlet Obstruction in Patients Undergoing Single Ventricle Palliation

Aortic Stenosis and Aortic Insufficiency in Children: Impact of Valvuloplasty and Modified Ross-Konno Procedure

How I Manage Mitral Stenosis in the Neonate and Infant

Fontan or Septation: When I Abandon Septation in Complex Lesions With Two Ventricles

MR and CT Imaging of the Pediatric Patient With Structural Heart Disease

Evaluation, Risk Stratification, and Management of Pulmonary Hypertension in Patients With Congenital Heart Disease

Percutaneous Pulmonary Valve Implantation

How We Approach Peripheral Pulmonary Stenosis in Williams-Beuren Syndrome

Management of the Aortic Root in Adult Patients With Conotruncal Anomalies

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 Ventricle

Repair, Replacement, Ross: How I Approach the Older Child With Mixed Aortic Stenosis/Aortic Insufficiency

Pulmonary Atresia, VSD and Mapcas: Repair Without Unifocalization

Surgical Management of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals: A Protocol-Based Approach

How We Manage Patients With Major Aorta Pulmonary Collaterals