Congenital Cardiac Lesions Research Articles

Evaluation of the Cyanotic Newborn: Part 2—A Cardiologist’s Perspective

Congenital heart disease is a major cause of cyanosis in the newborn, which should be considered and recognized promptly. Careful history and meticulous physical examination along with appropriate investigations are essential for the diagnosis of cyanotic congenital heart diseases. Although cardiac echocardiography is the diagnostic choice for congenital cardiac lesions, many centers may not have it readily available. In such cases, use of pulse oximetry, the hyperoxia test, chest radiography, and electrocardiograpy will usually differentiate heart disease from other causes of cyanosis. If a ductal-dependent lesion is suspected, the immediate initiation of prostaglandin E1 infusion may be life-saving. Understanding the pathophysiology of cyanotic congenital heart diseases is crucial in the initial management in such settings. A high index of suspicion must be maintained for the appropriate management and institution after birth-saving measures.

IMPROVING RATE OF FETAL DIAGNOSIS OF COARCTATION OF THE AORTA IN ALBERTA. DOES IT RELATE TO OBSTETRIC SCREENING GUIDELINES?

Coarctation of the aorta is one of the most difficult congenital cardiac lesions to detect in the fetus, and despite postnatal oximetry screening programs, remains the critical heart defect most likely to be undetected prior to neonatal discharge from hospital after birth. Since 2009 new formal Canadian obstetric ultrasound guidelines have been implemented that mandate evaluation of the ventricular outflow tracts and encourage views of the great arteries and arches. In 2013, the International Society of Ultrasound in Obstetrics and Gynaecology recommended further imaging where the ventricular outflow tracts could not be confirmed as normal. The rate of fetal detection of coarctation of the aorta in Alberta has not been audited over this era.

Therapeutic Utilities of Pediatric Cardiac Catheterization.

In an era when less invasive techniques are favored, therapeutic cardiac catheterization constantly evolves and widens its spectrum of usage in the pediatric population. The advent of sophisticated devices and well-designed equipment has made the management of many congenital cardiac lesions more efficient and safer, while providing more comfort to the patient. Nowadays, a large variety of heart diseases are managed with transcatheter techniques, such as patent foramen ovale, atrial and ventricular septal defects, valve stenosis, patent ductus arteriosus, aortic coarctation, pulmonary artery and vein stenosis and arteriovenous malformations. Moreover, hybrid procedures and catheter ablation have opened new paths in the treatment of complex cardiac lesions and arrhythmias, respectively. In this article, the main therapeutic utilities of cardiac catheterization in children are discussed.

Outcomes of deep hypothermic circulatory arrest in pediatric cardiac surgery: A single center experience

BackgroundDeep hypothermic circulatory arrest (DHCA) is a technique used in the repair of complex congenital cardiac lesions that require aortic arch or pulmonary vein repair. DHCA has been linked to adverse outcomes and neurologic complications. Selective cerebral perfusion (SCP) may be added to DHCA to prevent neurological complication. Air embolism and hyperperfusion injury may be encountered. The aim of this study was to evaluate the safety and efficacy of simple DHCA and to outline the early outcomes especially the neurological ones. MethodsTwenty nine patients underwent surgical repair of congenital cardiac lesion with DHCA at a single institution from January 2010 to November 2015. DHCA was conducted with a target esophageal temperature of 18° and placement of an ice pack on the head. No selective perfusion was done. Demographic, operative and postoperative data were reviewed. Mortality, any neurological complications including seizers, coma, and stroke were recorded. ResultsThe mean age was 20.6 ± 8.2 months (range: 9 days to 154 months). The majority were males (20, 69%). The mean weight was 5.57 ± 4.2 kg (range: 2.3–17.5 kg). DHCA time was 20.03 min (range 3–52 min). There were three (10.3%) deaths. Two deaths occurred after Norwood operation, and one after interrupted aortic arch repair. None of the deaths were related to neurological injury. None of the patients developed seizers, coma, abnormal movement or neurological deficits. ConclusionsSimple DHCA without SCP is a safe, expeditious and reliable method for brain protection during repair of complex cardiac lesions, with acceptable outcomes.

Shunt Lesions Part I: Patent Ductus Arteriosus, Atrial Septal Defect, Ventricular Septal Defect, and Atrioventricular Septal Defect

This review summarizes the current understanding of the pathophysiology and perioperative management of patent ductus arteriosus, atrial septal defect, ventricular septal defect, and atrioventricular septal defect. MEDLINE and PubMed. The four congenital cardiac lesions that are the subject of this review, patent ductus arteriosus, atrial septal defect, ventricular septal defect, and atrioventricular septal defect, are the most commonly found defects causing a left-to-right shunt. These defects frequently warrant transcatheter or surgical intervention. Although the perioperative care is relatively straightforward for many of these patients, there are a number of management strategies and complications associated with each intervention. The treatment outcomes for all of these lesions are very good in the current era.

A 20-year study on treating childhood infective endocarditis with valve replacement in a single cardiac center in China.

Children with infective endocarditis (IE) have to undergo valve replacement instead of valve repair in China due to severe valve damage. The present study is to review our experience on surgical treatment of children with IE in reference to the incidence, pathologic status, diagnosis, surgical strategies and outcomes. We reviewed 35 patients with a mean age of 13.7±2.2 years who were underwent valve replacement surgery for IE during the period from January 1993 to December 2013. Preoperative transthoracic echocardiographic (TTE) evaluation and transesophageal echocardiography during operation were performed in all patients. All the children underwent chart review and retrospective risk-hazard analysis. Among the patients surveyed congenital cardiac lesions were present in 15 (42.8%), rheumatic heart valve disease in 2 (5.7%) and previous heart surgery in 2 (5.7%). The median stay of intensive care unit was 6 days. Intraoperative findings showed that the endocarditis involved mostly the mitral and aortic valves (88.5%). Triple or quadruple valve involvement was found in one patient each. Ten-year freedom from IE-related death and re-intervention was 94.2% and 91.6%, respectively. Children undergoing surgery for IE frequently have advanced disease with embolic complications. Although valve replacement is not the primary option for pediatric IE, the rate of 5-year survival and freedom from re-operation was optimal prognostically. Pediatric physicians should pay attention to the common clinical features of IE so that the native valve is preserved well.

16. Optimal guidance of percutaneous device closure of PDA by transthoracic echocardiography

Patent ductus arteriosus (PDA) is common congenital cardiac lesion and the most accepted way of management is transcatheter occlusion by device which is usually done under fluoroscopy guidance. Transoesophageal echo cardiography and transaortic imaging were used in adult to guide the procedure which is with certain applications in pediatric age group transthoracic echocardiography (TTE) in pediatric population provides excellent images for PDA and may replace the use of fluoroscopy to guide PDA closure at least in special situations. To highlight the feasibility of device closure under guidance of TTE to be applied in sick patients who are not suitable for transfer to cardiac catheter laboratory or those with contraindication to contrast and or radiation application. 18 patients from July 2013 to May 2015 underwent TTE guidance device closure of PDA, 1 patient was excluded after device embolization which necessitate retrieval under fluoroscopy (fluoro.). Conscious sedation was used in 17 patients except 1 who was sick and already ventilated in ICU, there were 11 female and 7 male, antegrade approach was used in 10 patients with partial fluoro and retrograde approach in 8 patients without fluoro. Median age is 7 months, median weight is 8 kg (3.2–11 kg), 2 patients with renal impairment, 2 with Leukemia, median procedure time is 35 min, median fluoro. is 2.2 min, PDA size were small in 13 patients and moderate in 5, immediate closure is achieved in all. The devices were ADOI, ADOS, ADOII, Occlutech, and AVP2. Device embolization in 1 with successful retrieval and second device was used with complete closure percutaneous PDA closure under TTE guidance is feasible, safe and recommended in selected patients with certain situation.

Mesenchymal stem cells preserve neonatal right ventricular function in a porcine model of pressure overload.

Limited therapies exist for patients with congenital heart disease (CHD) who develop right ventricular (RV) dysfunction. Bone marrow-derived mesenchymal stem cells (MSCs) have not been evaluated in a preclinical model of pressure overload, which simulates the pathophysiology relevant to many forms of CHD. A neonatal swine model of RV pressure overload was utilized to test the hypothesis that MSCs preserve RV function and attenuate ventricular remodeling. Immunosuppressed Yorkshire swine underwent pulmonary artery banding to induce RV dysfunction. After 30 min, human MSCs (1 million cells, n = 5) or placebo (n = 5) were injected intramyocardially into the RV free wall. Serial transthoracic echocardiography monitored RV functional indices including 2D myocardial strain analysis. Four weeks postinjection, the MSC-treated myocardium had a smaller increase in RV end-diastolic area, end-systolic area, and tricuspid vena contracta width (P < 0.01), increased RV fractional area of change, and improved myocardial strain mechanics relative to placebo (P < 0.01). The MSC-treated myocardium demonstrated enhanced neovessel formation (P < 0.0001), superior recruitment of endogenous c-kit+ cardiac stem cells to the RV (P < 0.0001) and increased proliferation of cardiomyocytes (P = 0.0009) and endothelial cells (P < 0.0001). Hypertrophic changes in the RV were more pronounced in the placebo group, as evidenced by greater wall thickness by echocardiography (P = 0.008), increased cardiomyocyte cross-sectional area (P = 0.001), and increased expression of hypertrophy-related genes, including brain natriuretic peptide, β-myosin heavy chain and myosin light chain. Additionally, MSC-treated myocardium demonstrated increased expression of the antihypertrophy secreted factor, growth differentiation factor 15 (GDF15), and its downstream effector, SMAD 2/3, in cultured neonatal rat cardiomyocytes and in the porcine RV myocardium. This is the first report of the use of MSCs as a therapeutic strategy to preserve RV function and attenuate remodeling in the setting of pressure overload. Mechanistically, transplanted MSCs possibly stimulated GDF15 and its downstream SMAD proteins to antagonize the hypertrophy response of pressure overload. These encouraging results have implications in congenital cardiac pressure overload lesions.

Clinical characteristics and outcomes in pregnant women with Ebstein anomaly at the time of delivery in the USA: 2003–2012

Ebstein anomaly is an uncommon congenital cardiac lesion that may be associated with cyanosis, arrhythmias and right heart dysfunction. Investigation into patient characteristics and outcomes in pregnant women with Ebstein anomaly has been limited. To characterize patient characteristics and clinical events for pregnant women with Ebstein anomaly during hospitalization for delivery in the USA; also, to determine the effect of Ebstein anomaly on maternal clinical outcomes and individual predictors of poor outcome at time of delivery. We screened the Healthcare Cost and Utilization Project's National Inpatient Sample for hospital admissions of pregnant women for delivery (vaginal or caesarean section) in the USA from 2003-2012, and identified a cohort of 7,850,381. Clinical characteristics and maternal outcomes were identified in those with and without Ebstein anomaly. The primary outcome of interest was major adverse cardiac events (MACE), a composite of in-hospital death, acute myocardial infarction, cerebrovascular events, embolic events, cardiac complications of labour and delivery heart failure or arrhythmia. Our study population consisted of 82 hospitalizations of pregnant women with Ebstein anomaly and 7,850,299 without. The Ebstein cohort more frequently had ostium secundum-type atrial septal defect and/or patent foramen ovale and anomalous atrioventricular excitation (P<0.001 for both). The MACE rate was significantly higher among Ebstein patients (P<0.001). Preterm delivery, postpartum haemorrhage and caesarean delivery occurred more frequently among the Ebstein cohort (19.5% vs 7.2%, 8.5% vs 2.8% and 47.6% vs 31.1%, respectively; P≤0.001). In a multivariable analysis, anomalous atrioventricular excitation (odds ratio [OR] 21.75, 95% confidence interval [CI] 1.03-457.91) and preterm delivery (OR 11.71, 95% CI 1.39-98.89) were associated with MACE among those with Ebstein anomaly. Pregnant women with Ebstein anomaly are at higher risk of MACE during pregnancy and delivery. Preterm delivery occurred more frequently in women with Ebstein anomaly.

Hemodynamically Significant Congenital Cardiac Lesions in Pregnancy.

The incidence of congenital cardiac disease among reproductive-aged women is increasing. Understanding the unique physiology of pregnancy and the postpartum period is critical to helping women achieve successful pregnancy outcomes. Risk assessment models estimate the cardiac, obstetric, and neonatal risks a woman may face and influence the conversations regarding pregnancy and contraception management. This review focuses on some of the most common congenital cardiac lesion encountered during pregnancy, as well as key aspects of antepartum, intrapartum, and postpartum care for these women. A multidisciplinary team, with Maternal-Fetal Medicine, Cardiology and Obstetric Anesthesiology specialists, is critical to the care of these patients.

Clinical and Functional Developmental Outcomes in Neonates Undergoing Truncus Arteriosus Repair: A Cohort Study

Truncus arteriosus (TA) is an uncommon congenital cardiac lesion that portends an exceedingly poor prognosis if not repaired. The objective of this study was to assess the clinical and developmental outcomes in a prospective cohort of patients who underwent TA repair. All patients who underwent a TA repair between 1996 and 2012 were included. Follow-up clinical, neurologic, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Functional developmental outcomes were assessed at 21.1 ± 2.5 months of age with the Adaptive Behavior Assessment System-II, General Adaptive Composite (GAC) score. Survival and outcomes were compared between those with and without chromosomal abnormalities (CA). Survival and freedom from reintervention were assessed by Kaplan-Meier analysis. The study comprised 36 infants (19 male). CA was identified in 13, with 22q11.2 deletion in 10 patients. Patients underwent TA repair at a median age of 10 days; 5patients underwent concomitant interrupted arch repair. There were 8 deaths, 2 of which occurred in the hospital. The 5-year survival was 79.4%. Survival was similar between those with and without CA. At 5 years, freedom from reoperation was 77.2%. The mean GAC was higher in the patients without CA (93.6 ± 12.8 vs 76.1 ± 13.1, p= 0.0016). Patients with surgically repaired TA continue to have significant postoperative mortality. Reoperation and cardiac catheterization are eventualities for a quarter of patients in the first 5 years of life. Functional developmental outcome in patients without CA is good, although it is significantly impaired in those with CA.

Maximillian Muenke, Paul Kruszka, Craig A. Sable, John W. Belmont (eds): Congenital heart disease molecular genetics, principles of diagnosis and treatment

dedicated to chromosomal disorders with specific chapters on Down syndrome, Turner syndrome and the 22q11 deletion syndrome. Section 4 moves to discuss single gene disorders such as the Noonan RAS MAPK pathway syndromes and transcription factor-related heart conditions such as Holt–Oram syndrome and heterotaxy. In each chapter, there is a clear description of the genetic aetiology and embryogenesis and interaction between genotype and phenotype. Section 5 is dedicated to Arrhythmias, Cardiomyopathies and Connective Tissue disorders. This is a vast subject area to cover but the expert authors do well in providing concise up to date reviews which blend genetic and clinical data and provide an overview and understanding of pathogenesis. Section 6 is dedicated to evaluation. The initial chapter describes the genetic workup for congenital heart disease and the authors include a useful algorithm to aid the clinician in developing an approach to genetic workup. Various genetic techniques such as targeted gene sequencing, whole-genome and whole-exome sequencing are described and there are helpful sections on copy number and structural variants and their role in congenital heart disease. This particular chapter is a very helpful synopsis of modern genetic techniques for the non-geneticist. This chapter is followed by an equivalent chapter on cardiac imaging for the non-cardiologist. Although it purports to cover imaging in general, the main text is dedicated to a description of echocardiography as an imaging tool. An approach to the echocardiographic assessment of common congenital cardiac lesions is given. Whilst echocardiography is the prime diagnostic tool in children with congenital heart disease, future editions might also include other modalities such as magnetic resonance imaging which are of increasing value in older patients. A brief chapter on foetal diagnosis and intervention follows which gives a concise summary for the non-specialist. The final section is In the era of information overload with rapid electronic access to ever-changing scientific information, one might ask what is the purpose of a new textbook? More specifically, what is the purpose of a new textbook in a subject which is developing as rapidly as the molecular genetics of congenital heart disease? This book edited by Muenke and colleagues is a multiauthor volume by a series of experts from diverse backgrounds ranging from interventional cardiology to molecular genetics and bioethics. Together they have produced an outstanding synthesis of current research and practice in the molecular genetics of congenital heart disease. However, the book covers a much broader range than implied by its title. The book is divided into seven sections. The opening section comprises a fascinating historical overview of the remarkable developments in diagnosis and treatment of congenital heart disease that have occurred over the last 350 years. This is followed by an up to date summary of cardiac development illustrated beautifully with colourful diagrams, which are a model of clarity and make a complex subject understandable. Section 2 covers the often neglected topic of epidemiology. There is an overview chapter followed by a chapter which emphasises how epidemiology and management differ in the developing world. This is followed by chapters describing non-genetic risk factors and the specific and rapidly increasing population of adults with congenital heart disease. Section 3 is

Dual Coronary Artery Fistula from Left Anterior Descending and Right Coronary Artery to Pulmonary Trunk in a Patient with Myocardial Infarction—A Case Report

Coronary artery fistulas (CAF) are precapillary communications between a coronary artery and a cardiac chamber or vessel. CAF have been described as the most common hemodynamically significant congenital coronary anomal. However, it remains a relatively uncommon clinical problem. Coronary fistulas originates slightly more common from the right than from the left coronary artery, but the bilateral fistulas—those that originate from both coronary arteries—accounts for only 5% of total cases. These bilateral fistulas have a unique tendency to terminate in the pulmonary artery. More than half of the bilateral and only 17% of unilateral fistulas, terminates in this manner [1]. CAF are believed to be embryological remnants of sinusoidal connections between the lumens of the primitive tubular heart. This was first described by Maude Abbott in 1908 [2]. These fistulas are usually discovered incidentally upon coronary angiography [3]. Their incidence in the overall population is reported about 0.002% and constitutes 0.13% of congenital cardiac lesion, however, they are found in 0.05% to 0.25% of patients who undergo coronary angiography. The most common site of drainage is the right ventricle seen in 41% of patients. Congenital CAFs usually result from abnormal embryological development of the myocardial vascular system. Acquired CAFs are seen after trauma, endovascular procedures like coronary angiography, endomyocardial biopsy etcor cardiac transplantation. True fistulas of the circulatory system are characterized by an ectatic vascular segment that shows aberrant flow connecting two vascular territories governed by large pressure differences. We report a case of double coronary to pulmonary artery fistula discovered during emergent coronary angiography for acute inferior wall ST-elevation myocardial infarction (STEMI) in a patient with no prior cardiac symptoms.

Valve-sparing aortic root replacement in adult patients previously operated for congenital heart defects: an initial experience.

To review our experience with valve-sparing aortic root replacement (VSARR) in adult patients after the previous surgery for congenital heart disease. From August 2008 to February 2014, 11 patients (mean age: 30.5 ± 7.7 years), previously operated for various congenital cardiac defects, mostly conotruncal lesions, underwent VSARR for progressive aortic root dilatation. Five patients had also developed moderate, and 1 patient severe aortic valve (AoV) insufficiency. All aortic root procedures were performed by the same operating surgeon in two institutions. The mean interval between the initial procedure and aortic root surgery was 25.7 ± 7.7 years. At the time of reoperation, the mean aortic root diameter was 53.4 ± 6.2 mm. The reimplantation technique (David procedure) was used in all patients. Seven patients underwent concomitant procedures. The median follow-up was 32 months (range 12-78 months). No mortality occurred in hospital or during the follow-up period. Ten patients remain in NYHA functional class I; 1 patient with a single-ventricle circulation is in NYHA functional class II. During the immediate postoperative period, 2 patients underwent drainage of a pericardial collection and 1 patient required renal replacement therapy with eventual full recovery of renal function. No other reoperations or reinterventions were needed during the follow-up period. The most recent echocardiogram in 10 patients have demonstrated no or trivial AoV insufficiency. One patient, however, with severe aortic insufficiency preoperatively, developed mild AoV regurgitation shortly after the operation, which has progressed to moderate regurgitation 1 year following the operation. Analysis of our experience in a small group of patients confirms that valve-sparing aortic root surgery can be safely performed in adult congenital patients presenting with progressive aortic root dilatation following their previous surgery. Although we have analysed patients with different original congenital cardiac lesions and the follow-up period is not long, we can conclude that the use of the reimplantation technique (David procedure) have resulted in overall satisfactory AoV function at follow-up. Longer follow-up with larger series will be needed, however, before firm conclusions can be drawn.

Surgical repair of isolated total anomalous pulmonary venous connection is linked to favourable outcomes.

The operative mortality for total anomalous pulmonary venous connection repair is considered high when coexists with other congenital cardiac lesions. The isolated form however warrants excellent surgical outcomes.

Left Atrial Isomerism in Afro-Caribbean Adolescents and Adults with no Intervention: Echocardiographic Findings.

Left atrial isomerism (LAI) is a rare congenital anomaly usually associated with major or minor congenital cardiac lesions. Presentation beyond childhood is unusual as there is usually high morbidity of 63% associated with the co-morbid major congenital cardiac lesions in the first year of life. The four index cases highlight the myriad cardiac lesions facilitating survival into adolescence and adulthood of this rare cardiac condition. Presented are markedly different echocardiographic findings of four cases of LAI in Afro-Caribbeans who had no intervention. Their diagnostic transthoracic echocardiographic findings are the focussed features of this article and the first documentation of isolated patent ductus arteriosus with LAI. Fascinating diagnostic chest X-ray findings of the LAI index cases are presented. They are the first cases of LAI documented in Afro-Caribbeans.

An Overview of Pulmonary Atresia and Major Aortopulmonary Collateral Arteries.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a rare and complex congenital cardiac lesion that has historically carried a poor prognosis. With advancements in surgical management, we have seen an improvement in the outcomes for children affected by this disease. However, this population continues to present challenges due to the complex anatomy and physiology associated with PA/VSD/MAPCA. This summary of material presented during one of the nursing sessions of the 2014 Meeting of the Pediatric Cardiac Intensive Care Society provides an overview for those in cardiac intensive care units who do not have a large experience with this lesion. We will review the anatomy, physiology, surgical approach, postoperative management strategies, and cardiac catheter intervention options for PA/VSD/MAPCAs. We will also discuss recent innovations that may lead to continued improvement in outcomes for this challenging patient population.

Primary Prevention of Sudden Cardiac Death in Adults with Transposition of the Great Arteries: A Review of Implantable Cardioverter-Defibrillator Placement.

Transposition of the great arteries encompasses a set of structural congenital cardiac lesions that has in common ventriculoarterial discordance. Primarily because of advances in medical and surgical care, an increasing number of children born with this anomaly are surviving into adulthood. Depending upon the subtype of lesion or the particular corrective surgery that the patient might have undergone, this group of adult congenital heart disease patients constitutes a relatively new population with unique medical sequelae. Among the more common and difficult to manage are cardiac arrhythmias and other sequelae that can lead to sudden cardiac death. To date, the question of whether implantable cardioverter-defibrillators should be placed in this cohort as a preventive measure to abort sudden death has largely gone unanswered. Therefore, we review the available literature surrounding this issue.

The Impact of Infective Endocarditis Prophylaxis Recommendations on the Practices of Pediatric and Adult Congenital Cardiologists

BackgroundCompliance with the April 2007 American Heart Association (AHA) infective endocarditis (IE) guidelines is not well described. We sought to evaluate the impact of these guidelines on the clinical practices of pediatric and adult congenital cardiologists. MethodsWe conducted a 2-centre retrospective cohort study, including patients who had at least 1 outpatient cardiology visit after April 1, 2008 and had 1 of 11 prespecified high- or low-risk congenital cardiac lesions. High-risk lesions required prophylaxis per the AHA guidelines; low-risk patients no longer required prophylaxis. Cardiology clinic letters were reviewed to determine if antibiotic prophylaxis and oral hygiene were addressed and whether prophylaxis was recommended. Comparisons were made before April 2007 vs at least 1 year after guideline publication. ResultsWe included 238 high-risk and 201 low-risk patients. IE prophylaxis was recommended for all study patients before April 2007. After April 2008, IE prophylaxis recommendations were reduced by 44.9% for low-risk patients (P < 0.0001) and 9.3% for high-risk patients (P = 0.0156). IE prophylaxis recommendations were documented in the medical record of 92% of patients (95% confidence interval, 88%-95%) before April 2007 vs 81% (77%-84%) after April 2008. Oral hygiene was emphasized for only 44% and 28% of patients before and after guideline revision (P = 0.0005). ConclusionsThe 2007 AHA guidelines resulted in significantly fewer recommendations for IE prophylaxis in low-risk patients. However, cardiologists continue to recommend prophylaxis for some low-risk patients and advise some high-risk patients against prophylaxis. These findings suggest disagreement or uncertainty within the cardiology community regarding current IE guidelines.

Neurodevelopmental outcome in children with congenital heart disease: a work in progress.

When I began my residency in 1977, treatment of children with congenital heart defects was evolving in the context of an inspiring collaboration between pediatric cardiology, cardiac surgery, and cardiac anesthesia. In the ensuing decades, thousands of infants were saved by novel imaging, new operations, and refined supportive methods. The impact on these children was, in many instances, not only lifesaving but even miraculous. A similar phenomenon was occurring nationally and internationally, and advances in diagnosis, treatment, surgical interventions, and support techniques changed faster than in perhaps any other pediatric field. By the mid-1980s, many previously fatal congenital cardiac lesions had become operable, and survival rates had improved. In addition, new surgical techniques were developed to repair even the most complex lesions during early infancy rather than in 2 stages (ie, first palliative, then later reparative surgery), and morbidity and mortality declined. Dazzling progress in the sophistication of surgical repair is illustrated nowhere better than by the transition of surgery for dextrotransposition of the great arteries from atrial switch surgery (eg, … Address correspondence to Leonard Rappaport, MD, MS, Boston Children’s Hospital, Fegan 10, 300 Longwood Avenue, Boston, MA 02115. E-mail: leonard.rappaport{at}childrens.harvard.edu