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To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jtcvs.2006.03.053 Byline: Tirone E. David, Christopher M. Feindel, Gary D. Webb, Jack M. Colman, Susan Armstrong, Manjula Maganti Abbreviations: AI, aortic insufficiency; PTFE, polytetrafluoroethylene Abstract: To examine the results of aortic valve sparing for aortic root aneurysm. Author Affiliation: Divisions of Cardiovascular Surgery and Cardiology of Toronto General Hospital and University of Toronto, Toronto, Ontario, Canada. Article History: Received 5 February 2006; Revised 19 March 2006; Accepted 23 March 2006

This article will endeavour to accomplish four things: to point out some of the more common challenges in the assessment and management of adult patients with congenital heart defects (CHD); [...]

Byline: Erwin N. Oechslin, David A. Harrison, Louise Harris, Eugene Downar, Gary D. Webb, Samuel S. Siu, William G. Williams Abstract: Objective: The purpose of this study is to review indications, surgical procedures, and outcomes in adults with repaired tetralogy of Fallot referred for reoperation. Method: Sixty consecutive adults (age [greater than or equal to] 18 years) who underwent reoperation between 1975 and 1997 after previous repair of tetralogy of Fallot were reviewed. Mean age at corrective repair was 13.3 [+ or -] 9.6 years and at reoperation 33.3 [+ or -] 9.6 years. Mean follow-up after reoperation is 5.0 [+ or -] 4.9 years. Results: Long-term complications of the right ventricular outflow tract (n = 45, 75%) were the most common indications for reoperation: severe pulmonary regurgitation (n = 23, 38%) and conduit failure (n = 13, 22%) were most frequent. Less common indications were ventricular septal patch leak (n = 6) and severe tricuspid regurgitation (n = 3). A history of sustained ventricular tachycardia was present in 20 patients (33%) and supraventricular tachycardia occurred in 9 patients (15%). A bioprosthetic valve to reconstruct the right ventricular outflow tract was used in 42 patients. Additional procedures (n = 115) to correct other residual lesions were required in 46 patients (77%). There was no perioperative mortality. Actuarial 10-year survival is 92% [+ or -] 6%. At most recent follow-up, 93% of the patients are in New York Heart Association classification I or II. Sustained ventricular tachycardia occurred in 4 patients (7%) during follow-up. Conclusions: Long-term complications of the right ventricular outflow tract were the main reason for reoperation. Mid-term survival and functional improvement after reoperation are excellent. (J Thorac Cardiovasc Surg 1999;118:245-51) Article History: Received 27 May 1998; Revised 31 July 1998; Revised 1 October 1998; Accepted 2 April 1999 Article Note: (footnote) [star] From the Toronto Congenital Cardiac Centre for Adults, The Toronto Hospital, Toronto, Ontario, Canada., [star][star] Erwin Oechslin was supported by the Faculty of Medicine, University of Zurich, and the Stiefel Zangger Foundation, Zurich, Switzerland., a Address for reprints: William G. Williams, MD, Hospital for Sick Children, 555 University Ave, Toronto, ON M5G 1X8, Canada., aa 0022-5223/99 $8.00 + 0 12/1/99099

The growing body of medical literature in pediatric cardiology has made it increasingly difficult for individual providers to stay abreast of the most current, meaningful articles to help guide practice. Crowdsourcing represents a collaborative process of obtaining information from a large group of individuals, typically from an online or web‐based community, and could serve a potential mechanism to pool individual efforts to combat this issue. This study aimed to utilize crowdsourcing as a novel way to generate a list of the most relevant, current publications in congenital heart disease, utilizing input from an international group of professionals in the field of pediatric cardiology. All members of the PediHeartNet Google group, an international email distribution list of medical professionals with an interest in pediatric cardiology, were queried in 2017 to submit literature that they considered to be most relevant to their current practice. A Google Form submission platform was used. The articles were evaluated by a multi‐institutional panel of four experts in pediatric cardiology using the Delphi method via an electronic evaluation form until a consensus was reached regarding whether the article merited inclusion in the final list. In total, 260 articles were submitted by members of the PediHeartNet Google group. Expert review using the Delphi method resulted in a list of 108 articles. The final collection of articles was published on a publicly available educational website. Crowdsourcing represents a novel approach for generating a high‐yield, comprehensive, yet practical list of the most relevant recent publications in pediatric cardiology. The same techniques could be easily applied to any medical subspecialty. By enlisting the input of frontline providers, the value and relevance of such a list will be significant. A web‐based platform for publication of the list allows for real‐time updates to ensure continued relevance.

Objective. Hypoplastic left heart syndrome (HLHS) is a relatively common complex congenital heart defect. Prior to development of staged reconstruction (i.e., Norwood procedure), HLHS was almost universally fatal within months of birth. Early survivors of the Norwood procedure are now reaching reproductive age. We report successful pregnancies in two such women.

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease, and one of the first to be successfully repaired by congenital heart surgeons. Since the first procedures in the 1950s, advances in the diagnosis, perioperative and surgical treatment, and postoperative care have been such that almost all those born with tetralogy of Fallot can now expect to survive to adulthood. The startling improvement in outcomes for babies born with congenital heart disease in general—and for those with tetralogy of Fallot in particular—is one of the success stories of modern medicine. Indeed, in many countries adults with tetralogy of Fallot outnumber children. Consequently, new issues have emerged, ranging from hitherto unpredicted medical complications to issues with training for caregivers and resource allocation for this population of survivors. Therefore, evolution of treatment, recognition of late complications, research on disease mechanisms and therapies—with feedback to changes in care of affected children born nowadays—are templates on which the timely discussion of organisation of care of those affected by congenital heart diseases from the fetus to the elderly can be based. Here, we focus on new developments in the understanding of the causes, diagnosis, early treatment, and late outcomes of tetralogy of Fallot, emphasising the continuum of multidisciplinary care that is necessary for best possible lifelong treatment of the 1% of the population born with congenital heart diseases.

Individuals with repaired tetralogy of Fallot (TOF) comprise a substantial proportion of the current adult congenital heart disease population. Pulmonary regurgitation (PR) is one of the most prevalent postoperative sequelae, but timing of pulmonary valve replacement (PVR) in the asymptomatic TOF patient remains controversial.

Document the frequency of substance use and oral hygiene among adolescents and young adults with moderate to complex congenital heart disease (CHD).

22q11 Deletion Syndrome (22q11DS) is a common microdeletion syndrome with multisystem expression. Phenotypic features vary with age, ascertainment, and assessment. We systematically assessed 78 adults (36 M, 42 F; mean age 31.5, SD 10.5 years) with a 22q11.2 deletion ascertained through an adult congenital cardiac clinic (n = 35), psychiatric‐related sources (n = 39), or as affected parents of subjects (n = 4). We recorded the lifetime prevalence of features requiring attention, with 95% confidence intervals (CI) not overlapping zero. Subtle learning difficulties, hypernasality and facial gestalt were not included. We investigated ascertainment effects using non‐overlapping subgroups ascertained with tetralogy of Fallot (n = 31) or schizophrenia (n = 31). Forty‐three features met inclusion criteria and were present in 5% or more patients, including several of later onset (e.g., hypothyroidism, cholelithiasis). Number of features per patient (median 9, range 3–22) correlated with hospitalizations (P = 0.0002) and, when congenital features were excluded, with age (P = 0.02). Adjusting for ascertainment, 25.8% (95% CI, 9.5–42.1%) of patients had cardiac anomalies and 22.6% (95% CI, 7.0–38.2%) had schizophrenia. Ascertainment subgroups were otherwise similar in median number and prevalence of features. Non‐characteristic features are common in 22q11DS. Adjusting for ascertainment effects is important. Many treatable conditions may be anticipated and features may accumulate over time. The results have implications for clinical assessment and management, genetic counseling and research into pathophysiological mechanisms. © 2005 Wiley‐Liss, Inc.

As patients with congenital heart disease increase in number and age, arrhythmia is becoming a more prevalent and important clinical problem. Although catheter-based therapy has revolutionized the management of arrhythmia, there remains an increasing patient population with congenital heart disease presenting for repair or reoperation with associated atrial or ventricular arrhythmias. Arrhythmia ablation may be safely and effectively included as an adjunct to repair of underlying structural cardiac lesions. Successful electrophysiologic surgery requires accurate preoperative characterization of the arrhythmia. Copyright 2003 Elsevier, Inc. All rights reserved.

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Background. Congenital absence of the pericardium (CAP) is a rare clinical entity.

Interventions in Adults with Congenital Heart Disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigor to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies between from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision making. Excluded are recommendations for pacemakers, defibrillators and arrhythmia directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As a data grows on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons and patients making complex decisions relative to ACHD interventions.

Transposition complex refers to the reversal of the normal connection of the ventricles to the great arteries, and includes both complete transposition of the great arteries (TGA) and congenitally corrected TGA. Adults with complete TGA usually have had an atrial switch (Mustard or Senning), procedures now abandoned in many pediatric centers in favor of the arterial switch (Jatene). The course and treatment of patients with congenitally corrected TGA is much more variable, depending on which associated lesions are present. Such patients share the tendency to complete heart block, systemic tricuspid AV valve regurgitation, and systemic RV dysfunction.

We sought to assess the clinical outcome, functional status and complications of adult patients with congenitally corrected transposition of the great arteries.

The clinical status and exercise assessment of adult patients late after the Fontan operation were reviewed to determine cardiovascular function.

Objectives and Background. Sudden death has long been considered a major contributor to mortality in pediatric patients with corrected tetralogy of Fallot. However, this may not apply to the patient with repaired tetralogy of Fallot who has survived into adulthood. Consequently we followed up a cohort of such adults to establish the clinical outcome and risk factors affecting their survival.

Pulmonary valve incompetence is usually well tolerated after tetralogy of Fallot repair but may result in late progressive right heart failure as manifested by increasing fatigue, dyspnea, and frequently arrhythmias.

Objectives. We sought to determine the features associated with sustained monoform ventricular tachycardia (VT) in adult patients late after repair of tetralogy of Fallot (TOF) and to review their management.

The effect of prolonged support of the systemic circulation by the right ventricle was assessed in adult survivors of the Mustard operation. Forty five adult patients (32 male) aged 21.7 +/-3.3 years (mean +/-S.D) were studied. They had undergone Mustard's operation for correction of complete transposition 19.1 +/-2.6 years previously at 2.6 +/-3.1 years of age. Cardiopulmonary function was assessed using maximal bicycle ergometry with respiratory mass spectrometry. Systemic ventricular ejection fraction (SVEF) was measured at rest (r) and with exercise (ex) by radionuclide angiography. Thirtyfour patients additionally had magnetic resonance imaging to measure ventricular dimensions. Results were compared with normals [mean (SD)]Max. Work load kpmVO2 maxATSVEF (r)SVEF (ex)SVThSVD% NYHA 1 or 2Mustard673*17.1*11.5*45.4*51.1*14.0*40.4+92(179)(4.9)(4.0)(10.9)(14.7)(3.7)(7.4)Normal111743.424.259.970.69.332.5100(190)(10.1)(4.7)(8.7)(7.7)(1.0)(3.5)AT=anaerobic threshold, VO2 max=maximal oxygen consumption, SVD=systemic ventricular diameter (mm), SVTh=wall thickness (mm)*p<0.01+p<005