Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association.

It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals.

2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias: executive summary.

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias.

2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias.

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias.

Abnormalities in serum biomarkers correlate with lower cardiac index in the Fontan population.

BACKGROUND: Fontan survivors have depressed cardiac index that worsens over time. Serum biomarker measurement is minimally invasive, rapid, widely available, and may be useful for serial monitoring. The purpose of this study was to identify biomarkers that correlate with lower cardiac index in Fontan patients. Methods and results This study was a multi-centre case series assessing the correlations between biomarkers and cardiac magnetic resonance-derived cardiac index in Fontan patients ⩾6 years of age with biochemical and haematopoietic biomarkers obtained ±12 months from cardiac magnetic resonance. Medical history and biomarker values were obtained by chart review. Spearman's Rank correlation assessed associations between biomarker z-scores and cardiac index. Biomarkers with significant correlations had receiver operating characteristic curves and area under the curve estimated. In total, 97 cardiac magnetic resonances in 87 patients met inclusion criteria: median age at cardiac magnetic resonance was 15 (6-33) years. Significant correlations were found between cardiac index and total alkaline phosphatase (-0.26, p=0.04), estimated creatinine clearance (0.26, p=0.02), and mean corpuscular volume (-0.32, p<0.01). Area under the curve for the three individual biomarkers was 0.63-0.69. Area under the curve for the three-biomarker panel was 0.75. Comparison of cardiac index above and below the receiver operating characteristic curve-identified cut-off points revealed significant differences for each biomarker (p<0.01) and for the composite panel [median cardiac index for higher-risk group=2.17 L/minute/m2 versus lower-risk group=2.96 L/minute/m2, (p<0.01)]. CONCLUSIONS: Higher total alkaline phosphatase and mean corpuscular volume as well as lower estimated creatinine clearance identify Fontan patients with lower cardiac index. Using biomarkers to monitor haemodynamics and organ-specific effects warrants prospective investigation.

Sudden cardiac arrest following ventricular fibrillation attributed to anabolic steroid use in an adolescent.

Anabolic androgenic steroids are synthetic derivatives of testosterone that promote the growth of skeletal muscles and have many recognised cardiovascular effects. We report the clinical presentation and pathological findings of an adolescent male whose sudden cardiac arrest following ventricular fibrillation was attributed to anabolic androgenic steroid use. The age of our patient reflects the usage of anabolic androgenic steroids among younger athletes and highlights the need for increased awareness among practitioners.

Incidence, Predictors, and Impact of Postoperative Acute Kidney Injury Following Fontan Conversion Surgery in Young Adult Fontan Survivors.

Acute kidney injury (AKI) is a common complication following single ventricle congenital heart surgery. Data regarding AKI following Fontan conversion (FC) surgery are limited. This study evaluated the incidence, predictors of, and prognostic value of AKI following FC. Single-center retrospective cohort study, including consecutive FC patients from December 1994 to December 2016. Medical records were reviewed. AKI was classified into AKI-1/AKI-2/AKI-3 using Kidney Disease: Improving Global Outcomes criteria. Multivariable logistic regression identified risk factors for AKI≥2. Chi-square and 2-sample t-tests assessed associations between AKI≥2 and postoperative outcomes. Mid-term heart-transplant-free survival among AKI0-1 vs AKI2-3 groups was compared using Kaplan-Meier curves and log-rank test. We included 139 FC patients: age at FC 24 (25th-75th, 19-31) years; 81% initial atrio-pulmonary Fontan; follow-up 8.3 ± 5.3 years following FC. Post-FC, 63 patients (45%) developed AKI (AKI-1 = 37 [27%]; AKI-2 = 10 [7%]; AKI-3 = 16 [11%]). AKI recovered by hospital discharge in 86%, 80%, and 19% of patients with AKI-1/AKI-2/AKI-3, respectively. Independent risk factors for AKI≥2 included older age (OR 1.07, 95%CI 1.01-1.15; P = 0.027); ≥3 prior sternotomies (OR = 6.11; 95%CI = 1.59-23.47; P = 0.009); greater preoperative right atrial pressure (OR 1.19; 1.02-1.38; P = 0.024), and prior catheter ablation procedure (OR 3.45; 1.17-10.18; P = 0.036). AKI≥2 was associated with: longer chest tube duration (9 [5-57] vs 7 [3-28] days; P = 0.01); longer mechanical ventilation time (2 [1-117] vs 1 [1-6] days; P = 0.01); greater need for dialysis (31% v s0%; P < 0.001); and longer postoperative length of stay (18 [8-135] vs 10 [6-58] days; P < 0.001). AKI 2-3 patients had worse mid-term heart-transplant-free survival. Half of the patients undergoing FC develop AKI. AKI 2-3 is associated with worse early postoperative outcomes and reduced mid-term transplant-free survival following FC. Knowledge of AKI predictors may allow for improved FC risk stratification, patient selection, and perioperative management in this high-risk population.

Extracardiac versus intra-atrial lateral tunnel fontan: extracardiac is better.

This article was prepared to summarize the points made in a debate that the first author (C.L.B.) had with Dr. Richard Jonas at the American Association for Thoracic Surgery 90th Annual Meeting. The topic of the debate was the optimal surgical approach for functional single-ventricle patients: extracardiac versus intra-atrial lateral tunnel Fontan. My role was to take the viewpoint that the extracardiac Fontan is better. This review summarizes our results at Children's Memorial Hospital (Chicago, IL) with 180 patients undergoing a primary Fontan procedure and 126 patients undergoing an extracardiac Fontan as part of a Fontan conversion. The world literature was reviewed on outcomes following the Fontan procedure, focusing on six main areas supporting the superiority of the extracardiac Fontan: hemodynamics, arrhythmias, applicability to complex anatomy, use of cardiopulmonary bypass, complications of fenestration and thromboembolism, and operative mortality. Based on this review, it is our conclusion that the extracardiac Fontan is the procedure of choice for patients with a functional single ventricle based on a very low operative mortality, a lower incidence of early and late arrhythmias, improved hemodynamics, fewer postoperative complications, and applicability to a wide variety of complex cardiac anatomy.

Late sinus and atrial tachycardia after pediatric heart transplantation might predict poor outcome.

Our objective was to examine clinical/electrocardiogram (ECG) predictors and outcomes of arrhythmias beyond 1 year after pediatric heart transplantation (HTx). We performed a retrospective chart review of 94 1-year HTx survivors, 1988-2006. Clinical records identified patients with arrhythmias occurring >1 year after HTx requiring pharmacotherapy, excluding acute rejection. We reviewed preoperative diagnosis, gender, age at HTx, operative details, transplant coronary artery disease (TCAD), and mortality. We analyzed serial ECGs after HTx for HR, PR, QRS, QT, and QTc intervals. Our results found complete data in 58 patients, 14 (24%) with arrhythmia and 44 controls. Arrhythmias occurred 1.1-17.9 years after HTx (mean = 6.8): 11 focal atrial tachycardia, 1 atrial fibrillation/flutter, 1 atrioventricular node reentry tachycardia; only 1 patient had ventricular tachycardia (VT). Serial ECG intervals were similar between groups, as well as surgical technique, ischemic time, and rejection history. Seven patients (50%) with arrhythmias had death or graft death versus 11% of the controls (P = 0.006). Patients with arrhythmias were more likely to be diagnosed with TCAD (P = 0.007). The patient with VT had no TCAD. In conclusion, supraventricular arrhythmias were frequent (22%) in 1-year survivors of pediatric HTx. These patients were more likely to develop TCAD and/or graft loss/mortality.

Perspective: Childhood Obesity Requires New Strategies for Prevention.

The prevalence of obesity among youth in the USA is currently >18% with projections that more than half of today's children will be obese as adults. The growth trajectory of children more likely to become obese is determined by weight in earliest childhood, and childhood body mass index (BMI) tracks through adolescence and adulthood. Childhood consequences of obesity include increased risk of asthma, type 2 diabetes mellitus, orthopedic disorders, and reduced academic performance. Health implications of obesity in adulthood include premature coronary artery disease, hypertension, type 2 diabetes, stroke, and certain cancers, contributing to the leading causes of adult mortality. Early childhood obesity is influenced by prenatal exposure to maternal obesity and environmental obesogens, and is associated with poverty, food insecurity, and poor nutritional quality. New strategies for primordial prevention of early childhood obesity require focusing attention on growth parameters during the first 2 y of life, with support for increasing the duration of breastfeeding, and improvements in dietary quality and availability, particularly the reduced consumption of added sugars. Reducing the prevalence of obesity among adolescent females and reducing exposure to environmental obesogens may reduce the prevalence of transgenerational obesity. The reduction of early childhood obesity could improve population health, quality of life, and longevity throughout the life course.

2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias: Executive summary.

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias.

2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias.

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias.

2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias: Executive summary.

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias.

2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias.

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias.

2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias: Executive summary.

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias.

Surgical advances in the treatment of adults with congenital heart disease.

PURPOSE OF REVIEW: This article reviews three common abnormalities requiring surgical treatment in adults with congenital heart disease (CHD): Ebstein's anomaly, the failing Fontan, and pulmonary valve disease. RECENT FINDINGS: As of 2000, more adults than children are alive with CHD. Each year, more of these adults with CHD undergo surgery. Three common operations performed on adults with CHD are surgery for Ebstein's anomaly, Fontan revision, and pulmonary valve replacement (PVR). Recent advances have been made in each of these three operations. SUMMARY: Surgery for Ebstein's anomaly can now be offered to older patients at low risk and with good late outcome. The operation includes tricuspid valve repair or replacement and frequent concomitant procedures such as atrial septal defect closure, arrhythmia surgery (the maze procedure), and coronary artery bypass grafting. Fontan conversion involves: conversion of the previously created atriopulmonary connection to a total cavopulmonary artery, extracardiac Fontan; arrhythmia surgery, typically with a modified biatrial maze procedure along with placement of an antitachycardia, dual-chamber pacemaker with steroid-eluting epicardial leads in all patients; and concomitant procedures to treat associated lesions. The need for PVR is increasing for many adults with CHD. In the past, residual defects such as chronic pulmonary regurgitation following repair of tetralogy of Fallot were considered benign. Recent evidence suggests that pulmonary regurgitation causes significant morbidity, producing right ventricular dilatation and dysfunction, exercise intolerance, arrhythmias, and sudden death. Multiple options exist for PVR including several recent developments such as PVR with a man-made polytetrafluoroethylene bicuspid valve and percutaneous PVR.

2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias.

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias.