Fontan failure has been variably and inconsistently described in the literature, leading to challenges in comparing studies and evaluating treatments. Development of a conceptual framework to describe clinical phenotypes will aid in consistent terminology in the literature. In the heart failure literature, several key concepts have been described-"heart failure" is a clinical syndrome of various etiologies, with phenotype-specific response to therapies. As the congenital heart disease community struggles to grapple with "Fontan failure," these concepts come to light. Fontan failure in the context of four clinical phenotypes, including evaluation, potential management strategies, and future directions is discussed.

The transition and transfer from pediatric to adult care is becoming increasingly important as improvements in the diagnosis and management of congenital heart disease allow patients to live longer. Transition is a complex and continuous process that requires careful planning. Inadequate transition has adverse effects on patients, their families and healthcare delivery systems. Currently, significant gaps exist in patient care as adolescents transfer to adult care and there are little data to drive the informed management of transition and transfer of care in adolescent congenital heart disease patients. Appropriate congenital heart disease care has been shown to decrease mortality in the adult population. This paper reviews the transition and transfer of care processes and outlines current congenital heart disease specific guidelines in the United States and compares these recommendations to Canadian and European guidelines. It then reviews perceived and real barriers to successful transition and identifies predictors of success during transfer to adult congenital heart disease care. Lastly, it explores how disease-specific markers of outcomes and quality indicators are being utilized to guide transition and transfer of care in other chronic childhood illnesses, and identifies existing knowledge gaps and structural impediments to improving the management of transition and transfer among congenital heart disease patients.

This validation study demonstrates that 745.5 performed poorly across all ages. As 745.5 is widely used in population-level investigations and ICD-10-CM perpetuates the problem, future analyses utilizing CHD codes should consider separate analysis of those identified only through code 745.5.

Objective Transfer of congenital heart disease care from the pediatric to adult setting has been identified as a priority and is associated with better outcomes. Our objective is to determine what percentage of patients with congenital heart disease transferred to adult congenital cardiac care. Design A retrospective cohort study. Setting Referrals to a tertiary referral center for adult congenital heart disease patients from its pediatric referral base. Patients This resulted in 1514 patients age 16‐30, seen at least once in three pediatric Georgia health care systems during 2008‐2010. Interventions We analyzed for protective factors associated with age‐appropriate care, including distance from referral center, age, timing of transfer, gender, severity of adult congenital heart disease, and comorbidities. Outcome Measures We analyzed initial care by age among patients under pediatric care from 2008 to 2010 and if patients under pediatric care subsequently transferred to an adult congenital cardiologist in this separate pediatric and adult health system during 2008‐2015. Results Among 1514 initial patients (39% severe complexity), 24% were beyond the recommended transfer age of 21 years. Overall, only 12.1% transferred care to the referral affiliated adult hospital. 90% of these adults that successfully transferred were seen by an adult congenital cardiologist, with an average of 33.9 months between last pediatric visit and first adult visit. Distance to referral center contributed to delayed transfer to adult care. Those with severe congenital heart disease were more likely to transfer (18.7% vs 6.2% for not severe). Conclusion Patients with severe disease are more likely to transfer to adult congenital heart disease care than nonsevere disease. Most congenital heart disease patients do not transfer to adult congenital cardiology care with distance to referral center being a contributing factor. Both pediatric and adult care providers need to understand and address barriers in order to improve successful transfer.

Even though a significant number of adults with congenital heart disease are lost to appropriate follow-up in their third and fourth decades of life, pediatric cardiologists discussed transfer of care with moderate and complex congenital heart disease patients less frequently. Appropriate transfer of adults with congenital heart disease to an adult congenital cardiologist provides an opportunity to reinforce the importance of regular follow-up in adulthood and may improve outcomes as adult congenital cardiologists followed the adult congenital heart disease guidelines more consistently than pediatric or adult cardiologists.

Multicenter studies in pediatric cardiovascular magnetic resonance (CMR) improve statistical power and generalizability. However, a structured process for identifying important research topics has not been developed. We aimed to (1) develop a list of high priority knowledge gaps, and (2) pilot the use of a wiki survey to collect a large group of responses. Knowledge gaps were defined as areas that have been either unexplored or under-explored in the research literature. High priority goals were: (1) feasible and answerable from a multicenter research study, and (2) had potential for high impact on the field of pediatric CMR. Seed ideas were contributed by a working group and imported into a pairwise wiki survey format which allows for new ideas to be uploaded and voted upon (https://allourideas.org). Knowledge gaps were classified into 2 categories: ‘Clinical CMR Practice’ (16 ideas) and ‘Disease Specific Research’ (22 ideas). Over a 2-month period, 3,658 votes were cast by 96 users, and 2 new ideas were introduced. The 3 highest scoring sub-topics were myocardial disorders (9 ideas), translating new technology & techniques into clinical practice (7 ideas), and normal reference values (5 ideas). The highest priority gaps reflected strengths of CMR (e.g., myocardial tissue characterization; implementation of technologic advances into clinical practice), and deficiencies in pediatrics (e.g., data on normal reference values). The wiki survey format was effective and easy to implement, and could be used for future surveys.

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A 5-day-old neonate presented in shock, with signs and symptoms of severe coarctation of the aorta. He was diagnosed with a patent aortic arch, and a completely thrombosed abdominal aorta. The authors report the successful transcatheter removal of the entire thrombus using the AngioJet(®) Thrombectomy system. This represents the first published use of the AngioJet(®) catheter in the aorta of a pediatric patient.