Multicenter research priorities in pediatric CMR: results of a collaborative wiki survey.

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.

Counseling for Perinatal Outcomes in Women with Congenital Heart Disease.

As the adult congenital heart disease population grows, more women are reaching childbearing age. Women with moderate to complex congenital heart disease have an increased risk of morbidity and mortality than the general population. There is increased risk of prematurity and intrauterine growth restriction in infants. Regular preconceptual adult congenital heart disease care, contraception counseling, and multidisciplinary care during a pregnancy can help minimize the risk during pregnancy for both mother and baby.

The association between cardiac magnetic resonance-derived aortic stiffness parameters and aortic dilation in young adults with bicuspid aortic valve: With and without coarctation of aorta.

Background: Bicuspid aortic valve (BAV) is associated with progressive aortic dilation. Studies in aortopathies have shown a correlation between increased aortic stiffness and aortic dilation. We aimed to evaluate aortic stiffness measures as predictors of progressive aortic dilation by cardiac magnetic resonance (CMR) in BAV patients. Methods: This is a retrospective study of 49 patients with BAV (median age 21.1 years at first CMR visit) with ≥2 CMR at the Wisconsin Adult Congenital Heart Disease Program (WAtCH). Circumferential aortic strain, distensibility, and ß-stiffness index were obtained from CMR-derived aortic root cine imaging, and aortic dimensions were measured at aortic root and ascending aorta. A linear mixed-model and logistic regression were used to identify important predictors of progressive aortic dilation. Results: Over a median of 3.8 years follow-up, the annual growth rates of aortic root and ascending aorta dimensions were 0.25 and 0.16 mm/year, respectively. Aortic strain and distensibility decreased while ß-stiffness index increased with age. Aortic root strain and distensibility were associated with progressive dilation of the ascending aorta. Baseline aortic root diameter was an independent predictor of >1 mm/year growth rate of the aortic root (adjusted OR 1.34, 95 % CI 1.03-1.74, p = 0.028). Most patients (61 %) had coexisting coarctation of aorta. Despite the higher prevalence of hypertension in patients with aortic coarctation, hypertension or coarctation had no effect on baseline aorta dimensions, stiffness, or progressive aortic dilation. Conclusion: Some CMR-derived aortic stiffness parameters correlated with progressive aortic dilation in BAV and should be further investigated in larger and older BAV cohorts.

Preoperative Evaluation of Adult Congenital Heart Disease Patients for Non-cardiac Surgery.

PURPOSE OF REVIEW: Highlight the extracardiac comorbidities that adult congenital heart disease patients and summarize the current recommendations for non-cardiac surgery. RECENT FINDINGS: Adult congenital heart disease patients are living longer, becoming more complex and developing adult comorbidities as they age. These patients have multiorgan involvement including higher prevalence of kidney disease and decreased lung function. Non-cardiac comorbidities can complicate surgery in this patient population. Most patients have non-cardiac surgery in community settings. These settings may increase adverse events during and in the post-operative period. Survival is improved when moderate and complex patients are seen in regional referral centers. Improved awareness of long-term complications of congenital heart disease and extracardiac comorbidities for adult congenital heart disease patients is needed. Appropriate care settings with both congenital heart disease expertise and adult subspecialty care reduces morbidity and mortality in these complex patients.

The 745.5 issue in code-based, adult congenital heart disease population studies: Relevance to current and future ICD-9-CM and ICD-10-CM studies.

OBJECTIVE: Although the ICD-9-CM code 745.5 is widely used to indicate the presence of a secundum atrial septal defect (ASD), it is also used for patent foramen ovale (PFO) which is a normal variant and for "rule-out" congenital heart disease (CHD). The ICD-10-CM code Q21.1 perpetuates this issue. The objective of this study was to assess whether code 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 miscodes for CHD, and if true CHD positives decrease with age. DESIGN: Echocardiograms of patients with an ICD-9-CM code of 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 were reviewed to validate the true incidence of an ASD. This observational, cross-sectional record review included patients between 11 and 64 years of age. RESULTS: Medical charts and echocardiograms of 190 patients (47.9% males) were reviewed. The number of falsely coded patients with 745.5 (no ASD) was high (76.3%). Forty-five (23.7%) patients had a true ASD. Among the 145 patients without an ASD, 100 (52.6%) were classified as having a PFO, 37 (19.5%) had a normal non-CHD echocardiogram, and 8 (4.2%) had some other CHD anomaly. The likelihood that 745.5 coded for a true ASD was higher in children aged 11-20 (64.3%) than adults aged 21-64 years (20.6%). CONCLUSIONS: 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.

Improving the quality of transition and transfer of care in young adults with congenital heart disease.

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.

Compliance with Adult Congenital Heart Disease Guidelines: Are We Following the Recommendations?

OBJECTIVE: As the adult congenital heart disease population increases, poor transition from pediatric to adult care can lead to suboptimal quality of care and an increase in individual and institutional costs. In 2008, the American College of Cardiology and American Heart Association updated the adult congenital heart disease practice guidelines and in 2011, the American Heart Association recommended transition guidelines to standardize and encourage appropriate timing of transition to adult cardiac services. The objective of this study was to evaluate if patient age or complexity of congenital heart disease influences pediatric cardiologists' decision to transfer care to adult providers and to evaluate the compliance of different types of cardiology providers with current adult congenital heart disease treatment guidelines. DESIGN: A single-center retrospective review of 991 adult congenital heart disease patients identified by ICD-9 code from 2010 to 2012. SETTING: Academic and community outpatient cardiology clinics. PATIENTS: Nine hundred ninety-one patients who are 18 years and older with congenital heart disease. INTERVENTION: None. OUTCOMES MEASURES: The compliance with health maintenance and transfer of care recommendations in the outpatient setting. RESULTS: For patients seen by pediatric cardiologists, only 20% had transfer of care discussions documented, most often in younger simple patients. Significant differences in compliance with preventative health guidelines were found between cardiology provider types. CONCLUSION: 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.

Descending aorta thrombus in a neonate mimicking coarctation of the aorta: mechanical thrombectomy using the AngioJet(®) catheter.

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.