Mark D. Norris

Prevalence and patterns of retention in cardiac care in young adults with congenital heart disease

The population with adult congenital heart disease is expanding. Cardiac care retention and follow-up patterns were assessed in 153 adults with congenital heart disease (median age, 24.5 years), previously compliant as teenagers. The majority (125; 81.7%) were retained in care, most often by a pediatric cardiologist (69%). The rate of retention was surprisingly high.

Assessment of Transition Readiness in Adolescents and Young Adults with Heart Disease.

OBJECTIVES To evaluate transition readiness, including perceived knowledge deficits, self-efficacy, and self-management behaviors, in 13- to 25-year-olds with congenital heart disease or heart transplant and to examine the relationships between transition readiness assessment, information seeking behavior, and quality of life (QOL). STUDY DESIGN In this cross-sectional study, patients (n = 164) completed the Transition Readiness Assessment and the Pediatric Quality of Life Inventory using an e-tablet, web-based format at a routine clinic visit. RESULTS Median patient age was 18.1 years (range 13.0-25.5). Average perceived knowledge deficit score (% of items with no knowledge) was 25.7% (range 0%-75%). On a 100-point scale, the mean score was 72.0 ± 17.2 for self-efficacy and 49.7 ± 17.5 for self-management. Knowledge deficits were negatively correlated with self-efficacy (r = -0.45, P < .0001) and self-management (r = -0.36, P < .0001). Overall, 66% of patients requested information (73% ≥ 18 years old). Higher psychosocial QOL scores were correlated with lower knowledge deficit scores (r = -0.21, P = .01) and higher perceived self-efficacy scores (r = 0.41, P < .0001). CONCLUSIONS Transition knowledge deficits are common and associated with decreased self-efficacy and self-management skills in adolescents and young adults with heart disease. Greater transition knowledge and perceived self-efficacy are associated with better psychosocial QOL. Routine assessment of transition readiness is recommended to facilitate recognition of deficits and interventions to promote successful transition outcomes.

Relation of Increased Epicardial Fat After Fontan Palliation to Cardiac Output and Systemic Ventricular Ejection Fraction

Epicardial fat produces multiple proinflammatory cytokines and is associated with adverse cardiovascular events. Inflammation and resultant endothelial dysfunction may play a role in progressive myocardial dysfunction among adults with single ventricle physiology after Fontan palliation, but the potential impact of increased epicardial fat volume (EFV) has not been studied. This study sought to determine if there is greater EFV in Fontan patients compared with a group of repaired tetralogy of Fallot (rTOF) patients. We retrospectively measured EFV manually on cardiac magnetic resonance imaging in Fontan patients, ≥15 years, and 1:1 age, sex, and body mass index-matched patients with rTOF. EFV was indexed to body surface area. A random subset of studies was re-measured to assess intra- and interobserver reliability. Fontan patients (n = 63, median age 21.6 years, 51% male, mean body mass index 24.2 ± 5.6 kg/m2) had a larger indexed EFV compared with matched rTOF patients (75.3 ± 29.2 ml/m2 vs 60.0 ± 19.9 ml/m2, p = 0.001). In Fontan patients, indexed EFV was inversely correlated with ventricular ejection fraction (r = -0.26, p = 0.04) and cardiac index (r = -0.33, p = 0.01). Intra- and interobserver reliabilities of the indexed EFV measurements in both groups were excellent (intraclass correlation coefficient ranges from 0.93 to 0.97). In conclusion, indexed EFV is higher in Fontan patients compared with patients with rTOF and is associated with lower ventricular ejection fraction and cardiac index. Increased EFV could play a role in the failing Fontan circulation, but longitudinal studies are necessary to establish any causative role.

KOMMERELL DIVERTICULUM ACROSS THE AGES: DESTINED FOR DILATION?

Background: A Kommerell Diverticulum (KD) is the dilated proximal portion of an aberrant subclavian artery as a consequence of abnormal embryonic vascular development. Concern has arisen for aneurysmal KD dilation, dissection and rupture, with a trend toward invasive prophylactic intervention. This

Continuous Cardiac Care for Adolescents

Lifelong specialized care is indispensable to optimize life expectancy and quality of life for a growing number of chronic conditions including congenital cardiac conditions. Provision of seamless continuous care along the patients’ life spectrum, from birth until death, is challenging. Transfers of care from pediatric, over adolescent, to adult-focused and even geriatric care make patients vulnerable for prolonged gaps in cardiac care or for discontinuation of cardiac care. Care gaps are observed in all chronic medical conditions, and among those with congenital heart disease (CHD) studies reported that up to 76 % of adolescents experienced care gaps or are even completely lost to follow-up. A median proportion of 42 % of young persons with CHD experience such discontinuous cardiac care. Care gaps in chronically ill populations are associated with an increased risk for serious comorbidities and a need for urgent interventions. In patients with CHD, such care gaps might aggravate disease burden, as the complex nature of the heart defects and its inherent high risk for comorbidity onset warrant close and sustained follow-up. This chapter describes the phenomenon of (dis)continuity of care by reviewing the current body of evidence on operational definitions for care gaps and the magnitude of this problem in adolescents with CHD. Furthermore, the clinical impact of discontinuation of cardiac care is described as well as predictors identified in empirical studies. Finally, as strategies are developed for improving CHD outcomes including quality of life, the patients’ perspectives on reasons for leaving or returning to cardiac care are described.

A peculiar presentation of a large retrocardiac mass

![Figure][1] [![Graphic][3] ][3] A 13-year-old young man with anxiety presented for echocardiogram after multiple emergency room visitations for atypical chest pain. An echocardiogram (A to E) displayed a fluid filled, tube-like structure posterior to the left atrium (LA) and parallel