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Background— Transposition of the great arteries with intra-atrial baffle repair is among the congenital heart defects at highest risk of sudden death. Little is known about mechanisms of sudden death and the role of implantable cardioverter defibrillators. Methods and Results— We conducted a multicenter cohort study in patients with transposition of the great arteries to determine actuarial rates of implantable cardioverter defibrillator shocks, identify risk factors, assess underlying arrhythmias, and characterize complications. Overall, 37 patients (age, 28.0�7.6 years; 89.2% male) were enrolled from 7 sites. Implantable cardioverter defibrillators were implanted for primary prevention in 23 (62.1%) patients and secondary prevention in 14 patients (37.8%). Annual rates of appropriate shocks were 0.5% and 6.0% in primary and secondary prevention, respectively ( P =0.0366). Independent predictors were a secondary prevention indication (hazard ratio, 18.0; P =0.0341) and lack of β-blockers (hazard ratio, 16.7; P =0.0301). In patients with appropriate shocks, intracardiac electrograms documented supraventricular tachycardia preceding or coexisting with ventricular tachycardia in 50%. No patient with inducible ventricular tachycardia received an appropriate shock in comparison with 37.5% of noninducible patients ( P =0.0429). Inappropriate shocks occurred in 6.6% per year, more so in patients of lesser weight (hazard ratio, 0.91 per kg; P =0.0168). Additionally, 14 patients (37.8%) experienced complications: 5 (13.5%) acute, 1 (2.7%) late generator related, and 12 (32.4%) late lead related. Conclusion— In patients with transposition of the great arteries, high rates of appropriate shocks are noted in secondary but not primary prevention. Supraventricular arrhythmias may be implicated in the etiology of ventricular tachyarrhythmias; β-blockers seem protective, and inducible ventricular tachycardia does not seem to predict future events. Inappropriate shocks and late lead-related complications are common.

ObjectivesThe objective of this study was to document the prevalence of chronic venous insufficiency (CVI) and its associated factors in adults with Fontan physiology.BackgroundAs the population of adults with complex congenital heart disease and Fontan physiology increases, so does the occurrence of highly morbid and mortal outcomes, including heart failure and thromboembolism. The presence of abnormal peripheral hemodynamic conditions in this population and their potential contribution to adverse outcomes is not well known. The primary objective of this study was to document the prevalence of CVI in adults with Fontan physiology.MethodsA total of 159 adults with Fontan physiology from 7 adult congenital heart centers were prospectively assessed for lower extremity CVI, with the assignment of clinical, etiological, anatomical, and pathophysiological classification grades, and compared with age-matched and sex-matched controls. Leg photographs were independently reassessed to confirm interobserver reliability.ResultsThe prevalence of CVI was significantly greater in the Fontan population (60%; 95% confidence interval [CI]: 52% to 68%) compared with healthy controls (32%; 95% CI: 15% to 54%) (p = 0.008). Strikingly, the prevalence of severe CVI (clinical, etiological, anatomical, and pathophysiological grade ≥4) was significantly higher in the Fontan group (22%; 95% CI: 16% to 29%) versus the healthy controls (0%; 95% CI: 0% to 14%) (p = 0.005). In a multivariate analysis, several factors were independently associated with severe CVI, including increased numbers of catheterizations with groin venous access, lower extremity itching, and deep venous thrombosis.ConclusionsCVI is common in adult patients with congenital heart disease with Fontan physiology. The contribution of abnormal peripheral hemodynamic conditions to comorbidities, including thromboembolism and heart failure, and interventions to improve peripheral hemodynamic conditions require further exploration.

The objective of this study was to explore the current practice and attitudes of pediatric cardiologists in the United States and Europe on the transfer and transition of children with congenital heart disease (CHD). A survey among pediatric cardiology programs in the United States and Europe was undertaken. Sixty-nine centers completed and returned the 61-item questionnaire that was specifically devised for this survey. Of 69 participating centers, 74% reported that they transfer their patients to adult-focused care. When a center transfers its patients, 80% transfer them to a formalized Adult Congenital Heart Disease Program. The median age of transfer is 18 years. Comorbidities, pregnancy, and patient/family request to leave pediatric cardiology were identified as initiators for transfer. Complexity of the heart defect was relatively less important when deciding whether to transfer patients. Only one-third of the centers that transfer their patients provide a structured preparation for patients and family. Development of a formal transition program is planned at 59% of the centers that transfer patients. In conclusion, timely transfer and a structured transition process of children with CHD are not implemented in all pediatric cardiology programs. Health-care providers working in pediatric cardiology should make their transfer policies explicit and transition programs ought to be developed.

We thank Witte et al for their interest in our article and encourage the critical appraisal of implantable cardioverter-defibrillator (ICD) therapy at large and in tetralogy of Fallot (TOF) in particular. We concur that decisions regarding ICD implantation should be thoughtfully considered in light of anticipated risks, known complications, and potential benefits. Indeed, one of our stated objectives was to provide caregivers with evidence-based data regarding ICD-related complications, as derived from a multicenter experience. These complications were undeniably substantial. Inappropriate shocks were observed in 5.8% of patients per year, and 80% of those shocks were triggered by sinus or supraventricular tachycardia.1 Complications other than inappropriate shocks occurred in 30% and were predominantly late lead–related. From a psychosocial perspective, feelings of anxiety and depression have been previously characterized in youths with ICDs, although most remain …

Background— Tetralogy of Fallot is the most common form of congenital heart disease in implantable cardioverter-defibrillator (ICD) recipients, yet little is known about the value of ICDs in this patient population. Methods and Results— We conducted a multicenter cohort study in high-risk patients with Tetralogy of Fallot to determine actuarial rates of ICD discharges, identify risk factors, and characterize ICD-related complications. A total of 121 patients (median age 33.3 years; 59.5% male) were enrolled from 11 sites and followed up for a median of 3.7 years. ICDs were implanted for primary prevention in 68 patients (56.2%) and for secondary prevention in 53 (43.8%), defined by clinical sustained ventricular tachyarrhythmia or resuscitated sudden death. Overall, 37 patients (30.6%) received at least 1 appropriate and effective ICD discharge, with a median ventricular tachyarrhythmia rate of 213 bpm. Annual actuarial rates of appropriate ICD shocks were 7.7% and 9.8% in primary and secondary prevention, respectively ( P =0.11). A higher left ventricular end-diastolic pressure (hazard ratio 1.3 per mm Hg, P =0.004) and nonsustained ventricular tachycardia (hazard ratio 3.7, P =0.023) independently predicted appropriate ICD shocks in primary prevention. Inappropriate shocks occurred in 5.8% of patients yearly. Additionally, 36 patients (29.8%) experienced complications, of which 6 (5.0%) were acute, 25 (20.7%) were late lead-related, and 7 (5.8%) were late generator-related complications. Nine patients died during follow-up, which corresponds to an actuarial annual mortality rate of 2.2%, which did not differ between the primary and secondary prevention groups. Conclusions— Patients with tetralogy of Fallot and ICDs for primary and secondary prevention experience high rates of appropriate and effective shocks; however, inappropriate shocks and late lead-related complications are common.

ACC/AHA guidelines recommend a structured preparation for and transfer to adult-oriented cardiac care for adult survivors of pediatric onset heart disease (POHD). Given this, we sought to describe the transition and transfer practices for a cohort of young adults with POHD and to determine factors associated with successful transfer to adult-oriented cardiac care. We performed a single-center, retrospective chart review on patients ≥18 years of age, with POHD likely to require lifelong cardiac care, who were seen in outpatient pediatric cardiology (PC) between 2008 and 2011. Successful transfer was defined as the subsequent attendance at adult cardiology (AC) within 2 years of PC visit. We identified 118 patients who met study criteria. Mean age 22.4 ± 2.0 years, 59 % male, 64 % white and 40 % Hispanic. Mean transition education topics noted was 3.3 ± 1.8 out of 20 and covered the underlying cardiac disease (89 %), follow-up and current medications (56 %) and exercise limitations (34 %). Recommendations for follow-up were AC (57 %) and PC (33 %). Of those told to transfer to AC, 79 % successfully transferred. Characteristics of successful transfer included: prior cardiac surgery (p = 0.008), cardiac medication use (p = 0.006) and frequency of follow-up ≤1 year (p = 0.037). One-quarter of all subjects did not follow-up within at least 2 years. Despite published guidelines, transition education appears lacking and the approach to transfer to adult cardiac care is not consistent. Given the increased risk of morbidity and mortality in this patient population, standardization of transition education and transfer processes appear warranted.

To the Editor: Of the approximately 1.3 million adult survivors with congenital heart disease (CHD) living in the United States, only a small percentage appears to be receiving specialized care in accordance with the American College of Cardiology (ACC) 2008 Guidelines for the Management of Adult

Guidelines for the successful orchestration of transitioning of the adolescent and young and older adult patient with congenital heart disease to a health care system appropriate for their long-term congenital heart disease care and counseling appear necessary to improve patient and family confidence, education, therapy, life quality, and survival outcomes. Schema for care organization and delivery for adult patients with congenital heart disease remain primitive and largely unimplemented. The presence of a strong central care oversight organization and the establishment of a multi-institutional database to assist in assessment of care outcomes and guidelines appears requisite to these needs and for the establishment of transitioning guidelines for these patients as they assume a greater and deeper shared control of their futures with their caregivers.

10088 Background: Pediatric oncologists are responsible for facilitating the transition of childhood cancer survivors to risk based adult focused care. This study describes transition practices, pe...

WOMEN WITH CONGENITAL HEART DISEASE DAVID W. OUYANG, PAUL KHAIRY, SUSAN FERNANDES, KATHERINE ECONOMY, AVIVA LEE-PARRITZ, MICHAEL LANDZBERG, Brigham and Women’s Hospital, Maternal Fetal Medicine, Boston, Massachusetts, Children’s Hospital, Boston, Massachusetts, Boston Medical Center, Maternal Fetal Medicine, Boston, Massachusetts OBJECTIVE: A composite risk index to predict maternal cardiac complications has been proposed as a contemporary approach to risk stratify pregnant women with heart disease. We sought to independently validate this risk index in women with congenital heart disease. STUDY DESIGN: We conducted a retrospective, single center, cohort study examining maternal outcomes in women with congenital heart disease who delivered between 1998-2004. Patients received 1 point for each of the following predictors: Previous cardiac event (h/o CHF/stroke/arrhythmia); baseline NYHA class >2 or central cyanosis; LV outflow tract obstruction; and LVEF ! 40%. The cardiac event rates were stratified by the risk index scoring system and compared to the predicted rate. The predicted rate of a primary cardiac event in pregnancies with 0, 1, and R2 points is 5%, 27% and 75% respectively. Primary events were defined as CHF; sustained arrhythmia; stroke; cardiac arrest; and cardiac death. RESULTS: 53 patients were studied who completed a total of 83 pregnancies. All patients had a baseline NYHA class %2. Six patients had a h/o CHF, eleven had a h/o arrhythmia and one patient had a h/o stroke. No patients had central cyanosis or LVEF !40%. A primary cardiac event occurred in 15 pregnancies, all of which were cases of CHF (n = 9) and arrhythmia (n = 8). In two pregnancies, both CHF and arrhythmia were observed. The predicted rate of a primary cardiac event based on the composite risk index was similar to the observed rate (Table). CONCLUSION: This study represents the largest single institution experience evaluating cardiac outcomes in pregnant women with congenital heart disease. It provides the first independent validation of the risk index scoring system proposed to predict adverse cardiac events in pregnant women with heart disease.