Adults With Congenital Heart Disease Patients Research Articles

Adequacy of Cancer Screening in Adult Women with Congenital Heart Disease

Adults with congenital heart disease (ACHD) face noncardiac healthcare challenges as the population ages. We assessed whether women with ACHD have comparable cancer screening rates to non-ACHD women in a cardiac practice and to the general population. We performed a retrospective review of 175 adult women seen in a cardiac care center in 2009–2011. Data on Pap tests, mammography, and colonoscopies, were collected through electronic medical records and primary care provider records. Adequate documentation was available for 100 individuals with ACHD and 40 comparator cardiac patients. The adequacy of screening was determined using guidelines set forth by the American Cancer Society in 2010. Compared with the national average, ACHD patients had significantly lower rates of Pap tests (60% versus 83%, P < 0.001) and mammography (48% versus 72%, P < 0.001). Compared with non-ACHD women in the same practice, ACHD patients had consistently lower rates of mammography (48% versus 81%, P = 0.02) and colonoscopies (54% versus 82%, P = 0.23). As the population of ACHD individuals ages, attention to cancer screening becomes increasingly important but may be overlooked in this population. Primary care physicians and cardiologists should collaborate to ensure appropriate cancer screening for this growing population.

Physical activity is associated with improved aerobic exercise capacity over time in adults with congenital heart disease

BackgroundImpaired exercise capacity is common in adults with congenital heart disease (ACHD). This impairment is progressive and is associated with increased morbidity and mortality. We studied the influence of the frequency of at least moderately strenuous physical activity (PhysAct) on changes in exercise capacity of ACHD patients over time. MethodsWe studied ACHD patients ≥21years old who had repeated maximal (RER≥1.09) cardiopulmonary exercise tests within 6 to 24months. On the basis of data extracted from each patient's clinical records, PhysAct frequency was classified as (1) Low: minimal PhysAct, (2) Occasional: moderate PhysAct <2 times/week, or (3) Frequent: moderate PhysAct ≥2 times/week. ResultsPhysAct frequency could be classified for 146 patients. Those who participated in frequent exercise tended to have improved pVO2 (∆pVO2=+1.63±2.67ml/kg/min) compared to those who had low or occasional activity frequency (∆pVO2=+0.06±2.13ml/kg/min, p=0.003) over a median follow-up of 13.2months. This difference was independent of baseline clinical characteristics, time between tests, medication changes, or weight change. Those who engaged in frequent PhysAct were more likely to have an increase of pVO2 of ≥1SD between tests as compared with sedentary patients (multivariable OR=7.4, 95%CI 1.5–35.7). Aerobic exercise capacity also increased for patients who increased activity frequency from baseline to follow-up; 27.3% of those who increased their frequency of moderately strenuous physical activity had a clinically significant (at least +1SD) increase in pVO2 compared to only 11% of those who maintained or decreased activity frequency. ConclusionsACHD patients who engage in frequent physical activity tend to have improved exercise capacity over time.

Congenital heart disease beyond the age of 60: emergence of a new population with high resource utilization, high morbidity, and high mortality

The population of adults with congenital heart disease (ACHD) is growing and ageing. Limited information about the diagnostic spectrum of this emerging population, its resource utilization at tertiary ACHD centres, and especially about prognostic parameters is available. Retrospective cohort study on all ACHD patients ≥60 years of age under active follow-up. All-cause mortality was the primary outcome measure. Out of a total population of 7315 ACHD patients, 375 [190 females (50.7%), mean age 64.8 ± 5.9 years] fulfilled the inclusion criteria. During a median follow-up of 5.5 (IQR 3.1-8.6) years, 55 of the 375 patients died. The number of interventions (P = 0.0006), the number and length of hospitalization (P < 0.0001), and the number of outpatient clinic visits (P < 0.0001) were significantly higher in patients ≥60 compared with patients aged between 20 and 60 years. Patients ≥60 years of age with moderate or severe congenital heart defects had worse survival prospects than their age- and gender-matched comparison population. On multivariate Cox analysis, coronary artery disease [hazard ratio (HR): 5.04; 95%CI: 1.88-13.51, P = 0.0014], symptoms of heart failure (HR: 2.36; 95%CI: 1.05-5.29, P < 0.05), NYHA class (HR: 1.96; 95%CI: 1.18-3.26, P < 0.01), and moderate to severe reduction in systemic ventricular systolic function (HR: 1.90; 95%CI: 1.20-2.99, P < 0.001) were the strongest prognostic factors. There is a growing number of elderly ACHD patients with high mortality rates and a higher utilization of healthcare resources compared with younger patients. Acquired morbidities, such as coronary artery disease, seem to be key determinants of outcome in this older population in conjunction with the underlying congenital heart disease.

Outcome of Adults with Congenital Heart Disease Evaluated for Heart Transplant: A Multi-Center Study

<h3>Purpose</h3> The prevalence of adults with congenital heart disease (ACHD) has increased with improved surgical outcomes in childhood. Heart transplant (HT) is an option for ACHD with heart failure (HF) but studies have shown higher 30-day mortality vs. other adult recipients. This study reviews outcomes of ACHD referred for HT. <h3>Methods and Materials</h3> All ACHD (≥18 yrs) referred for HT since 2000 to 4 large volume centers were identified. Characteristics were compared among those listed and not listed for HT. Survival from time of evaluation for each group was examined. <h3>Results</h3> Of 45 ACHD patients evaluated for HT from 2000-2012, diagnosis was single ventricle in 21 (47%) of which 17 had Fontan, transposition of great arteries (15), Tetralogy of Fallot (5), and other (4). Mean age was 29±11 years. Groups were similar in age, sex, and PRA status, but fewer listed patients were single ventricle (33% vs 67%, p=0.03). Among 27 listed, 26 had HT at a median wait time of 168 days (IQR: 22-458). At HT, 6 (23%) were UNOS status 1A, 3 on mechanical circulatory support, 2 ventilated, 10 on iv inotropes, and 7 hospitalized. Among 18 evaluated but not listed, 11 were deemed too high risk including 6 for liver disease and 1 for pulmonary hypertension, 5 too well, and 2 unknown. Survival after evaluation was similar Figure 1. <h3>Conclusions</h3> The 1- and 10-year HT survival for ACHD in this cohort was comparable to published survival in the overall adult HT population. HT is a viable option for ACHD, but careful candidate selection is critical to ensure optimal outcomes for this high risk population. Similar survival in ACHD patients who were not listed underscores the need for careful selection as evidence for HF management and transplant suitability are lacking in ACHD.

Heart Failure Associated With Adult Congenital Heart Disease

The population of adults with congenital heart disease (ACHD) now exceeds that of children with congenital heart disease. Within this relatively young population of adults in their third decade of life exist a population of patients with heart failure (HF) who, in functional status, closely resemble patients in their fifth and sixth decades of life with HF caused by coronary artery disease and myocardial ischemia. The ACHD patients currently at greatest risk for HF are those with single-ventricle physiology, those with a 2-ventricle circulation with the right ventricle as the systemic ventricle, and those with repaired tetralogy of Fallot and pulmonary valve insufficiency. This article will review the demographics, diagnosis, functional capacity, and treatment of HF in the ACHD population.

Prosthetic valves in adult patients with congenital heart disease: Rationale and design of the Dutch PROSTAVA study

Data on long-term complications in adult patients with congenital heart disease (ACHD) and a prosthetic valve are scarce. Moreover, the influence of prosthetic valves on quality of life (QoL) and functional outcome in ACHD patients with prosthetic valves has not been studied. The primary objective of the PROSTAVA study is to investigate the relation between prosthetic valve characteristics (type, size and location) and functional outcome as well as QoL in ACHD patients. The secondary objectives are to investigate the prevalence and predictors of prosthesis-related complications including prosthesis-patient mismatch. The PROSTAVA study, a multicentre cross-sectional observational study, will include approximately 550 ACHD patients with prosthetic valves. Primary outcome measures are maximum oxygen uptake during cardiopulmonary exercise testing and QoL. Secondary outcomes are the prevalence and incidence of valve-related complications including prosthesis-patient mismatch. Other evaluations are medical history, physical examination, echocardiography, MRI, rhythm monitoring and laboratory evaluation (including NT-proBNP). Identification of the relation between prosthetic valve characteristics in ACHD patients on one hand and functional outcome, QoL, the prevalence and predictors of prosthesis-related complications on the other hand may influence the choice of valve prosthesis, the indication for more extensive surgery and the indication for re-operation.

Management of Adults With Congenital Heart Disease Using Videoconferencing Across Western Canada: A 3-Year Experience

BackgroundManagement of adults with congenital heart disease (ACHD) requires quaternary centres with cardiologists and cardiac surgeons who have expertise in the diagnosis and management of this patient population. We report on the feasibility of the management of ACHD patients using videoconferencing and streaming through 1 regional referral centre covering 4 western Canadian provinces, roughly 30% of Canada's land and population. MethodsVideoconferencing sessions from January 2008 to December 2010 were systematically reviewed. Case presentations were classified as successful or unsuccessful. All patients were followed to assess whether the recommendations were acted upon. The hosting institution used the Alberta Health Services internet protocol network, while other connections used integrated service digital network. The videoconference equipment at the different sites includes Polycom HDX 9000 (Polycom, San Jose, CA), Tandberg Edge 95 (Tandberg, San Jose, CA), and Tandberg 990 (Tandberg). ResultsFrom January 2008 to December 2010 there were 26 sessions, 213 case presentations, and 177 patients discussed with an average 8.2 case presentations per session. Thirty-two case presentations were deferred, 10 of which were because of transmission errors and the remainder were because of unavailability of staff or images. Of the 177 recommendations, 124 procedures (91 surgical, 29 percutaneous, and 4 electrophysiological) were booked directly at the regional referral centre. Only 6 recommendations were not carried out (4 because of premature deaths, and 2 because of patient logistic issues). ConclusionsThe results of this study illustrate that telehealth is a feasible medium for arriving at consensus recommendation in the management of ACHD patients living in a geographically diverse area.

Making Complex Adult Congenital Heart Disease a Little Simpler

Advances in pediatric cardiothoracic surgery and cardiology have enabled most patients born with severe congenital cardiovascular malformations to survive into adulthood. Physicians are, therefore, increasingly likely to encounter adults with congenital heart disease (ACHD). Tetralogy of Fallot (TOF), 1 D-transposition of the great arteries (DTGA), 2 and single functional ventricle states 3 (eg, hypoplastic left heart syndrome) are three of the most prevalent of the complex cyanotic congenital defects, occurring in 17, 4, and 3 of 100,000 adults, respectively. 4 Most, but not all, patients with complex ACHD underwent palliative or reparative surgery early in life. As they survive into adulthood, they may require intervention to alter the expected course of disease progression, to treat complications of repair, or to address residual abnormalities. ACHD patients are also at increased risk of acquired heart disease. 5 Imaging plays a role in both diagnosis of symptomatic exacerbations and longterm maintenance. Imagers should become familiar with the underlying anatomy and pathophysiology of the common forms of ACHD, prior and current surgical repairs and their associated complications, as well as postoperative imaging guidelines. This article focuses on 3 ACHD complexes: TOF, DTGA, and single functional ventricle.

Range and Distribution of NT-proBNP Values in Stable Corrected Congenital Heart Disease of Various Types

BackgroundElevated levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) are an expected finding in many adults with congenital heart disease (ACHD) but no reports have described the range of values within different diagnostic groups. MethodsBetween the years 2003 and 2011 we measured NT-proBNP in consecutive ACHD patients attending the outpatient department. Further NT-proBNP analysis was performed on 705 blood samples from 394 stable patients with the defect corrected. The results were compared among different diagnostic groups and with a control group of healthy volunteers. ResultsThe median value of NT-proBNP in the whole cohort was signifinatly higher than in the control group (211 vs 42 pg/mL; P < 0.0001). The median value of NT-proBNP in pg/mL and the percentage of normal NT-proBNP values in the listed diagnostic groups were as follows: coarctation of the aorta (COA), 97 (64%); pulmonary stenosis (PS),160 (48%); secundum atrial septal defect (ASD), 254 (44%); incomplete atrioventricular septal defect (AVSD), 211 (32%); ventricular septal defect (VSD), 154 (58%); tetralogy of Fallot (TOF), 177 (38%); transposition of the great arteries (TGA) after Mustard, 237 (20%) or Senning correction, 143 (39%); Ebstein anomaly 287, (26%); pulmonary atresia (PA), 327 (18%); and complex defects after Fontan procedure, 184 (31%). ConclusionsNormal levels of NT-proBNP were found most often in patients after the correction of coarctation of the aorta and ventricular septal defect with the highest levels found in pulmonary atresia and Ebstein anomaly. Our results may serve as reference values for different ACHD groups.

Cardiothoracic ratio from postero-anterior chest radiographs: A simple, reproducible and independent marker of disease severity and outcome in adults with congenital heart disease

ObjectiveThe wide spectrum of intracardiac anatomy and reparative surgery available for adults with congenital heart disease (ACHD) makes uniform measurement of cardiac size and disease severity challenging. The aim of this study was to assess the prognostic potential of cardiothoracic ratio, a simple marker of cardiomegaly, in a large cohort of ACHD. Patients and settingChest radiographs from 3033 ACHD patients attending our institution between 1998 and 2007 and 113 normal controls of similar age were analyzed blindly. DesignCardiothoracic ratio derived from plain postero-anterior chest radiographs, was compared between ACHD patients and controls, different diagnostic subgroups and different functional classes. Relationship between cardiothoracic ratio and survival was assessed using Cox regression. ResultsAverage cardiothoracic ratio in ACHD was 52.0±7.6% (over 50% in 56.4%), significantly higher in all ACHD diagnostic subgroups compared to controls (42.3±4.0%, p<0.0001) and highest in the “complex” cardiac anatomy, Ebstein's anomaly and Eisenmenger subgroups. Cardiothoracic ratio related to functional class, but was high even in asymptomatic patients. During a median follow-up of 4.2years, 164 patients died. Patients with a cardiothoracic ratio >55% had an 8-fold increased risk of death compared to those in the lowest tertile (<48%). Even patients with mildly increased cardiothoracic ratio (48–55%) had an adjusted 3.6-fold increased mortality compared to the lowest tertile. ConclusionsCardiothoracic ratio derived from postero-anterior chest radiographs is a simple, and reproducible marker, which relates to functional class and predicts independently mortality risk in ACHD patients.

Abstract 17062: Adequacy of Primary Care Screening of Women with Adult Congenital Heart Disease

Introduction: Adults with congenital heart disease (ACHD) face non-cardiac healthcare challenges as the population ages. The 2008 ACC/AHA guidelines called for every ACHD patient to have a primary care physician and receive preventive screening, often underemphasized in chronic disease populations. Hypothesis: We assessed the hypothesis that women with ACHD have lower compliance rates for preventive screening compared with control subjects seen in a cardiac practice and with norms in the US population. Methods: IRB approved retrospective review of females over age 18 years seen in a cardiac care center in 2009-2011. Data on history, Pap smears, mammograms, colonoscopy, and bone densitometry were obtained from medical records. Adequacy of screening was defined according to US guidelines (as of November 2009). Results: We reviewed data of 164 women with ACHD, with 89 women meeting criteria with adequate data, and compared with 39 age-matched control subjects. Women with ACHD had lower rates of preventive screening compared with controls (p &lt; 0.001 for comparison across 4 screening categories, see Table 1). Compliance rates of both groups with colonoscopies were better than nationwide statistics, surpassing the Healthy People 2010 target. Women with ACHD had low screening rates for cervical cancer, breast cancer, and osteoporosis, below national averages. Follow-up for abnormal results was adequate, with lowest rates of follow up for colonoscopies. Individuals with “moderate” ACHD per Bethesda conference guidelines had the lowest rates of adequate screening (37%) compared with simple (50%) or complex (47%) disease patients. Conclusions: Women with ACHD need increased primary care screening with Pap smears, mammograms, and DEXA scans. Primary care physicians, cardiologists, and gynecologists should collaborate to ensure appropriate health maintenance screening for this growing and aging patient population.

Geriatric Congenital Heart Disease: Burden of Disease and Predictors of Mortality

The study sought to measure the prevalence, disease burden, and determinants of mortality in geriatric adults with congenital heart disease (ACHD). The population of ACHD is increasing and aging. The geriatric ACHD population has yet to be characterized. Population-based cohort study using the Quebec Congenital Heart Disease Database of all patients with congenital heart disease coming into contact with the Quebec healthcare system between 1983 and 2005. Subjects with specific diagnoses of congenital heart disease and age 65 years at time of entry into the cohort were followed for up to 15 years. The primary outcome was all-cause mortality. The geriatric ACHD cohort consisted of 3,239 patients. From 1990 to 2005, the prevalence of ACHD in older adults remained constant from 3.8 to 3.7 per 1,000 indexed to the general population (prevalence odds ratio: 0.98; 95% confidence interval [CI]: 0.93 to 1.03). The age-stratified population prevalence of ACHD was similar in older and younger adults. The most common types of congenital heart disease lesions in older adults were shunt lesions (60%), followed by valvular lesions (37%) and severe congenital heart lesions (3%). Type of ACHD and ACHD-related complications had a minor impact on mortality, which was predominantly driven by acquired comorbid conditions. The most powerful predictors of mortality in the Cox proportional hazards model were: dementia (hazard ratio [HR]: 3.24; 95% CI: 1.53 to 6.85), gastrointestinal bleed (HR: 2.79; 95% CI: 1.66 to 4.69), and chronic kidney disease (HR: 2.50; 95% CI: 1.72 to 3.65). The prevalence of geriatric ACHD is substantial, although severe lesions remain uncommon. ACHD patients that live long enough acquire general medical comorbidities, which are the pre-eminent determinants of their mortality.

A guide for identification and continuing care of adult congenital heart disease patients in primary care

BackgroundSurgical and other advances in the treatment and care of congenital heart disease have resulted in a significant increase in the number of adults with congenital heart disease (ACHD), many of whom have no regular cardiology follow-up. Optimised care for ACHD patients requires continuity of specialist and shared care and education of practitioners and patients. The challenges for managing ACHD were identified by a Health Needs Assessment in the North West and are addressed within the UK Department of Health's ACHD Commissioning Guide. Materials and methodsAn ACHD model of care was recommended in the North West of England and developed by the three North West Cardiac & Stroke Networks. Within this, a Task Group focused on the role of primary care in the identification and continuing care of ACHD patients. A feasibility study demonstrated that existing diagnostic Read Codes can identify ACHD patients on general practice registers. An ACHD Toolkit was developed to provide algorithms to guide the appropriate management of ACHD patients through primary, secondary and/or specialist ACHD care and to improve education/knowledge amongst primary care staff about ACHD and its wider implications. ResultsEarly findings during the development of this Toolkit illustrate a wide disparity of provision between current and optimal management strategies. Patients lost to follow-up have already been identified and their management modified. ConclusionsBy focusing on identifying ACHD patients in primary care and organising/delivering ACHD services, the ACHD Toolkit could help to improve quality, timeliness of care, patient experience and wellbeing.

Hyponatraemia: a strong predictor of mortality in adults with congenital heart disease

We studied the prevalence of hyponatraemia and its prognostic implications in a large population of adult patients with congenital heart disease (ACHD). A total of 1004 ACHD patients were retrospectively entered in this study (mean age 36.2 +/- 14.4 years, 48.7% male). Cox regression was used to estimate mortality associated with hyponatraemia, adjusted for potential confounders using both multivariable regression and propensity score matching. Mean sodium concentration in this ACHD cohort was 137.6 +/- 2.6 mmol/L. The overall prevalence of hyponatraemia in this cohort was 15.5% and was highest in congenitally corrected transposition (33.3%), after Fontan operation (29.6%), and in patients with Eisenmenger syndrome (22.0%). Predictors of hyponatraemia were worse functional class, cyanosis, higher serum creatinine levels, and treatment with diuretics. Patients were followed for a median of 4.1 years, during which there were 96 deaths. Hyponatraemia was a strong predictor of death, independent of age, previous surgery, functional class, systemic ventricular function, creatinine levels, and the use of diuretics (adjusted HR 2.82, 95% CI: 1.72-4.63, P < 0.0001). Hyponatraemia is relatively common in ACHD. Hyponatraemia carries a three-fold higher risk of death in ACHD and is a simple, cheap but powerful marker of mortality.

Anemia in Adults With Congenital Heart Disease Relates to Adverse Outcome

To assess the relation of anemia in noncyanotic adults with congenital heart disease (ACHD) to functional capacity and mortality. Anemia is common in acquired heart failure and affects prognosis. The presence of anemia and its relation to outcome in ACHD remain unknown. Data were collected on consecutive noncyanotic ACHD patients attending our tertiary center between 2001 and 2006 in whom hemoglobin concentration was measured. Anemia was defined as hemoglobin concentration <13 g/dl in males and <12 g/dl in females. Cyanotic patients were excluded to avoid confounding from secondary erythrocytosis. Overall, 830 noncyanotic ACHD patients (age 36.5 +/- 15.0 years, 49.6% male) fulfilled the inclusion criteria. The prevalence of anemia was 13.1% and was highest in patients with congenitally corrected transposition of great arteries and Ebstein anomaly of the tricuspid valve. Anemic patients were more likely to be receiving diuretics (p < 0.0001) and have a lower mean corpuscular volume (p = 0.0001), with a trend toward a higher New York Heart Association functional class (p = 0.06). During a median follow-up of 47 months, 55 patients died. Anemic patients had a 3-fold higher mortality risk compared with nonanemic patients, even after propensity score adjustment for clinical variables such as systemic ventricular function, renal impairment, and diuretic therapy (adjusted hazard ratio: 3.00; 95% confidence interval: 1.46 to 6.13). Anemia is not uncommon in ACHD patients attending tertiary services and is associated with a 3-fold increased risk of death. Screening for anemia should be part of the routine assessment of ACHD patients for risk stratification and treatment when correctable causes are identified.

Morbidity and Mortality Risk Factors in Adults With Congenital Heart Disease Undergoing Cardiac Reoperations

Reoperations represent relatively frequent events in adults with congenital heart disease (ACHD). Cardiac operations in these patients present major difficulties in management and technique. Although reoperations in ACHD are becoming increasingly frequent, limited knowledge exists regarding perioperative risk factors. The study included 164 ACHD patients who underwent cardiac reoperations between January 2002 and December 2007 at our institution. Preoperative and intraoperative data were analyzed to identify morbidity and mortality risk factors. Reoperations included pulmonary valve implantation or conduit replacement in 60, aortic valve/root procedures in 36, residual atrial or ventricular septal defect closure in 19, and Fontan operation/conversion in 19. Hospital mortality was 3.6%. The mean mechanical ventilation time was 26 hours. Mean intensive care unit stay was 3.1 days. Severe postoperative complications occurred in 24 (15.1%). Cardiopulmonary bypass time (p = 0.001), Fontan operation/conversion (p = 0.001), preoperative hematocrit (p = 0.004), previous number of operations (p = 0.001), and preoperative congestive heart failure (p = 0.021) were associated with severe morbidity. No factor was associated with death. Reoperations in ACHD are mostly due to right ventricular outflow tract lesions and were associated with a low mortality rate if performed in a center with a considerable activity and a dedicated program. Severe morbidity is relatively frequent and is generally associated with the preoperative (high hematocrit due to cyanosis, congestive heart failure, and the number of previous operations) and operative (Fontan operation/conversion and cardiopulmonary bypass duration) conditions of the patient.

Quality outcomes of ACHD patients undergoing cardiovascular procedures and hospital admissions in a free-standing children's hospital

Describe quality outcomes of adults with congenital heart disease (ACHD) undergoing cardiovascular procedures and admissions in a free-standing children's hospital with a multi-disciplinary ACHD program and compared with pediatric outcomes. A challenge for the U.S. healthcare system is where to treat the >1 million ACHD patients (pts): adult hospitals without CHD care, or pediatric hospitals without adult services. We reviewed all CHD pts ≥ 18 yrs of age from 2002-2007. Procedural and hospital related morbidity and mortality were recorded. ANOVA and t-test compared adult with pediatric pts. Overall, 782 pts, mean age of 29.8 ± 9.9 yrs, encountered 1490 procedures/admissions. For 178 cardiac surgeries (72% reoperations), mortality was 1.8% and complication rate was 7.3%. There was 0% mortality for 412 cardiac catheterizations, 311 electrophysiological procedures, 401 transesophageal echocardiograms (TEE), 244 exercise tests (ETT) and 54 medical admissions. Major adverse event rate was 0.6% for cardiac catheterization and electrophysiological procedures. No adverse events occurred during TEE and ETT. Only 4 pts required transfer to an adult institution (0.25%). There was no significant difference in mortality or adverse events between pediatric and adult CHD pts, p>0.05. The optimal setting to provide ACHD care remains a complex issue. Our study is the first to demonstrate 1) a low incidence of morbidity and mortality for ACHD pts undergoing cardiovascular procedures or admissions at a free-standing children's hospital, 2) outcomes comparable to pediatric CHD pts. Future models incorporating ACHD programs within pediatric heart centers should be considered to care for this complex population.

High prevalence of abnormal glucose metabolism in young adult patients with complex congenital heart disease

Abnormal glucose metabolism (AGM) adversely impacts morbidity and mortality in patients with chronic heart failure. No data on AGM in adult patients with congenital heart disease (ACHD) are available. To assess the AGM in ACHD and compare the results with their clinical characteristics, we performed a 75-g oral glucose tolerance test to detect AGM, that is, insulin resistance, impaired glucose tolerance, and diabetes mellitus, in 205 consecutive ACHD (24 +/- 8 years), including 16 unrepaired patients, 67 Fontan patients, 122 postbiventricular (BV) patients, and 27 healthy controls (27 +/- 5 years). All ACHD groups had a high prevalence of AGM (unrepaired, 43.8%; Fontan, 43.3%; BV, 46.7%; control, 3.7%; P < .001). In the 2 postoperative groups, the Matsuda index was decreased (P < .0001), and greater waist circumference, liver dysfunction, higher plasma renin activity, and diuretic use were associated with AGM. Although male gender was associated with AGM (P < .01), baseline glucose and lipid metabolic variables did not correlate with the 75-g oral glucose tolerance test-induced hyperglycemia (area under the plasma glucose curve [AUC-PG]) in the Fontan patients but did correlate in the BV patients. The AUC-PG correlated inversely with exercise capacity (P < .05) in the 2 postoperative ACHD groups, and the AGM ACHD had a high incidence of future cardiac events (P < .05), especially the Fontan patients with diabetes mellitus (P < .01). Complex ACHD have a high prevalence of AGM, and this newly recognized pathophysiology should be considered in managing long-term survivors of complex ACHD.

Abstract 4686: Noncontrast Three Dimensional Dark Blood Magnetic Resonance Imaging in Adult Congenital Heart Disease Patients After Transcatheter Intervention

Background : Adults with congenital heart disease (ACHD) often require transcatheter therapies as a result of residual lesions. CT is well-suited for evaluation of stents but exposes young adults to radiation. Gradient echo MRI suffers from artifact limiting assessment of stented segments. We investigate the use of a novel 3D turbo spin echo (TSE) MRI sequence in these patients. Methods: ACHD patients presenting for MRI with prior interventions underwent an additional T1-weighted 3D dark blood navigator respiratory gated TSE scan on a1.5 Tesla system. Type of CHD, prior CT, radiation dose, length of time between examinations, and luminal diameters on each study were obtained. Continuous variables analyzed using Student’s t-test and Bland-Altman plots. Results: Eleven patients underwent both MRI and CT. Diagnoses were coarctation of the aorta (n=6) and tetralogy of Fallot (n=5) with intervention in the aorta or pulmonary arteries respectively. Average radiation dose was 19.57mSv; average time between CT and MRI was 99 ± 160 days. Luminal diameters of stented vessels correlated closely between TSE MRI and CT (r 2 = .85) with a small bias toward overestimation with MRI (mean 22.4 +/− 4.3 mm and 20.9 +/− 3.7 mm, p&lt; .01). There were no cases of in-stent stenosis demonstrated by CT. Conclusion: Metallic artifact is significantly reduced with 3D navigator respiratory gated TSE MRI allowing visualization of anatomy in the vicinity of stents, providing a potential method for assessment of complex anatomy without radiation exposure or use of contrast agents. Further studies of this technique are warranted, particularly in patients with in-stent stenosis.

Abstract 4680: Rapid Growth in Hospitalizations for Adults with Congenital Heart Disease in the United States

Background: Adults with congenital heart disease (ACHD) constitute a growing population due to advances in pediatric care and diagnostic testing. This study aimed to better define the growth in ACHD hospitalizations and to provide data on the cost of hospitalization for these patients. Methods: We utilized data from the 1998 –2005 Nationwide Inpatient Survey, the largest all-payer nationally representative hospital discharge database, to analyze trends in ACHD hospitalizations. We included all patients over 17 years of age with Clinical Classification Software diagnostic code 213 (Cardiac and Circulatory Anomalies). National estimates of hospitalizations and hospital charges by year for ACHD diagnoses were calculated, as were equivalent estimates for specific subsets of patients. Results: The number of ACHD hospitalizations increased 67% between 1998 and 2005, from 53,212 to 88,610 per year. Over this period, the annual number of admissions increased for both simple (44,781 to 75,911) and complex diagnoses (8,431 to 12,699)(Table ). Patients 55 years or older made up approximately 51% of all ACHD patients, and 40% of those with complex disease. Rates of hospitalization increased similarly for patients under and over 55 years old among all diagnostic categories. Inpatient mortality decreased from 2.6+/−0.2% to 1.7+/−0.1% for simple diagnoses, and 4.4+/−0.5% to 3.7+/−0.4% for complex diagnoses. Mean hospital charges per hospitalization increased 66% from $21,485 to $35,565, and the estimated total national charges for these hospitalizations increased from $1.1 billion to $3.1 billion in 2005 (all in 1998 dollars). In 1998, admissions for ACHD accounted for 0.31% of estimated total national charges for hospitalizations of patients 18 years or older, as compared with 0.47% in 2005. Conclusions: There has been a striking increase in hospitalizations for ACHD, and the cost of these admissions is rising out of proportion to overall hospital costs. Number of Adult Congenital Heart Disease Admissions per Year