Administrative health databases for addressing emerging issues in adults with CHD: a systematic review

The need for population-based studies of adults with CHD has motivated the growing use of secondary analyses of administrative health data in a variety of jurisdictions worldwide. We aimed at systematically reviewing all studies using administrative health data sources for adult CHD research from 2006 to 2016. Using PubMed and Embase (1 January, 2006 to 1 January, 2016), we identified 2217 abstracts, from which 59 studies were included in this review. These comprised 12 different data sources from six countries. Of these, 55% originated in the United States of America, 28% in Canada, and 17% in Europe and Asia. No study was published before 2007, after which the number of publications grew exponentially. In all, 41% of the studies were cross-sectional and 25% were retrospective cohort studies with a wide variation in the availability of patient-level compared with hospitalisation-level episodes of care; 58% of studies from eight different data sources linked administrative data at a patient level; and 37% of studies reported validation procedures. Assessing resource utilisation and temporal trends of relevant epidemiological and outcome end points were the most reported objectives. The median impact factor of publication journals was 4.04, with an interquartile range of 3.15, 7.44. Although not designed for research purposes, administrative health databases have become powerful data sources for studying adult CHD populations because of their large sample sizes, comprehensive records, and long observation periods, providing a useful tool to further develop quality of care improvement programmes. Data linkage with electronic records will become important in obtaining more granular life-long adult CHD data. The health services nature of the data optimises the impact on policy and public health.

Related papers:

title: Temporal Trends in Survival to Adulthood Among Patients Born With Congenital Heart Disease From 1970 to 1992 in Belgium
abstract: Over the past decades, the life expectancy of individuals with congenital heart disease (CHD) has increased significantly. However, precise estimates for survival to adulthood are scarce for patients with CHD. We investigated the proportion of CHD patients born between 1990 and 1992 who survived into adulthood. We also compared their survival with that of CHD patients born in earlier eras and evaluated survival as a function of the type of heart defect.  We reviewed the CHD program administrative and clinical database at the University Hospitals Leuven (Leuven, Belgium) and analyzed the records of 7497 CHD patients born from 1970 to 1992. Survival to 18 years of age in patients born between 1990 and 1992 was 88.6% (95% confidence interval [CI], 86.3% to 90.5%), which was significantly greater than that of patients born in previous decades (P<0.0001). For patients born between 1990 and 1992, survival into adulthood for those with mild heart defects was 98.0% (95% CI, 95.8% to 99.1%), whereas survival for those with moderate- and severe-complexity heart defects was 90.0% (95% CI, 86.8% to 92.5%) and 56.4% (95% CI, 47.4% to 64.5%), respectively. Analysis per heart defect confirmed these findings, demonstrating that patients with univentricular heart (49.1% [95% CI, 30.8% to 65.1%]) and hypoplastic left heart syndrome (7.5% [95% CI, 0.6% to 26.6%]) had the poorest survival rate.  This study demonstrates that almost 90% of children with CHD have the prospect of surviving into adulthood.

title: Health care resource utilization in adults with congenital heart disease.
abstract: The number of adults with congenital heart disease (CHD) is increasing. However, rates of health care resource utilization in this population are unknown. The objectives of this study were to describe the use of general health care resources in adults with CHD and to examine the impact of CHD severity on resource utilization. The study consisted of adults alive in 1996 who had > or = 1 diagnosis of a CHD lesion conforming to the International Classification of Disease, Ninth Revision, in the physician's claims database of the province of Quebec from 1983 to 2000. From 1996 to 2000, rates of health care utilization were measured. The impact of the severity of CHD on the use of health care resources was determined using multivariate models to adjust for age, gender, Charlson co-morbidity score, and duration of follow-up. The study population consisted of 22,096 adults with CHD (42% men). From 1996 to 2000, 87% received outpatient care from specialists, 68% visited emergency rooms, 51% were hospitalized, and 16% were admitted to critical care units. Patients with severe CHD had higher adjusted rates of outpatient cardiologist care (rate ratio [RR] 2.24, 95% confidence interval [CI] 2.06 to 2.45), emergency department utilization (RR 1.09, 95% CI 1.03 to 1.17), hospitalization (RR 1.30, 95% CI 1.19 to 1.43), and days in critical care (RR 2.12, 95% CI 1.80 to 2.50) than patients with other congenital cardiac lesions. Hospitalization rates were higher than in the general Quebec adult population (RR 2.08, 95% CI 2.00 to 2.17). In conclusion, adults with CHD have high rates of health care resource utilization, particularly those with severe lesions. Appropriate resource allocation is required to serve this growing population.

title: Trends in hospitalizations for adults with congenital heart disease in the U.S.
abstract: The purpose of this study was to better define the epidemiology of hospitalizations for adults with congenital heart disease (ACHD) in the U.S.



There is a growing population of ACHD as the result of advances in pediatric care and diagnostic testing.



We used nationally representative data from the 1998 to 2005 Nationwide Inpatient Sample to identify patients > or =18 years of age admitted to an acute care hospital with an International Classification of Diseases-9th Revision code designating a CHD diagnosis. National estimates of hospitalizations and total hospital charges by year were calculated.



The number of ACHD hospitalizations increased 101.9% from 35,992 +/- 2,645 in 1998 to 72,656 +/- 5,258 in 2005. During this period, the annual number of admissions grew for both simple (19,448 +/- 1,614 to 44,707 +/- 3,644) and complex (12,507 +/- 1,172 to 19,973 +/- 1,624) diagnoses. The percentage of admissions originating in the emergency department (41.7 +/- 0.8%) or involving cardiac surgery (17.7 +/- 0.7%) remained stable during the study period. The average patient age (52.3 +/- 0.8 years to 53.8 +/- 0.6 years, p < 0.0001) and proportion of patients with > or =2 medical comorbidities (23.3 +/- 0.9% to 33.0 +/- 0.7%, p < 0.0001) increased. Mean hospital charges per hospitalization increased 127% from 19,186 +/- 803 to 43,496 +/- 2,166 US dollars, and the estimated total national charges for these hospitalizations increased 357% from 691 million US dollars in 1998 to 3.16 billion US dollars in 2005 (in inflation-adjusted 2005 dollars).



The number of hospital admissions for ACHD in the U.S. more than doubled between 1998 and 2005. Hospital charges attributable to these admissions have grown even more dramatically.

title: Major adverse cardiovascular events in adult congenital heart disease: a population-based follow-up study from Taiwan
abstract: BackgroundThe aim of the present study was to identify the long-term major adverse cardiovascular events (MACE) in adult congenital heart disease (ConHD) patients in Taiwan.MethodsFrom the National Health Insurance Research Database (1997-2010), adult patients (≥18 years) with ConHD were identified and compared to non-ConHD control patients. The primary end point was the incidence of MACE. Cox proportional hazards models were used to compute hazard ratios as estimates for multivariate adjusted relative risks with or without adjusting for age and sex. total of 3,267 adult patients with ConHD were identified between 2000 and 2003 with a median follow-up of 11 years till December 31, 2010. The five most common types of ConHD were atrial septal defects, ventricular septal defects, patent ductus arteriosus, tetralogy of Fallot, and pulmonary stenosis. Overall, the incidence of MACE was 4.0-fold higher in the ConHD group compared with the controls. After adjustment for age and gender, the patients with ConHD had an increased risk of heart failure, malignant dysrhythmia, acute coronary syndrome, and stroke. The adult ConHD patients had a decreased life-long risk of MACE if they received surgical correction, especially in the patients with atrial septal defects.ConclusionsAfter a median of 11 years of follow-up, the Taiwanese patients with ConHD were at an increased risk of life-long cardiovascular MACE, including heart failure, stroke, acute coronary syndrome, and malignant dysrhythmia. Surgical correction may help to decrease long-term MACE in ConHD patients, especially those with ASD.

title: Children and Adults With Congenital Heart Disease Lost to Follow-Up: Who and When?
abstract: Many patients with congenital heart disease (CHD) require lifelong care. However, the duration of cardiology follow-up in children and adults with CHD is unknown. We sought to determine the proportion of children and young adults with CHD receiving outpatient cardiology care and to identify predictors of lack of follow-up.  The study population consisted of individuals born in 1983 and alive at age 22 years who were diagnosed with CHD in Quebec, Canada, before 6 years of age (n=643). Patients and outpatient visits were identified with the use of the provincial physician's claims database. Three age groups were examined for the presence of outpatient cardiology follow-up: 6 to 12, 13 to 17, and 18 to 22 years. CHD lesions were classified as severe (n=84; 13%), simple shunts (n=390; 61%), and “other” lesions (n=169; 26%). Failure to receive cardiac follow-up after the 6th, 13th, and 18th birthday occurred in 28%, 47%, and 61%, respectively. Among those with severe lesions, only 79% were seen after the 18th birthday. However, the majority of subjects visited primary care physicians in all age groups, and 93% remained in contact with the healthcare system into early adulthood. Predictors of lack of cardiology follow-up in adulthood included male sex, a nonsevere lesion, and a history of follow-up outside a university hospital setting.  Lack of cardiology follow-up begins during childhood, even among those with severe lesions. This occurs despite patients being in contact with other healthcare providers. Improved communication with primary care physicians may reduce the proportion of patients lost to cardiac follow-up.

title: Specialized Adult Congenital Heart Disease Care: The Impact of Policy on Mortality
abstract: Clinical guidelines recommend specialized care for adult congenital heart disease (ACHD) patients. In reality, few patients receive such dedicated care. We sought to examine the impact of specialized care on ACHD patient mortality.  We examined referral rates to specialized ACHD centers and ACHD patient mortality rates between 1990 and 2005 in the population-based Quebec Congenital Heart Disease database (n=71 467). This period covers several years before and after the publication of guidelines endorsing specialized care for ACHD patients. A time-series design, based on Joinpoint and Poisson regression analyses, was used to assess the changes in annual referral and patient mortality rates. The association between specialized ACHD care and all-cause mortality was assessed in both case–control and cohort studies. The time-series analysis demonstrated a significant increase in referral rates to specialized ACHD centers in 1997 (rate ratio, +7.4%; 95% confidence interval [CI], +6.6% to +8.2%). In parallel, a significant reduction in expected ACHD patient mortality was observed after year 2000 (rate ratio, −5.0%;95% CI, −10.8% to −0.8%). In exploratory post hoc cohort and case–control analyses, specialized ACHD care was independently associated with reduced mortality (hazard ratio, 0.78; 95% CI, 0.65–0.94) and a reduced odds of death (adjusted odds ratio, 0.82; 95% CI, 0.08–0.97), respectively. This effect was predominantly driven by patients with severe congenital heart disease (hazard ratio, 0.38; 95% CI, 0.22–0.67).  A significant increase in referrals to specialized ACHD centers followed the introduction of the clinical guidelines. Moreover, referral to specialized ACHD care was independently associated with a significant mortality reduction. Our findings support a model of specialized care for all ACHD patients.

title: Lapse of care as a predictor for morbidity in adults with congenital heart disease.
abstract: There is a need for timely transition of patients with congenital heart disease who have reached adulthood. While the American Heart Association guidelines dictate that patients with moderate or great complexity lesions be seen at least every 2 years, lapse in care is common. We sought to assess the frequency and clinical impact of lapse of medical care in adults with moderate or great complexity cardiac lesions diagnosed in childhood.



All patients, fulfilling the above criteria, who were seen in an Adult Congenital Heart Disease regional clinic from 2002 to 2005 were questioned as to the length of time from leaving care at a pediatric institution to establishment of subsequent cardiac care. Lapse of medical care, defined as > or = 2 year interval from leaving a pediatric cardiac care facility, was determined. Variables associated with lapse of medical care were sought. Variables associated with adverse outcome were identified.



Of 400 patients seen, 158 met inclusion criteria. Of these, lapse of medical care was present in 99 (63%) with a median duration of lapse of medical care of 10 (2-50 years). The most common reason cited for lapse of medical care was that the patient was told there was no need for follow-up (32%). Patients with lapse of medical care were 3.1x(p=0.003) more likely to require urgent cardiac intervention (p=0.003).



Lapse of medical care is common in adults with congenital heart disease and is associated with adverse outcome.

title: Congenital Heart Disease in 56,109 Births Incidence and Natural History
abstract: Within a prospective study of 56,109 total births, 457 youngsters have been found to have congenital heart disease. The overall incidence is 8.14/1000 total births, 8.0/1000 for the Negro and 8.3/1000 for the white. A specific lesion has been identified for each patient and lesion frequencies given for each class of patient, stillbirth, neonatal death, infant death, childhood death, and survivors. The percentage of autopsies was 93% in the stillbirths, 89% in the neonatal deaths, and 76% for those dying after 28 days of age. Of those classified as having definite congenital heart disease, 93% have been examined by a pediatric cardiologist. The average follow-up time for the 272 survivors is 3 years. Thirty-five per cent of patients with ventricular septal defect surviving more than 6 months had their lesion close spontaneously; one-half of the survivors with tetralogy of Fallot were “atypical,” and essentially equal numbers of blacks and whites had all types of coarctation of the aorta in line with the study population, which is 47% black and 53% white.

title: Heart transplantation for adults with congenital heart disease: analysis of the United network for organ sharing database.
abstract: Congenital heart disease (CHD) in the adult is an uncommon indication for heart transplantation but has been increasing. We assessed survival and predictors of death after heart transplantation for adults with CHD.



Adult primary heart transplant recipients (aged > 17 years) reported to the United Network for Organ Sharing (1987 to 2006) were reviewed and categorized by diagnosis of CHD vs other diagnoses. Kaplan-Meier survival analysis and Cox regression modeling were performed.



During the study period, 35,334 adults underwent primary heart transplantation, and 689 (2%) had CHD. Adult CHD recipients had longer mean waiting list time (218 vs 195 days; p = 0.004), longer ischemic time (3.5 vs 2.9 hours, p < 0.0001), and were more likely to have pretransplant pulmonary vascular resistance exceeding 4 Woods Units (62% vs 51%, p < 0.0001) vs other recipients. Thirty-day mortality was 16% vs 6% (p < 0.0001), although Kaplan-Meier survival did not differ between groups (p = 0.92) out to 10 years. Ischemic time (hazard ratio [HR], 1.2; 95% confidence interval [CI], 1.02 to 1.35; p = 0.02), African American race (HR, 1.9; 95% CI, 1.04 to 3.58; p = 0.03), and pulmonary vascular resistance exceeding 4 Woods Units (HR, 1.5; 95% CI, 1.01 to 2.19; p = 0.04) were predictors of death for adult CHD recipients.



Heart transplantation for adults with CHD is effective and has good long-term prognosis. The 30-day mortality rate is high, but 5- and 10-year survival is not statistically different from patients without CHD.

title: Atrial arrhythmias in adult patients with right- versus left-sided congenital heart disease anomalies.
abstract: Atrial arrhythmias (AAs) are a common complication in adult patients with congenital heart disease. We sought to compare the lifetime prevalence of AAs in patients with right- versus left-sided congenital cardiac lesions and their effect on the prognosis. A congenital heart disease diagnosis was assigned using the International Disease Classification, Ninth Revision, diagnostic codes in the administrative databases of Quebec, from 1983 to 2005. Patients with AAs were those diagnosed with an International Disease Classification, Ninth Revision, code for atrial fibrillation or intra-atrial reentry tachycardia. To ensure that the diagnosis of AA was new, a washout period of 5 years after entry into the database was used, a period during which the patient could not have received an International Disease Classification, Ninth Revision, code for AA. The cumulative lifetime risk of AA was estimated using the Practical Incidence Estimators method. The hazard ratios (HRs) for mortality, morbidity, and cardiac interventions were compared between those with right- and left-sided lesions after adjustment for age, gender, disease severity, and cardiac risk factors. In a population of 71,467 patients, 7,756 adults developed AAs (isolated right-sided, 2,229; isolated left-sided, 1,725). The lifetime risk of developing AAs was significantly greater in patients with right- sided than in patients with left-sided lesions (61.0% vs 55.4%, p <0.001). The HR for mortality and the development of stroke or heart failure was similar in both groups (HR 0.96, 95% confidence interval [CI] 0.86 to 1.09; HR 0.94, 95% CI 0.80 to 1.09; and HR 1.10, 95% CI 0.98 to 1.23, respectively). However, the rates of cardiac catheterization (HR 0.63, 95% CI 0.55 to 0.72), cardiac surgery (HR 0.40, 95% CI 0.36 to 0.45), and arrhythmia surgery (HR 0.77, 95% CI 0.6 to 0.98) were significantly less for patients with right-sided lesions. In conclusion, patients with right-sided lesions had a greater lifetime burden of AAs. However, their morbidity and mortality were no less than those with left-sided lesions, although the rate of intervention was substantially different.

title: A United Network for Organ Sharing analysis of heart transplantation in adults with congenital heart disease: outcomes and factors associated with mortality and retransplantation.
abstract: Heart transplantation in patients with adult congenital heart disease is increasing, yet no large studies have defined how this subgroup differs from other adult recipients. We investigated outcomes and risk factors for mortality and retransplantation among patients with adult congenital heart disease compared with adult recipients.



A review was performed of 18- to 45-year-old patients undergoing heart transplantation from 1990-2008 reported to the United Network for Organ Sharing database. Trends were compared between 2 eras: era 1 (1990-1998) and era 2 (1999-2008). Multivariable semiparametric hazard models identified factors associated with time-related death and retransplantation.



Of 8496 patients identified, 575 had adult congenital heart disease. The prevalence of heart transplantation among adult recipients decreased by 28% over time (P < .001) and increased among patients with adult congenital heart disease by 41% (P < .001). Induction therapy use was less in patients with adult congenital heart disease (66%) compared with that seen in adult recipients (71%, P = .02). Steroid maintenance was less in patients with adult congenital heart disease (92%) compared with that seen in adult recipients (97%, P < .001). Post-heart transplantation survival among adult recipients improved over time (P = .02) but not among patients with adult congenital heart disease (P = .81). Overall post-heart transplantation mortality (P = .006) and retransplantation (P = .03) were significantly higher for patients with adult congenital heart disease than for adult recipients, mainly because of an early hazard phase. Adult congenital heart disease was a risk factor for both death (P < .001) and retransplantation (P = .04). Any induction therapy and steroid maintenance was associated with improved survival for all recipients (P = .001).



Adult congenital heart disease represent an increasing proportion of heart transplant recipients. Compared with adult recipients, patients with adult congenital heart disease experience higher post-heart transplantation mortality and retransplantation. Immunosuppression differs among patients with adult congenital heart disease and adult recipients. Further studies should investigate whether post-heart transplantation outcomes would be improved by more aggressive induction therapy or judicious steroid tapers.

title: Changing mortality in congenital heart disease.
abstract: This study sought to characterize temporal trends in all-cause mortality in patients with congenital heart disease (CHD).



Historically, most deaths in patients with CHD occurred in early childhood. Notable advances have since been achieved that may impact on mortality trends.



We conducted a population-based cohort study of patients with CHD in Quebec, Canada, from July 1987 to June 2005. A total of 8,561 deaths occurred in 71,686 patients with CHD followed for 982,363 patient-years.



The proportion of infant and childhood deaths markedly declined from 1987 to 2005, with a reduction in mortality that exceeded that of the general population. Distribution of age at death transitioned from a bimodal to unimodal, albeit skewed, pattern, more closely approximating the general population. Overall, mortality decreased by 31% (mortality rate ratio: 0.69, 95% confidence interval [CI]: 0.61 to 0.79) in the last (2002 to 2005) relative to the first (1987 to 1990) period of observation. Mortality rates decreased in all age groups below 65 years, with the largest reduction in infants (mortality rate ratio: 0.23, 95% CI: 0.12 to 0.47). In adults 18 to 64 years, the mortality reduction (mortality rate ratio: 0.84, 95% CI: 0.73 to 0.97) paralleled the general population. Gains in survival were mostly driven by reduced mortality in severe forms of CHD, particularly in children (mortality rate ratio: 0.33, 95% CI: 0.19 to 0.60), and were consistent across most subtypes.



Deaths in CHD have shifted away from infants and towards adults, with a steady increase in age at death and decreasing mortality.

title: Long-term outcomes after surgical versus transcatheter closure of atrial septal defects in adults.
abstract: The purpose of this study was to assess the comparative effectiveness and long-term safety of transcatheter versus surgical closure of secundum atrial septal defects (ASD) in adults.



Transcatheter ASD closure has largely replaced surgery in most industrialized countries, but long-term data comparing the 2 techniques are limited.



We performed a retrospective population-based cohort study of all patients, ages 18 to 75 years, who had surgical or transcatheter ASD closure in Québec, Canada's second-largest province, using provincial administrative databases. Primary outcomes were long-term (5-year) reintervention and all-cause mortality. Secondary outcomes were short-term (1-year) onset of congestive heart failure, stroke, or transient ischemic attack, and markers of health service use.



Of the 718 ASD closures performed between 1988 and 2005, 383 were surgical and 335 were transcatheter. The long-term reintervention rate was higher in patients with transcatheter ASD closure (7.9% vs. 0.3% at 5 years, p = 0.0038), but the majority of these reinterventions occurred in the first year. Long-term mortality with the transcatheter technique was not inferior to surgical ASD closure (5.3% vs. 6.3% at 5 years, p = 1.00). Secondary outcomes were similar in the 2 groups.



Transcatheter ASD closure is associated with a higher long-term reintervention rate and long-term mortality that is not inferior to surgery. Overall, these data support the current practice of using transcatheter ASD closure in the majority of eligible patients and support the decision to intervene on ASD with significant shunts before symptoms become evident.

title: Long-term cost-effectiveness of transcatheter versus surgical closure of secundum atrial septal defect in adults.
abstract: The most common congenital anomaly in adults is secundum, which can be closed using a surgical or transcatheter approach. Despite the growing use of transcatheter ASD closure, few studies have examined the cost-effectiveness of this strategy. We sought to compare the long-term cost effectiveness of transcatheter and surgical closure of secundum in adults.



A decision-analytic model was used with all clinical outcome parameter estimates obtained from the province-wide Québec Congenital Heart Disease Database. Costs were obtained from a single academic centre (Canadian dollars). A cost-effectiveness analysis using a discrete event Monte Carlo simulation model from the perspective of a single third party payer and multiple sensitivity analyses were performed. Patients were followed for a maximum of 5 years after ASD closure.



Between l998 and 2005, we identified 718 adults (n=335 transcatheter; n=383 surgical) who underwent ASD closure in Quebec. The 5-year cost of surgical closure was $15,304 SD $4581 versus $11,060 SD $5169 for the transcatheter alternative. At 5 years, transcatheter closure was marginally more effective than surgery (4.683 SD 0.379 life-years versus 4.618 SD 0.638 life-years). Probabilistic sensitivity analyses demonstrated that transcatheter ASD closure was a dominant strategy with an 80% probability of cost savings and equal or greater efficacy compared to surgical treatment.



Although definitive conclusions are limited given the observational nature of the primary data sources, transcatheter ASD closure appeared to be a cost-effective strategy associated with slightly improved clinical outcomes and reduced costs compared to surgical closure at 5-years follow-up.

title: In-Hospital and 4-Year Clinical Outcomes Following Transcatheter Versus Surgical Closure for Secundum Atrial Septal Defect in Adults
abstract: AbstractAtrial septal defect (ASD) closure is major therapy for patients with secundum ASD. Although surgical closure (SC) and transcatheter closure (TC) are usually performed in such patients, data on the long-term outcomes comparing TC and SC in adults are limited.Data on the participants of this cohort study were retrieved from Taiwan's National Health Insurance Research Database from 2004 to 2011. Secundum ASD patients > 18 years of age who underwent TC or SC were initially enrolled, and those with associated comorbidities were excluded. After propensity score matching, the clinical outcomes between the TC and SC groups were analyzed.There were 595 patients recruited in the TC group and 308 patients in the SC group. The SC group had a higher incidence of systemic thromboembolism (P < 0.001), ischemic stroke (P = 0.002), and all-cause mortality (P = 0.013) when compared with those of the TC group at the index hospitalization, and similar phenomena could also be seen in a around 4-year follow-up period after the procedures (systemic thromboembolism (P < 0.001, HR = 11.48, 95% CI: 3.29–40.05), ischemic stroke (P = 0.005, HR = 9.28, 95% CI: 1.94–44.39), and all-cause mortality (P = 0.035, HR = 2.28, 95% CI: 1.06–4.89). In addition, atrial fibrillation (P = 0.005) and atrial flutter (P = 0.049) more frequently developed in the SC group than in the TC group at the index hospitalization.The adult secundum ASD patients had lower incidence rates of systemic thromboembolism, ischemic stroke, and all-cause mortality after TC than those after SC in the 4-year follow-up after procedures. Transcatheter ASD closure should therefore be given priority

title: Pregnancy outcomes in Marfan syndrome: a retrospective cohort study.
abstract: Marfan syndrome (MFS) is a rare connective tissue disease with significant risk for adverse cardiovascular outcomes. Our objective was to evaluate pregnancy and cardiovascular outcomes in pregnant women with MFS.



We conducted a population-based retrospective cohort study using the Healthcare Cost and Utilization Project Nationwide Inpatient Sample (HCUP NIS) database from 2003 to 2010. We used unconditional regression analyses to compare maternal and fetal outcomes among pregnancies in women with and without MFS.



Out of the 7,094,400 births in our cohort, 339 deliveries were to women with MFS. There was one maternal death and six aortic dissections among women with MFS. Births to women with MFS were more likely to be premature, odds ratio (OR) 2.15 (1.60-2.89), have intrauterine growth restricted and small for gestational age infants, OR 2.06 (1.24-3.43). Women with MFS were more likely to deliver by cesarean section, OR 1.91 (1.53-2.38) and were at higher risk of major morbidities including cardiac arrhythmias, OR 10.64 (5.49-20.61) and pneumothorax, OR 51.95 (6.18, 437.10).



Pregnant women with MFS are at a particularly high risk of adverse pregnancy and cardiovascular events. Preconception counseling should take these risks into consideration and appropriate pregnancy care in tertiary centers should be considered.

title: Stroke in Adults With Congenital Heart Disease: Incidence, Cumulative Risk, and Predictors.
abstract: Stroke is an important cause of morbidity and mortality, although there is a lack of comprehensive data on its incidence, cumulative risk, and predictors in patients with adult congenital heart disease.



This retrospective study of 29 638 Quebec patients with adult congenital heart disease aged 18 to 64 years between 1998 and 2010 was based on province-wide administrative data. The cumulative risk of ischemic stroke estimated up to age 64 years was 6.1% (95% confidence interval [CI], 5.0-7.0%) in women and 7.7% (95% CI, 6.4-8.8%) in men; the risk of hemorrhagic stroke was 0.8% (95% CI, 0.4-1.2%) and 1.3% (95% CI, 0.8-1.8%), respectively. Compared with rates reported for the general Quebec population, age-sex standardized incidence rates of ischemic stroke were 9 to 12 times higher below age 55 years and 2 to 4 times higher in the age group 55 to 64 years; hemorrhagic stroke rates were 5 to 6 times (age <55 years) and 2 to 3 times higher. Using a combination of stepwise model selection and Bayesian model averaging, the strongest predictors of ischemic stroke were heart failure (odds ratio for age group 18-49 years, 5.94 [95% CI, 3.49-10.14], odds ratio for age group 50-64 years, 1.68 [95% CI, 1.06-2.66]), diabetes mellitus (odds ratio, 2.33 [95% CI, 1.66-3.28]), and recent myocardial infarction (odds ratio, 8.38 [95% CI, 1.77-39.58]).



Among patients with adult congenital heart disease, 1 in 11 men and 1 in 15 women experienced a stroke between ages 18 and 64 years. Stroke incidence was considerably higher than in the general population, especially at a younger age. The most important predictors of ischemic stroke were heart failure, diabetes mellitus, and recent myocardial infarction. Additional research is required to see whether advances in the management of adult congenital heart disease may reduce this substantial stroke rate.

title: The Risk of Cancer in Patients with Congenital Heart Disease: A Nationwide Population-Based Cohort Study in Taiwan
abstract: The relationship between congenital heart disease (CHD) and malignancies has not been determined. This study aimed to explore the association of CHD with malignancies and examine the risk factors for the development of cancer after a diagnosis of CHD.  This nationwide, population-based cohort study on cancer risk evaluated 31,961 patients with newly diagnosed CHD using the Taiwan National Health Insurance Research Database (NHIRD) between 1998 and 2006. The standardized incidence ratios (SIRs) for all and specific cancer types were analyzed, while the Cox proportional hazard model was used to evaluate risk factors of cancer occurrence.  Among patients with newly diagnosed CHD regardless of ages, 187 (0.6%) subsequently developed cancers after a diagnosis of CHD. Patients with CHD had increased risk of cancer (SIR, 1.45; 95% CI, 1.25–1.67), as well as significantly elevated risks of hematologic (SIR, 4.04; 95% CI, 2.76–5.70), central nervous system (CNS) (SIR, 3.51; 95% CI, 1.92–5.89), and head and neck (SIR, 1.81; 95% CI, 1.03–2.94) malignancies. Age (HR, 1.06; 95% CI, 1.05–1.06) and co-morbid chronic liver disease (HR, 1.91; 95% CI, 1.27–2.87) were independent risk factors for cancer occurrence among CHD patients.  Patients with CHD have significantly increased cancer risk, particularly hematologic, CNS, and head and neck malignancies. Physicians who care for patients with CHD should be aware of their predisposition to malignancy after the diagnosis of CHD. Further studies are warranted to clarify the association between CHD and malignancies.

title: Complications of catheter-based electrophysiology procedures in adults with congenital heart disease: a national analysis.
abstract: To determine whether adult congenital heart disease patients (ACHD) undergoing catheter-based electrophysiology (EP) procedures have an increased risk for complications compared with adults without congenital heart disease.



Retrospective cohort study of a national administrative database.



Nationwide Inpatient Sample, 1998 through 2011.



All admission records of patients who underwent a catheter-based electrophysiology procedure, categorized based on the presence or absence of ACHD.



ACHD and non-ACHD cohorts were compared with respect to baseline, procedural, and outcome characteristics.



ACHD patients accounted for n=15,133 (1.7%) of n=873,437 EP procedure admissions and comprised a significantly increasing proportion over the study period (from 0.8% in 1998 to 2.4% in 2011, p<0.0001). ACHD patients were younger than non-ACHD patients (52.5±0.3 years v 61.9±0.04 years; p<0.0001), had a longer length of stay (4.6±0.1 days v 4.4±0.01 days, p=0.013), higher total hospital charges ($89,485±$1,543 v $70,456±$175, p<0.0001), and a higher rate of procedure-related complications (odds ratio 1.66, 95% confidence interval 1.49-1.85, p<0.0001). On multivariate analysis, ACHD patients continued to demonstrate an increased risk of procedural complications (odds ratio 1.95, 95% confidence interval 1.75-2.19, p<0.0001).



ACHD patients experienced greater morbidity after catheter-based EP procedures. This finding will be of increasing significance as ACHD patients occupy a growing segment of the population undergoing these procedures. Further investigations are warranted to determine whether this increased risk is modifiable, with the aim of improving patient safety.

title: Risk of Systemic Hypertension and Cerebrovascular Accident in Patients With Aortic Coarctation Aged <60 Years (from a National Database Study).
abstract: Patients with coarctation of the aorta (CoA) may develop hypertension and cerebrovascular accident (CVA) late after intervention, but the risk is still unclear. Therefore, we studied the epidemiologic profile from a general population aged <60 years to assess the relative risk. Our study population consisted of 126,943 patients with congenital heart disease (CHD) derived from Taiwan's National Health Insurance database from 2000 to 2010 (population 22,765,535). There were 2,295 patients with CoA (50.3% men) (prevalence: 0.116/1000). Associated cardiac lesions in 44% of the patients with CoA included ventricular septal defect (VSD; 23.3%), patent ductus arteriosus (7.5%), atrial septal defect (9.0%), and so forth. Hypertension occurred in 190 patients (8.3%) and was related to age, without associated VSD (odds ratio [OR] 5.90, 95% confidence interval [CI] 2.12 to 16.39, p = 0.001) and male gender (OR 1.59, 95% CI 1.09 to 2.30, p = 0.015). The incidence of hypertension increased rapidly in the young adulthood and was 1.36% in pediatric age group and 28.9% and 45.7% in age group 20 to 29 and 30 to 39, respectively. CVA occurred in 29 patients (1.67%), and the coexisting hypertension was the single predictor (OR 3.19, 95% CI 1.13 to 9.00, p = 0.029). Furthermore, in all patients with CHD, CoA was an independent risk factor for hypertension (OR 15.65, 95% CI 12.44 to 19.68, p <0.001) and CVA (OR 6.55, 95% CI 4.41 to 9.74, p <0.001). Comparing with non-CoA CHD patients, patients with CoA, particularly men, adult, and patients without VSD, have high risk of hypertension, which would further increase the risk of CVA. Early recognition and timely intervention for the hypertension in patients with CoA is mandatory.

title: Arrhythmic burdens in patients with tetralogy of Fallot: a national database study.
abstract: Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease with increasingly recognized late morbidity.



The purpose of this study was to explore the long-term outcome by using a national database of Taiwan, a country with national health insurance and easily accessible medical care.



Data on TOF patients were retrieved from database records from 2000 to 2010. Complications and therapies were identified by their respective codes.



We identified 4781 TOF patients (prevalence 0.63/1000 in pediatric patients and 0.06/1000 in adult patients). Arrhythmias were identified in 219 patients (8.3% for adult patients and 2.8% for pediatric patients): 160 tachycardia and 59 bradycardia (4 with tachy-bradycardia syndrome). The occurrence of arrhythmias was associated with higher mortality (excluding cardiac surgical death, 15.6% vs 8.6%, P = .001). Patients with atrial fibrillation were the oldest (median age 44.3 years), followed by those with tachy-bradycardia syndromes (32.4 years) and atrial flutter (31.5 years). The incidence of nonperioperative tachycardia increased with age (1.4%, 1.7%, 3.3%, 5.2%, 10.2%, and 16.9% in age group 0-9, 10-19, 20-29, 30-39, 40-49, and ≥50 years, respectively). Tachycardia therapy (ablation and implantable cardioverter-defibrillator) was administered in 20.4% (annually 2.4%) of patients with nonperioperative tachycardia. In the subgroup born 2000-2010 with complete postnatal data, mortality was 15.1% (296/1960), and 1-, 5-, and 10-year survival was 0.911, 0.826, and 0.788, respectively. Risk of atrioventricular block requiring a pacemaker was 0.6%.



Arrhythmias are common in TOF patients and increase mortality risk. Medical needs because of tachycardia often appear late in adulthood.

title: Hospital Readmission of Patients With Congenital Heart Disease in Canada.
abstract: Risk factors for hospital readmission in children or adults with congenital heart disease (CHD) are poorly understood. We assessed readmission rates among patients with CHD stratified by age, sex, and severity of CHD and identified risk factors associated with hospital readmission.



A retrospective cohort study was conducted among patients with CHD identified from the Discharge Abstract Database of the Canadian Institute for Health Information from fiscal years 2003-2013. Index hospitalizations were identified and patients followed to identify readmissions. Poisson regression was used to analyze readmission rates by age, sex, and severity of CHD. Logistic regression analysis was performed to identify risk factors associated with readmission within 1 month after discharge.



There were 67,940 index hospitalizations. The readmission rate per 1000 patients with CHD was 48 within 2 weeks, 83 within 1 month, 163 within 3 months, and 351 within 1 year. Patients aged ≥ 65 years had the highest readmission rate at 1 month (12.6%) followed by 40- to 64-year old adults (8.5%), and infants (8.3%). Readmission rates were 2.3-4.1 times higher in patients with complex CHD (P < 0.001) and 1.3-1.8 times higher in patients with moderate CHD (P < 0.001) than in simple patients with CHD. Risk factors for readmission were age ≥ 40 years, age < 1 year, male sex, longer index hospitalization stay, and complex CHD. Increasing Charlson comorbidity score was a risk factor for readmission among adults.



Greater than one third of hospitalized patients with CHD were readmitted within 1 year. Age, sex, and CHD complexity influence readmission risk. Future studies should investigate the reasons for unplanned readmission.

title: Congenital Heart Disease Hospitalizations in Canada: A 10-Year Experience.
abstract: The effect of the growing population of children and adults with congenital heart disease (CHD) on inpatient services in Canada is not known. We sought to assess temporal changes in hospitalizations of CHD patients.



We identified all patients with a CHD diagnosis who received inpatient care in Canada between fiscal years 2003 and 2012 according to the Discharge Abstract Database of the Canadian Institute for Health Information. Poisson regression was performed to assess temporal changes in the annual hospitalization rate. Hospitalization rates were indexed to the general population and the estimated CHD population.



A total of 103,034 hospitalizations occurred in 61,051 patients from fiscal years 2003 to 2012. The absolute number of hospitalizations increased by 4.0% per year in adults and 1.3% per year in children. The greatest increase was in patients aged ≥ 65 years followed by those 40-64 years. However, the hospitalization rate in adults varied between 39 and 55 per 1000 CHD population with a reduction of 4% per year (95% confidence interval, 0.95-0.96; P < 0.001). The hospitalization rate in children ranged from 79 to 87 per 1000 CHD population and did not change significantly over time (rate ratio, 1.00; 95% confidence interval, 1.00-1.01; P = 0.035). Men accounted for 53.5% of hospitalizations.



The absolute number of hospitalizations of patients with CHD increased over time in children and adults. However, the hospitalization rate relative to the CHD population decreased among adults, possibly reflecting improved outpatient management. The absolute increase in CHD hospitalizations will pose a financial burden on health care systems.

title: Congenital Heart Disease in the General Population: Changing Prevalence and Age Distribution
abstract: Empirical data on the changing epidemiology of congenital heart disease (CHD) are scant. We determined the prevalence, age distribution, and proportion of adults and children with severe and other forms of CHD in the general population from 1985 to 2000.  Where healthcare access is universal, we used administrative databases that systematically recorded all diagnoses and claims. Diagnostic codes conformed to the International Classification of Disease, ninth revision. Severe CHD was defined as tetralogy of Fallot, truncus arteriosus, transposition complexes, endocardial cushion defects, and univentricular heart. Prevalence of severe and other CHD lesions was determined in l985, 1990, 1995, and 2000 using population numbers in Quebec. Children were subjects <18 years of age. The prevalence was 4.09 per 1000 adults in the year 2000 for all CHD and 0.38 per 1000 (9%) for those with severe lesions. Female subjects accounted for 57% of the adult CHD population. The median age of all patients with severe CHD was 11 years (interquartile range, 4 to 22 years) in 1985 and 17 years (interquartile range, 10 to 28 years) in 2000 (P<0.0001). The prevalence of severe CHD increased from 1985 to 2000, but the increase in adults was significantly higher than that observed in children. In the year 2000, 49% of those alive with severe CHD were adults.  The prevalence in adults and median age of patients with severe CHD increased in the general population from 1985 to 2000. In 2000, there were nearly equal numbers of adults and children with severe CHD.

title: Ten-year epidemiological review of in-hospital patients with Marfan syndrome.
abstract: Marfan syndrome is a connective tissue disorder with a worldwide prevalence of 1 in 5,000, without any racial predilection. Major cardiovascular manifestations of Marfan disease often require surgical intervention. The aim of this study was to examine the demographics of patients with Marfan syndrome admitted to Hong Kong hospitals over a 10-year period from 1997 to 2006. We retrospectively reviewed the prospectively collected Hong Kong Health Authority's Clinical Data Analysis and Reporting System and Clinical Medical System. Statistical analysis was performed using SPSS, version 15. A total of 525 patients with Marfan syndrome (310 male, 215 female) were included in this study. For males, mean age at first hospital admission was 19.8 years (range 0-78) and for females, 18.7 years (range 0-60). One hundred and twelve (21.3%) patients (56 male, 56 female) had documented aortoiliac aneurysms and/or dissection, with 74 (66.7%) cases involving the thoracic aorta. Forty-nine (9.3%) patients had major cardiac or aortoiliac operations, with an operative mortality of 5/49 (10.2%). Thirty-seven (7.0%) patients (23 male, 14 female) died during this period, with a mean age at death of 41.0 years (range 0-83) for males and 29.9 years (range 0-59) for females. The majority of these patients died of cardiovascular causes, with four aortic dissections, two ruptured aneurysms, seven with sudden collapse and cardiac arrest, and five with heart failure. In addition, there were other causes of mortality: five perioperative, one congenital, and four pulmonary causes, three with malignancy and one of stroke. The cause of death was unknown in five patients. Patients with aortoiliac diseases have a statistically significant higher mortality rate (p < 0.05). This population-based study shows that significant numbers of patients with Marfan syndrome are admitted to hospital per year, with a significant proportion requiring admissions at a young age. Life span in Marfan patients is markedly shortened, and aortoiliac disease is probably underdiagnosed. A standardized diagnostic and therapeutic follow-up program should be offered to these patients and their families.

title: Inpatient admissions and costs of congenital heart disease from adolescence to young adulthood.
abstract: Health care utilization patterns and associated costs of congenital heart disease (CHD) in young adulthood are not well understood.



California State Inpatient Databases 2005 to 2009 were used to conduct a retrospective study on inpatient admissions of CHD patients 10 to 29 years old (n = 7,419) and of all patients of the same age (n = 1,195,233).



Compared with all patients, annual admission costs of CHD patients monotonically decreased, from $13.9 million at age 10 to 14 years to $7.2 million at age 25 to 29 years, which were due to lower costs per admission and somewhat fewer annual hospitalizations. Admissions from the emergency department in CHD patients increased with age regardless of insurance status: at age 25 to 29 years, 62% of admissions with public insurance, 43% with private insurance, and 78% with no insurance were admitted from the emergency department. Cardiac surgery, catheterization, and electrophysiologic study admissions decreased with age in CHD patients, whereas admissions due to arrhythmia and congestive heart failures became more prevalent. Results from multivariate linear regression in CHD patients showed that cardiac surgery was the most costly factor, associated with $29,543 more in costs per admission, followed by the use of a children's hospital, at $10,533. Factors predicting lower costs included female gender, low-complexity CHD, and shorter stay, all P < .001.



Compared with adolescents, young adults with CHD use fewer resources because the natural history of CHD results in fewer surgical admissions and more frequent but less expensive medical admissions.

title: Decentralization of Care for Adults with Congenital Heart Disease in the United States: A Geographic Analysis of Outpatient Surgery
abstract: Guidelines recommend that adults with congenital heart disease (CHD) undergo noncardiac surgery in regionalized centers of expertise, but no studies have assessed whether this occurs in the United States. We hypothesized that adults with CHD are less likely than children to receive care at specialized CHD centers.  Using a comprehensive state ambulatory surgical registry (California Ambulatory Surgery Database, 2005–2011), we calculated the proportion of adult and pediatric patients with CHD who had surgery at a CHD center, distance to the nearest CHD center, and distance to the facility where surgery was performed.  Patients with CHD accounted for a larger proportion of the pediatric population (n = 11,254, 1.0%) than the adult population (n = 10,547, 0.07%). Only 2,741 (26.0%) adults with CHD had surgery in a CHD center compared to 6,403 (56.9%) children (p<0.0001). Adult CHD patients who had surgery at a non-specialty center (11.9±15.4 miles away) lived farther from the nearest CHD center (37.9±43.0 miles) than adult CHD patients who had surgery at a CHD center (23.2±28.4 miles; p<0.0001). Pediatric CHD patients who had surgery at a non-specialty center (18.0±20.7 miles away) lived farther from the nearest CHD center (35.7±45.2 miles) than pediatric CHD patients who had surgery at a CHD center (22.4±26.0 miles; p<0.0001).  Unlike children with CHD, most adults with CHD (74%) do not have outpatient surgery at a CHD center. For both adults and children with CHD, greater distance from a CHD center is associated with having surgery at a non-specialty center. These results have significant public health implications in that they suggest a failing to achieve adequate regional access to specialized ACHD care. Further studies will be required to evaluate potential strategies to more reliably direct this vulnerable population to centers of expertise.

title: Valvular operations in patients with congenital heart disease: increasing rates from 1988 to 2005.
abstract: The congenital heart disease population is aging. We hypothesized that changes in rates of congenital, valvular, and noncongenital surgical operations in congenital heart patients varied with age and disease severity over the last two decades.



We performed time trend analysis using a Quebec congenital heart disease database constructed from administrative data. We included congenital heart patients of all ages having cardiac surgical operations. Heart lesions were classified as "severe" and "other." Cardiac surgical operations were grouped as congenital, valvular (including aortic), and noncongenital (arrhythmia surgery, coronary artery bypass grafting, and cardiac transplants). An adapted Aristotle score was developed to classify procedures based on surgical risk. Yearly surgical rates were measured as surgical operations per 1,000 person-years and analyzed over time using Poisson regression models stratified by age, lesion severity, and cardiac surgery category.



From 1988 to 2005 we followed 71,979 patients for 1,009,430 person-years. We identified 17,444 cardiac surgical operations. There was a 31% increase in volumes and a 5% increase in surgical rates over time. In children, congenital surgical operations remained constant, accounting for 80% of all surgical operations. In adults, valvular operations were the most common type of surgical operations, increasing from 42% to 63% of all procedures over time. Rates of valvular operations increased significantly in all adult subgroups and in children with severe lesions.



The need for valvular interventions has increased in the last two decades in congenital heart disease patients. These findings should be taken into account when allocating resources that will optimize outcomes for this growing population.

title: Atrial fibrillation and stroke in adult patients with atrial septal defect and the long-term effect of closure
abstract: To estimate the risk of atrial fibrillation (AF) and stroke and the impact of closure in patients with atrial septal defect (ASD) compared with a general population cohort.  All adult Danish patients (>18 years) diagnosed with ASD from 1977 to 2009 (N=1168) were identified through population-based registries. Using Cox regression, we compared ASD patients’ risk of AF and stroke with an age-matched and gender-matched comparison cohort. We computed prevalence proportions of anticoagulation and antiarrhythmic medicine use before and after closure and described stroke-related mortality.  Median follow-up was 9.6 years (range 1–33 years). Patients with ASD had a higher risk of first-time AF (adjusted HR 8.2; 95% CI 6.6 to 10.2) after closure than the comparison cohort, but with no difference between transcatheter and surgical closure (HR 1.5, 95% CI 0.6 to 3.5). Patients without prevalent AF had a 10-year cumulative incidence of AF of 11% (95% CI 9% to 14%) after closure compared with 2% (95% CI 1.8% to 2.5%) in the comparison cohort. Patients with ASD with prevalent AF continued to use anticoagulation medicine after closure/diagnosis. Patients with ASD had increased risk of stroke without closure (adjusted HR 2.6; 95% CI 1.4 to 3.0) and with closure (adjusted HR 2.0; 95% CI 1.4 to 2.7). Risk of stroke after closure was related to AF (HR adjusted for AF 1.3; 95% CI 0.9 to 1.9).  Patients with ASD had a higher risk of first-time AF after closure than the comparison cohort. There was no effect of closure on the use of AF-related medicine in patients with prevalent AF.

title: Risk of pneumonia in adults with closed versus unclosed atrial septal defect (from a nationwide cohort study).
abstract: Atrial septal defect (ASD) is associated with pulmonary hyperperfusion and inflammation and has been linked with increased risk of pneumonia. We investigated the risk of pneumonia in patients with ASD and the impact of ASD closure in a nationwide cohort study. All adults in Denmark (n = 1,168) diagnosed with ASD from 1977 to 2009 were identified through Danish public registries. We compared the risk of hospitalization for pneumonia, use of antibiotics, and mortality with age- and gender-matched general population controls. The median follow-up from time of diagnosis was 9.6 years (range 1 to 33). Cox regression analysis showed that both patients with closed (n = 863) and unclosed (n = 305) ASD had a higher risk of hospitalization for pneumonia (adjusted hazard ratios [HRs] 2.8, 95% confidence interval [CI] 2.3 to 3.5 and 3.1, 95% CI 2.4 to 4.0, respectively) than controls. Patients with ASD also used more antibiotics (adjusted HR for closure 1.4, 95% CI 1.3 to 1.5 and adjusted HR for no closure 1.4, 95% CI 1.2 to 1.6) than the controls. Antibiotic use normalized (adjusted HR 1.1, 95% CI 1.0 to 1.3) and the risk of pneumonia decreased (adjusted HR 1.8, 95% CI 1.1 to 3.0) within 5 years after closure. The proportion of patients with ASD with pneumonia-related death (8.4%) was comparable with that of the general population (9.5%). In conclusion, patients with ASD had a substantially higher risk of hospitalization for pneumonia and used more antibiotics than the general population. The pneumonia risk diminished, but did not completely normalize, 5 years after closure.

title: Perioperative Outcomes of Major Noncardiac Surgery in Adults with Congenital Heart Disease
abstract: An increasing number of patients with congenital heart disease are surviving to adulthood. Consensus guidelines and expert opinion suggest that noncardiac surgery is a high-risk event, but few data describe perioperative outcomes in this population. By using the Nationwide Inpatient Sample database (years 2002 through 2009), the authors compared patients with adult congenital heart disease (ACHD) who underwent noncardiac surgery with a non-ACHD comparison cohort matched on age, sex, race, year, elective or urgent or emergency procedure, van Walraven comborbidity score, and primary procedure code. Mortality and morbidity were compared between the two cohorts. A study cohort consisting of 10,004 ACHD patients was compared with a matched comparison cohort of 37,581 patients. Inpatient mortality was greater in the ACHD cohort (407 of 10,004 [4.1%] vs. 1,355 of 37,581 [3.6%]; unadjusted odds ratio, 1.13; P = 0.031; adjusted odds ratio, 1.29; P < 0.001). The composite endpoint of perioperative morbidity was also more commonly observed in the ACHD cohort (2,145 of 10.004 [21.4%] vs. 6,003 of 37,581 [16.0%]; odds ratio, 1.44; P < 0.001). ACHD patients comprised an increasing proportion of all noncardiac surgical admissions over the study period (P value for trend is <0.001), and noncardiac surgery represented an increasing proportion of all ACHD admissions (P value for trend is <0.001). Compared with a matched control cohort, ACHD patients undergoing noncardiac surgery experienced increased perioperative morbidity and mortality. Within the limitations of a retrospective analysis of a large administrative dataset, this finding demonstrates that this is a vulnerable population and suggests that better efforts are needed to understand and improve the perioperative care they receive.

title: Atrial Arrhythmias in Adults With Congenital Heart Disease
abstract: Atrial arrhythmias increase disease burden in the general adult population. Adults with congenital heart lesions constitute a rapidly growing group of patients with cardiovascular disease. We hypothesized that atrial arrhythmias increase with age and impair health outcomes in this population.  We conducted a population-based analysis of prevalence, lifetime risk, mortality, and morbidity associated with atrial arrhythmias in adults with congenital heart disease from l983 to 2005. In 38 428 adults with congenital heart disease in 2005, 5812 had atrial arrhythmias. Overall, the 20-year risk of developing atrial arrhythmia was 7% in a 20-year-old subject and 38% in a 50-year-old subject. More than 50% of patients with severe congenital heart disease reaching age 18 years developed atrial arrhythmias by age 65 years. In patients with congenital heart disease, the hazard ratio of any adverse event in those with atrial arrhythmias compared with those without was 2.50 (95% confidence interval, 2.38 to 2.62; P<0.0001), with a near 50% increase in mortality (hazard ratio, 1.47; 95% confidence interval, 1.37 to 1.58; P<0.001), more than double the risk of morbidity (stroke or heart failure) (hazard ratio, 2.21; 95% confidence interval, 2.07 to 2.36; P<0.001), and 3 times the risk of cardiac interventions (hazard ratio, 3.00; 95% confidence interval, 2.81 to 3.20; P<0.001).  Atrial arrhythmias occurred in 15% of adults with congenital heart disease. The lifetime incidence increased steadily with age and was associated with a doubling of the risk of adverse events. An increase in resource allocation should be anticipated to deal with this increasing burden.

title: Medical and Obstetric Outcomes Among Pregnant Women With Congenital Heart Disease
abstract: To estimate nationwide trends in the prevalence of maternal congenital heart disease (CHD) and determine whether women with CHD are more likely than women without maternal CHD to have medical and obstetric complications.  The 2000–2010 Nationwide Inpatient Sample was queried for International Classification of Diseases, 9th Revision, Clinical Modification codes to identify delivery hospitalizations of women with and without CHD. Trends in the prevalence of CHD were determined and then rates of complications were reported for CHD per 10,000 delivery hospitalizations. For Nationwide Inpatient Sample 2008–2010, logistic regression was used to examine associations between CHD and complications.  From 2000 to 2010, there was a significant linear increase in the prevalence of CHD from 6.4 to 9.0 per 10,000 delivery hospitalizations (P<.001). Multivariable logistic regression demonstrated that all selected medical complications, including mortality (17.8 compared with 0.7/10,000 deliveries, adjusted odds ratio [OR] 22.10, 95% confidence interval [CI] 13.96–34.97), mechanical ventilation (91.9 compared with 6.9/10,000, adjusted OR 9.94, 95% CI 7.99–12.37), and a composite cardiovascular outcome (614 compared with 34.3/10,000, adjusted OR 10.54, 95% CI 9.55–11.64) were more likely to occur among delivery hospitalizations with maternal CHD than without. Obstetric complications were also common among women with CHD. Delivery hospitalizations with maternal CHD that also included codes for pulmonary circulatory disorders had higher rates of medical complications compared with hospitalizations with maternal CHD without pulmonary circulatory disorders.  The number of delivery hospitalizations with maternal CHD in the United States is increasing, and although we were not able to determine whether correction of the cardiac lesion affected outcomes, these hospitalizations have a high burden of medical and obstetric complications.

title: Geriatric congenital heart disease: burden of disease and predictors of mortality.
abstract: 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.

title: Diagnosis of pulmonary hypertension in the congenital heart disease adult population impact on outcomes.
abstract: The aim of this study was to assess the impact of the diagnosis of pulmonary hypertension (PH) on mortality, morbidity, and health services utilization (HSU) in an adult congenital heart disease (CHD) population.



Although PH is a well-recognized complication of CHD, population-based studies of its significance on the survival and functional capacity of patients are uncommon.



A retrospective longitudinal cohort study was conducted in an adult CHD population with 23 years of follow-up, from 1983 to 2005. The prevalence of PH was measured in 2005. Mortality, morbidity, and HSU outcomes were compared between patients with and without diagnoses of PH using multivariate Cox (mortality and morbidity) and Poisson (HSU) regression models within a subcohort matched for age and CHD lesion type.



Of 38,430 adults alive with CHD in 2005, 2,212 (5.8%) had diagnoses of PH (median age 67 years, 59% women). The diagnosis of PH increased the all-cause mortality rate of adults with CHD more than 2-fold compared with patients without PH (hazard ratio [HR]: 2.69; 95% confidence interval [CI]: 2.41 to 2.99). Morbid complications including heart failure and arrhythmia occurred with a 3-fold higher risk compared with patients without PH (HR: 3.01; 95% CI: 2.80 to 3.22). The utilization of inpatient and outpatient services was increased, especially cardiac catheterization, excluding the index diagnostic study (rate ratio: 5.04; 95% CI: 4.27 to 5.93) and coronary and intensive care hospitalizations (rate ratio: 5.03; 95% CI: 4.86 to 5.20).



A diagnosis of PH in adults with CHD is associated with a more than 2-fold higher risk for all-cause mortality and 3-fold higher rates of HSU, reflecting high morbidity.

title: Outcomes of heart failure-related hospitalization in adults with congenital heart disease in the United States.
abstract: Heart failure (HF) accounts for >3 million hospital admissions annually in adults with acquired cardiovascular disease, but there are limited data on HF admissions in adults with congenital heart disease (ACHD). The purpose of this study was to test the hypotheses that HF admissions are common in ACHD and associated with significant morbidity and mortality.



The 2007 Nationwide Inpatient Sample was used to assess national prevalence, morbidities, and risk factors for mortality during hospitalizations among ACHD with HF.



Of the 84,308 (95% CI 71,345-97,272) ACHD admissions in the United States in 2007, 17,193 (95% CI 14,157-20,229) had a diagnosis of HF (20%). ACHD with HF was associated with an increased risk of death compared to ACHD without HF (OR 3.3, 95% CI 2.6-4.1). On multivariable analysis independent risk factors for mortality included nonoperative intubation (OR 6.1, 95% CI 3.3-11.4), sepsis (OR 4.3, 95% CI 2.4-7.4), and acute myocardial infarction (OR 3.2, 95% CI 1.8-5.7). Cardiac defects associated with an increased risk of mortality included ventricular septal defects (VSDs) (OR 1.8, 95% CI 1.0-3.4).



In this large population-based study, HF-related hospitalizations were common in ACHD and associated with an increased risk of death compared to non-HF admissions. The risk of mortality is increased with the diagnoses of VSDs and the presence of specific comorbidities such as respiratory failure and sepsis.

title: Maternal cardiovascular events during childbirth among women with congenital heart disease
abstract: To define the epidemiology of adverse cardiovascular events among women with congenital heart disease (CHD) hospitalised for childbirth in the USA.  Main outcome measures Logistic regression was used to estimate ORs for cardiovascular outcomes (arrhythmia, heart failure, cerebrovascular accident, embolism, death or a combined outcome) for women with and without CHD. Covariates included age, number of medical comorbidites, pulmonary hypertension, hospital teaching status, insurance status and method of delivery.  Annual deliveries for women with CHD increased 34.9% from 1998 to 2007 compared with an increase of 21.3% in the general population. Women with CHD were more likely to sustain a cardiovascular event (4042/100 000 vs 278/100 000 deliveries, univariate OR 15.1, 95% CI 13.1 to 17.4, multivariable OR 8.4, 95% CI 7.0 to 10.0). Arrhythmia, the most common cardiovascular event, was more frequent among women with CHD (2637/100 000 vs 210/100 000, univariate OR 12.9, 95% CI 10.9 to 15.3, multivariable OR 8.3, 95% CI 6.7 to 10.1). Death occurred in 150/100 000 patients with CHD compared with 8.2/100 000 patients without CHD (multivariable OR 6.7, 95% CI 2.9 to 15.4). Complex CHD was associated with greater odds of having an adverse cardiovascular event than simple CHD (8158/100 000 vs 3166/100 000, multivariable OR 2.0, 95% CI 1.4 to 3.0).  Maternal CHD is associated with a markedly increased risk of adverse cardiovascular events and death during admission for delivery.

title: Single ventricle anatomy is associated with increased frequency of nonalcoholic cirrhosis.
abstract: Single ventricle (SV) patients with Fontan physiology have multiple risk factors for liver disease but the prevalence of liver disease remains unknown in this population. We sought to determine whether hospitalized patients with a SV diagnosis have higher rates of nonalcoholic cirrhosis than patients without congenital heart disease.



We used the 1998-2009 Healthcare Cost and Utilization Project's Nationwide Inpatient Sample, a nationally representative dataset, to identify patients 18-49 years old admitted to an acute care hospital. We compared the rate of nonalcoholic cirrhosis between those with a SV diagnosis and patients without congenital heart disease.



There were 7968 hospitalizations of patients with a SV diagnosis and 13,602,149 hospitalizations of patients without congenital heart disease. SV patients were more likely to have nonalcoholic cirrhosis than those without congenital heart disease (4.3 ± 0.7 vs. 0.3 ± 0.01%, univariate OR 15.2, 95%CI 10.9-21.3), even after adjusting for viral or chronic hepatitis and other cirrhosis risk factors (multivariable OR 21.6, 95%CI 4.3-32.5). The proportion of all hospitalizations among SV patients for nonalcoholic cirrhosis increased by 173% between 1998/9 and 2008/9, from 2.3% to 6.2% (p=0.009). Among those with nonalcoholic cirrhosis, SV patients were more likely to have congestive hepatopathy (6.6 ± 3.1 vs. 0.1 ± 0.0001%, OR 63.2, 95%CI 19.2-207.8), longer hospital stays and higher hospital charges.



A single ventricle diagnosis is associated with markedly higher risk for nonalcoholic cirrhosis in a population-based sample of hospitalized patients. The proportion of patients with single ventricle anatomy admitted for nonalcoholic cirrhosis or its complications is increasing rapidly.

title: Changes in hospitalization patterns among patients with congenital heart disease during the transition from adolescence to adulthood.
abstract: This study was designed to evaluate hospitalization patterns of congenital heart disease (CHD) patients surrounding the transition from adolescence to adulthood.



Few population data exist on hospitalizations among adolescent and adult CHD patients.



Patients ages 12 to 44 years with CHD were selected from the 2000 to 2003 California hospital discharge database. Patient demographics, hospitalization patterns, emergency department (ED) admissions, CHD complexity, and insurance patterns were described. Data were analyzed in 3-year age increments and compared between patients over and under age 21. Predictors of admission via the ED were determined using multivariate regression analysis.



There were 9,017 hospitalizations at 368 hospitals. For patients ages 12 to 20 years, 12 hospitals accounted for 70% of hospitalizations; for patients ages 21 to 44 years, 25 hospitals accounted for only 44.8% of cases. Regarding insurance, 53% of admissions were private, 44% public, and <4% were self-pay. Sixty-five percent of patients had complex CHD and 19% had a cardiac procedure during hospitalization. The proportion of patients admitted via the ED nearly doubled surrounding the transition to adulthood. The positive predictors of admission via the ED included public insurance, self-pay, and age >17 years, whereas having a procedure and being female decreased the likelihood.



Congenital heart disease hospitalizations occur at a wide variety of hospitals and disperse as patients enter adulthood. Those without private insurance and >17 years old are at higher risk of being admitted via the ED. These findings require further investigation to examine access to care and possible disparities, as they are important for future healthcare planning.

title: Trends in utilization of specialty care centers in California for adults with congenital heart disease.
abstract: The American College of Cardiology and American Heart Association guidelines recommend that management of adult congenital heart disease (ACHD) be coordinated by specialty ACHD centers and that ACHD surgery for patients with moderate or complex congenital heart disease (CHD) be performed by surgeons with expertise and training in CHD. Given this, the aim of this study was to determine the proportion of ACHD surgery performed at specialty ACHD centers and to identify factors associated with ACHD surgery being performed outside of specialty centers. This retrospective population analysis used California's Office of Statewide Health Planning and Development's discharge database to analyze ACHD cardiac surgery (in patients 21 to 65 years of age) in California from 2000 to 2011. Designation as a "specialty ACHD center" was defined on the basis of a national ACHD directory. A total of 4,611 ACHD procedures were identified. The proportion of procedures in patients with moderate and complex CHD delivered at specialty centers increased from 46% to 71% from 2000 to 2011. In multivariate analysis among those discharges for ACHD surgery in patients with moderate or complex CHD, performance of surgery outside a specialty center was more likely to be associated with patients who were older, Hispanic, insured by health maintenance organizations, and living farther from a specialty center. In conclusion, although the proportion of ACHD surgery for moderate or complex CHD being performed at specialty ACHD centers has been increasing, 1 in 4 patients undergo surgery at nonspecialty centers. Increased awareness of ACHD care guidelines and of the patient characteristics associated with differential access to ACHD centers may help improve the delivery of appropriate care for all adults with CHD.

title: Percutaneous closure of patent foramen ovale and atrial septal defect in adults: the impact of clinical variables and hospital procedure volume on in-hospital adverse events.
abstract: Percutaneous closure of patent foramen ovale/atrial septal defect (PFO/ASD) is an increasingly common procedure perceived as having minimal risk. There are no population-based estimates of in-hospital adverse event rates of percutaneous PFO/ASD closure.



We used nationally representative data from the 2001-2005 Nationwide Inpatient Sample to identify patients >or-=20 years old admitted to an acute care hospital with an International Classification of Diseases, Ninth Revision code designating percutaneous PFO/ASD closure on the first or second hospital day. Variables analyzed included age, sex, number of comorbidities, year, same-day use of intracardiac or other echocardiography, same-day left heart catheterization, hospital size and teaching status, PFO/ASD procedural volume, and coronary intervention volume. Outcomes of interest included length of stay, charges, and adverse events.



The study included 2,555 (weighted to United States population: 12,544 +/- 1,987) PFO/ASD closure procedures. Mean age was 52.0 +/- 0.4 years, and 57.3% +/- 1.0% were women. Annual hospital volume averaged 40.8 +/- 7.7 procedures (range, 1-114). Overall, 8.2 +/- 0.8% of admissions involved an adverse event. Older patients and those with comorbidities were more likely to sustain adverse events. Use of intracardiac echocardiography was associated with fewer adverse events. The risk of adverse events was inversely proportional to annual hospital volume (odds ratio [OR] 0.91, 95% confidence interval [CI] 0.86-0.96, per 10 procedures), even after limiting the analysis to hospitals performing >or=10 procedures annually (OR 0.91, 95% CI 0.85-0.98). Adverse events were more frequent at hospitals in the lowest volume quintile as compared with the highest volume quintile (13.3% vs 5.4%, OR 2.42, 95% CI 1.55-3.78).



The risk of adverse events of percutaneous PFO/ASD closure is inversely correlated with hospital volume. This relationship applies even to hospitals meeting the current guidelines, performing >or=10 procedures annually.

title: High resource use among adult congenital heart surgery admissions in adult hospitals: risk factors and association with death and comorbidities.
abstract: Adult hospitals are a common location of adult congenital heart disease (ACHD) admissions, including cardiac surgical admissions. Understanding the patterns and predictors of resource use could aid these institutions by identifying and targeting potentially modifiable determinants of high resource use (HRU). Our objectives were to examine resource use during adult congenital heart surgical admissions in adult hospitals, determine the association of HRU with mortality, and identify risk factors for HRU.



Population-based retrospective study We obtained data from the Nationwide Inpatient Sample 2005-2009 and examined ACHD surgical admissions ages 18-49 years (n = 16 231).



We defined HRU as admissions with >90th percentile for total hospital charges.



Despite representing 10% of admissions, HRU admissions accounted for 32% of total charges. HRU admissions had a higher mortality rate (9.7% vs. 1.8%, P < .001). Multivariable analysis demonstrated that HRU is associated with government insurance adjusted odds ratio (AOR) 2.0 (95% confidence interval [CI] 1.6,2.4), emergency admissions AOR 3.9 (95% CI 3.1,4.8), complications AOR 4.2 (95% CI 3.3,5.2), renal failure AOR 1.8 (95% CI 1.4,2.2), congestive heart failure AOR 1.2 (95% CI 1,1.4), surgical complexity risk category-2 AOR 2.0 (95% CI 1.0,3.6), and category-3+ AOR 2.3 (95% CI 1.4,3.8).



HRU admissions for adult congenital heart surgery consumed a disproportionate amount of resources and were associated with higher mortality. HRU risk factors included nonelective admissions, government insurance, heart failure, surgical complexity, renal failure, and complications. Complications, if preventable, may be a target for improvement strategies to decrease resource use. Other risk factors may require a broader patient care approach.

title: Resource Use Among Adult Congenital Heart Surgery Admissions in Pediatric Hospitals: Risk Factors for High Resource Utilization and Association With Inpatient Death
abstract: Pediatric hospitals frequently perform congenital heart surgery in adults with congenital heart disease. The impact of these admissions on pediatric hospital resources is unknown. Our goals were to examine resource use by adults undergoing congenital heart surgery in pediatric hospitals, explore the association between high resource use (HRU) and inpatient death, and identify HRU risk factors. We obtained inpatient data from 42 pediatric hospitals from 2000 to 2008 and selected adult congenital heart (ACH) surgery admissions. We defined HRU admissions as those exceeding the 90th percentile for total hospital charges. We performed multivariable analyses using generalized estimating equations to identify risk factors for HRU. Of 97 563 congenital heart surgery admissions to pediatric hospitals, 3061 (3.1%) were adults, accounting for 2.2% of total hospital charges. The threshold for HRU was total hospital charges ≥$213 803. Although HRU admissions comprised 10% of admissions, they accounted for 34% of charges for all ACH surgery admissions. Mortality rate was 16% for HRU admissions and 0.7% for others (P<0.001). Multivariable analysis demonstrated higher case complexity: risk category 2 (adjusted odds ratio [AOR], 3.6; P=0.02), risk category 3 (AOR, 13.7; P<0.001), and risk category 4+ (AOR, 30.7; P<0.001) as compared with risk category 1; DiGeorge syndrome (AOR, 4.2; P=0.006); depression (AOR, 3.1; P<0.001); weekend admission (AOR, 2.6; P<0.001); and government insurance (AOR, 2.0; P<0.001) as risk factors for HRU. High resource use ACH surgery admissions are associated with significantly greater mortality rates. ACH admissions with greater surgical complexity, government insurance, DiGeorge syndrome, weekend admission, and depression were more likely to result in HRU.

title: Impact of Hospital Volume on Outcomes of Endovascular Stenting for Adult Aortic Coarctation.
abstract: Use of transcatheter endovascular stenting has been increasing in the treatment of coarctation of aorta (CoA). The present study was undertaken on adults with CoA who underwent stent placement from 2000 to 2011 to analyze the relation of hospital volumes to the outcomes of stenting in adults with CoA. It was a retrospective study based on Healthcare Cost and Utilization Project's Nationwide Inpatient Sample (NIS) database from 2000 to 2011 and identified subjects using the International Classification of Diseases, Ninth Revision, Clinical Modification procedure code of 747.10 (CoA). Annual hospital volume was calculated using unique hospital identifiers. Weights provided by the Nationwide Inpatient Sample were used to generate national estimates. A total of 105 (weighted 521) subjects were identified with International Classification of Diseases, Ninth Revision, code of 39.90 (Endovascular stent). Hospital volumes were divided into tertiles. We compared the highest tertile (≥3 procedures annually) with other tertiles (<3 procedure annually). The composite outcomes of the analysis were procedure-related complications, length of stay (LOS), and cost in relation to the hospital volume. No inhospital death was reported in either group. Hospitals with ≥3 procedures annually had significantly lower incidence of complications (9.5% vs 23.0%) compared to the hospitals with <3 procedures annually (p-value 0.002). Similar results were obtained after multivariate regression analysis in relation to hospital volume. Shorter LOS and lower cost were observed with annual hospital volume of ≥3 procedures. In conclusion, stenting adults for CoA is remarkably safe, and the outcomes of the procedure have improved in centers with annual hospital volume of ≥3 procedures. There is also decreasing trend of procedure-related complications, shorter LOS, and lower costs compared to centers with annual volume <3 procedures.

title: Device complications in adult congenital heart disease.
abstract: Pacemakers and implantable cardioverter-defibrillators (ICDs) are increasingly implanted in adults with congenital heart disease (CHD), but little is known about implant-related complications and mortality.



The purpose of this study was to compare pacemaker and ICD implantation complication rates between adults with and those without CHD using a comprehensive, statewide database.



We used the Healthcare Cost and Utilization Project database to identify initial transvenous pacemaker and ICD implantations and implant-related complications in California hospitals from January 1, 2005, to December 31, 2011. We calculated relative risks of implant-related complications by comparing those with and those without CHD using Poisson regression with robust standard errors, adjusting for age and medical comorbidities.



We identified 105,852 patients undergoing pacemaker implantation, 1465 with noncomplex CHD and 66 with complex CHD. CHD was not associated with increased risk of pacemaker implant-related complications: adjusted risk ratio (aRR) 0.92, 95% confidence interval (CI) 0.74-1.14, P = .45. We identified 32,948 patients undergoing ICD implantation, 815 with noncomplex CHD and 87 with complex CHD. Patients with CHD had increased risk of ICD implant-related complications: aRR 1.36, 95% CI 1.05-1.76, P = .02. Patients with complex CHD had greater increased risk of ICD implant-related complications: aRR 2.14, 95% CI 1.16-3.95, P = .02. In patients receiving devices, CHD was associated with a trend toward lower 30-day in-hospital mortality after pacemaker (P = .07) and ICD (P = .19) implantation.



Among adult patients undergoing device implantation in California, CHD was associated with increased risk of ICD implant-related complications, but not pacemaker implant-related complications or higher 30-day in-hospital mortality.

title: Influence of hospital volume on outcomes of percutaneous atrial septal defect and patent foramen ovale closure: A 10‐years us perspective
abstract: Contemporary data regarding percutaneous closure of atrial septal defect/patent foramen ovale (ASD/PFO) are lacking. We evaluated the current trends in utilization of ASD/PFO closure in adults and investigated the effect of annual hospital volume on in‐hospital outcomes.  We queried the Nationwide Inpatient Sample between the years 2001 and 2010 using the International Classification of Diseases (ICD‐9‐CM) procedure code for percutaneous closure of ASD/PFO with device. Hierarchical mixed effects models were generated to identify the independent multivariate predictors of outcomes.  A total of 7,107 percutaneous ASD/PFO closure procedures (weighted n = 34,992) were available for analysis. A 4.7‐fold increase in the utilization of this procedure from 3/million in 2001 to 14/million adults in 2010 in US (P < 0.001) was noted. Overall, percutaneous ASD/PFO closure was associated with 0.5% mortality and 12% in‐hospital complications. The utilization of intracardiac echocardiography (ICE) increased 15 fold (P < 0.001) during the study period. The procedures performed at the high volume hospitals [2nd (14–37 procedures/year) and 3rd (>38 procedures/year) tertile] were associated with significant reduction in complications, length of stay and cost of hospitalization when compared to those performed at lowest volume centers (<13 procedures/year). Majority (70.5%) of the studied hospitals were found to be performing <10 procedures/year hence deviating from the ACC/AHA/SCAI clinical competency guidelines.  Low hospital volume is associated with an increased composite (mortality and procedural complications) adverse outcome following ASD/PFO closure. In the interest of patient safety, implementation of the current guidelines for minimum required annual hospital volume to improve clinical outcomes is warranted.

title: CONCOR, an initiative towards a national registry and DNA-bank of patients with congenital heart disease in the Netherlands: Rationale, design, and first results
abstract: Survival of patients with congenital heart disease has dramatically improved after surgical repair became available 40 years ago. Instead of a mortality of 85% during childhood following the natural course, over 85% of these infants are now expected to reach adulthood. However, data on long-term outcome is scarce due to the lack of large, national registries. Moreover, little is known about the genetic basis of congenital heart defects. In 2000, the Interuniversity Cardiology Institute of the Netherlands and the Netherlands Heart Foundation have taken the initiative to develop a national registry and DNA-bank of patients with congenital heart disease in the Netherlands named CONCOR.Objectives: The aims of the CONCOR-project are to facilitate investigation of the prevalence and long-term outcome of specific congenital heart defects and their treatment, to develop an efficient organisational structure for the improvement of healthcare for patients with congenital heart disease, and to allow investigation of the molecular basis of congenital heart defects.  After informed consent, research nurses enter data of participating patients into the CONCOR database using a web application. Data is transferred over the Internet via a secure connection. About 20 ml blood is withdrawn from the patient, and the DNA is isolated and stored. From each participating patient family history on congenital heart disease is obtained.  Within two and a half years more than 4200 patients have agreed to participate. More than 99% of the patients that were asked have given their consent to participate in CONCOR. From 60% of these patients DNA has already been obtained. Mean age of the patients included is 34 years; more than 85% of the patients are younger than 45 years. Late complications occur frequently and the incidence increases with advancing age. 18% of the patients are known with supraventricular or ventricular arrhythmias. 2% of the included patients suffered a cerebrovascular accident, 139 (3%) had endocarditis. 6% of the patients has pulmonary hypertension or Eisenmenger syndrome. More than 15% of the patients reported an affected family member with congenital heart disease in the first, second, or third degree. 6% has an affected first-degree relative, and 4% a second-degree relative. Already 10 research projects have started using the CONCOR data and DNA.  The population of patients with congenital heart disease is young and rapidly growing. Late complications occur frequently and the incidence increases with advances age. The CONCOR registry and DNA-bank facilitates research on prevalence and long-term outcome and allows investigation of the molecular basis of congenital heart disease.

title: Body mass index in adult congenital heart disease
abstract: Abnormal body mass index (BMI) is associated with higher mortality in various cardiovascular cohorts. The prognostic implications of BMI in adults with congenital heart disease (ACHD) are unknown. We aim to assess the distribution of BMI and its association with symptoms and survival in the ACHD population.  We included 3069 ACHD patients (median age 32.6 years) under follow-up at our institution between 2001 and 2015. Patients were classified based on BMI as underweight (<18.5), normal weight (18.5–25), overweight (25–30) or obese (>30), and symptoms, exercise capacity and mortality were assessed.  Overall, 6.2% of patients were underweight, 51.1% had normal weight, 28.2% were overweight and 14.6% were obese. Higher BMI values were associated with lower all-cause and cardiac mortality on univariable Cox analysis, and this effect persisted after adjustment for age, defect complexity, cyanosis and objective exercise capacity. Higher BMI was especially associated with better prognosis in symptomatic ACHD patients (HR 0.94 (95% CI 0.90 to 0.98), p=0.002) and those with complex underlying cardiac defects (HR 0.96 (95% CI 0.91 to 0.997), p=0.048) In patients with a complex cardiac defect who had repeated weight measurements, weight loss was also associated with a worse survival (HR 1.82 (95% CI 1.02 to 3.24), p=0.04).  ACHD patients with a higher BMI had a lower mortality. The association between BMI and mortality was especially pronounced in symptomatic patients with complex underlying cardiac defects, suggesting that cardiac cachexia may play a role. Indeed, weight loss in complex ACHD patients was linked to an even higher mortality.

title: Linking clinical registry data with administrative data using indirect identifiers: implementation and validation in the congenital heart surgery population.
abstract: The use of clinical registries and administrative data sets in pediatric cardiovascular research has become increasingly common. However, this approach is limited by relatively few existing datasets, each of which contain limited data, and do not communicate with one another. We describe the implementation and validation of methodology using indirect patient identifiers to link The Society of Thoracic Surgeons Congenital Heart Surgery (STS-CHS) Database to The Pediatric Health Information Systems (PHIS) Database (a pediatric administrative database).



Centers submitting data to STS-CHS and PHIS during 2004 to 2008 were included (n=30). Both data sets were limited to patients 0 to 18 years old undergoing cardiac surgery. An exact match was defined as an exact match on each of the following: date of birth, date of admission, date of discharge, sex, and center. Likely matches were defined as an exact match for all variables except ±1 day for one of the date variables.



Of 45,830 STS-CHS records, 87.4% matched to PHIS using the exact match criteria and 90.3% using the exact or likely match criteria. Validation in a subset of patients revealed that 100% of exact and likely matches were true matches.



This analysis demonstrates that indirect identifiers can be used to create high-quality link between a clinical registry and administrative data set in the congenital heart surgery population. This methodology, which can also be applied to other data sets, allows researchers to capitalize on the strengths of both types of data and expands the pool of data available to answer important clinical questions.

title: In‐Hospital Vital Status and Heart Transplants After Intervention for Congenital Heart Disease in the Pediatric Cardiac Care Consortium: Completeness of Ascertainment Using the National Death Index and United Network for Organ Sharing Datasets
abstract: The long‐term outcomes of patients undergoing interventions for congenital heart disease (CHD) remain largely unknown. We linked the Pediatric Cardiac Care Consortium (PCCC) with the National Death Index (NDI) and the United Network for Organ Sharing Dataset (UNOS) registries to study mortality and transplant occurring up to 32 years postintervention. The objective of the current analysis was to determine the sensitivity of this linkage in identifying patients who are known to have died or undergone heart transplant.  We used direct identifiers from 59 324 subjects registered in the PCCC between 1982 and 2003 to test for completeness of case ascertainment of subjects with known vital and heart transplant status by linkage with the NDI and UNOS registries. Of the 4612 in‐hospital deaths, 3873 were identified by the NDI as “true” matches for a sensitivity of 84.0% (95% CI, 82.9–85.0). There was no difference in sensitivity across 25 congenital cardiovascular conditions after adjustment for age, sex, race, presence of first name, death year, and residence at death. Of 455 known heart transplants in the PCCC, there were 408 matches in the UNOS registry, for a sensitivity of 89.7% (95% CI, 86.9–92.3). An additional 4851 deaths and 363 transplants that occurred outside the PCCC were identified through 2014.  The linkage of the PCCC with the NDI and UNOS national registries is feasible with a satisfactory sensitivity. This linkage provides a conservative estimate of the long‐term death and heart transplant events in this cohort.

title: From numbers to guidelines.
abstract: Advances in pediatric cardiology and cardiac surgery have resulted in a change in the mortality, prevalence, and age distribution of patients with congenital heart disease (CHD). In this chapter, we review the changing epidemiology of CHD and the impact of these trends on health services utilization and delivery in this population. We demonstrate not only that adult CHD (ACHD) patients have high utilization rates but also that care gaps exist where disease is expected to be lifelong. We outline the components of quality improvement for the care of ACHD patients. We review the newly published guidelines for the management of CHD conditions in adults, anchoring them to structure, process, and outcome indexes of quality of care. We highlight the manpower needs and the importance of an appropriately trained cardiology workforce to provide ACHD care. Finally, we review recommended health care systems ideally suited to deliver care to this population.

title: Planning the specialized care of adult congenital heart disease patients: from numbers to guidelines; an epidemiologic approach.
abstract: Guidelines published in 2001 recommended 1 regional adult congenital heart disease (ACHD) center per 3 to 10 million population. Our objective was to determine if published guidelines on the numbers of regional ACHD centers are sufficient to meet the needs of adults with congenital heart disease in the general population. Population data were examined to evaluate the recommendations for the number of regional centers. We extrapolated a population prevalence of 4.09 per 1,000 adults corresponding to 847,896 and 87,375 patients with ACHD in the United States and Canada, respectively. We reviewed the information currently available on the numbers of ACHD facilities of any kind indexed to continental populations. We examined the distribution of disease and health services in pediatric and adult populations and examined the evidence for pressure points during the transition process. Published data on 6 of the largest regional ACHD centers were used to model regional center care. We reviewed determinants and recommendations for follow-up in regional centers. We explore 3 scenarios of referral patterns to regional centers, examining their impact of the number of centers required per country population. In conclusion, we demonstrate that 1 regional ACHD center for a population of 2.0 million adults appears to be closer to what is required for improving access to specialized care for patients with ACHD in the United States and Canada.

title: Databases for Congenital Heart Defect Public Health Studies Across the Lifespan
abstract: In a 2012 meeting at the Centers for Disease Control and Prevention (CDC), key experts and stakeholders identified public health knowledge gaps about congenital heart defects (CHDs), namely prevalence of CHDs across the life span, long‐term outcomes of persons with CHDs, and health services

title: Impact of single-ventricle physiology on death after heart transplantation in adults with congenital heart disease.
abstract: Prevalence of univentricular (1V) anatomy over time and whether 1V anatomy is associated with early death after heart transplant (HTx) among recipients with adult congenital heart disease (ACHD) is unknown. We investigated changes in case-mix over time, 1V vs biventricular (2V) status, and the effect of 1V anatomy on death after HTx among ACHD recipients.



The Nationwide Inpatient Sample (NIS) was used to identify ACHD HTx recipients in the United States aged 14 years or older from 1993 to 2007, divided into era 1 (1993 to 2000) and era 2 (2001 to 2007). In-hospital death was compared among recipients with 1V and 2V anatomy. Multivariable determinants associated with an increased risk of in-hospital death were sought with logistic regression models.



From a national estimate of 509 ACHD recipients, 143 were 1V and 366 were 2V. Overall, 1V in-hospital mortality (23%) was higher than for 2V (8%; p<0.001) and remained associated with in-hospital death after adjustment for other factors (odds ratio, 3.9; 95% confidence interval, 1.29 to 11.74; p=0.02). All 1V diagnoses had higher mortality than all 2V diagnoses. Despite minor fluctuations, the proportion of 1V patients did not increase over time (era 1, 36%; era 2, 30%; p=0.46).



Overall case-mix of ACHD recipients (1V vs 2V) has not changed over time. Initial 1V anatomy increases post-HTx death among ACHD recipients, whereas 2V patients have mortality rates similar to non-CHD recipients. National and international transplant registries should include specific CHD diagnoses because this factor plays such a large role in determining early outcomes.

title: Impact of anatomical subtype and medical comorbidities on hospitalizations in adults with single ventricle congenital heart disease.
abstract: Most patients with single ventricle congenital heart disease (SV) are now expected to survive to adulthood. Medical comorbidities are common in SV.



We used data from 43 pediatric hospitals in the 2004 to 2011 Pediatric Health Information System database to identify patients ≥18 years of age admitted with International Classification of Diseases-9th Revision codes for a diagnosis of either hypoplastic left heart syndrome (HLHS), tricuspid atresia (TA) or common ventricle (CV). Primary (PD) and secondary diagnoses (SD), length of stay (LOS) and hospital charges were determined. Multilevel models were used to evaluate differences in demographics, diagnoses, and admission outcomes among the three subgroups (HLHS, TA, and CV). Interactions of charges with PD and admission year were examined using ANOVA.



There were 801 SV patients with 1330 admissions during the study period. Mean age was 24.8±6.2 years (55% male) and mean LOS was 6.8±11.3 days. Total hospital charges were $135 million with mean charge per admission of $101,131±205,808. The mean charge per day was $15,407±16,437. Hospital charges correlated with PD group (p<0.001). Admission rate remained stable (~180/year) from 2006 to 2011. LOS decreased (p=0.0308) and hospital charges per day increased across the study period (p<0.001). PD was non-cardiac in 28% of admissions. Liver-related conditions were more common in patients with HLHS (p<0.001).



Hospitalization costs in adults with SV are significant and are impacted by comorbid medical conditions. Hospitalization rates for adults with SV are not increasing. Gastroenterologic comorbidities including protein-losing enteropathy (PLE) are common in HLHS.

title: Hospital Utilization in Adults with Single Ventricle Congenital Heart Disease and Cardiac Arrhythmias
abstract: The study sought to identify the impact of cardiac arrhythmias on hospitalizations in adults with single ventricle (SV) congenital heart disease (CHD).

title: Hospital Resource Utilization for Common Noncardiac Diagnoses in Adult Survivors of Single Cardiac Ventricle.
abstract: Single ventricle congenital heart disease (SV CHD) has transformed from a nearly universally fatal condition to a chronic illness. As the number of adults living with SV CHD continues to increase, there needs to be an understanding of health care resource utilization (HCRU), particularly for noncardiac conditions, for this patient population. We performed a retrospective database review of the University HealthSystem Consortium Clinical Database/Resource Manager for adult patients with SV CHD hospitalized for noncardiac conditions from January 2011 to November 2014. Patients with SV CHD were identified using International Classification of Disease (ICD)-9 codes associated with SV CHD (hypoplastic left heart, tricuspid atresia, and SV) and stratified into 2 groups by age (18 to 29 years and 30 to 40 years). Direct cost, length of stay (LOS), intensive care unit (ICU) admission rate and mortality data were compared with age-matched patients without CHD. There were 2,083,651 non-CHD and 590 SV CHD admissions in Group 1 and 2,131,046 non-CHD and 297 SV CHD admissions in Group 2. There was no difference in LOS in Group 1, but there were higher costs for several diagnoses. LOS and costs were higher for several diagnoses in Group 2. ICU admission rate and in-hospital mortality were higher for several diagnoses for patients with SV CHD in both groups. In conclusion, adults with SV CHD admitted for noncardiac diagnoses have higher HCRU (longer LOS and higher ICU admission rates) compared with similarly aged patients without CHD. These findings stress the importance of good primary care in this population with complex, chronic cardiac disease to prevent hospitalizations and higher HCRU.

title: National Trends in Hospitalizations for Patients With Single-Ventricle Anatomy.
abstract: Patients with single-ventricle (SV) anatomy now live to adulthood. Little is known about the cost of care and outcomes for patients with SV anatomy, especially those who develop heart failure (HF) cared for in adult hospitals in the United States. We analyzed the Nationwide Inpatient Sample from 2000 to 2011 for patients >14 years admitted to adult hospitals with the International Classifications of Diseases, Ninth Revision, codes for SV anatomy. Demographics, outcomes, co-morbidities, and cost were assessed. From 2000 to 2011, the number of SV admissions was stable with a trend toward increased cost per admission over time. Coexistent hypertension, obesity, and liver, pulmonary, and renal diseases significantly increased over time. The most common reason for admission was atrial arrhythmia followed by HF. Patients with SV with HF had significantly higher inhospital mortality, length of stay, and more medical co-morbidities than those with SV and without HF. In conclusion, the cohort of patients with SV admitted to adult hospitals has changed in the modern era. Patients with SV have medical co-morbidities including renal and liver diseases, hypertension, and obesity at a surprisingly young age. Aggressive and proactive management of HF and arrhythmia may reduce cost of care for this challenging population. Patients with SV with HF have particularly high mortality, more medical co-morbidities, and increased cost of care and deserve more focused attention to improve outcomes.

title: Completeness of state administrative databases for surveillance of congenital heart disease.
abstract: Tracking birth prevalence of cardiac defects is essential to determining time and space clusters, and identifying potential associated factors. Resource limitations on state birth defects surveillance programs sometimes require that databases already available be used for ascertaining such defects. This study evaluated the data quality of state administrative databases for ascertaining congenital heart defects (CHD) and specific diagnoses of CHD.



Children's Hospital of Wisconsin (CHW) medical records for infants born 1997-1999 and treated for CHD (n = 373) were abstracted and each case assigned CHD diagnoses based on definitive diagnostic reports (echocardiograms, catheterizations, surgical or autopsy reports). These data were linked to state birth and death records, and birth and postnatal (< 1 year of age) hospital discharge summaries at the Wisconsin Bureau of Health Information (WBHI). Presence of any code/checkbox indicating CHD (generic CHD) and exact matches to abstracted diagnoses were evaluated.



Fifty-eight percent of cases with generic CHD were identified by state databases. Postnatal hospital discharge summaries identified 48%, birth hospital discharge summaries 27%, birth certificates 9% and death records 4% of these cases. Exact matches were found for 52% of 633 specific diagnoses. Postnatal hospital discharge summaries provided most matches.



State databases identified 60% of generic CHD and exactly matched about half of specific CHD diagnoses. The postnatal hospital discharge summaries performed best in both in identifying generic CHD and matching specific CHD diagnoses. Vital records had limited value in ascertaining CHD.

title: Validity of Hospital Discharge Data for Identifying Infants with Cardiac Defects
abstract: To examine validity of the International Classification of Diseases, 9th Edition, Clinical Modification (ICD-9-CM) codes in discharge data for identifying infants with cardiac defects according to surveillance guidelines.Retrospective medical record review of infants born in 2001 at one hospital in Minneapolis, Minnesota. Infants were identified using ICD-9-CM codes from hospital discharge data, and keywords in medical records.Of 2697 children, ICD-9-CM codes identified 66 infants coded with cardiac defects; physician review confirmed 24 had cardiac defects. Only 35 of 85 (41.2%) ICD-9-CM codes accurately reflected the cardiac defect diagnoses. Additional case finding located four infants with five cardiac defects. Sensitivity of ICD-9-CM codes for identifying these infants was 0.857, predictive value positive was 0.364.CONCLUSIONS:ICD-9-CM codes from hospital discharge data identified most infants with cardiac defects, but many were false positives. ICD-9-CM codes were inaccurate for specific cardiac defects.

title: Exposure to Low-Dose Ionizing Radiation From Cardiac Procedures in Patients With Congenital Heart Disease: 15-Year Data From a Population-Based Longitudinal Cohort.
abstract: The burden of low-dose ionizing radiation (LDIR) exposure from medical procedures among individuals with congenital heart disease (CHD) is unknown. In this longitudinal population-based study, we sought to determine exposure to LDIR-related cardiac imaging and therapeutic procedures in children and adults with CHD.



In an analysis of the Quebec CHD database, exposure to the following LDIR-related cardiac procedures was recorded: catheter-based diagnostic procedures, structural heart interventions, coronary interventions, computed tomography scans of the chest, nuclear procedures, and pacemaker/implantable cardioverter-defibrillator insertion and repair. From 1990 to 2005, there were 16 253 LDIR-exposed patients with CHD with 317 988 patient-years of available follow-up. The total number of LDIR-related procedures increased from 18.5 to 51.9 per 1000 CHD patients per year (P<0.0001). This increase was attributable to increases in rates per 1000 CHD patients in diagnostic cardiac catheterizations (11.7 to 13.7 per 1000), structural heart interventions (1.0 to 5.2 per 1000), coronary interventions (1.0 to 2.4 per 1000), pacemaker/implantable cardioverter-defibrillator insertions (1.6 to 4.4 per 1000), nuclear procedures (4.2 to 13.8 per 1000), and computed tomography scans of the chest (2.5 to 12.3 per 1000). Over time, among children with CHD, the median age at first LDIR procedure decreased from 5.0 years to 9.6 months. Severity of CHD significantly predicted extent of exposure.



From 1990 to 2005, patients with CHD were exposed to increasing numbers of LDIR-emitting cardiac procedures. This exposure occurred at progressively younger ages. These findings provide an important perspective on longitudinal LDIR exposure in this at-risk population.

title: Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010.
abstract: Because of advancements in care, there has been a decline in mortality from congenital heart defects (CHDs) over the past several decades. However, there are no current empirical data documenting the number of people living with CHDs in the United States. Our aim was to estimate the CHD prevalence across all age groups in the United States in the year 2010.



The age-, sex-, and severity-specific observed prevalence of CHDs in Québec, Canada, in the year 2010 was assumed to equal the CHD prevalence in the non-Hispanic white population in the United States in 2010. A race-ethnicity adjustment factor, reflecting differential survival between racial-ethnic groups through 5 years of age for individuals with a CHD and that in the general US population, was applied to the estimated non-Hispanic white rates to derive CHD prevalence estimates among US non-Hispanic blacks and Hispanics. Confidence intervals for the estimated CHD prevalence rates and case counts were derived from a combination of Taylor series approximations and Monte Carlo simulation.



We estimated that ≈2.4 million people (1.4 million adults, 1 million children) were living with CHDs in the United States in 2010. Nearly 300 000 of these individuals had severe CHDs.



Our estimates highlight the need for 2 important efforts: planning for health services delivery to meet the needs of the growing population of adults with CHD and the development of surveillance data across the life span to provide empirical estimates of the prevalence of CHD across all age groups in the United States.

title: The care of adults with congenital heart disease across the globe: Current assessment and future perspective: A position statement from the International Society for Adult Congenital Heart Disease (ISACHD).
abstract: The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD.