Background— 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. Methods and Results— 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. Conclusions— 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.

Background-Our objective was to obtain contemporary lifetime estimates of congenital heart disease (CHD) prevalence using population-based data sources up to year 2010. Methods and Results-The Quebec CHD database contains 28 years of longitudinal data on all individuals with CHD from 1983 to 2010. Severe CHD was defined as tetralogy of Fallot, truncus arteriosus, transposition complexes, endocardial cushion defects, and univentricular hearts. We used latent class bayesian models combining case definitions from physician claims, hospitalization, and surgical data to obtain point and interval prevalence estimates of CHD in the first year of life, in children (<18 years of age) and in adults. We identified 107 559 CHD patients from 1983 to 2010. Prevalence of CHD in the first year of life was 8.21 per 1000 live births (95% confidence interval, 7.47-9.02) from 1998 to 2005. In 2010, overall prevalence of CHD was 13.11 per 1000 (95% confidence interval, 12.43-13.81) in children and 6.12 per 1000 (95% confidence interval, 5.69-6.57) in adults. CHD prevalence increased by 11% in children and 57% in adults from 2000 to 2010. Prevalence in the severe CHD subgroup increased by 19% (95% confidence interval, 17%-21%) in children and 55% (51%-62%) in adults. By 2010, adults accounted for 66% of the entire CHD population. Conclusions-With

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

Background-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. Methods and Results-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 (nϭ643). 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 (nϭ84; 13%), simple shunts (nϭ390; 61%), and "other" lesions (nϭ169; 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. Conclusions-Lack

Background-Left ventricular (LV) hypertrophy (LVH) in children is widely defined as a left ventricular mass index (LVMI, g/m 2.7 ) Ͼ95th percentile. However, LVMI increases with decreasing height in young children; thus, the 95th percentile LVMI will depend on the height distribution of the reference population. The objective of this study was to compare the performance of a novel method of expressing LV mass relative to body size (centile curves) with the LVMI method. Methods and Results-LV mass was estimated by M-mode echocardiography in 440 healthy nonobese reference children (birth to 21 years) and 239 children at risk for LVH; the LVMI was calculated for all children. Three samples of 270 children, each with different height distributions, were drawn from the reference population. A sample-specific 95th percentile LVMI was determined for each reference sample. At-risk children were classified as having LVH or not based on each sample-specific 95th percentile. Four LV mass-for-height centile curves were constructed with the Cole lambda-mu-sigma method and data from each reference sample. At-risk children were each assigned an LV mass-for-height percentile with these curves and were reclassified as having LVH if LV mass-for-height was Ͼ95th percentile. The centile method provided a stable estimate of the proportion of at-risk children with LVH regardless of reference group, whereas proportion estimates varied significantly depending on the reference population when the LVMI method was used.

This large-scale, international study found that overall QOL in adults with CHD was generally good. Variation in QOL was related to patient characteristics but not country-specific characteristics. Hence, patients at risk for poorer QOL can be identified using uniform criteria. General principles for designing interventions to improve QOL can be developed.

Background-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. Methods and Results-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). Conclusions-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. (Circulation. 2014;129:1804-1812.)

BackgroundThe late cardiotoxic effects of anthracycline chemotherapy influence morbidity and mortality in the growing population of childhood cancer survivors. Even with lower anthracycline doses, evidence of adverse cardiac remodeling and reduced exercise capacity exist. We aim to examine the relationship between cardiac structure, function and cardiovascular magnetic resonance (CMR) tissue characteristics with chemotherapy dose and exercise capacity in childhood cancer survivors.MethodsThirty patients (15 ± 3 years), at least 2 years following anthracycline treatment, underwent CMR, echocardiography, and cardiopulmonary exercise testing (peak VO2). CMR measured ventricular function, mass, T1 and T2 values, and myocardial extracellular volume fraction, ECV, a measure of diffuse fibrosis based on changes in myocardial T1 values pre- and post-gadolinium. Cardiac function was also assessed with conventional and speckle tracking echocardiography.ResultsPatients had normal LVEF (59 ± 7%) but peak VO2 was 17% lower than age-predicted normal values and were correlated with anthracycline dose (r = −0.49). Increased ECV correlated with decreased mass/volume ratio (r = −0.64), decreased LV wall thickness/height ratio (r = −0.72), lower peak VO2(r = −0.52), and higher cumulative dose (r = 0.40). Echocardiographic measures of systolic and diastolic function were reduced compared to normal values (p < 0.01), but had no relation to ECV, peak VO2 or cumulative dose.ConclusionsMyocardial T1 and ECV were found to be early tissue markers of ventricular remodeling that may represent diffuse fibrosis in children with normal ejection fraction post anthracycline therapy, and are related to cumulative dose, exercise capacity and myocardial wall thinning.