Andrew S. Mackie

Congenital Heart Disease in the General Population Changing Prevalence and Age Distribution

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.

Changing Mortality in Congenital Heart Disease

OBJECTIVESnThis study sought to characterize temporal trends in all-cause mortality in patients with congenital heart disease (CHD).nnnBACKGROUNDnHistorically, most deaths in patients with CHD occurred in early childhood. Notable advances have since been achieved that may impact on mortality trends.nnnMETHODSnWe 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.nnnRESULTSnThe 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.nnnCONCLUSIONSnDeaths in CHD have shifted away from infants and towards adults, with a steady increase in age at death and decreasing mortality.

Planning the specialized care of adult congenital heart disease patients: from numbers to guidelines; an epidemiologic approach

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.

Hospital readmissions in children with congenital heart disease: a population-based study.

BACKGROUNDnLittle is known about the factors that predispose children with congenital heart disease (CHD) to readmission soon after hospital discharge. We sought to determine rates and risk factors for hospital readmission within 1 month among children with CHD.nnnMETHODSnData were obtained from administrative databases that record all hospitalizations within the province of Quebec. We included children 0 to 17 years with CHD who were hospitalized with a cardiac diagnosis (an index hospitalization) between 1990 and 2005. Cox proportional hazards analysis was used to identify predictors of readmission.nnnRESULTSnAmong 3675 hospitalizations, there were 518 readmissions (15%) within 31 days. Median time to readmission was 12 days (interquartile range 5-19 days). Readmissions occurred in the same hospital as the index hospitalization 86% of the time. The most common diagnoses at readmission were cardiac (59%) and respiratory illnesses (12%). Independent predictors of readmission were severe CHD lesion, younger age, Friday or Saturday discharge, having an emergency department visit within the preceding 6 months, length of index hospitalization > 14 days, and multiple (> or = 4) diagnoses (either cardiac diagnoses or comorbid conditions). Having an invasive procedure during the index hospitalization reduced the likelihood of readmission.nnnCONCLUSIONSnReadmission occurred after 1 in 7 hospitalizations. Infants and patients with severe CHD lesions were among those more likely to be readmitted. The risk of readmission may be reduced by avoiding hospital discharge at the beginning of a weekend. Further studies are needed to determine if targeted interventions will reduce readmission rates in high-risk patients.

Prevalence and Severity of Hypertensive Retinopathy in Children

The National High Blood Pressure Education Program (NHBPEP) report recommends a retinal exam seeking evidence of target organ damage in hypertensive children. This study aimed to determine the prevalence and severity of hypertensive retinopathy among hypertensive children, evaluated by pediatric ophthalmologists in the “real world” clinical setting using direct ophthalmoscopy. The authors retrospectively reviewed the medical records of the 83 children diagnosed with hypertension by a pediatric nephrologist between 1999 and 2006. Of the 35 children examined by an ophthalmologist within 12 months of the diagnosis of hypertension, only 3 (8.6%; 95% CI, 1.8%-23.1%) were diagnosed with hypertensive retinopathy. Despite the fact that those selected for retinal examination were likely at higher risk for retinopathy, the prevalence of retinopathy was low, and only mild abnormalities were detected. Given the lack of evidence linking mild retinal abnormalities with adverse outcomes, the NHBPEP recommendation for retinal examinations in hypertensive children should be reconsidered.

LIFETIME PREVALENCE OF CONGENITAL HEART DISEASE IN THE GENERAL POPULATION IN 2010

There is a paucity of data to estimate the number of people living with congenital heart disease (CHD) in the general population. The most recent estimates were for the years 2000-2005 when increasing CHD prevalence was documented in adults and children. Our goal was to provide current estimates of