Liming Guo

Lifetime Prevalence of Congenital Heart Disease in the General Population from 2000 to 2010

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 107u2009559 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 an increase of >50% in CHD prevalence since 2000, by 2010 adults accounted for two thirds of patients with severe and other forms of CHD in the general population. Our findings should inform allocation of resources and the planning of workforce needs for the predominantly adult CHD population.

Coarctation of the Aorta and Coronary Artery Disease: Fact or Fiction?

Background— Aortic coarctation (CoA) is reported to predispose to coronary artery disease (CAD). However, our clinical observations do not support this premise. Our objectives were to describe the prevalence of CAD among adults with CoA and to determine whether CoA is an independent predictor of CAD or premature CAD. Methods and Results— The study population was derived from the Quebec Congenital Heart Disease Database. We compared patients with CoA and those with a ventricular septal defect, who are not known to be at increased risk of CAD. The prevalence of CAD in patients with CoA compared with those with ventricular septal defect was determined. We then used a nested case-control design to determine whether CoA independently predicted for the development of CAD. Of 756 patients with CoA who were alive in 2005, 37 had a history of CAD compared with 224 of 6481 patients with ventricular septal defect (4.9% versus 3.5%; P=0.04). Male sex (odds ratio [OR], 2.13; 95% confidence interval [CI], 1.62–2.80), hypertension (OR, 1.95; 95% CI, 1.44–2.64), diabetes mellitus (OR, 1.68; 95% CI, 1.09–2.58), age (OR per 10-year increase, 2.28; 95% CI, 2.09–2.48), and hyperlipidemia (OR, 11.58; 95% CI, 5.75–23.3) all independently predicted for the development of CAD. CoA did not independently predict for the development of CAD (OR, 1.04; 95% CI, 0.68–1.57) or premature CAD (OR for CoA versus ventricular septal defect, 1.44; 95% CI, 0.79–2.64) after adjustment for other factors. Conclusions— Although traditional cardiovascular risk factors independently predicted for the development of CAD, the diagnosis of CoA alone did not. Our findings suggest that cardiovascular outcomes of these patients may be improved with tight risk factor control.

Incidence, Predictors, and Mortality of Infective Endocarditis in Adults With Congenital Heart Disease Without Prosthetic Valves

Congenital heart disease (CHD) increases the risk of infective endocarditis (IE), though the lesion-specific risk and mortality are poorly defined. Using the population-based Quebec CHD database, we sought to describe the predictors of IE and to evaluate if IE was associated with mortality among adult CHD (ACHD) patients without prior valve replacement surgery. We extracted data on ACHD patients with IE and assessed the lesion-specific incidence of IE, risk factors for IE acquisition, and all-cause 1-year mortality. Among 29,866 ACHD patients, 285 (0.95%) developed IE during follow-up period of 378,901 patient-years, from 1988-2010. The highest and lowest lesion-specific incidences of IE were observed with left-sided lesions (1.61/1000 patient-years) and patent ductus arteriosus (0.24/1000 patient-years), respectively. The following predicted the risk of IE acquisition (odds ratio (OR), 95% confidence interval [CI]): cardiac surgery in the previous 6 months (9.07, 3.98-20.67), endocardial cushion defects (6.65, 3.84-11.53), left-sided lesions (5.11, 3.60-7.25), cyanosis at birth (4.82, 3.12-7.46), ventricular septal defect (2.81, 1.87-4.21), diabetes mellitus (1.65, 1.10-2.48), and recent medical interventions (12.52, 5.23-29.97). Twenty-five (8.77%) patients died within 1-year of IE diagnosis, a substantially elevated rate compared to patients without IE (OR 31.07, 95%CI 16.23-59.49). The risk of death following IE diagnosis was similarly elevated among patients with left-sided, cyanotic and other CHD lesions. In conclusion, the risk of IE in ACHD patients is lesion-specific and is greatest in the context of recent medical interventions. IE is associated with increased 1-year mortality, irrespective of broad CHD lesion grouping.

Exposure to Low-Dose Ionizing Radiation From Cardiac Procedures in Patients With Congenital Heart DiseaseCLINICAL PERSPECTIVE

Background— 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.nnMethods and Results— 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.nnConclusions— 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.nn# CLINICAL PERSPECTIVE {#article-title-22}Background— 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. Methods and Results— 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. Conclusions— 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.

Stroke in Adults With Congenital Heart DiseaseCLINICAL PERSPECTIVE: Incidence, Cumulative Risk, and Predictors

Background— 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. Methods and Results— 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]). Conclusions— 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.Background— 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.nnMethods and Results— 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]).nnConclusions— 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.nn# CLINICAL PERSPECTIVE {#article-title-46}

Stroke in Adults With Congenital Heart DiseaseCLINICAL PERSPECTIVE

Background— 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. Methods and Results— 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]). Conclusions— 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.Background— 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.nnMethods and Results— 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]).nnConclusions— 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.nn# CLINICAL PERSPECTIVE {#article-title-46}

Coarctation of the Aorta and Coronary Artery DiseaseClinical Perspective: Fact or Fiction?

Background— Aortic coarctation (CoA) is reported to predispose to coronary artery disease (CAD). However, our clinical observations do not support this premise. Our objectives were to describe the prevalence of CAD among adults with CoA and to determine whether CoA is an independent predictor of CAD or premature CAD. Methods and Results— The study population was derived from the Quebec Congenital Heart Disease Database. We compared patients with CoA and those with a ventricular septal defect, who are not known to be at increased risk of CAD. The prevalence of CAD in patients with CoA compared with those with ventricular septal defect was determined. We then used a nested case-control design to determine whether CoA independently predicted for the development of CAD. Of 756 patients with CoA who were alive in 2005, 37 had a history of CAD compared with 224 of 6481 patients with ventricular septal defect (4.9% versus 3.5%; P=0.04). Male sex (odds ratio [OR], 2.13; 95% confidence interval [CI], 1.62–2.80), hypertension (OR, 1.95; 95% CI, 1.44–2.64), diabetes mellitus (OR, 1.68; 95% CI, 1.09–2.58), age (OR per 10-year increase, 2.28; 95% CI, 2.09–2.48), and hyperlipidemia (OR, 11.58; 95% CI, 5.75–23.3) all independently predicted for the development of CAD. CoA did not independently predict for the development of CAD (OR, 1.04; 95% CI, 0.68–1.57) or premature CAD (OR for CoA versus ventricular septal defect, 1.44; 95% CI, 0.79–2.64) after adjustment for other factors. Conclusions— Although traditional cardiovascular risk factors independently predicted for the development of CAD, the diagnosis of CoA alone did not. Our findings suggest that cardiovascular outcomes of these patients may be improved with tight risk factor control.

Coarctation of the Aorta and Coronary Artery DiseaseClinical Perspective

Background— Aortic coarctation (CoA) is reported to predispose to coronary artery disease (CAD). However, our clinical observations do not support this premise. Our objectives were to describe the prevalence of CAD among adults with CoA and to determine whether CoA is an independent predictor of CAD or premature CAD. Methods and Results— The study population was derived from the Quebec Congenital Heart Disease Database. We compared patients with CoA and those with a ventricular septal defect, who are not known to be at increased risk of CAD. The prevalence of CAD in patients with CoA compared with those with ventricular septal defect was determined. We then used a nested case-control design to determine whether CoA independently predicted for the development of CAD. Of 756 patients with CoA who were alive in 2005, 37 had a history of CAD compared with 224 of 6481 patients with ventricular septal defect (4.9% versus 3.5%; P=0.04). Male sex (odds ratio [OR], 2.13; 95% confidence interval [CI], 1.62–2.80), hypertension (OR, 1.95; 95% CI, 1.44–2.64), diabetes mellitus (OR, 1.68; 95% CI, 1.09–2.58), age (OR per 10-year increase, 2.28; 95% CI, 2.09–2.48), and hyperlipidemia (OR, 11.58; 95% CI, 5.75–23.3) all independently predicted for the development of CAD. CoA did not independently predict for the development of CAD (OR, 1.04; 95% CI, 0.68–1.57) or premature CAD (OR for CoA versus ventricular septal defect, 1.44; 95% CI, 0.79–2.64) after adjustment for other factors. Conclusions— Although traditional cardiovascular risk factors independently predicted for the development of CAD, the diagnosis of CoA alone did not. Our findings suggest that cardiovascular outcomes of these patients may be improved with tight risk factor control.