Adults With Congenital Heart Disease: Trends in Event-Free Survival Past Middle Age.

BACKGROUND: The survival of children with congenital heart disease has increased substantially over the past decades, with 97% currently reaching adulthood. The total effect of advanced treatment on future mortality and morbidity in adult survivors with congenital heart disease (CHD) is less well described. METHODS: We used data from the Swedish National Inpatient, Outpatient, and Cause of Death Register to identify patients with CHD who were born between 1950 and 1999 and were alive at 18 years of age. Ten controls identified from the Total Population Register were matched for year of birth and sex and with each patient with CHD. Follow-up was from 1968 and 18 years of age until death or at the end of the study (2017). Survival percentage with 95% CI for all-cause mortality were performed with Kaplan-Meier survival function. Cox proportional hazard regression models with hazard ratios (HRs) and 95% CI were used to estimate the risk of all-cause mortality. RESULTS: We included 37 278 patients with adult CHD (ACHD) and 412 799 controls. Mean follow-up was 19.2 years (±13.6). Altogether, 1937 patients with ACHD (5.2%) and 6690 controls (1.6%) died, a death rate of 2.73 per 1000 person-years and 0.84 per 1000 person years, respectively. Mortality was 3.2 times higher (95% CI, 3.0-3.4; P<0.001) among patients with ACHD compared with matched controls. Up to the maximum of 50 years of follow-up, >75% of patients with ACHD were still alive. Mortality was highest among patients with conotruncal defects (HR, 10.13 [95% CI, 8.78-11.69]), but also significantly higher for the more benign lesions, with the lowest risk in patients with atrial septal defects (HR, 1.36 [95% CI, 1.19-1.55]). At least 75% of patients with ACHD alive at 18 years of age lived past middle age and became sexagenerians. CONCLUSIONS: In this large, nationwide, register-based cohort study of patients with ACHD surviving to 18 years of age, the risk of mortality up to 68 years of age was >3 times higher compared with matched controls without ACHD. Despite this, at least 75% of patients with CHD alive at 18 years of age lived past middle age and became sexagenerians. A notable risk decline in the mortality for patients with ACHD was seen for those born after 1975.

Long-term outcomes after myocardial infarction in middle-aged and older patients with congenital heart disease-a nationwide study.

AIMS: We aimed to describe the risk of myocardial infarction (MI) in middle-aged and older patients with congenital heart disease (ACHD) and to evaluate the long-term outcomes after index MI in patients with ACHD compared with controls. METHODS AND RESULTS: A search of the Swedish National Patient Register identified 17 189 patients with ACHD (52.2% male) and 180 131 age- and sex-matched controls randomly selected from the general population who were born from 1930 to 1970 and were alive at 40 years of age; all followed up until December 2017 (mean follow-up 23.2 ± 11.0 years). Patients with ACHD had a 1.6-fold higher risk of MI compared with controls [hazard ratio (HR) 1.6, 95% confidence interval (CI) 1.5-1.7, P < 0.001] and the cumulative incidence of MI by 65 years of age was 7.4% in patients with ACHD vs. 4.4% in controls. Patients with ACHD had a 1.4-fold increased risk of experiencing a composite event after the index MI compared with controls (HR 1.4, 95% CI 1.3-1.6, P < 0.001), driven largely by the occurrence of new-onset heart failure in 42.2% (n = 537) of patients with ACHD vs. 29.5% (n = 2526) of controls. CONCLUSION: Patients with ACHD had an increased risk of developing MI and of recurrent MI, new-onset heart failure, or death after the index MI, compared with controls, mainly because of a higher incidence of newly diagnosed heart failure in patients with ACHD. Recognizing and managing the modifiable cardiovascular risk factors should be of importance to reduce morbidity and mortality in patients with ACHD.

Cumulative Incidence of Infective Endocarditis in Patients with Congenital Heart Disease: A Nationwide, Case-Control Study Over Nine Decades.

BACKGROUND: Congenital heart disease (CHD) is a lifelong predisposing condition for infective endocarditis (IE). As a consequence of advances in pediatric care, the number of adults with CHD is now exceeding the number of children. The goal of the present study was to determine the cumulative incidence of IE in patients with CHD and detect temporal changes compared with controls. METHODS: Nationwide registry-based case-control study of patients with CHD born 1930-2017 matched with 10 random controls. Infective endocarditis episodes were linked using the Swedish 10-digit personal identification number. RESULTS: In total, 89 541 patients with CHD and 890 470 matched controls were included. In patients with CHD, 1477 IE episodes were registered and 447 episodes in controls. Patients with CHD had 8.5% cumulative incidence of IE at age 87 years, compared with 0.7% in matched controls. Incidence rate of IE per 100 000 person-years was 65.5 (95% confidence interval [CI] 62.2-68.9) and 1.8 (95% CI: 1.7-2.0) in CHD patients and controls, respectively. By age 18 years, patients with CHD had an IE incidence similar to that of 81-year-old controls. Incidence of IE differed by age but not by birth year. Bacterial etiology was registered from 1997 in half of the IE episodes; among CHD IE cases, 43.3% were caused by streptococci and 29.8% by Staphylococcus aureus. CONCLUSIONS: Infective endocarditis remains an important complication in patients with CHD. Incidence correlate with age and the number of IE episodes are expected to increase as the CHD population grow older.

The risk of dementia after coronary artery bypass grafting in relation to age and sex.

INTRODUCTION: We examined the long-term risk of dementia after coronary artery bypass grafting (CABG) in relation to age and sex. METHODS: All CABG patients in Sweden 1992-2015 (n = 111,335), and matched controls (n = 222,396) were included in a population-based study. Adjusted hazard ratios (aHR) for all-cause dementia, vascular dementia, and Alzheimer's disease were calculated. RESULTS: There was no difference in the risk for all-cause dementia between CABG patients and control subjects (aHR 0.98 [95% confidence interval 0.95 to 1.02]). CABG patients <65 years and 65 to 74 years had higher risk (aHR 1.29 [1.17-1.42] and 1.08 [1.02-1.13], respectively), and patients ≥75 years had lower risk (aHR 0.76 [0.71-0.81]). The highest risk was observed in women <65 years (aHR 1.64 [1.31-2.05]). DISCUSSION: Overall, the long-term risk for all-cause dementia does not differ between CABG patients and the general population. Younger patients have a higher risk, while older patients have a lower risk, compared to controls.

Long-Term Risk of Hemorrhagic Stroke in Young Patients With Congenital Heart Disease.

BACKGROUND AND PURPOSE: The risk of ischemic stroke is increased in patients with congenital heart disease (CHD); however, data on the risk of hemorrhagic stroke, including intracerebral hemorrhage (ICH) and subarachnoid hemorrhage (SAH), are lacking. METHODS: The Swedish Patient Register was used to identify all patients who were born with a diagnosis of CHD between 1970 and 1993. Each patient was compared with 10 randomly selected controls from the general population, matched for age, sex, and county. Follow-up data were collected until December 2011 for both cases and controls. RESULTS: Of 21 982 patients with CHD, 70 developed ICH and 57 developed SAH up to the age of 42 years. CHD patients had more than an 8× higher risk (incidence rate ratio, 8.23; 95% confidence interval, 6-11.2) of developing ICH and almost an 8× higher risk of developing SAH (incidence rate ratio, 7.64; 95% confidence interval, 5.41-10.7) compared with controls. The absolute risk of ICH and SAH was low, with incidence rates of 1.18 and 0.96 cases per 10 000 person-years, respectively. Patients with severe nonconotruncal defects (incidence rate ratio, 16.5; 95% confidence interval, 5.63-51.2) or coarctation of the aorta (incidence rate ratio, 17.3; 95% confidence interval, 6.63-51.8) had the highest relative risk of developing hemorrhagic stroke, with incidence rates of 3.22 and 2.79 cases per 10 000 person-years, respectively. CONCLUSIONS: The relative risk of hemorrhagic stroke among children and young adults with CHD was almost 8× higher than that of matched controls from the general population, although the absolute risk was low. The highest risk of ICH and SAH occurred in patients with severe nonconotruncal defects and coarctation of the aorta.

Risk of Heart Failure in Obese Patients With and Without Bariatric Surgery in Sweden-A Registry-Based Study.

BACKGROUND: Obesity is a known risk factor for heart failure. The prevalence of both conditions has increased in Sweden during the past several decades. Obesity surgery has been shown to improve cardiac function. We therefore investigated whether the risk of heart failure was lower in obese patients after bariatric surgery compared with obese patients without surgical intervention. METHODS AND RESULTS: From the Swedish National Patient Registry. we created a cohort including 47,859 patients aged 18-74 years with a primary diagnosis of obesity from 2000 to 2011. Of these, 22,295 (46.6%) underwent bariatric surgery (mean age 40.7 (standard deviation [SD] 10.7) years, 75.9% female). There were 25,564 (53.4%) nonsurgical obese patients (mean age 44.3 (SD 13.2) years, 66.8% female). Patients who underwent bariatric surgery had a markedly reduced risk of heart failure compared with nonsurgical obese patients (age- and sex-adjusted hazard ratio [HR] 0.37, 95% confidence interval [CI] 0.29-0.46). The lower risk persisted after further adjustment for baseline differences in known risk factors for heart failure (HR 0.37, 95% CI 0.30-0.46). CONCLUSION: Patients who underwent bariatric surgery had a reduced risk of heart failure after surgery compared with nonsurgical obese patients.

Body weight in midlife and long-term risk of developing heart failure-a 35-year follow-up of the primary prevention study in Gothenburg, Sweden.

BACKGROUND: This study aimed to determine whether midlife obesity predicts heart failure (HF) over an extended follow-up into old age. METHODS: We studied 7495 men (from a population sample of 9,998 men) without HF, who were 47-55 years old when investigated in 1970 to 1973. All participants were followed up for 35 years, or until death, using the Swedish National Inpatient Register (IPR) and the Cause of Death Register. Over follow-up, 1855 men (24.7%) were discharged from hospital or died with a diagnosis of HF. RESULTS: There was a strong relation between obesity and future risk of HF, which was accentuated over the last years of the long follow-up. After adjusting for age, the risk of HF increased stepwise with increasing body mass index (BMI), even in those with a normal BMI (22.5-24.9) The subdistribution hazard ratio (SHR) was 1.20 (95% CI: 1.02-1.39) in men with a normal BMI, 1.29 (95% CI: 1.11-1.50) for a BMI of 25-27.49, 1.50 (95% CI: 1.27-1.77) for a BMI of 27.5-29.99, and 1.62 (95% CI: 1.33-1.97) for a BMI >30. After adjusting for, age, smoking, occupational class, and physical activity, the results were unchanged. CONCLUSION: Obesity in midlife is strongly related to the long-term risk of developing HF extending into old age where the risk is highest. Even normal body weight (BMI <25) was related to an increased risk of developing HF during life. Because overweight and obesity are largely preventable, our findings further emphasize the importance of public health interventions against the development of obesity.

The association between job strain and atrial fibrillation in Swedish men.

OBJECTIVES: The purpose of this study was to investigate whether psychosocial stress defined as high strain based on the job demand-control model increases risk for atrial fibrillation. METHODS: The present study comprised 6035 men born between 1915 and 1925 and free from previous coronary heart disease, atrial fibrillation and stroke at baseline (1974-1977). Work-related psychosocial stress was measured using a job-exposure matrix for the job demand-control model based on occupation at baseline. The participants were followed from baseline examination until death, hospital discharge or 75 years of age, using the Swedish national register on cause of death and the Swedish hospital discharge register for any registration for atrial fibrillation, resulting in the identification of 436 cases. Data were analysed with Cox regression models with atrial fibrillation as the outcome using high strain as the explanatory variable adjusted for age, smoking, body mass index, hypertension, diabetes and socioeconomic status. RESULTS: There was an increased risk for atrial fibrillation in relation to high strain (HR 1.32, 95% CI 1.003 to 1.75). When the four categories of the job-strain model were included and low strain was used as reference, the risk for high strain decreased (HR 1.23, 95% CI 0.84 to 1.82). CONCLUSIONS: Exposure to occupational psychosocial stress defined as high strain may be associated with increased risk for atrial fibrillation. The observed increase in risk is small and residual confounding may also be present.

Stroke and coronary heart disease: predictive power of standard risk factors into old age--long-term cumulative risk study among men in Gothenburg, Sweden.

AIMS: The aim of this study was to examine the short-term and long-term cumulative risk of coronary heart disease (CHD) and stroke separately based on age, sex, smoking status, systolic blood pressure, and total serum cholesterol. METHODS AND RESULTS: The Primary Prevention Study comprising 7174 men aged between 47 and 55 free from a previous history of CHD, stroke, and diabetes at baseline examination (1970-73) was followed up for 35 years. To estimate the cumulative effect of CHD and stroke, all participants were stratified into one of five risk groups, defined by their number of risk factors. The estimated 10-year risk for high-risk individuals when adjusted for age and competing risk was 18.1% for CHD and 3.2% for stroke which increased to 47.8 and 19.6%, respectively, after 35 years. The estimates based on risk factors performed well throughout the period for CHD but less well for stroke. CONCLUSION: The prediction of traditional risk factors (systolic blood pressure, total serum cholesterol, and smoking status) on short-term risk (0-10 years) and long-term risk (0-35 years) of CHD of stroke differs substantially. This indicates that the cumulative risk in middle-aged men based on these traditional risk factors can effectively be used to predict CHD but not stroke to the same extent.

Outcomes after cancer diagnosis in children and adult patients with congenital heart disease in Sweden: a registry-based cohort study.

OBJECTIVE: Patients with congenital heart disease (CHD) have an increased cancer risk. The aim of this study was to determine cancer-related mortality in CHD patients compared with non-CHD controls, compare ages at cancer diagnosis and death, and explore the most fatal cancer diagnoses. DESIGN: Registry-based cohort study. SETTING AND PARTICIPANTS: CHD patients born between 1970 and 2017 were identified using Swedish Health Registers. Each was matched by birth year and sex with 10 non-CHD controls. Included were those born in Sweden with a cancer diagnosis. RESULTS: Cancer developed in 758 out of 67814 CHD patients (1.1%), with 139 deaths (18.3%)-of which 41 deaths occurred in patients with genetic syndromes. Cancer was the cause of death in 71.9% of cases. Across all CHD patients, cancer accounted for 1.8% of deaths. Excluding patients with genetic syndromes and transplant recipients, mortality risk between CHD patients with cancer and controls showed no significant difference (adjusted HR 1.17; 95% CI 0.93 to 1.49). CHD patients had a lower median age at cancer diagnosis-13.0 years (IQR 2.9-30.0) in CHD versus 24.6 years (IQR 8.6-35.1) in controls. Median age at death was 15.1 years (IQR 3.6-30.7) in CHD patients versus 18.5 years (IQR 6.1-32.7) in controls. The top three fatal cancer diagnoses were ill-defined, secondary and unspecified, eye and central nervous system tumours and haematological malignancies. CONCLUSIONS: Cancer-related deaths constituted 1.8% of all mortalities across all CHD patients. Among CHD patients with cancer, 18.3% died, with cancer being the cause in 71.9% of cases. Although CHD patients have an increased cancer risk, their mortality risk post-diagnosis does not significantly differ from non-CHD patients after adjustements and exclusion of patients with genetic syndromes and transplant recipients. However, CHD patients with genetic syndromes and concurrent cancer appear to be a vulnerable group.

Corrigendum to "Risk of cancer in young and older patients with congenital heart disease and the excess risk of cancer by syndromes, organ transplantation and cardiac surgery: Swedish health registry study (1930-2017)" [The Lancet Regional Health-Europe 18 (2022) 100407].

[This corrects the article DOI: 10.1016/j.lanepe.2022.100407.].

Incidence of diabetes mellitus and effect on mortality in adults with congenital heart disease.

BACKGROUND: Worldwide, 1-2% of children are born with congenital heart disease (CHD) with 97% reaching adulthood. OBJECTIVES: This study aims to demonstrate the risk of diabetes in patients with CHD, and the influence of incident diabetes on mortality in CHD patients and controls. METHODS: By combining data from patient registries, the incidence of adult-onset diabetes registered at age 35 or older, and subsequent mortality risk were analysed in two successive birth cohorts (born in 1930-1959 and 1960-1983), by type of CHD lesion and sex, compared with population-based controls matched for sex and year of birth and followed until a maximum of 87 years of age. RESULTS: Out of 24,699 patients with CHD and 270,961 controls, 8.4% and 5.6%, respectively, were registered with a diagnosis of diabetes at the age of 35 or older, hazard ratio (HR) 1.47 (95% CI 1.40-1.54). The risk of diabetes was higher in the second birth cohort (HR of 1.74, 95% CI 1.54-1.95) and increased with complexity of CHD. After onset of DM, the total mortality among patients with CHD was 475 compared to 411/ 10,000 person-years among controls (HR 1.16, 95% CI 1.07-1.25). CONCLUSIONS: In this nationwide cohort of patients with CHD and controls, the incidence of diabetes was almost 50% higher in patients with CHD, with higher risk in the most recent birth cohort and in those with conotruncal defects, with the combination of CHD and diabetes associated with a significantly increased mortality compared to diabetic controls.

Impact of Down Syndrome on Survival Among Patients With Congenital Heart Disease.

BACKGROUND: Increasing survival among patients with congenital heart disease (CHD) has recently been reported. However, the impact of Down syndrome (DS) in patients with CHD is still debated. We aimed to estimate survival in patients with CHD with versus without DS compared with matched controls from the general population without CHD or DS. METHODS AND RESULTS: We linked data from Swedish health registries to identify patients with CHD born between 1970 and 2017. Data from the Total Population Register were used to match each patient with CHD by sex and birth year with 8 controls without CHD or DS. A Cox proportional regression model was used to estimate mortality risk, and Kaplan-Meier curves were analyzed for the survival analysis. We identified 3285 patients with CHD-DS, 64 529 patients with CHD without DS, and 26 128 matched controls. The mortality risk was 25.1 times higher (95% CI, 21.3-29.5) in patients with CHD-DS versus controls. The mortality rate was 2 times higher (95% CI, 1.94-2.31) for patients with CHD with versus without DS. Lower mortality was found during the second versus first birth periods in patients with CHD-DS compared with controls; hazard ratio: 46.8 (95% CI, 29.5-74.0) and 17.7 (95% CI, 12.8-24.42) in those born between 1970 and 1989 versus 1990 and 2017, respectively. CONCLUSIONS: In this retrospective cohort study, the mortality risk among patients with CHD-DS was 25 times higher compared with matched controls and 2 times higher compared with patients with CHD without DS. Survival was higher in patients with CHD-DS born after versus before 1990, coinciding with the modern era of congenital heart care.

Twenty-four-year trends in the incidence of ischemic stroke in Sweden from 1987 to 2010.

BACKGROUND AND PURPOSE: The incidence of stroke in Sweden increased between 1989 and 2000 among people aged ≤65 years, but more recent data on those aged >65 years are lacking. METHODS: Through the Swedish Hospital Discharge and Cause of Death registries, we identified all cases of nonfatal and fatal ischemic stroke (IS) among people aged 18 to 84 years during 1987-2010 in Sweden. RESULTS: Of the 391 081 stroke cases identified, 1.6% were 18 to 44 years, 16.7% were 45 to 64 years, and 81.7% were 65 to 84 years. Among people aged 18 to 44 years, there was a continuous increase in the incidence of stroke of 1.3% (95% confidence interval, 0.8%-1.8%) per year for men and 1.6% (1.0%-2.3%) per year for women. Among men and women aged 45 to 64 years, slightly declining rates were observed from the late 1990s, with a mean annual decrease of 0.4% (0.1%-0.7%) among men and 0.6% (0.2%-1.0%) among women. Among men aged 65 to 84 years, a decrease of 3.7% in IS (3.4%-4.0%) per year was observed from the late 1990s. This was more marked in women, where an initial decrease of 2.5% (2.1%-2.9%) per year was followed by an accelerated decrease of 5.1% (4.4%-5.8%) after 2005. Mortality from IS decreased markedly in all age groups. CONCLUSIONS: The incidence of IS in elderly people in Sweden is now decreasing, whereas the decline in IS incidence in the middle-aged people is much less steep. The increasing incidence of stroke in the young, particularly if carried forward to an older age, is concerning.

Twenty-year trends in long-term mortality risk in 17,149 survivors of ischemic stroke less than 55 years of age.

BACKGROUND AND PURPOSE: The purpose of the present study was to investigate the 4-year mortality risk among patients<55 years with a first ischemic stroke during 1987-2006. METHODS: A total of 17,149 cases (37.4% women) aged 18 to 54 years who survived≥28 days after a first ischemic stroke were identified in the Swedish Inpatient Register from 1987 to 2006. All patients were followed for 4 years or until death. The standardized mortality ratio was calculated by comparing the mortality rates with those of the general population of equivalent age, sex, and calendar year. RESULTS: During the period, there were 1265 deaths. Long-term survival improved over time in both men and women. Among men, the mortality risk decreased by 32% (hazard ratio=0.68 [95% confidence interval, 0.56-0.82]) from the first 5-year period to the last 5-year period (1987-1991 versus 2002-2006), and among women, the mortality risk decreased by 45% (0.55 [0.41-0.75]). Despite an overall decrease in mortality, the standardized mortality ratios for the last 5-year period remained high: 5.88 (95% confidence interval, 5.10-6.71) for men and 5.91 (4.68-7.29) for women with an absolute excess risk of 1.60 and 0.97 per 100 person-years, respectively, with nearly half of all deaths related to cardiovascular disease. CONCLUSIONS: During the 20-year period, 4-year mortality decreased by one third but was still 6-fold higher than that of the general population in the most recent period, emphasizing the importance of secondary prevention in young persons who have had a stroke.

Survival Trends in Children With Tetralogy of Fallot in Sweden From 1970 to 2017.

Importance: Mortality in patients with tetralogy of Fallot (TOF) has decreased substantially since the start of surgical correction of this abnormality in the 1950s. However, nationwide data in Sweden comparing survival trends among pediatric patients with TOF with the general population are still limited. Objective: To study survival trends in pediatric patients with TOF and compare them with matched controls. Design, Setting, and Participants: A Swedish registry-based, nationwide, matched cohort study was conducted; data were collected from national health registers from January 1, 1970, to December 31, 2017. Patients with a registered diagnosis of TOF as well as controls without TOF matched by birth year and sex were included in the study. Follow-up data were collected from birth to age 18 years, death, or the end of follow-up (December 31, 2017), whichever occurred first. Data analysis was performed from September 10 to December 20, 2022. Survival trends among patients with TOF were compared with matched controls using Cox proportional hazards regression and Kaplan-Meier survival analyses. Main Outcomes and Measures: All-cause mortality during childhood in patients with TOF and matched controls. Results: The population included 1848 patients (1064 [57.6%] males; mean [SD] age, 12.4 [6.7] years) with TOF and 16 354 matched controls. The number of patients who underwent congenital cardiac surgery (henceforth, surgery group) was 1527 (897 [58.7%] males). In the whole TOF population from birth until age 18 years, 286 patients (15.5%) died during a mean (SD) follow-up time of 12.4 (6.7) years. In the surgery group, 154 of 1527 patients (10.1%) died during a follow-up time of 13.6 (5.7) years with a mortality risk of 21.9 (95% CI, 16.2-29.7) compared with matched controls. When stratified by birth period, a substantial decrease in the mortality risk was noted in the surgery group, from 40.6 (95% CI, 21.9-75.4) in those born in the 1970s to 11.1 (95% CI, 3.4-36.4) in those born in the 2010s. Survival increased from 68.5% to 96.0%. The risk of mortality for surgery decreased from 0.52 in the 1979s to 0.19 in the 2010s. Conclusions and Relevance: The findings of this study suggest there has been substantial improvement in survival in children with TOF who underwent surgery from 1970 to 2017. However, the mortality rate is still significantly higher in this group compared with matched controls. Predictors of good and poor outcomes in this group need to be further explored, with the modifiable ones evaluated for further outcome improvements.

Congenital heart disease: changes in recorded birth prevalence and cardiac interventions over the past half-century in Sweden.

AIMS: Our objective was to assess changes in the birth prevalence of CHD over a half-century in a high-resource, nationwide setting, as well as changes in the prevalence of cardiac interventions in this population. METHODS AND RESULTS: The Swedish National Patient and Cause of Death registers were linked to estimate the annual rates of CHD and cardiac interventions among live-born infants from 1970 to 2017. Additionally, separate estimates were obtained by lesion complexity, from mild to the most complex forms of CHD. Overall, the numbers of live-born infants with a CHD identified varied from 624 to 2459 annual cases, with rates increasing steadily from 5.7 to an average of 20 per 1000 live births at the end of the study period, and with a more pronounced increase from 1996 to 2005. The largest increase over time was observed for mild CHD lesions. Overall, the proportion of cardiac interventions among patients with CHD declined from 40.7% in 1970 to below 15.0% after 2014. However, in the most complex CHD lesion groups, overall cardiac interventions increased from 57.1 to 76.8% in patients with conotruncal defects and from 32.8 to 39.5% in those with severe non-conotruncal defects. CONCLUSION: The live-birth prevalence of CHD in Sweden more than tripled during the past half-century, most likely resulting from more accurate diagnostic capabilities. The largest increase over time was observed among patients with simple defects. During the same period, overall cardiac interventions decreased whereas interventions for the most complex CHD groups increased.

Socioeconomic factors and long-term mortality risk after surgical aortic valve replacement.

Background: There is scarce knowledge about the association between socioeconomic status and mortality in patients undergoing surgical aortic valve replacement. This study explores the associations between income, education and marital status, and long-term mortality risk. Methods: In this national registry-based observational cohort study we included all 14,537 patients aged >18 years who underwent isolated surgical aortic valve replacement for aortic stenosis in Sweden 1997-2020. Socioeconomic status and comorbidities were collected from three mandatory national registries. Cox regression models adjusted for patient characteristics and comorbidities were used to estimate the mortality risk. Results: Mortality risk was higher for patients in the lowest versus the highest income quintile (adjusted hazard ratio [aHR] 1.36, 95 % confidence interval [CI]: 1.11-1.65), for patients with <10 years education versus >12 years (aHR 1.20, 95 % CI:1.08-1.33), and for patients who were not married/cohabiting versus those who were (aHR 1.24, 95 % CI:1.04-1.48). Patients with the most unfavorable socioeconomic status (lowest income, shortest education, never married/cohabiting) had an adjusted median survival of 2.9 years less than patients with the most favorable socioeconomic status (14.6 years, 95 % CI: 13.2-17.4 years vs. 11.7 years, 95 % CI: 9.8-14.4). Conclusions: Low socioeconomic status in patients undergoing surgical aortic valve replacement is associated with shorter survival and an increased long-term adjusted mortality risk. These results emphasize the importance of identifying surgical aortic valve replacement patients with unfavorable socioeconomic situation and ensure sufficient post-discharge surveillance.

Risk of cancer in young and older patients with congenital heart disease and the excess risk of cancer by syndromes, organ transplantation and cardiac surgery: Swedish health registry study (1930-2017).

Background: Increasing survival of patients with congenital heart disease (CHD) will result in an increased risk of age-dependent acquired diseases later in life. We aimed to investigate the risk of cancer in young and older patients with CHD and to evaluate the excess risk of cancer by syndromes, organ transplantation and cardiac surgery. Methods: Patients with CHD born between 1930 and 2017 were identified using Swedish Health Registers. Each patient with CHD (n = 89,542) was matched by sex and birth year with ten controls without CHD (n = 890,472) from the Swedish Total Population Register. Findings: 4012 patients with CHD (4·5%) and 35,218 controls (4·0%) developed cancer. The median follow-up time was 58·8 (IQR 42·4-69·0) years. The overall cancer risk was 1·23 times higher (95% confidence interval (CI) 1·19-1·27) in patients with CHD compared with matched controls, and remained significant when patients with syndromes and organ transplant recipients were excluded. The risk of cancer was higher in all CHD age groups, and in patients that underwent cardiac surgery during the first year after birth (Hazard Ratio 1·83; 95% CI 1·32-2·54). The highest risk was found in children (0-17 years), HR 3·21 (95% CI 2·90-3·56). Interpretation: The cancer risk in patients with CHD was 23% higher than in matched controls without CHD. The highest risk was found in children and in the latest birth cohort (1990-2017). Funding: Funding by the Swedish state (Grant Number: 236611), the Swedish Research Council (Grant Number: 2019-00193), the Swedish Childhood Cancer Fund (Grant Number: SP2017-0012) and the Swedish Heart-Lung Foundation (Grant Number: 20190724).