Swedish National Patient Register Research Articles

Identification of irritable bowel syndrome in the Swedish National Patient Register: a validation study

Background and objective National patient registers are valuable in epidemiological studies. To ensure high-quality data for studies of irritable bowel syndrome (IBS), this study aimed to validate the ICD-10 code for IBS in the Swedish National Patient Register. Methods The positive predictive values (PPV) for IBS defined by the Rome criteria were calculated based on a review of medical records of randomly selected individuals with a first-ever diagnostic listing of IBS in the Swedish National Patient register in the year 2005 (Rome II criteria) or 2010 (Rome III criteria). Key results 340 medical records were reviewed (172 from 2005 and 168 from 2010). The majority of patients were females (74%), and the mean age was 42 years. IBS used in any type of department had a PPV of 76% (95% confidence interval 71–80%), which increased to 80% (76–84%) when we included individuals likely to have IBS but where information about some aspects of the Rome criteria was lacking in the medical record. Two highly specialized gastroenterological departments had the best PPV, 96%, while departments of internal medicine in general had a PPV of 82% (80–95%). The PPV for the IBS subtype was 62% (55–67%). The PPVs were not significantly different comparing the two time periods investigated. Conclusion and inferences The validity of a register-based definition of IBS in the Swedish National Patient register is high and can be used to identify patients with IBS in observational research. The data source, i.e., type of hospital and department, influences reliability.

P180 Ileorectal anastomosis preserves female fertility after colectomy in Ulcerative Colitis

Abstract Background Colectomy and reconstruction in patients with inflammatory bowel disease (IBD) may adversely affect fertility, but few population-based studies are available. Methods Fertility was assessed in 2,989 women and 3,771 men with IBD and prior colectomy 1964-2014, identified from the Swedish National Patient Register, and 35,092 matched population-based individuals. Results Reconstruction with ileoanal pouch anastomosis (IPAA) was as common as ileorectal anastomosis (IRA) in ulcerative colitis (UC) and IBD-unclassified (IBD-U) while rare in Crohn’s disease (CD). Compared with the matched non-IBD cohort, women with IBD had lower fertility overall post colectomy (HR 0.65, CI 0.61-0.69). The impact was least in patients with colectomy only with the rectum left intact (HR 0.79, CI 0.70–0.90) and more pronounced after reconstructive surgery. Comparing the surgical options within the cohort of women with colectomy for IBD using colectomy only as reference, fertility in female patients remained unaffected after IRA (HR 0.86, CI 0.63-1.17 for UC, 0.86, CI 0.68-1.08 for IBD-U and 1.07, CI 0.70-1.63 for CD), but was impaired after IPAA, especially in UC (HR 0.67 CI 0.50-0.88), and after completion proctectomy (HR 0.65, CI 0.49-0.85 for UC, 0.68, CI 0.55-0.85 for IBD-U and 0.61, CI 0.38-0.96 for CD). In men, fertility was marginally reduced in the entire cohort post colectomy (HR 0.89, CI 0.85-0.94), regardless of reconstruction. Conclusion Fertility was reduced in women after colectomy for IBD. The least impact was seen with a deviated rectum left in situ. IRA was associated with no further reduction in fertility, whereas proctectomy with or without IPAA were associated with the strongest impairment. IRA therefore seems to be the preferred reconstruction to preserve fertility in selected female patients. Fertility in men was only moderately reduced after colectomy and reconstructive choice.

Economic Burden of Palmoplantar Pustulosis in Sweden: A Population-based Register Study

The aim of this study was to estimate the economic burden of palmoplantar pustulosis, a chronic relapsing skin condition commonly occurring in combination with psoriasis vulgaris. Using data from the Swedish National Patient Register and Swedish Prescribed Drug Register for 2015, the study estimated all-cause and palmoplantar pustulosis-specific healthcare resource use (inpatient stays, physician visits and drug use) for 14,715 patients with palmoplantar pustulosis, and compared these both with matched controls from the general population and with patients with psoriasis vulgaris (without palmoplantar pustulosis). Mean annual direct costs for a patient with palmoplantar pustulosis was higher compared with costs for the general population (3,000 vs 1,700 Euro, p < 0.001). Compared with psoriasis vulgaris, more patients with palmoplantar pustulosis had inpatient stays, but fewer had physician visits and psoriasis-related drugs; the overall costs were similar. Only a small fraction of the costs of physician visits and inpatient stays for patients with palmoplantar pustulosis were attributable to specific palmoplantar pustulosis problems, indicating a clear comorbidity burden in palmoplantar pustulosis.

Low Apgar score and need for resuscitation increased the probability of receiving therapeutic hypothermia more strongly than acidosis at birth.

The aim of this study was to investigate how individual markers for birth asphyxia, so-called A criteria, were associated with the probability of receiving therapeutic hypothermia. This population-based cohort study included 1336 live-born singleton term infants with any A criterion in the Stockholm-Gotland Region, Sweden during 2008 to 2014. The Swedish Neonatal Quality Register and National Patient Register were used for data collection. Results were presented as adjusted odds ratios (aORs) with 95% confidence intervals (CIs). There were 89 infants, 44 boys and 45 girls with mean gestational age 40.5 weeks, who received therapeutic hypothermia. Low Apgar score, aOR 12.44 (95% CI 5.99-25.86), and resuscitation, aOR 9.18 (95% CI 3.77-22.34), were strongly associated with therapeutic hypothermia. A pH <7.0 was less associated with the outcome, aOR 2.02 (95% CI 1.02-4.0). No infant who received therapeutic hypothermia fulfilled the criteria of base deficit ≥16 mmol/L only. A low Apgar score of and/or a need for resuscitation is more relevant for identifying infants eligible for therapeutic hypothermia, compared to other A criteria. This knowledge could be used clinically to identify cases for review and avoid unnecessary monitoring of infants.

Abstract P074: Pregnancy and neonatal outcomes among early-onset colorectal cancer survivors: a nationwide case-control study

Abstract Purpose: Early-onset colorectal cancer (CRC diagnosed before age 50) has risen worldwide, with an increasing number of survivors of reproductive age. We aimed to investigate the risk of adverse pregnancy and neonatal outcomes among early-onset CRC survivors. Methods: We conducted a nationwide case-control study of 207 births in women with early-onset CRC and 1019 births in women without CRC from the general Swedish population (1992-2019), matched on age, calendar year of birth, parity, and county of residence. To control for genetic susceptibility and shared childhood exposures, we additionally identified 146 births in female siblings of the CRC survivors. Early-onset CRC cases were identified through the Swedish Cancer Register (1969-2019), and siblings were identified through the Swedish Multi-generation Register. Maternal characteristics and neonatal outcomes were retrieved from the Swedish Medical Birth Register and National Patient Register. Using conditional logistic regression, we estimated adjusted odds ratios (aORs) and 95% confidence intervals (CIs). Results: Compared to women without prior CRC, early-onset CRC survivors who gave birth had increased risk of pre-eclampsia (aOR 2.52, 95% CI 1.25 to 5.08), induced labor (aOR 1.64, 95% CI 1.09 to 2.46) and emergency Cesarean section (aOR 1.79, 95% CI 1.17 to 2.75), after adjustment for maternal education level, country of birth, body mass index and smoking in early pregnancy, and comorbidities. Sibling analyses showed similar trends, especially for higher risk of induced labor (aOR 2.58, 95% CI 1.03 to 6.49). Maternal history of early-onset CRC was also associated with offspring preterm birth (aOR 2.31, 95% CI 1.34 to 3.99) and low birth weight (aOR 2.95, 95% CI 1.40 to 6.21), and risks were particularly pronounced for medically indicated preterm birth (aOR 4.48, 95% CI 2.05 to 9.79). No significant associations were found for small for gestational age birth, low Apgar score, and congenital malformations. Conclusion: In this population-based study, history of early-onset CRC was associated with increased risk of adverse pregnancy outcomes, including pre-eclampsia, induction of labor, and emergency Cesarean section. Offspring to mothers with early-onset CRC also had increased risk of medically indicated preterm birth and neonatal low birth weight. Citation Format: Yin Cao, Stephanie Zhao, Tomas S. Bexelius, Jonas Söderling, Mengyao Shi, Bjorn Roelstraete, Barbara B. Warner, Olof Stephansson, Jonas F. Ludvigsson. Pregnancy and neonatal outcomes among early-onset colorectal cancer survivors: a nationwide case-control study. [abstract]. In: Proceedings of the AACR Special Conference: Precision Prevention, Early Detection, and Interception of Cancer; 2022 Nov 17-19; Austin, TX. Philadelphia (PA): AACR; Can Prev Res 2023;16(1 Suppl): Abstract nr P074.

Risk of Cancer and Subsequent Mortality in Primary Biliary Cholangitis: A Population-based Cohort Study of 3052 Patients.

Primary biliary cholangitis (PBC) is a rare cholestatic liver disease. Incident cancer is a concern. Previous studies have described an increase in hepatocellular carcinoma (HCC), but the risk of nonhepatic cancer and cancer risk across subgroups is largely unknown. We used the Swedish National Patient Register to identify all patients diagnosed with PBC between 2002 and 2019. Patients were matched for age, sex, and municipality with up to 10 reference individuals from the general population. Incident cancer was recorded from the National Cancer Register. Cox regression was used to investigate the rates of cancer and postcancer mortality, adjusted for potential confounders. The cumulative incidence of cancer was calculated while accounting for the competing risk of death. At 10 years of follow-up, the cumulative incidence of any cancer in patients with PBC (n= 3052) was 14.3% (95% confidence interval (CI)= 12.8-15.9), compared to 11.8% (95% CI 11.3-12.2) in the reference population (n= 26,792) (adjusted hazard ratio aHR= 1.4, 95% CI= 1.2-1.5). The rate of HCC was particularly high (aHR 30.9; 95% CI= 14.8-64.6). The rate of cancer was higher in patients with cirrhosis (aHR 2.1; 95% CI 1.4-3.0), but similar across categories of age and sex. Increased rates of other cancer subtypes, including gastrointestinal (aHR= 1.5, 95% CI= 1.1-1.9), lung (aHR= 1.5, 95% CI= 1.1-2.2), and lymphoma (aHR= 2.9, 95% CI= 1.9-4.6) were seen. Following a diagnosis of cancer, patients with PBC had higher mortality rates compared to reference individuals (aHR= 1.4, 95% CI= 1.2-1.7). This was mainly driven by HCC (non-HCC-related mortality: aHR= 1.1, 95% CI= 0.9-1.5). Patients with PBC have a significantly higher risk of HCC compared to matched individuals from the general population, but only a low risk increase of non-HCC cancer.

The joint effect between fetal growth and health behaviors on the risk of cardiovascular diseases in young adulthood

To investigate the individual and the joint effect of impaired fetal growth and adult health behaviors on the risk of cardiovascular diseases (CVDs). A total of 15,618 individuals were included from three sub-cohorts of the Stockholm Public Health Cohort. Data on participants' birthweight and gestational age were retrieved from the Medical Birth Register. Data on the diagnoses of CVDs were extracted from the Swedish National Patient Register and the Cause of Death Register. Data on health behaviors were identified from self-reported questionnaires, and health behavioral profile was defined based on the recommendations of the American Health Association. The associations of fetal growth and health behaviors with the risk of CVDs were analyzed using Cox proportional hazard model. Individuals born small for gestational age (SGA) had a higher risk of CVDs than those born appropriate for gestational age (AGA), and the adjusted hazard ratio (HR) and 95% confidence interval (CI) was 1.88 (1.44, 2.47). Participants born SGA and having poor health behavioral profile in adulthood had a higher risk of CVDs than those born AGA and having ideal health behaviors with adjusted HR (95% CI) being 3.58 (1.95, 6.56). Impaired fetal growth was associated with an increased risk of CVDs in adulthood, and the risk was highest in individuals with both impaired fetal growth and poor health behaviors in adulthood.

Reoperations and Long-term Survival of Kock's Continent Ileostomy in Patients With IBD: A Population-Based National Cohort Study From Sweden.

Kock's continent ileostomy is an option after proctocolectomy for patients not suitable for IPAA or ileorectal anastomosis. Ulcerative colitis is the most common indication for continent ileostomy. The aim of this study was to evaluate the long-term outcome of continent ileostomy. Retrospective cohort register study. Data were obtained from the Swedish National Patient Registry. All patients with IBD and a continent ileostomy were identified. Data on demographics, diagnosis, reoperations, and excisions of the continent ileostomy were obtained. Patients with inconsistent diagnostic coding were classified as IBD-unclassified. The main outcome measures were number of reoperations, time to reoperations, and time to excision of continent ileostomy. We identified 727 patients, 428 (59%) with ulcerative colitis, 45 (6%) with Crohn's disease, and 254 (35%) with IBD-unclassified. After a median follow-up time of 27 (interquartile range, 21-31) years, 191 patients (26%) never had revision surgery. Some 1484 reoperations were performed on 536 patients (74%), and the median number of reoperations was 1 (interquartile range, 0-3) per patient. The continent ileostomy was excised in 77 patients (11%). Reoperation within the first year after reconstruction was associated with a higher rate of revisions (incidence rate ratio, 2.90; p < 0.001) and shorter time to excision (HR 2.38; p < 0.001). Constructing the continent ileostomy after year 2000 was associated with increased revision and excision rates (incidence rate ratio, 2.7; p < 0.001 and HR 2.74; p = 0.013). IBD-unclassified was associated with increased revisions (incidence rate ratio, 1.3; p < 0.001)' and the proportion of IBD-unclassified patients almost doubled from the 1980s (32%) to after 2000 (50%). Retrospective design, data from a register, and no data on quality of life were available were the limitations of this study. Continent ileostomy is associated with substantial need for revision surgery, but most patients keep their reconstruction for a long time. See Video Abstract at http://links.lww.com/DCR/C122 . ANTECEDENTES:La ileostomía continente de Kock es una opción después de la proctocolectomía para los pacientes que no son aptos para la anastomosis ileoanal con reservorio o la anastomosis ileorrectal. La colitis ulcerativa es la indicación más común para la ileostomía continente.OBJETIVO:El objetivo de este estudio fue evaluar el resultado a largo plazo de la ileostomía continente.DISEÑO:Estudio de registro de cohorte retrospectivo.AJUSTES:Los datos se obtuvieron del Registro Nacional de Pacientes de Suecia.PACIENTES:Se identificaron todos los pacientes con enfermedad inflamatoria intestinal e ileostomía continente. Se obtuvieron datos demograficos, diagnóstico, reoperaciones y extirpaciones de la ileostomía continente. Los pacientes con codificación diagnóstica inconsistente se clasificaron como no clasificados con EII.PRINCIPALES MEDIDAS DE RESULTADO:Las principales medidas de resultado fueron el número de reoperaciones, el tiempo hasta las reoperaciones y el tiempo hasta la escisión de la ileostomía continente.RESULTADOS:Identificamos 727 pacientes, 428 (59%) con colitis ulcerativa, 45 (6%) con enfermedad de Crohn y 254 (35%) con EII no clasificada. Después de una mediana de tiempo de seguimiento de 27 (IQR 21-31) años, 191 (26%) pacientes nunca se habían sometido a una cirugía de revisión. Se realizaron 1.484 reintervenciones en 536 (74%) pacientes, la mediana de reintervenciones fue de 1 (RIC 0-3) por paciente. La ileostomía continente se extirpó en 77 (11%) pacientes. La reoperación dentro del primer año después de la reconstrucción se asoció con una mayor tasa de revisiones (IRR 2,90 p < 0,001) y un tiempo más corto hasta la escisión (HR 2,38 p < 0,001). La construcción de la ileostomía continente después del año 2000 se asoció con mayores tasas de revisión y escisión (IRR 2,7 p < 0,001 y HR 2,74 p = 0,013). La EII no clasificada se asoció con un aumento de las revisiones (IRR 1,3 p < 0,001) y la proporción de pacientes con EII no clasificada casi se duplicó desde la década de 1980 (32%) hasta después de 2000 (50%).LIMITACIONES:Diseño retrospectivo, datos de registro. No hay datos disponibles sobre la calidad de vida.CONCLUSIÓN:La ileostomía continente se asocia con una necesidad sustancial de cirugía de revisión, pero la mayoría de los pacientes logran mantener su reconstrucción durante mucho tiempo. Consulte Video Resumen en http://links.lww.com/DCR/C122 . (Traducción-Dr. Yolanda Colorado ).

One versus two biological parents with mental disorders: Relationship to educational attainment in the next generation.

Both maternal and, separately, paternal mental illness are associated with diminished academic attainment among children. However, the differential impacts of diagnostic type and degree of parental burden (e.g. one v. both parents affected) on these functional outcomes are unknown. Using the Swedish national patient (NPR) and multi-generation (MGR) registers, 2 226 451 children (1 290 157 parental pairs), born 1 January 1973-31 December 1997, were followed through 31 December 2013. Diagnostic status of all cohort members was defined for eleven psychiatric disorders, and families classed by exposure: (1) parents affected with any disorder, (2) parents affected with a disorder group (e.g. neuropsychiatric disorders), and (3) parents affected with a specific disorder (e.g. ADHD). Pairs were further defined as 'unaffected,' 'single-affected,', or 'dual-affected.' Among offspring, the study evaluated fulfillment of four academic milestones, from compulsory (primary) school through University (college). Sensitivity analyses considered the impact of child's own mental health, as well as parental education, on main effects. Marked reductions in the odds of achievement were observed, emerging at the earliest levels of schooling for both single-affected [adjusted odds ratio (aOR), 0.50; 95% CI 0.49-0.51] and dual-affected (aOR 0.29, 95% CI 0.28-0.30) pairs and persisting thereafter [aOR range (single), 0.52-0.65; aOR range (dual), 0.30-0.40]. This pattern was repeated for analyses within diagnosis/diagnostic group. Main results were robust to adjustment for offspring mental health and parent education level. Parental mental illness is associated with profound reductions in educational attainment in the subsequent generation, with children from dual-affected families at uniquely high risk.

Adherence to CPAP therapy in Down syndrome: the population-based DISCOVERY study.

Persons with Down syndrome (DS) have an increased risk of obstructive sleep apnea (OSA) needing continuous positive airway pressure (CPAP), but data on the therapy and outcomes in this population are scarce. We aimed to compare patient characteristics and outcomes of CPAP treatment for patients with OSA with and without DS. This was a population-based, longitudinal study on patients initiating CPAP therapy between July 2010 and March 2018 in Sweden and a population-based sex- and age-matched control group (control:case ratio 5:1), with linked data from the Swedish National Patient Registry and the Prescribed Drug Registry (DISCOVERY study cohort). Patients with DS (n = 64) had a higher apnea-hypopnea index (51.7 ± 30.3 vs 36.8 ± 29.1 events/h, P < .001), Epworth Sleepiness Scale score (13.7 ± 5.9 vs 11.0 ± 4.9, P = .001), rate of previous surgery of tonsils and/or adenoids (21.9% vs 8.2%, P = .001), and more thyroid replacement hormone therapy (45.3% vs 7.8%, P < .001), but lower use of cardiovascular drugs (7.8% vs 22.3%, P = .003) compared with controls. At follow-up after 1.3 ± 0.9 years, there were no differences in nocturnal CPAP usage time (5.6 ± 2.4 vs 5.5 ± 2.0 hours, P = .77), CPAP adherence ≥ 4 hours/night (62% vs 65%, P = .93), or improvement in Epworth Sleepiness Scale score (-5.4 ± 6.8 vs -5.0 ± 2.0, P = .84) between DS and non-DS patients. OSA severity was substantially higher in patients with DS despite an increased rate of tonsil surgery. Treatment outcomes in terms of adherence and improved daytime sleepiness were comparable between groups, underlining the importance of both OSA diagnosis and treatment in patients with DS. Svensson M, Ekström M, Sundh J, Ljunggren M, Grote L, Palm A. Adherence to CPAP therapy in Down syndrome: the population-based DISCOVERY study. J Clin Sleep Med. 2023;19(3):453-458.

Postoperative morbidity and mortality after adenoidectomy: A national population-based study of 51 746 surgeries.

To investigate postoperative morbidity and mortality after paediatric adenoidectomy. This was a retrospective national population-based cohort study of data from the Swedish National Patient Register (NPR) and The Swedish Cause of Death Register (CDR). All patients aged 0-18 years who underwent adenoidectomy from 2007 to 2017 (without concomitant tonsil surgery) were included in this study. To evaluate postoperative morbidity and mortality, all diagnostic and surgical codes registered in the NPR for health care contacts within 30 days of surgery were analysed. The patients retrieved from the NPR were matched with the CDR to identify any deaths occurring within 30 days of the surgery. A total of 51746 adenoidectomies were included in this study. No deaths related to adenoidectomy were identified. All types of haemorrhagic complications were rare. Only 0.1% of the surgeries resulted in an outpatient contact due to postoperative haemorrhage and only 0.1% of the adenoidectomies resulted in a readmission due to haemorrhage. The rarest haemorrhagic complication was RTT (return to theatre), with only 4 events (0.01%). Postoperative haemorrhage was most frequent on the first day after surgery. Other complications were rare as well, requiring a total of 922 (2.6%) outpatient visits and 75 (0.2%) readmissions in the adenoidectomy group, with postoperative infection being the most commonly reported. Overall, adenoidectomy should be considered a safe surgical procedure associated with few postoperative complications. No deaths related to adenoidectomy were found. Severe complications, such as late postoperative haemorrhage after adenoidectomy, were rare, and haemorrhage resulting in RTT was even rarer. The highest rate of postoperative haemorrhage was observed the first day after surgery, and most haemorrhagic complications occurred within a week. Comparisons with studies on tonsil surgery show that adenoidectomy is associated with substantially lower postoperative morbidity.

Mortality Among Pediatric Patients With Acute Lymphoblastic Leukemia in Sweden From 1988 to 2017

Acute lymphoblastic leukemia (ALL) constitutes 20% to 30% of all pediatric cancers. The 5-year overall survival among pediatric patients with ALL in high-income countries such as Sweden is currently more than 90%, but long-term unselected nationwide mortality data and mortality data in relation to the general population are lacking. To compare mortality between pediatric patients with ALL and the general population during a 30-year period in Sweden and to assess the incidence of ALL in Sweden. This cohort study included pediatric patients (aged <18 years) with a morphologically verified ALL diagnosis in the Swedish Cancer Register and/or at least 2 ALL diagnoses in the Swedish National Patient Register between January 1, 1988, and December 31, 2017. Data were cross-linked to the Swedish Cause of Death Register. Data were analyzed from May 2019 to January 2022. The main outcomes were mortality among patients with ALL compared with that in the general population and mortality in different subgroups within the cohort. Standardized mortality ratios (SMRs) were calculated using the general Swedish population as a reference. Within-cohort survival analyses were performed. A total of 2397 patients (1354 [56%] male; mean [SD] age at diagnosis, 6.1 [4.7] years) were included in the study. The mean (SD) incidence of pediatric ALL during the study period was 4.11 (0.60) cases per 100 000 persons per year (females, 3.68 [0.65] cases per 100 000 persons per year; males, 4.52 [0.81] cases per 100 000 persons per year; P < .001). The observed number of deaths among pediatric patients with ALL was 409 vs the 9.5 deaths expected in the general population, resulting in an overall SMR of 43.1 (95% CI, 39.0-47.5); females had a higher SMR than males (57.8 [95% CI, 49.5-67.2] vs 34.5 [95% CI, 32.0-41.4]; P < .001). Analysis within the cohort showed a continued decrease in survival throughout the 30-year follow-up. The association between calendar year of ALL diagnosis, corresponding with different ALL treatment protocols, and mortality showed the lowest survival for the 1988-1991 group and the highest for the 2008-2017 group (χ2 = 20.3; P < .001). In this cohort study, a consistently high SMR was seen among pediatric patients with ALL. Within the ALL cohort, survival evolved to a similar extent as in the young general population of Sweden. Furthermore, survival among patients with ALL decreased throughout the whole follow-up period without any trend difference after the 5-year follow-up time point. The changes in ALL treatment protocols were associated with overall improved absolute survival over time.

Cardiovascular Risk Factors before Onset of Rheumatoid Arthritis Are Associated with Cardiovascular Events after Disease Onset: A Case-Control Study.

The increased comorbidity and mortality in rheumatoid arthritis (RA) patients are largely due to cardiovascular disease (CVD). Previously, we demonstrated increased frequencies of risk factors for CVD (elevated body mass index (BMI), elevated apoliprotein (Apo) B:ApoA1 ratio, and smoking) in pre-RA individuals compared with matched controls. Assess the impact of traditional CV risk factors present before the onset of RA on the risk of CV events (CVE) after diagnosis in comparison with matched controls. A case-control study including 521 pre-symptomatic individuals and 1566 controls identified within the Health Surveys of the Medical Biobank was performed. CVD risk factors were hypertension, elevated ApoB:A1 ratio, BMI, diabetes, and smoking. Information on comorbidities was requested from the Swedish National Patient Register and Cause of Death Register. Pre-RA individuals had a higher risk of future CVE compared with matched controls (HR [95% CI] 1.70 [1.31-2.21]), which remained after adjustments for risk factors for CVD (HR [95% CI] 1.73 [1.27-2.35]). Most risk factors were associated with CVE after diagnosis, and a combination resulted in a higher risk in RA compared with controls; two risk factors, HR [95% CI] 2.70 [1.19-6.13] vs. 1.26 [0.75-2.13]; and three to four risk factors, HR [95% CI] 6.32 [2.92-13.68] vs. 3.77 [2.34-6.00]. Risk factors for CVD present in pre-RA individuals were associated with future CVE, and even after adjustments for these risk factors and treatments after RA onset, pre-RA individuals had a higher risk of CVE compared with controls. These findings further highlight the importance of the early assessment of risk for CVD.

Overall survival of individuals with metastatic cancer in Sweden: a nationwide study

AimsConsistent improvements for overall survival (OS) have been reported for individuals with metastatic cancer. Swedish population-based registers allow national coverage and long follow-up time. The aim of this study was to estimate and explore long-term OS of individuals diagnosed with metastatic cancer using Swedish nationwide health registers.MethodsIndividuals with metastatic breast (MBC), non-small cell lung (MNSCLC), ovary (MOC) or colorectal cancer (MCRC) or metastatic malignant melanoma (MMM) were identified in the Swedish national cancer register and national patient registers. Survival was estimated and stratified by available variables. Potential cure fractions were estimated using mixture cure models.ResultsIn total, approximately 69,000 individuals were identified. The most common cancers were MCRC (36.2%) and MNSCLC (29.5%). Men were more frequently diagnosed with MNSCLC, MCRC, and MMM compared to women. Except for MOC, about 50% of individuals were 70 years or older at diagnosis. Throughout the study period survival differed across cancers. The longest median OS was observed for individuals with MOC and MBC. At 10 years of follow-up, the survival curves flatten at a survival rate of approximately 10% for all cancers except MNSCLC. The youngest age groups had the longest median OS. Increased survival was also observed for individuals diagnosed in 2015 and 2018 compared to individuals diagnosed during earlier years. The estimated cure fractions were 4% for MBC, 1.5% for MNSCLC, 6.8% for MCRC, 8.6% for MOC and MMM.ConclusionsLong-term survival has been assessed across all indications except for NSCLC.. The findings may be relevant for healthcare planning to meet the needs of future patients and potential long-term survivors.

BJS-02 LONG-TERM REOPERATION RATE FOLLOWING PRIMARY VENTRAL HERNIA REPAIR: A REGISTER-BASED STUDY

Abstract Aim The aim of this study was to analyse the risk for reoperation following primary ventral hernia repair. Methods The study was based on umbilical hernia and epigastric hernia repairs registered in the population-based Swedish National Patient Register (NPR) 2010–2019. Reoperation was defined as repeat repair after primary repair. Results Altogether 30,253 umbilical hernia repairs and 7407 epigastric hernia repairs were identified. There were 624 reoperations registered following primary umbilical repair and 137 following primary epigastric repairs. In multivariable Cox proportional hazard analysis, the hazard ratio (HR) for reoperation was 0.284 (95% confidence interval (CI) 0.106–0.760) after open onlay mesh repair, 0.476 (CI 0.359–0.629) after open interstitial mesh repair, 0.368 (CI 0.230–0.590) after open sublay mesh repair, 0.446 (CI 0.167–1.194) after open intraperitoneal onlay mesh repair, 0.931 (CI 0.639–1.357) after laparoscopic repair, and 0.939 (CI 0.502–1.757) after other (unknown) techniques, when compared to open suture repair as reference method. Following umbilical hernia repair, the risk for reoperation was also significantly higher for patients aged ≤49 years (HR 1.669, CI 1.391–2.002), for women (HR 1.390, CI 1.178–1.641), and for patients with liver cirrhosis (HR 2.546, CI 1.050–6.174). For patients undergoing epigastric hernia repair, the only significant risk factor for reoperation was age ≤49 years (HR 2.079, CI 1.380–3.134). Conclusions All types of open mesh repair were associated with lower reoperation rates than open suture repair and laparoscopic repair. Female sex, young age and liver cirrhosis were risk factors for reoperation due to hernia recurrence, regardless of method.

Dyspnea and perfusion defects (V/Q scan) are common after an acute pulmonary embolus whereas chronic thromboembolic pulmonary hypertension (CTEPH) is a rare sequele

Abstract Introduction Acute pulmonary embolism (PE), a common disease with substantial morbidity and mortality. Many previous studies have focused on the short-term consequences after an acute PE, but less attention has been directed to the long-term effects of a PE. The most feared complication is chronic thromboembolic pulmonary hypertension (CTEPH), with reported incidence rates ranging from 0.6 to 4.8%. Even though CTEPH is a rare complication after PE, dyspnea and functional impairment are common complaints, and a syndrome named PPES (post-PE-syndrome) has been suggested. The aim of this study was to follow a large national-wide cohort of post-PE-patients and to gather information regarding long-term symptoms and to determine the prevalence of CTEPH. Patients and methods We identified all patients diagnosed with acute PE in 2005 (ICD-10-SE: I26.0 and I26.9) that were registered in the Swedish National Patient Register (NPR), which has nearly 100% coverage. In 2007, all surviving individuals were invited to participate by letter. They were asked to complete a questionnaire regarding symptoms and risk factors for long-term sequele including CTEPH. Individuals who reported dyspnea or had known risk factors for CTEPH were invited to a follow-up with blood tests (NT-pro-BNP) and pulmonary scintigraphy. If any perfusion defects were detected, an echocardiography was performed. If signs of pulmonary hypertension, a referral was done to the regional PAH-center. Results In 2005, 5793 unique patients were admitted with a PE diagnosis in Sweden. Of those, 3510 were still alive in 2007, and 3226 individuals were contacted and asked to complete the questionnaire. Altogether 2105 (65%) individuals returned the questionnaire, and 53% reported dyspnea and 15% were asymptomatic but had risk factors for CTEPH. Altogether 944 individuals were referred for further follow-up including a recommendation to perform a V/Q-scan. Altogether 530 V/Q-scans were performed and 44% showed perfusion defects, whereof 85% had an echocardiography. Twenty-seven % of them registered a systolic right ventricular pressure of at least 30 mmHg. Referrals resulted in 6 cases with proved CTEPH. In parallel, 10 patients with CTEPH diagnosed before 2005 were detected, and 8 patients were diagnosed after 2005 and outside the study.Altogether 24 patients with CTEPH were thus identified and the cumulative incidence of CTEPH ranged between 0.7 and 1.2%. Conclusion Dyspnea is common after an acute PE and over 40% of them have perfusion defects on a V/Q scan, suggesting the presence of a post PE-syndrome (PPES). The incidence of CTEPH is however low. More research is needed to better define PPES. Funding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Actelion PharamceuticalsSwedish Heart and Lung Foundation

Survival in patients with congenital heart disease and down syndrome

Abstract Background Increasing survivorship among patients with congenital heart disease (CHD) has been reported during the past decades. However, a number of patients with CHD have an associated Down syndrome diagnosis and the long-term prognosis is still debated. Purpose The aim of the present study was to estimate the mortality risk in CHD patients with Down syndrome compared to a matched control population without CHD. Methods We linked data from the Swedish National Patient Register and Cause of Death Register to identify all CHD patients born between January 1970 and December 2017. Then we identified all CHD patients with Down syndrome. Each CHD patient with Down syndrome was matched for sex and birth year with eight controls without CHD or Down syndrome from the Total Population Register. A Cox proportional regression model was used to estimate the mortality risk and Kaplan Meier for survival analysis. Results In total, 3,285 patients with CHD and Down syndrome and 26,128 matched controls were identified. During a mean follow-up of 16.0 (±12.6) years, 558 CHD patients (17%) with Down syndrome, and 198 controls (0.76%) died. CHD patients with Down syndrome had 25.1 times higher risk of mortality (Hazard ratio 25.1, 95%, confidence interval (CI) 21.3–29.5) compared with controls. Patients with the most complex CHD were found to have the highest mortality risk (Hazard ratio 32.3, 95% CI 26.1–39.9). A decrease in risk of mortality during the first year of life was found between the two birth periods, risk of mortality was 46.8 (CI 29.5–74.0) in 1970–1989 and 17.7 (CI 12.8–24.42) 1990–2017. In addition, the mortality risk was two times (95% CI 1.94–2.31) higher for CHD patients with Down syndrome when compared to CHD patients without Down syndrome. Conclusion The risk of mortality among CHD patients with Down syndrome was 25 times higher compared to controls without CHD or Down syndrome. The highest risk of mortality was found in patients with complex CHD. The survivorship rate was exponentially increased in CHD patients with Down syndrome that were born after 1990, determining the modern era of congenital heart care. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Swedish state under an agreement between the Swedish Government and county Councils (ALF)

Sex-related differences in long-term outcome of heart failure in low-risk patients with atrial fibrillation. A Swedish registry case-control study

Abstract Background Knowledge about sex-related differences regarding long-term risk of heart failure (HF) among patients with atrial fibrillation (AF) is limited. Aim To evaluate the impact of sex on risks for new onset HF in patients with AF. Methods All patients from the Swedish National Patient Register, with a first-time diagnosis of AF between 1987 and 2018 were identified and compared with two matched controls without AF from the Total Population Register. Patients &amp;lt;18 years, or any previous cardiovascular disease, diabetes mellitus and renal failure at the baseline were excluded. Results In total 227,811 patients and 452,712 controls were included; 44.5% were women. The mean age (SD) for men was 65.5 (15) vs. 72.7 (13) in women (p&amp;lt;0.0001). The incidence rate for HF onset per 1000 person-years within one and five years after AF diagnosis was 77.3 (75.5–79.1) and 45.0 (44.3–45.7) in women vs. 66.5 (65.0–68.0) and 35.3 (34.8–35.9) in men, respectively. The incidence rate for HF onset increased with age in both patients with AF and controls, but was generally more pronounced in women. Women had 26% and 34% higher risk for HF onset, within five and thirty years, respectively. The highest risk for HF onset was found in women 18–34 years and 35–49 years of age, HR 24.64 (95%, confidence interval (CI) 7.59–80.0) and 8.09 (95%, CI 6.34–10.33) vs. 9.86 (95%, CI 6.81–14.27) and 6.52 (95%, CI 5.87–7.25) in equally old men. The mortality rate after HF was 42.3% and 33.1% in women and men with AF (p&amp;lt;0.0001). Conclusion In this nationwide, register-based cohort study, when compared to matched controls we found that the risk for HF onset was higher in women with AF, particularly in reproductive age, highlighting great importance of further research for prevention of HF in young women with AF but without any other cardiovascular risk factors. Funding Acknowledgement Type of funding sources: None.

Long-term survival in adults with congenital heart disease: a nationwide, register-based cohort study

Abstract Background The survival of children with congenital heart disease has increased exponentially over the last decades, with 97% currently reaching adulthood. However, the total impact of advanced treatment on mortality and morbidity in adult survivors in relation to the general population is less well described. Purpose We aimed to investigate long term outcomes of adults with congenital heart disease (ACHD) in Sweden, from the age of 18 years through middle-age. Methods Data from the Swedish National Patient Register and Cause of Death Register were linked to identify all ACHD patients born from 1950 to 1999 and alive at the age of 18 years. Each ACHD patient was matched by birth year and sex with more than ten controls without congenital heart malformations from the Total Population Register in Sweden, with follow-up time for cases and controls until December 2017. Results Altogether 37,278 ACHD patients and 412,799 matched controls were included in the study. The mean follow-up was 19.2 (± 13.6 years). The risk of death after the age of 18 years was 3.2 times higher (95%, confidence interval (CI) 3.04–3.37) among ACHD patients compared to controls. At the age of 68 years, more than 75% of ACHD patients were still alive. The risk of death was significantly higher also for the more benign lesions i.e. atrial septal defects, ventricular septal defects and coarctation of the aorta. Starting in the mid 1970ies, the risk of death declined over time and for those born after 1975 the risk decreased steadily with each successive birth year. More patients with ACHD developed atrial fibrillation, heart failure, non-fatal myocardial infarction, non-fatal stroke or died in comparison to controls, adjusted hazard ratio for any of these ranged between 20.58 (95%, CI 18.74–22.60) for patients with complex disease and 9.89 (95%, CI 9.53–10.27) for patients with mild or moderate disease. Conclusions In this large, nationwide, register-based cohort study of patients with ACHD surviving until the age of 18 years, the risk of mortality up to the age of 68 years was more than three times higher compared to matched controls. A notable decline in the risk of death was found in patients with ACHD born after 1970. Despite this substantially high relative mortality risk during adulthood, at least 75% of ACHD patients will become sexagenerians. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Swedish state under an agreement between the Swedish Government nad county Councils (ALF)

Long-term incidence of pacemaker implantations and bradycardia among cross-country skiers

Abstract Background Bradycardia is more common among well-trained athletes. The associations of training with pacemaker implantations are less known. We aim to investigate the association of endurance training with incidence of bradycardia and pacemaker implantations in a cohort of endurance athletes. Furthermore, we aim to establish potential sex differences of such associations. Methods Swedish skiers (209,072) that completed 1 or more races in the 30 to 90 km cross-country skiing event Vasaloppet (1989–2011) and a matched sample (n=531,949) of nonskiers were followed until first event of bradycardia or pacemaker implantation. The Swedish National Patient Register of in-patient and specialized outpatient care was used to obtain the date of diagnoses. Cox regression was used to investigate associations of number of completed races and finishing time with incidence of bradycardia and pacemaker implantations. Results Male skiers had higher incidence of bradycardia (hazard ratio [HR], 1.14; 95% CI, 0.99–1.30) and pacemaker implantations (HR, 1.14; 95% CI, 1.02–1.28) than male nonskiers. Male skiers completing most races or with the fastest finishing times had the highest incidence. The incidence of bradycardia (HR, 0.96; 95% CI, 0.70–1.30) and pacemaker implantations (HR, 0.97; 95% CI, 0.75–1.25) among female skiers in Vasaloppet was not different to that of female nonskiers. There was a trend that female skiers completing most races or with fastest finishing times had lower incidence. Conclusions Male skiers had higher incidence of bradycardia and pacemaker implantations. Female skiers in Vasaloppet had non-different incidence of bradycardia and pacemaker implantations. Men with the highest number of races and fastest finishing times had the highest incidence of pacemaker implantations. This indicates that bradycardia associated with training is not always as benign as previously suggested. The response appears to be different between sexes. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): Uppsala UniversityCKF Dalarna