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Background: As more women with congenital heart disease (CHD) are reaching childbearing age, it becomes more common for their symptoms to be evaluated during pregnancy. However, pregnancy-related symptoms are similar to those caused by heart disease. This study investigated the prevalence of factors associated with symptoms during pregnancy in women with CHD. Methods: The national birth register was searched for primiparous women with CHD who were registered in the national quality register for patients with CHD. Results: Symptoms during the third trimester were reported in 104 of 465 evaluated women. The most common symptom was palpitations followed by dyspnea. Factors associated with symptoms were tested in a univariable model; higher NYHA classification (>1) (OR 11.3, 95%CI 5.5-23.2), low physical activity (≤3 h/week) (OR 2.1 95%CI 1.3-3.6) and educational level ≤ 12 years (OR 1.9 95%CI 1.2-3.0) were associated with having symptoms. In multivariable analysis, low physical activity level (OR 2.4 95%CI 1.2-5.0) and higher NYHA class (OR 11.3 95%CI 5.0-25.6) remained associated with symptoms during pregnancy. There were no cases with new onset of impaired systemic ventricular function during pregnancy. Conclusion: Symptoms during pregnancy are common in women with CHD but are often already present before pregnancy. Because ordinary symptoms during pregnancy often overlap with symptoms of heart disease, it is important to know if symptoms were present before pregnancy and if they became worse during pregnancy. These results should be included in pre-pregnancy counselling and considered in the monitoring during pregnancy.

Background. Poor maternal self-rated health in healthy women is associated with adverse neonatal outcomes, but knowledge about self-rated health in pregnant women with congenital heart disease (CHD) is sparse. This study, therefore, investigated self-rated health before, during, and after pregnancy in women with CHD and factors associated with poor self-rated health. Methods. The Swedish national registers for CHD and pregnancy were merged and searched for primiparous women with data on self-rated health; 600 primiparous women with CHD and 3062 women in matched controls. Analysis was performed using descriptive statistics, chi-square test and logistic regression. Results. Women with CHD equally often rated their health as poor as the controls before (15.5% vs. 15.8%, p  = .88), during (29.8% vs. 26.8% p  = .13), and after pregnancy (18.8% vs. 17.6% p  = .46). None of the factors related to heart disease were associated with poor self-rated health. Instead, factors associated with poor self-rated health during pregnancy in women with CHD were ≤12 years of education (OR 1.7, 95%CI 1.2-2.4) and self-reported history of psychiatric illness (OR 12.6, 95%CI 1.4-3.4). After pregnancy, solely self-reported history of psychiatric illness (OR 5.2, 95%CI 1.1-3.0) was associated with poor self-rated health. Conclusion. Women with CHD reported poor self-rated health comparable to controls before, during, and after pregnancy, and factors related to heart disease were not associated with poor self-rated health. Knowledge about self-rated health may guide professionals in reproductive counselling for women with CHD. Further research is required on how pregnancy affects self-rated health for the group in a long-term perspective.

Introduction. Congenital heart disease (CHD) is the most common type of birth defect today. The adult congenital heart disease (ACHD) population is constantly growing and becoming older and more patients require cardiac surgery. The objective of this study was to review the surgical outcome of the open heart procedures performed on ACHD patients in the last 10 years at Sahlgrenska University Hospital (SUH) through a retrospective descriptive cohort study. Methods. A retrospective data collection was performed for 421 patients who underwent a total of 439 surgical procedures between 2009 and 2018 at the Cardiothoracic department in SUH. The primary outcomes were early (<30 days) and late survival. Secondary outcomes were postoperative complications and independent risk factors for postoperative complications. Results. 30-day mortality was 1.9%. Long-term survival after 3, 5 and 10 years were 96% ± 1, 94.3% ± 1.3 and 92.4% ± 1.8. 82 major complications occurred after 46 procedures (11.6%). The most common major complication was re-exploration due to hemorrhage. Risk factors for major complications were acute surgery and prolonged extracorporeal circulation time. 173 minor complications occurred after 90 procedures (22.5%). The most common minor complication was prolonged intensive care unit stay (>48 h). Conclusion. This study presents satisfactory early and midterm survival. The survival and frequency of major postoperative complications are well in line with what other studies have presented. Patients undergoing resternotomies had no increased risk for mortality or postoperative complications.

Objectives: Implantable cardiac devices are common in patients with tetralogy of Fallot (ToF) (18.3-21.3%) according to previous reports from large centres. We conducted this study to investigate the prevalence and incidence of cardiac devices in a less selected population of patients with ToF and assess factors other than arrhythmia associated with having a device. Design: 530 adult (≥18 years) patients with repaired ToF were identified in the national registry of congenital heart disease (SWEDCON) and matched with data from the Swedish pacemaker registry. Patients with implantable cardiac devices were compared with patients without devices. Results : Seventy-five patients (14.2%) had a device; 51 (9.6%) had a pacemaker and 24 (4.5%) had an implantable cardioverter defibrillator. The incidence in adult age (≥18 years) was 5.9/1000 patient years. Estimated device free survival was 97.5% at twenty, 87.2% at forty and 63.5% at sixty years of age. Compared with previous studies, the prevalence of devices was lower, especially for ICD. In multivariate logistic regression, cardiovascular medication (odds ratio [OR] 3.5, 95% confidence interval [CI] 1.8-6.8), impaired left ventricular function, (OR 2.6, 95%CI 1.3-5.0) and age (OR 1.02, 95%CI 1.002-1.05) were associated with having a device. Conclusion : The prevalence of devices in our population, representing a multicenter register cohort, was lower than previously reported, especially regarding ICD. This can be due to differences in treatment traditions with regard to ICD in this population, but it may also be that previous studies have reported selected patients with more severe disease.

Objective: Congenitally corrected and surgical atrial redirected transposition of the great arteries (TGA) represents states where the morphological right ventricle serves as a systemic ventricle (S-RV). The S-RV is prone to failure, but data on medical treatment on this problem is limited. The purpose of this study was to evaluate the survival in adults with S-RV, with or without heart failure treatment. Design: The SWEDCON registry was used to collect data. All adults with S-RV and minimum follow-up of 1 year were included retrospectively. Medical treatment was defined as taking beta-blockers and/or ACE inhibitors and/or ARBs for more than 50% of the time. Results: We identified 343 patients with S-RV (median age: 21 years). Surgical atrial redirected TGA was present in 58% and congenitally corrected TGA in 42% of patients. The medically treated group ( n  = 126) had higher rates of impaired S-RV function, use of diuretics, pacemaker and higher NYHA functional class at baseline compared to controls. The proportion of patients with impaired functional class did not change over time in the medically treated group, but increased in controls (21% vs. 30%, p  = .015). In Kaplan-Meier analysis, the mean follow-up was 10.3 years, no difference in survival was seen between the groups. Conclusions: Medical treatment may be beneficial in patients with S-RV and impaired functional class and appears to be safe in the long term. The treatment group had equal survival to controls, despite worse baseline characteristics, which might be a result of slower progression of disease in this group.

Objectives: To evaluate the incidence of atrial fibrillation (AF) as well as the value of thumb electrocardiography (ECG) for identification of paroxysmal AF in a Swedish cohort of middle-aged men from the general population., Design: A population based random cohort of 798 men underwent screening at the age of 50 and re-examined at the age of 60 and 71 years. At the last examination, a thumb ECG recording was conducted for 2 weeks twice a day in 479 men from the original cohort. Registered hospital AF diagnoses were retrieved from the Swedish Patient Registry from 1993 to 2014., Results: During a 21-year follow-up, 77 men (9.6%) were diagnosed with AF; of these men, 49.4% (38 of 77) had permanent AF. Fifteen of 479 (3.1%) patients had paroxysmal AF. Of those, seven had been previously diagnosed with paroxysmal AF through Patient Registry. The incidence of AF increased from 2.2 per 1000 years at risk at the age of 50-54 years to 9.3 per 1000 years at risk at the age 65-70 years. The prevalence of AF at the age of 71 years was increased from 7.1% to 9.9% using thumb ECG., Conclusions: In addition to medical history, patient register and 12-lead -ECG, the use of thumb ECG increased the number of detected paroxysmal AF by 21%.

Background: Reconstruction of the right ventricular outflow tract with a conduit is an established surgical procedure in congenital heart disease and reinterventions are common., Objective: An increasing number of patients have a conduit, but there are few population-based studies of long-term outcomes after conduit surgery, reoperations, and transcatheter pulmonary valve replacement., Methods: In April 2015, all adult patients with a conduit were identified in the Swedish National Registry for Congenital Heart Disease (SWEDCON). Data on patients who died before age of 16 years are not included in the registry and thus not included in the study., Results: We found 574 patients with a mean age 36.1 years. The largest proportion had tetralogy of Fallot (45%). In total there were 762 operations and 50 transcatheter pulmonary valve replacements. Mean age at first conduit operation was 20.2 years. Long-term survival up to 48 years including perioperative mortality (<1%) was 93% at 20 years. The most common cause of death was cardiac-related. Higher age at first conduit operation was associated with increased mortality risk. Reintervention-free survival was 77% and 54% at 10 and 20 years, respectively. Conduit reinterventions were common. Ten-year reintervention-free survival after first conduit reintervention (n = 176) was significantly lower than after first conduit operation (70% vs 77% p = .04). Higher age at first conduit operation was associated with a reduced risk of reintervention, whereas male sex and complex malformations were associated with increased risk of reintervention., Conclusions: The mortality of repeated conduit reinterventions is low. The need for reintervention of conduits is considerable, and reintervention-free survival after the first conduit reintervention is poorer than after first conduit implantation. The findings in this study only applies for patients reaching 16 years of age.

Objective: The use of a conduit is an established surgical method for reconstruction of the right ventricular outflow tract in congenital heart disease; however, its limited durability makes reintervention almost inevitable. We studied the trends in new implantation, reoperation, and transcatheter pulmonary valve replacement (TPVR) from a Swedish national perspective., Design and Results: The Swedish registry of congenital heart disease (SWEDCON) was used to collect data. From 2000-2014, there was an increase in adult patients with conduits from 122 to 536. There were 60 surgical conduit replacements, 40 TPVRs and 176 new conduit implantations in the study period. Perioperative mortality was <1%. The yearly number of new implantations and reoperations both doubled over the study period. Patients with new implantations were older (mean age, 36 years) compared with the reoperation and TPVR groups (mean age, 26 years) with the majority of patients having tetralogy of Fallot. The majority of conduit reinterventions were surgical also after the introduction of TPVR in 2007, with no significant difference regarding diagnosis, gender, age, or previous number or longevity of conduits., Conclusion: The number of adults with conduits increased steadily with most conduit-related operations being new implantations in patients with tetralogy of Fallot. Surgical conduit replacements increased significantly and represented the majority of conduit reinterventions after the introduction of TPVR, indicating that TPVR is not used for the majority of patients with conduit failure. Diagnosis, gender, age, or previous number or longevity of conduits had no impact on choosing surgical replacement vs. TPVR.

Introduction: Participation in clinical trials by patients with cardiovascular disease is paramount to the development of new treatments. Capturing and keeping patients in the trials until the end is very important and trials are often of long duration and include patients in a clinically stable condition, with few symptoms and a low risk of recurrent disease. We investigated what motivates patients to participate in long-term cardiovascular trials. Increased knowledge may enhance inclusion and retention and minimize lost to follow-up or withdrawal of consent., Materials and Methods: A questionnaire with 11 statements to elucidate the reasons for participation and retention in long-term clinical trials was used and replies from 135 participants in trials, 78% men, mean age was 68 years., Results: The two most important reasons for participation were: "I am able to see the same doctor and nurse at the visits", indicated by 89 patients (66%), followed by "I want to promote science", which was indicated by 74 patients (55%). The least important reason was "The visits are free of cost"., Conclusion: Patients who participate in cardiovascular clinical trials do so because it may provide access to more continuous care but equally important are altruistic motives including a wish to promote science.

Objectives: To examine causes of death for men above 80 years of age, and health status in centenarians in a cohort of men followed from age 50 years. Factors of importance for survival were studied., Design: A representative sample of men born in 1913 was first examined in 1963 and re-examined at ages 54, 60, 67, 75, 80 and 100 years., Results: Of 973 selected men, 855 (88%) were examined at age 50, and 10 were alive at age 100.Twenty-seven percent lived until 80 years. Cardiovascular disease was the most common cause of death after this age. Dementia was recorded in two of ten men at age 100. Long survival was related to the mothers' high age at death, to non-smoking, high social class at age 50 and high maximum working capacity at age 54 years. At age 100, the seven examined men had low/normal blood pressure. Serum values of troponin T, N-terminal pro-brain natriuretic peptides and C-reactive protein were elevated, but echocardiographic findings were normal., Conclusions: Ten men experienced their 100th birthday. Survival was related to non-smoking, mothers' high age at death, high social class and previous high physical working capacity. Age-adjusted reference levels for laboratory tests are needed for centenarians.

Objectives: With improving prognosis the prevalence of adult congenital heart disease (ACHD) is increasing. Patients with type 2 diabetes mellitus (T2DM) have a shorter life expectancy compared with the general population. We investigated, in a large national diabetes registry, the prevalence of ACHD in combination with T2DM to estimate the associated clinical risk, outcome and patient characteristics., Design: Data from the Swedish National Diabetes Register (NDR) were linked with the Swedish National Patient Register (NPR) and the Cause of Death Register., Results: 833 ACHD patients were matched with 5 controls each. ACHD patients had significantly lower body mass index or BMI, higher creatinine and were more sedentary as compared with patients with T2DM but without congenital heart disease. The overall mortality was 26.2% for ACHD patients as compared with 19.9% (P < 0.001) for the control group, and five-year mortality rates were 5.2 versus 3.4%, P = 0.014., Conclusions: Congenital heart disease and secondary risk factors for cardiovascular disease frequently coexist and the development of T2DM also in the ACHD population is not uncommon with an estimated prevalence of between 4 and 8%. Treatment of conventional cardiovascular risk factors in patients with congenital heart disease could be considered secondary prevention given the relatively high morbidity and high risk for mortality observed in patients with the combination of ACHD and T2DM.

Objectives: The presence of a patent foramen ovale (PFO) has been associated with recurrent cryptogenic cerebrovascular event (CVE). The BioSTAR is a partly biodegradeable atrial septal repair implant. We investigated the feasibility and efficacy of the BioSTAR PFO-occluder., Design: From October 2007 to December 2008, 59 consecutive patients underwent PFO closure at our institution with a history of at least one cryptogenic CVE defined by a neurologist. During the study period, all patients, who fulfilled our institutional criteria for PFO closure, were included. No patients were lost to follow-up., Results: Of the 59 patients treated, a BioSTAR device could be implanted in 30 and in 29 patients another device, almost exclusively an Amplatzer, had to be used. No serious complications were observed during implantation of either. Four of 30 patients suffered a recurrent CVE after BioSTAR implantation as compared to 2/29 in the comparison group. At long-term follow-up 29/30 patients in the BioSTAR group had complete closure of their PFO as compared to 23 of 29 in the comparison group., Conclusions: The BioSTAR device could be selected for use in small shunts less than 10 mm while the Amplatzer may be chosen for larger defects or more complicated anatomy.

Objective: The aims of this study were to investigate the prognostic influence of diabetes after an episode of unstable angina pectoris or non-Q-wave myocardial infarction (MI) and to investigate whether diabetes is independently associated with increased short- and long-term mortality risk following these episodes., Design: Consecutive patients with a diagnosis of unstable angina pectoris or non-Q-wave MI, admitted to the Coronary Care Unit at Ostra Hospital, Göteborg, Sweden during 1988-1998 were included. The primary endpoint was 2-year mortality collected from the Swedish cause-specific mortality register., Results: The study included 4341 patients, 722 (17%) had diabetes. Diabetes was associated with increased mortality during initial hospitalization (10.2% vs 5.7%, p < 0.0001), after 30 days (13% vs 7.5%, p < 0.0001), and at 2 years (33.7% vs 20.2%, p < 0.0001). After adjustment for potentially confounding factors, diabetes remained an independent predictor of 2-year mortality following unstable coronary syndromes, the hazard ratio (HR) of death (HR = 1.6; 95% CI 1.4-1.9)., Conclusions: Among patients with unstable coronary syndromes, diabetes is an independent risk factor associated with increased mortality during hospitalization, short- and long-term follow-up.

Continuous ST-segment monitoring by either continuous 12-lead ECG or continuous vectorcardiography provides reliable information regarding ST changes in patients with ongoing myocardial ischemia with or without concurrent chest pain. ST-segment monitoring enables the clinician to continuously follow the dynamic changes that characterize unstable angina and acute myocardial infarction syndromes. It provides important information for risk stratification in unstable coronary syndromes and helps in differentiating between extra-cardiac chest pain and acute coronary disease. The use of VECG for detection of perioperative myocardial damage is promising but much work still needs to be done to clarify the prognostic and clinical value of VECG in this setting.

Objective: To study early diagnosis, treatment and outcome in patients with bundle branch block and clinically suspected acute myocardial infarction., Design: A prospective multicenter study including 14 Swedish coronary care units. The study included 257 consecutive patients with bundle branch block and clinical suspicion of acute myocardial infarction., Results: Left bundle branch block was present in 62% of patients and right bundle branch block in 38%. Thrombolytic treatment of acute myocardial infarction in the left and right bundle branch block was 16% and 36%, respectively. Of those undergoing thrombolytic therapy, 20% of patients with left and 13% with right bundle branch block did not develop an acute myocardial infarction. Patients with left bundle branch block had higher mortality rates than those with right bundle branch block. After one year there was no difference in mortality rates between patients with and those without acute myocardial infarction on admission., Conclusion: Patients with bundle branch block and suspected acute myocardial infarction receive suboptimal treatment. Thus better diagnostic regimes are needed to identify those patients with bundle branch block and acute myocardial infarction who are suitable for thrombolytic treatment.

Standard 12-lead electrocardiogram (ECG) criteria were evaluated and compared with dynamic vectorcardiography for diagnosing acute myocardial infarction in 33 patients with chronic left bundle-branch block. In 14 patients a clinical diagnosis of acute myocardial infarction was made, but it was found that none of the seven most promising ECG criteria suggested in the literature could alone or in combination diagnose acute myocardial infarction. QRS vector difference evolution showed the same kind of pattern as that for patients with narrow QRS-complex. By using a predefined specific pattern, a diagnostic accuracy of 79% was achieved. The results indicate that dynamic vectorcardiography is a better tool for diagnosing and monitoring acute myocardial infarction in patients with left bundle-branch block than standard 12-lead ECGs taken on admission and after 12-24 h.

Reports on gender differences in the management of acute coronary syndromes indicate that women do not receive as much active treatment as men. Other conflicting findings have also been published. To investigate whether previously reported gender differences in the treatment of acute myocardial infarction (AMI) still persist, we included all patients admitted to our coronary care unit (CCU) in 1984-1995, and discharged with a diagnosis of AMI, in a retrospective study. A total of 1991 female admissions was compared with 4067 male admissions. The time-span was divided into two-year periods comprising approximately 1000 patients each. During one period, 1988 to 1989, women received significantly less thrombolytic therapy which, however, could reflect that women admitted with AMI were older than men. Analysis of time-trends showed a significant increase in the use of thrombolytic treatment in women and elderly men. In clinical praxis there has been a gender-gap in acute treatment of AMI, but age-dependent or not, this is now no longer apparent.