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Aims: To determine whether atrial-based pacing prevents atrial arrhythmias in adults with congenital heart disease (CHD) compared with ventricular pacing., Methods and Results: All adult CHD patients from four participating centres with a permanent pacemaker were identified. Patients with permanent atrial arrhythmias at pacemaker implantation and patients who received a pacemaker for treatment of drug-refractory atrial arrhythmias were excluded. The final study population consisted of 211 patients (52% male, 36% complex CHD) who received a first pacemaker for sick sinus dysfunction (n = 82) or atrioventricular block (n = 129) at a median age of 24 years [interquartile range (IQR), 12-34]. A history of atrial arrhythmias at implantation was present in 49 patients (23%). Atrial-based pacing was the initial pacing mode in 139 patients (66%) while the others (34%) received ventricular pacing. During a median follow-up of 13 years (IQR, 7-21), 90 patients (43%) developed an atrial arrhythmia. Multivariate analysis demonstrated no significant effect of atrial-based pacing on subsequent atrial arrhythmias [hazard ratio (HR), 1.53; 95% confidence interval (CI), 0.91-2.56; P = 0.1]. Independent predictors of atrial arrhythmia were history of atrial arrhythmias (HR, 5.55; 95% CI, 3.47-8.89; P< 0.0001), older age (≥18 years) at pacemaker implantation (HR, 2.29; 95% CI, 1.29-4.04; P = 0.005), and complex CHD (HR, 1.57; 95% CI, 1.01-2.45; P = 0.04)., Conclusion: In contrast to the general population, atrial-based pacing was not associated with a lower incidence of atrial arrhythmia in adults with CHD.

Objective: To investigate the psychosocial impact of having an implantable cardioverter defibrillator (ICD) in adults with Tetralogy of Fallot (ToF)., Methods: Included were 26 ToF-patients with an ICD (age 44 ± 12 years), and two control groups consisting of 28 ToF-patients without an ICD (age 40 ± 10 years) and a group of 35 ICD-patients of older age without ToF (age 72.0 ± 8 years). This last control group was chosen to represent the "older general ICD population" with acquired heart disease seen at the out-patient clinic. Psychosocial functioning encompassed daily functioning, subjective health status, quality of life, anxiety, depression, coping and social support., Results: ToF-patients with ICD showed diminished psychosocial functioning in comparison to ToF-patients without ICD. This was reflected by diminished subjectively perceived physical functioning (p = 0.01), general health perception (p < 0.01) and a lower satisfaction with life (p = 0.02). In comparison to older ICD-patients, ToF-patients with ICD showed less satisfaction with life (p = 0.03), experienced more anxiety (p = 0.01) and showed less favourable coping styles, although physical functioning was better for ToF-patients with ICD than for older ICD-patients (p = 0.01). More inappropriate shocks were found in ToF-patients with ICD compared to the older ICD-patients., Conclusion: In patients with ToF, ICD implantation had a major impact on psychosocial functioning which should be taken into account when considering ICD implantation in these young patients. To help improve psychosocial functioning, psychological counselling attuned to the specific needs of these patients may be useful.

Objective: To compare the risks of complications during pregnancy in women with repaired and unrepaired atrial septal defects (ASDs) without associated complex cardiac lesions., Design: A retrospective multicentre study., Setting: Tertiary centres in the Netherlands and Belgium., Population: Women with ASD without associated complex cardiac lesions., Methods: Women were identified using two congenital heart disease registries. One hundred women were identified who had 243 pregnancies, including 49 miscarriages and six terminations of pregnancy. Detailed information on each completed pregnancy (n = 188; unrepaired ASD, n = 133; repaired ASD, n = 55) was obtained using medical records and telephone interviews. In addition, data from the Generation R database (a prospective cohort study; n = 9667) were used to determine the background risk (control group)., Main Outcome Measures: Adjusted odds ratios (AORs) for cardiac, obstetric and neonatal events controlled for multiple pregnancies per woman using general estimating equation analysis., Results: Women with an unrepaired ASD had a higher risk of neonatal events (AOR = 2.99, 95% confidence interval [CI] 1.14-7.89, P = 0.027) than women with a repaired ASD. The risk of cardiac and obstetric complications was comparable between women with unrepaired and repaired ASDs. Compared with the general population, women with an unrepaired ASD had higher risks of pre-eclampsia (AOR = 3.54, 95% CI 1.26-9.98, P = 0.017), small-for-gestational-age births (AOR = 1.95, 95% CI 1.15-3.30, P = 0.013) and fetal mortality (AOR = 5.55, 95% CI 1.77-17.4, P = 0.003). By contrast, no differences were observed when comparing women with a repaired ASD versus controls., Conclusions: Women with an unrepaired ASD are at increased risk of neonatal events in comparison with women with a repaired ASD. Compared with the general population, women with an unrepaired ASD are at increased risk of pre-eclampsia, small-for-gestational-age births and fetal mortality.