Three risk estimation methods for predicting the cardiac outcomes of pregnancy in women with heart disease have been proposed. This study was designed to compare their prediction performance in an Asian cohort with congenital heart disease (CHD). This study enrolled pregnant women with CHD who delivered their babies after the 20th gestational week between 1985 and 2011. Of 268 pregnancies in 190 women with CHD, 18 (6.7%) had cardiac complications. The incidence of maternal cardiac events among women with a CARPREG index of 0, 1 or 2 was 3.4%, 27.3% and 100%. The incidence was 2.7%, 8.6%, 11.1%, 40% and 17.6% for those with a ZAHARA score 0-0.5, 0.51-1.5, 1.51-2.5, 2.51-3.5 and >3.5. Among patients with a modified World Health Organization (WHO) classification I, II, III and IV, the incidence of maternal cardiac events was 0%, 4.0%, 12.2% and 25.7%. The c-statistic was 0.732 (95% confidence interval (CI): 0.589, 0.876; P<0.001) for the CARPREG score, 0.737 (95% CI: 0.611, 0.864; P=0.001) for the ZAHARA score and 0.827 (95% CI: 0.745, 0.909; P<0.001) for the WHO classification. All 3 risk estimation methods had good performance in predicting maternal cardiac outcomes; however, the modified WHO classification demonstrated superior discrimination and calibration.

Objectives To assess the quality of life (QoL) in adults with congenital heart disease (ACHD) and to explore the sex-related differences and biopsychosocial determinants in an Asian cohort. Design Prospective cross-sectional clinical study. Setting One tertiary medical centre in Taiwan. Patients and methods The QoL of ACHD (≥20 years) was investigated using the Taiwanese version of the QoL questionnaire designed by the WHO, which assesses four domains of QoL (physical, psychological, social and environmental). Personality, psychological distress and family support were assessed using the Maudsley Personality Inventory, Brief Symptom Rating Scale, and the Family APGAR score, respectively. Results A total of 289 patients (age 33.2±10.6 years; 36% men) were studied. ACHD women had significantly lower QoL scores in the physical and psychological domains compared to the age-matched general population, whereas no differences were observed between ACHD men and the general population. Multivariate analysis showed that female gender was associated with poorer physical QoL; the sex difference in the psychological QoL was mediated by psychological distress. Interaction analysis showed that the effect of family support on the psychological domain of QoL may be different by sex. The determinants of QoL varied between different domains. Extroversion personality trait, psychological distress and family support were common determinants of most domains of QoL. Conclusions In ACHD, female gender was associated with poor physical and psychological QoL. The common denominators for QoL were primarily personality trait, psychological distress and family support, but not disease severity.

A number of randomized trials are underway, which will address the effects of angiotensin receptor blockers (ARBs) on aortic root enlargement and a range of other end points in patients with Marfan syndrome. If individual participant data from these trials were to be combined, a meta-analysis of the resulting data, totaling approximately 2,300 patients, would allow estimation across a number of trials of the treatment effects both of ARB therapy and of β-blockade. Such an analysis would also allow estimation of treatment effects in particular subgroups of patients on a range of end points of interest and would allow a more powerful estimate of the effects of these treatments on a composite end point of several clinical outcomes than would be available from any individual trial. A prospective, collaborative meta-analysis based on individual patient data from all randomized trials in Marfan syndrome of (i) ARBs versus placebo (or open-label control) and (ii) ARBs versus β-blockers will be performed. A prospective study design, in which the principal hypotheses, trial eligibility criteria, analyses, and methods are specified in advance of the unblinding of the component trials, will help to limit bias owing to data-dependent emphasis on the results of particular trials. The use of individual patient data will allow for analysis of the effects of ARBs in particular patient subgroups and for time-to-event analysis for clinical outcomes. The meta-analysis protocol summarized in this report was written on behalf of the Marfan Treatment Trialists' Collaboration and finalized in late 2012, without foreknowledge of the results of any component trial, and will be made available online (http://www.ctsu.ox.ac.uk/research/meta-trials).