Setting and Design: Retrospective observational cohort study in the State of Johor, Malaysia.
Subjects and Methods: All infants born between January 2006 and December 2015 with a diagnosis of CCHD, defined as infants with duct-dependent lesions or cyanotic heart disease who may die without early intervention. The late diagnosis was defined as a diagnosis of CCHD after 3 days of age.
Results: Congenital heart disease was diagnosed in 3557 of 531,904 live-born infants and were critical in 668 (18.7%). Of 668, 347 (52%) had duct-dependent pulmonary circulation. The birth prevalence of CCHD was 1.26 (95% confidence interval: 1.16-1.35) per 1000 live births, with no significant increase over time. The median age of diagnosis was 4 days (Q1 1, Q3 26), with 61 (9.1%) detected prenatally, and 342 (51.2%) detected late. The highest rate of late diagnosis was observed in coarctation of the aorta with a rate of 74%. Trend analysis shows a statistically significant reduction of late diagnosis and a significant increase in prenatal detection. However, Cox regression analysis shows the timing of diagnosis does not affect the outcome of CCHD.
Conclusions: Due to limited resources in the MIC, the late diagnosis of CCHD is high but does not affect the outcome. Nevertheless, the timing of diagnosis has improved over time.
METHODS: A cross sectional study by adopting European Quality of Life scale (EQ-5D) for the assessment of HRQoL was conducted. All registered HB patients attending two public hospitals in Quetta, Pakistan were approached for study. Descriptive statistics were used to describe demographic and disease related characteristics of the patients. HRQoL was scored using values adapted from the United Kingdom general population survey. EQ-5D scale scores were compared with Mann-Whitney and Kruskal-Wallis test. Standard multiple regression analysis was performed to identify predictors of HRQoL. All analyses were performed using SPSS v 16.0.
RESULTS: Three hundred and ninety HB patients were enrolled in the study. Majority of the participants (n = 126, 32.3%) were categorized in the age group of 18-27 years (36.07 ± 9.23). HRQoL was measured as poor in the current study patients (0.3498 ± 0.31785). The multivariate analysis revealed a significant model (F(10, 380) = 40.04, P
METHODS: Parental-proxy scores of the Pediatric Quality of Life Inventory 4.0 core scales were obtained for 179 children with CHD and 172 siblings. Intra-class coefficients were derived to determine the levels of proxy-child agreement in 66 children aged 8-18 years. Multiple regression analysis was used to determine factors that impacted Pediatric Quality of Life Inventory scores.
RESULTS: Proxy scores were lower in children with CHD than siblings for all scales except physical health. Maximum differences were noted in children aged 5-7 years, whereas there were no significant differences in the 2-4 and 13-18 years age groups. Good levels of proxy-child agreement were found in children aged 8-12 years for total, psychosocial health, social, and school functioning scales (correlation coefficients 0.7-0.8). In children aged 13-18 years, the level of agreement was poor to fair for emotional and social functioning. The need for future surgery and severity of symptoms were associated with lower scores.
CONCLUSION: Differences in proxy perception of quality of life appear to be age related. The level of proxy-child agreement was higher compared with other reported studies, with lower levels of agreement in teenagers. Facilitating access to surgery and optimising control of symptoms may improve quality of life in this group of children.