User requirement analysis: online thalassemia management system for Hospital Sultanah Aminah, Malaysia

The care process of patients suffering from thalassemia involves continuous and complex care procedures, which produce a large volume of diverse data. This makes the management of the patient records using paper-based system cumbersome. Information technology (IT) is an essential factor in the administration of healthcare where it can improves health care quality and service. Besides that, it provides more accurate and timely information regarding patient care. Consequently, the adoption of IT tools such as Electronic Medical Records (EMR) in hospitals is a solution to reduce some barriers in the healthcare services. This solution may improve the effectiveness of chronic disease management such as thalassemia, since a lot of the patient’s data will be stored and retrieved due to the frequent monitoring and treatment. In order to facilitate successful adoption of an EMR, involvement of end-users would be essentially required during the designing, implementation and usage phase. In this study, an EMR specifically design for thalassemia patient management is proposed to be implemented in Hospital Sultanah Aminah, Johor, Malaysia. The user requirement analysis (URA) of the proposed system, Online Thalassemia Management System (OTMS), is investigated and discussed in this article

Survival and Associated Risk Factors for Mortality Among Infants with Critical Congenital Heart Disease in a Developing Country

Critical congenital heart disease (CCHD) is associated with significant morbidity and mortality. However, data on survival of CCHD and the risk factors associated with its mortality are limited. This study examined CCHD survival and the risk factors for CCHD mortality. Using a retrospective cohort study of infants born with CCHD from 2006 to 2015, survival over 10 years was estimated using Kaplan–Meier analysis, and the risk factors for mortality were analyzed using multivariate Cox proportional hazards regression. A total of 491 CCHD cases were included in the study, with an overall mortality rate of 34.8% (95% confidence interval [CI] 30.6–39.2). The intervention/surgical mortality rate was 9.8% ≤ 30 days and 11.5% > 30 days after surgery, and 17% died before surgery or intervention. The median age at death was 2.7 months [first quartile: 1 month, third quartile: 7.3 months]. The CCHD survival rate was 90.4% (95% CI 89–91.8%) at 1 month, 69.3% (95% CI 67.2–71.4%) at 1 year, 63.4% (95% CI 61.1–65.7%) at 5 years, and 61.4% (95% CI 58.9–63.9%) at 10 years. Weight of < 2 kg at diagnosis, associated syndromes, poor pre-operative condition, and non-duct-dependent CCHD were independent risk factors for poor survival, with hazard ratios of 2.61, 2.10, 2.22, and 1.70, respectively. CCHD is associated with a high mortality rate. Low weight, poor pre-operative condition, associated syndromes, and non-duct-dependent CCHD are significant risk factors affecting the survival of infants with CCHD

Ten-year trend in prevalence and outcome of Down syndrome with congenital heart disease in a middle-income country

Limited data are available on the survival of patients with Down syndrome and congenital heart disease (CHD) from middle-income countries. This retrospective cohort study was performed to determine the trends in the prevalence and survival of such patients born from January 2006 to December 2015 in Malaysia. Among 754 patients with Down syndrome, 414 (55%) had CHD, and no significant trend was observed during the 10 years. Of these 414 patients, 30% had lesions that closed spontaneously, 35% underwent surgery/intervention, 9% died before surgery/intervention, and 10% were treated with comfort care. The overall mortality rate was 23%, the median age at death was 7.6 months, and no significant changes occurred over time. The early and late post-surgery/intervention mortality rates were 0.7% and 9.0%, respectively. Most deaths were of non-cardiac causes. The overall 1-, 5-, and 10-year survival rates were 85.5%, 74.6%, and 72.9%, respectively. Patients with severe lesions, persistent pulmonary hypertension of the newborn, atrioventricular septal defect, and pulmonary hypertension had low survival at 1 year of age. Conclusion: The prevalence of CHD in patients with Down syndrome is similar between Malaysia and high-income countries. The lower survival rate is attributed to limited expertise and resources which limit timely surgery.What is Known:• The survival of patients with Down syndrome with congenital heart disease (CHD) has improved in high-income countries. However, little is known about the survival of patients with Down syndrome with CHD from middle-income countries.• In the Caucasian population, atrioventricular septal defect is the most common type of CHD associated with Down syndrome.What is New:• In middle-income countries, the prevalence of CHD is the same as in high-income countries, but with a lower survival rate.• In the Asian population, ventricular septal defect is the most common type of CHD in patients with Down syndrome

The birth prevalence, severity, and temporal trends of congenital heart disease in the middle-income country: A population-based study

Objectives: There is limited data on congenital heart disease (CHD) from the lower- and middle-income country. We aim to study the epidemiology of CHD with the specific objective to estimate the birth prevalence, severity, and its trend over time. Design: A population-based study with data retrieved from the Pediatric Cardiology Clinical Information System, a clinical registry of acquired and congenital heart disease for children. Setting: State of Johor, Malaysia. Patients: All children (0-12 years of age) born in the state of Johor between January 2006 and December 2015. Intervention: None. Outcome measure: The birth prevalence, severity, and temporal trend over time. Results: There were 531,904 live births during the study period with 3557 new cases of CHD detected. Therefore, the birth prevalence of CHD was 6.7 per 1000 live births (LB) (95% confidence interval [CI]: 6.5-6.9). Of these, 38% were severe, 15% moderate, and 47% mild lesions. Hence, the birth prevalence of mild, moderate, and severe CHD was 3.2 (95% CI: 3.0-3.3), 0.9 (95% CI: 0.9- 1.1), and 2.6 (95% CI: 2.4-2.7) per 1000 LB, respectively. There was a significant increase in the birth prevalence of CHD, from 5.1/1000 LB in 2006 to 7.8/1000 LB in 2015 (P <.0001) due to increase in detection of both mild (1.9/1000 LB in 2006 to 3.9/1000 LB in 2015, P <.001) and severe CHD (1.8/1000 LB in 2005 to 2.9/1000 LB in 2015, P <.001). Conclusions: The birth prevalence of CHD was 6.7 per 1000 live births, and two in five were severe and significantly associated with syndrome and extracardiac defect. There was a significant increase in the detection of severe lesions in recent years leading to more burden to resources that are already limited in the middle-income country. Therefore, strategic and comprehensive pediatric and congenital heart surgery program is required