METHODS: This population-based cohort study included all children with CHD registered in the Pediatric Cardiology Clinical Information System born between 2006 and 2020 in Johor, Malaysia. The mortality rate was calculated, and Cox proportional hazard regression analysis was used to determine factors associated with mortality. The Kaplan-Meier analysis was used to estimate the survival rates at 1, 5, 10 and 15 years.
RESULTS: There were 5728 patients with CHD studied, with 1543 (27%) lesions resolved spontaneously, 322 (5.6%) were treated with comfort care, 1189 (21%) required no intervention, and 2674 (47%) needed surgery or intervention. The overall mortality rate was 15%, with a median age of death of 3.7 months (IQR 0.9-9.8 months). Preoperative/intervention death was observed in 300 (11%), and 68 (3.2%) children died within 30 days of surgery or intervention. The overall estimated survival at 1, 5, 10 and 15 years was 88%, 85%, 84% and 83%, respectively. The independent factors associated with mortality were male gender, associated syndrome or extra-cardiac defect, pulmonary hypertension, antenatal diagnosis and severe lesions.
CONCLUSIONS: Eight out of 10 patients with CHDs survived up to 15 years of age. However, 10% of CHDs who require intervention die before the procedure. Thus, improving congenital cardiac surgery and enhancing the overall healthcare system are crucial to improve survival.
METHODS: Between October 2013, when our service of paediatric cardiac surgery was opened, and December 2014, 24 patients were referred for surgical closure of VSDs. Among them, 17 patients (71%), with the median age of 6 years, ranging from 2 to 9 years, and with a median body weight of 19 kg, ranging from 11 to 56 kg, underwent surgical repair for doubly committed defects. In terms of size, the defect was considered moderate in 4 and large in 13. Aortic valvular regurgitation (AoVR) was present in 11 patients (65%) preoperatively, with associated malformations found in 14 (82%), with 5 patients (29%) having two or more associated defects.
RESULTS: After surgery, there was trivial residual shunting in 2 patients (12%). AoVR persisted in 6 (35%), reducing to trivial in 5 (29%) and mild in 1 (6%). Mean stays in the intensive care unit and hospital were 2.6 ± 1.2 days, ranging from 2 to 7 days, and 6.8 ± 0.8 days, ranging from 6 to 9 days, respectively. The mean follow-up was 14 ± 4 months, ranging from 6 to 20 months, with no early or late deaths and without clinical deterioration.
CONCLUSIONS: The incidence of doubly committed lesions is high in our experience, frequently associated with AoVR and other associated malformation. Early detection is crucial to prevent further progression of the disease. Patch closure remains the gold standard in management, not least since it allows simultaneous repair of associated intracardiac defects.
OBJECTIVES: To determine the efficacy of desmopressin acetate in preventing and treating acute bleeds during pregnancy in women with congenital bleeding disorders.
SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coaguopathies Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant and abstract books of conferences proceedings. We also searched for any randomised controlled trials in a registry of ongoing trials and the reference lists of relevant articles and reviews.Date of most recent search: 18 June 2015.
SELECTION CRITERIA: Randomised and quasi-randomised controlled trials investigating the efficacy of desmopressin acetate versus tranexamic acid or factor VIII or rFactor VII or fresh frozen plasma in preventing and treating congenital bleeding disorders during pregnancy were eligible.
DATA COLLECTION AND ANALYSIS: No trials matching the selection criteria were eligible for inclusion.
MAIN RESULTS: No trials matching the selection criteria were eligible for inclusion.
AUTHORS' CONCLUSIONS: The review did not identify any randomised controlled trials investigating the relative effectiveness of desmopressin acetate for bleeding during pregnancy in women with congenital bleeding disorders. In the absence of high quality evidence, clinicians need to use their clinical judgement and lower level evidence (e.g. from observational trials) to decide whether or not to treat women with congenital bleeding disorders with desmopressin acetate.Given the ethical considerations, future randomised controlled trials are unlikely. However, other high quality controlled studies (such as risk allocation designs, sequential design, parallel cohort design) to investigate the risks and benefits of using desmopressin acetate in this population are needed.
METHODS: A total of 8 and 7 children who had gradual GGS and ACO correction, respectively, for angular deformities due to rickets from 2002 to 2022 were recalled for follow-up. Demographic data, types of rickets, data on pharmacological treatment, biochemical parameters, recurrence of angular deformity and postoperative complications were obtained from the medical records. A radiographic evaluation of the leg was performed to determine the tibiofemoral angle. For functional evaluation, the Active Scale for Kids (ASK) and Lower Extremity Functional Scale (LEFS) instruments were used for children below and above 15 years old, respectively.
RESULTS: In terms of the tibiofemoral angle, the GGS group documented greater angle changes compared to the ACO group, but the difference was not significant. In terms of functional outcomes, the overall score percentage of both groups was comparable with the GGS group showing a trend of higher score percentage compared to the ACO group. The GGS group presented no complication while 2 neurovascular injuries and 1 implant failure were recorded in the ACO group.
CONCLUSION: Both GGS and ACO procedures resulted in similar radiographic and functional outcomes for the treatment of rickets in children. GGS may be advantageous in terms of reducing complications of surgery. Nevertheless, the choice of surgical intervention should be made based on the patient's circumstances and the surgeon's preference.
METHODS: We included 33 case-mother dyads and 2 mother-only (child deceased) cases of CHA in a case-only study. Ten genes important in determining fetal exposure to serotonin reuptake inhibitors were examined: CYP1A2, CYP2C9, CYP2C19, CYP2D6, ABCB1, SLC6A4, HTR1A, HTR1B, HTR2A and HTR3B.
RESULTS: Among the exposed cases, polymorphisms that tended to be associated with an increased risk of CHA were SLC6A4 5-HTTLPR and 5-HTTVNTR, HTR1A rs1364043, HTR1B rs6296 and rs6298 and HTR3B rs1176744, but none reached statistical significance due to our limited sample sizes.
CONCLUSION: We identified several polymorphisms that might potentially affect the risk of CHA among exposed fetuses, which warrants further investigation.
DESIGN: Retrospective cohort analysis of children who underwent a systemic-to-pulmonary shunt after excluding those who had it for Norwood or Damus-Kaye-Stansel procedure.
SETTING: The Royal Children's Hospital, Melbourne, VIC, Australia.
PATIENTS: From 2008 to 2015, 201 children who had a systemic-to-pulmonary shunt were included.
INTERVENTIONS: Major adverse event is defined as one or more of cardiac arrest, chest reopening, or requirement for extracorporeal membrane oxygenation. Study outcome is a "composite poor outcome," defined as one or more of acute kidney injury, necrotizing enterocolitis, brain injury, or in-hospital mortality.
MEASUREMENTS AND MAIN RESULTS: Median (interquartile range) age was 12 days (6-38 d) and median (interquartile range) time to major adverse event was 5.5 hours (2-17 hr) after admission. Overall, 36 (18%) experienced a major adverse event, and reasons were over-shunting (n = 17), blocked shunt (n = 13), or other (n = 6). Fifteen (88%) in over-shunting group suffered a cardiac arrest compared with two (15%) in the blocked shunt group (p < 0.001). The composite poor outcome was seen in 15 (88%) in over-shunting group, four (31%) in the blocked shunt group, and 56 (34%) in those who did not experience a major adverse event (p < 0.001). By multivariable analysis, predictors for composite poor outcome were major adverse event due to over-shunting (no major adverse event-reference; over-shunting odds ratio, 18.60; 95% CI, 3.87-89.4 and shunt-block odds ratio, 1.57; 95% CI, 0.46-5.35), single ventricle physiology (odds ratio, 4.70; 95% CI, 2.34-9.45), and gestation (odds ratio, 0.84/wk increase; 95% CI, 0.74-0.96).
CONCLUSIONS: Infants who suffer major adverse event due to over-shunting experience considerably poorer outcomes than those who experience events due to shunt block. A mainly hypoxic event with maintenance of systemic perfusion (as often seen in a blocked shunt) is less likely to result in poorer outcomes than those after a hypoxic-ischemic event (commonly seen in over-shunting).