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.
Subject and Methods: A total of 27 patients underwent surgery between 2001 and 2015, and they were followed up for a mean period of 6.4 ± 4.1 years. Out of the 27 patients, 14 (51.9%) were infants. The median age at repair was 9.8 months. Preoperative intubation was required in six patients (22.2%), and 11 patients (40.7%) had symptoms of respiratory distress. The pulmonary valve was replaced with a valved conduit in 15 patients (55.6%), monocusp valve in 6 patients (22.2%), and a transannular patch in 6 patients (22.2%). Reduction pulmonary arterioplasty was done in all patients.
Results: The overall 10-year survival was 82.1%. There was 81.1% overall freedom from re-intervention at 10 years. No statistically significant difference was found in 10-year survival (P = 0.464) and reoperation rates (P = 0.129) between valved conduit, monocusp, or transannular patch techniques. Older children had statistically significantly longer survival (P = 0.039) and freedom from re-intervention (P = 0.016) compared to infants. Patients without respiratory complications had 100% 10-year survival and 93.3% freedom from reoperation at 10 years compared to 55.6% and 60.1%, respectively, for patients with respiratory complications.
Conclusion: There has been improvement in surgical results for APVS over the years. However, it still remains a challenge to manage infants and patients with persistent respiratory problems.
METHODS: This retrospective observational study of children with KD was conducted at two tertiary hospitals in Malaysia from January 2014 to December 2019. Multivariable binary logistic regression was used to analyze the risk factors associated with IVIG resistance.
RESULTS: A total of 174 patients, 118 males (67.8%) with a median age of 1.4-year-old (interquartile range: 0.1-12.1-year-old), were analyzed. Early (<5 days) and late (>10 days) IVIG treatments were observed in 14 (8.1%) and 19 (11.0%), respectively. Thirty-two patients (18.4%) had IVIG resistance. The independent factors associated with IVIG resistance were high white cell count, hypoalbuminemia, and extremities changes with an odd ratio of 4.7, 3.0, and 4.0, respectively. In addition, CAA was significantly higher in IVIG resistance compared to IVIG responder patients (57.5% [19/33] vs. 23.4% [33/141], P < 0.001). The sensitivity was high in Harada (93.8%) but low in Kobayashi and Egami (46.9% and 34.4%, respectively). The specificity was high with Egami (79.6%) but low in Harada and Kobayashi (22.5% and 64.1%, respectively).
CONCLUSION: Leukocytosis, hypoalbuminemia, and extremities changes were independent risk factors for IVIG resistance. The variation in sensitivity and specificity of the Japanese scoring makes it unsuitable for predicting IVIG resistance in Malaysian children.
METHODS AND RESULTS: This was a retrospective review of 198 patients who underwent VA-ECMO after congenital cardiac surgeries at our institute between 2004 and 2023. Patients were divided into pre-ECMO protocol (2004-2017) and post-ECMO protocol (2018-2023) implementation. There were 107 patients in the preprotocol era and 91 in the postprotocol era. We compared weaning from ECMO and survival to hospital discharge between the two eras. An analysis of the factors influencing survival to hospital discharge was also done. ECMO was initiated through the central cannulation technique through median sternotomy in all patients. The median age and weight at initiation were 4 months (interquartile range [IQR] 1-33.5 months) and 4.4 kg (IQR 3.3-10.1 kg), respectively. The successful weaning of the ECMO (n = 67/91, 73.6%) and survival to discharge (n = 43/91, 47.3%) were higher in patients of the postprotocol era. However, it was not statistically significant. Higher risk adjustment for congenital heart surgery-1 >3 and acute kidney injury were independent predictors of poorer survival to hospital discharge.
CONCLUSIONS: A protocol-based ECMO program may improve outcomes of successful weaning and survival to discharge in patients undergoing congenital cardiac surgeries.
MATERIALS AND METHODS: A retrospective cohort study from January 2000 to December 2015, we analyzed management trends and focused on 53 patients with both VSD and moderate MR. This cohort was subdivided into four groups: first by their MV pathology, and then stratified by the receipt of intervention towards the diseased MV. Our primary goal was to identify correlations, especially concerning surgical outcomes such as mortality and need for re-operation. Statistical significance is determined when the P value is lower than 0.05.
RESULTS: There were no notable differences in preoperative variables across four cohort groups, apart from the cross-clamp duration which was longest in Group B intervention. Outcome analysis showed survival rates that were higher in cohort groups that underwent intervention toward the disease MV regardless of existing MV morphology, although results were not statistically significant. Cox model analysis found no correlation between the cohort groups and postoperative outcomes, but cross-clamp duration significantly correlated with mortality.
CONCLUSIONS: In cases of VSD with moderate MR with associated pathologies of the valvular apparatus, opting for MV repair (MVr) appears to improve survival outcomes without significantly increasing postoperative morbidity. Similarly, for patients with moderate MR and isolated annular dilatation, surgical MVr is observed to have better survival trend compared to the control group.