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.

DESIGN: A single-center retrospective cross-sectional study.

SETTING: Tertiary referral PICU in Johor, Malaysia.

PATIENTS: All children admitted to the PICU over 8 years were included. Patients readmitted into PICU after the first PICU discharge during the hospitalization period were categorized into "early" (within 48 hr) and "late" (after 48 hr), and factors linked to the readmissions were identified. The mortality rate was determined and compared between no, early, and late readmission.

INTERVENTIONS: None.

MEASUREMENTS AND MAIN RESULTS: There were 2,834 patients in the study with 70 early and 113 late readmissions. Therefore, the rate of early and late PICU readmission was 2.5% (95% CI, 1.9-3.0%) and 3.9% (95% CI, 3.2-4.7%), respectively. The median length of stay of the second PICU admission for early and late readmissions was 2.7 days (interquartile range, 1.1-7.0 d) and 3.2 days (interquartile range, 1.2-7.5 d), respectively. The majority of early and late readmissions had a similar diagnosis with their first PICU admission. Multivariable multinomial logistic regression revealed a Pediatric Index Mortality 2 score of greater than or equal to 15, chronic cardiovascular condition, and oxygen supplement upon discharge as independent risk factors for early PICU readmission. Meanwhile, an infant of less than 1 year old, having cardiovascular, other congenital and genetic chronic conditions and being discharged between 8 AM and 5 PM was an independent risk factor for late readmission. There was no significant difference in the mortality rate of early (12.9%), late (13.3%), and no readmission (10.7%).

METHODS: This retrospective, observational, population-based study involved all children with KD registered in Johor Kawasaki Clinical Registry over 10 years (January 2010 and December 2019). The prevalence of delayed diagnosis and its trend over time was calculated. Multivariable binary logistic regression was used to identify the independent risk factors for delayed diagnosis.

RESULTS: There were 556 cases of KD, with 28% having incomplete criteria, 11% atypical presentation, while 10% developed a coronary aneurysm. The overall prevalence of delayed diagnosis was 9.9% (95% confidence interval (CI): 7.6-12.7%). There was a statistically significant decrease in delayed diagnosis over time (P = 0.008), with the latest rate of 4.6%. The majority of delayed diagnoses were due to failure to diagnose the disease during the initial consultation. Independent risk factors for delayed diagnosis were children older than 1 year, diagnosis before 2015, atypical presentation, and incomplete KD, with adjusted odds ratios (ORs) of 2.7, 2.3, 4.3, and 3.6, respectively. Compared to early diagnosis of KD, delayed diagnosis was significantly associated with coronary aneurysms (27.3% vs. 8.2%, P < 0.001, OR 4.2, [95% CI: 2.1-8.3]).

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.

STUDY DESIGN: This observational cross-sectional study includes 195 confirmed PPHN with a gestational age of ≥34 weeks without congenital heart disease. Multivariable logistic regression was used to identify risk factors for mortality.

RESULTS: The mortality rate was 16.4%, with the highest mortality with pulmonary hypoplasia. Of 195, 65% received iNO; 18% were iNO non-responders with the majority having pulmonary hypoplasia. Independent risk factors for mortality were the presence of reversal of flow at the descending aorta, pulmonary hypoplasia, APGAR scores ≤ 5 at 5 min, and idiopathic PPHN with an adjusted odds ratio of 15.9, 7.5, 6.7, and 6.4, respectively.