A 10-month-old boy was referred for tachypnea and heart murmur. An echocardiogram showed unexplained left heart dilation without evidence of an intracardiac shunt. A 64-slice computed tomographic contrast-enhanced angiography showed a large tortuous anomalous artery arising from the descending thoracic aorta and supplying the lower lobe of the left lung. The venous return into the left atrium was normal. The affected lobe had normal lung parenchyma, and its bronchial tree was connected normally with the left main bronchus. Hence, it was not a sequestrated lobe. The boy underwent surgical lobectomy of the left lower lobe and improved. Anomalous arterial supply of a lobe without sequestration of its bronchial tree is a rare pathologic entity. It also is a very rare cause of congestive heart failure in children. Computed tomographic angiography was a useful tool for evaluation of the intrathoracic anomalous vessel in this case.
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
Limited data are available concerning supraventricular tachycardia (SVT) recurrence. Hence, this study aimed to determine the incidence, outcome, and factors associated with SVT recurrence. This retrospective, observational, population-based study was conducted among children with SVT from 2006 to 2020. The primary outcome measure was SVT recurrence. Kaplan Meier analysis was used to estimate SVT-free at 1, 5, and 10 years after diagnosis. Cox regression analysis was used to identify independent factors associated with recurrence. There were 156 patients with SVT with a median age at diagnosis of 1.9 years (Interquartile range [IQR] 11 days to 8.7 years) and follow-up for a median of 3.5 years (IQR 1.7 to 6.1 years). 35 patients (22%) had recurrent SVT at a median age of 7.8 years (IQR 4.4 to 12 years). Infants with Wolff-Parkinson-White Syndrome (WPW) had the highest recurrence (11/16, 68%), with 33% SVT-free at 5 years follow-up. The lowest recurrence rate was observed in neonatal diagnosis (2/54, 3.7%) with 98% SVT-free at 5 years follow-up. The independent factors associated with the recurrence of SVT were the diagnosis of WPW with an adjusted hazard ratio (aHR) of 5.2 (95% CI 2.4-11.2), age of more than 1 year at diagnosis (aHR 3.7 95% CI 1.4-9.7), and combine with or need second-line therapy (aHR 4.0 95% CI 1.5-10.7). One in five children with SVT experienced a recurrence, which is more likely for those with WPW, multiple maintenance therapy, and older age at first presentation. Whereas neonates with non-WPW may benefit from shorter maintenance therapy.
Patent Ductus Arteriosus (PDA) stenting is an alternative to Modified Blalock-Taussig-Thomas Shunt (BT) to increase pulmonary blood flow in ductal-dependent pulmonary circulation. The objective of this study is to compare the immediate Intensive Care Unit (ICU) outcomes of PDA stent versus BT shunt in infants with ductal-dependent pulmonary circulation. This is a single center, retrospective study in infants less than 6 months with ductal-dependent pulmonary circulation palliated with either PDA stenting or BT shunt from January 2015 till December 2023. Data were reviewed from pediatric ICU database. Demographics details, ICU parameters, mortality and morbidity were compared. The propensity score with inverse probability weighting was used to control the effect of covariates. A total of 302 patients with PDA stenting and 100 patients with BT shunt were included. Majority of the patients had intervention at first month of life. 126 (41.7%) patient underwent PDA stenting while 36 (36%) had BTS. The PDA stenting group has shorter ICU stay, 3 (2.0,6.0) days versus 8 (4.8,13.0) days (p