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Heritable pulmonary arterial hypertension (HPAH) is a rare progressive condition that includes patients with an identified genetic cause of pulmonary arterial hypertension (PAH). HPAH and idiopathic PAH (IPAH) have an estimated combined incidence of 0.5-0.9 cases per million children-years. Several pathogenic variants have been associated with HPAH in children and adults, including genes BMPR2, TBX4, and ACVRL1, and more rarely with variants in genes such as SOX17. HPAH is often difficult to manage and has poor prognosis despite advances in medical therapy with many patients progressing to lung transplantation, right heart failure and death. Surgical and transcatheter Potts shunt creation can reduce systolic burden and has shown reduction in morbidity and mortality in children. Early genetic testing can provide both diagnostic and prognostic value in managing and counseling children with severe PAH and it can guide transcatheter or surgical management in refractory cases despite maximal medical therapies. We describe a patient with HPAH (SOX17 mutation) who underwent percutaneous patent ductus arteriosus stent for right ventricle decompression at 2 months of age with clinical management guidance by genetic testing results.

Background: Antenatal corticosteroids (ACS) are administered to prevent neonatal complications and death in women at risk of imminent preterm birth. Internationally, the optimal interval from ACS to delivery (ACS-to-delivery interval) is within seven days; however, evidence in Asian populations specifically is limited. This study aimed to investigate the association between ACS-to-delivery interval and the incidence of neonatal complications in Japan. Methods: This retrospective observational study enrolled singleton neonates born preterm at < 32 weeks of gestational age between 2012 and 2020 at two tertiary centers. A total of 625 neonates were divided into the following four groups according to the timing of ACS (measured in days): no ACS (n = 145), partial ACS (n = 85), ACS 1–7 (n = 307), and ACS ≥ 8 (n = 88). The following outcomes were compared between the groups: treated respiratory distress syndrome (RDS), severe intraventricular hemorrhage (IVH), treated patent ductus arteriosus (PDA), necrotizing enterocolitis, sepsis, bronchopulmonary dysplasia (BPD), treated retinopathy of prematurity (ROP), periventricular leukomalacia, and death discharge. Results: The ACS 1–7 group had significantly decreased adjusted odds ratios (ORs) for treated RDS (0.37 [95% confidence interval: 0.23, 0.57]), severe IVH (0.21 [0.07, 0.63]), treated PDA (0.47 [0.29, 0.75]), and treated ROP (0.50 [0.25, 0.99]) compared with the no ACS group. The ACS ≥ 8 group also showed significantly reduced adjusted ORs for RDS (0.37 [0.20, 0.66]) and treated PDA (0.48 [0.25, 0.91]) compared with the no ACS group. However, the adjusted ORs for BPD significantly increased in both the ACS 1–7 (1.86 [1.06, 3.28]) and ACS ≥ 8 groups (2.94 [1.43, 6.05]) compared to the no ACS group. Conclusions: An ACS-to-delivery interval of 1–7 days achieved the lowest incidence of several complications in preterm neonates born at < 32 weeks of gestational age. Some of the favorable effects of ACS seem to continue even beyond ≥ 8 days from administration. In contrast, ACS might be associated with an increased incidence of BPD, which was most likely to be prominent in neonates delivered ≥ 8 days after receiving ACS. Based on these findings, the duration of the effect of ACS on neonatal complications should be studied further. [ABSTRACT FROM AUTHOR]

Background: To evaluate the impact of implementation of 2019 European respiratory distress syndrome (RDS) guidelines on the incidence of bronchopulmonary dysplasia (BPD). Method: We retrospectively collected the clinical data of very preterm infants (VPIs) born before 32 gestational weeks from January 1st 2018 to December 31st 2021. VPIs were divided into group A and group B according to their birth date which was before or at/after January 1st 2020, when the 2019 European RDS guidelines were introduced. BPD is considered as primary outcome. We statistically analyzed all the data, and we compared the general characteristics, ventilation support, medication, nutrition and the outcomes between the two groups. Results: A total of 593 VPIs were enrolled, including 380 cases in group A and 213 cases in group B. There were no statistic differences regarding to gender ratio, gestational age, birth weight and delivery mode between the two groups. Compared with group A, group B showed higher rate of antenatal corticosteroid therapy (75.1% vs. 65.5%). The improvement of ventilation management in these latter patients included lower rate of invasive ventilation (40.4% vs. 50.0%), higher rate of volume guarantee (69.8% vs. 15.3%), higher positive end expiratory pressure (PEEP) [6 (5, 6) vs. 5 (5, 5) cmH2O] and higher rate of synchronized nasal intermittent positive pressure ventilation (sNIPPV) (36.2% vs. 5.6%). Compared with group A, group B received higher initial dose of pulmonary surfactant [200 (160, 200) vs. 170 (130, 200) mg/Kg], shorter antibiotic exposure time [13 (7, 23) vs. 17 (9, 33) days], more breast milk (86.4% vs. 70.3%) and earlier medication for hemodynamically significant patent ductus arteriosus (hsPDA) treatment [3 (3, 4) vs. 8 (4, 11) days] (p < 0.05). As the primary outcome, the incidence of BPD was significantly decreased (16.9% vs. 24.2%) (p < 0.05), along with lower extrauterine growth retardation (EUGR) rate (39.0% vs. 59.7%), while there were no statistic differences regarding to other secondary outcomes, including mortality, intraventricular hemorrhage (IVH), periventricular leukomalacia (PVL), retinopathy of preterm (ROP) and necrotizing enterocolitis (NEC). However, in the subgroups of infants less than 28 gestational weeks or infants less than 1,000 g, the incidence of BPD was not significantly decreased (p > 0.05). Conclusions: After implementation of 2019 European RDS guidelines, the overall incidence of BPD was significantly decreased in VPIs. Continuous quality improvement is still needed in order to decrease the incidence of BPD in smaller infants who are less than 28 gestational weeks or less than 1,000 g. [ABSTRACT FROM AUTHOR]

Objective Magnesium sulfate (MgSO 4) provides effective fetal neuroprotection. However, there is conflicting evidence regarding the association between antenatal MgSO 4 exposure and patent ductus arteriosus (PDA). Thus, herein, we aimed to evaluate the association between antenatal MgSO 4 exposure and PDA. Study Design Preterm infants born between 24 0/7 and 31 6/7 weeks of gestation were included in this retrospective study. Infants who died within the first 72 hours of life and those with significant congenital anomalies were excluded from the study. Echocardiographic and clinical assessment parameters were used to define PDA and hemodynamically significant PDA (hsPDA). Treatments were planned according to the standard protocols of the unit. The following data were collected from hospital medical records: perinatal characteristics, neonatal outcomes, detailed PDA follow-up findings, and maternal characteristics including MgSO 4 exposure and doses. Results Of the 300 included infants, 98 (32.6%) were exposed to antenatal MgSO 4. hsPDA rates were similar in the infants exposed and not exposed to antenatal MgSO 4 , when adjusted for antenatal steroid administration, gestational age, and birth weight (OR: 1.6, 95% CI: 0.849–3.118, p = 0.146). The rates of PDA ligation and open PDA at discharge were similar between the groups. A cumulative MgSO 4 dose of >20 g was associated with an increased risk of hsPDA (crude OR: 2.476, 95% CI: 0.893–6.864, p = 0.076; adjusted OR: 3.829, 95% CI: 1.068–13.728, p = 0.039). However, the cumulative dose had no effect on the rates of PDA ligation or open PDA at discharge. Rates of prematurity-related morbidities and mortality were similar between the groups. Conclusion Although antenatal MgSO 4 exposure may increase the incidence of hsPDA, it may not affect the rates of PDA ligation or open PDA at discharge. Further studies are required to better evaluate the dose-dependent outcomes and identify the MgSO 4 dose that not only provides neuroprotection but also has the lowest risk of adverse effects. Key Points Antenatal exposure of MgSO 4 may cause PDA. Antenatal MgSO 4 exposure may not increase the rates of PDA ligation or open PDA at discharge. Further studies are required to better evaluate the dose-dependent outcomes and optimal MgSO 4 dose. [ABSTRACT FROM AUTHOR]

Objective Hemodynamically significant patent ductus arteriosus (hsPDA) in preterm neonates is associated with end-organ injury including intraventricular hemorrhage. Early treatment may reduce morbidities but may result in overtreatment. This study aimed to examine the association between commonly obtained echocardiographic markers within the first 12 hours of life and eventual treatment of an hsPDA. Study Design Patients with <32 weeks' gestational age had blinded echocardiograms done within the first 12 hours of life as part of research protocols. Subsequent treatment of the patent ductus arteriosus (PDA) was determined by the clinical team independent of echocardiogram results. t -tests and chi-square tests were done for continuous data and categorical outcomes. A receiver operating curve was created to optimize cutoff values. Results Among 199 neonates studied (mean time of echocardiogram 6.7 h after birth), those needing PDA treatment had higher left ventricular output (LVO), right ventricular output (RVO), and superior vena cava (SVC) flow (p -values 0.007, 0.044, and 0.012, respectively). Cutoffs for predicting PDA treatment were LVO > 204 mL/kg/min (63% sensitivity, 66% specificity), RVO > 221 mL/kg/min or SVC flow > 99 mL/kg/min (sensitivities 70 and 43%, specificities 48 and 73%, respectively). Conclusion Preterm neonates with higher markers of cardiac output in the first 12 hours of birth later required PDA treatment. These data are the first to use standard cardiac output measures in the first 12 hours of life to predict the need for future PDA treatment. Further prospective studies will need to be performed to corroborate these associations between echocardiographic markers and clinical outcomes/morbidities. Key Points Early diagnosis of hsPDA may prevent severe morbidity and death. There are echocardiographic markers beyond duct size and flow direction that may aid early diagnosis. Cardiac output markers within the first 12 hours of life may predict need for treatment of hsPDA. [ABSTRACT FROM AUTHOR]

The document "Viscosity-Corrected Pulmonary Vascular Resistance Index in Pediatric Pulmonary Arterial Hypertension" explores the impact of blood viscosity on the measurement of pulmonary vascular resistance index (PVRI) in children with pulmonary arterial hypertension (PAH). The study suggests that correcting PVRI for blood viscosity could lead to different results compared to raw PVRI measurements and potentially influence treatment decisions. By assessing PVRI corrected for blood viscosity (cPVRI) in a large group of pediatric PAH patients, the researchers discovered that the median cPVRI was higher than the uncorrected PVRI. This study emphasizes the importance of considering blood viscosity when evaluating PVRI in pediatric PAH patients. [Extracted from the article]

The preterm infants are at risk of cerebellar injury and the risk factors for necrotizing enterocolitis (NEC) associated cerebellar injury are not fully understood.Determine the risk factors of cerebellar injury in infants with surgical necrotizing enterocolitis (NEC).Retrospective study compared clinical/pathological information between surgical NEC infants with and those without cerebellar injury detected on brain MRI obtained at term equivalent age. Cerebellar Injury patterns that we identified on MRI brain were cerebellar hemorrhage, siderosis and/or cerebellar volume loss.Cerebellar injury (21/65, 32.3%) in preterm infants with NEC was associated with patent ductus arteriosus (PDA) (18/21(85.7%) vs. 25/44(56.8%); p = 0.021), blood culture positive sepsis (13/21 (61.9%) vs. 11/44 (25%); p = 0.004) following NEC, predominantly grew gram positive bacteria (9/21(42.9%) vs. 4/44(9.1%); p = 0.001), greater red cell transfusion, higher rates of cholestasis following NEC and differences in intestinal histopathology (more hemorrhagic and reparative lesions) on univariate analysis. Those with cerebellar injury had higher grade white matter injury (14/21 (66.7%) vs. 4/44(9.1%) p = 0.0005) and higher-grade ROP (70.6% vs. 38.5%; p = 0.027) than those without cerebellar injury.On multilogistic regression, the positive blood culture sepsis (OR 3.9, CI 1.1–13.7, p = 0.03), PDA (OR 4.5, CI 1.0–19.9, p = 0.04) and severe intestinal pathological hemorrhage (grade 3-4) (OR 16.9, CI 2.1–135.5, p = 0.007) were independently associated with higher risk of cerebellar injury.Preterm infants with surgical NEC with positive blood culture sepsis, PDA, and severe intestinal hemorrhagic lesions (grade 3-4) appear at greater risk for cerebellar injury. [ABSTRACT FROM AUTHOR]

Aim. The present study sought to evaluate the cardiovascular morphology and associated anomalies in patients with interrupted aortic arch (IAA) on CT angiography. Materials and Methods. A retrospective review of our departmental database was conducted to identify patients diagnosed with IAA on CT angiography between January 2014 and September 2022. The demographic information, anatomic characteristics, and associated cardiovascular abnormalities in patients with IAA were assessed. The morphological types of IAA were described in accordance with Celoria–Patton classification. Results. IAA was seen in 49 patients (32 males and 17 females; mean age: 15.9 months). The most common type of IAA based on the site of interruption was type A seen in 27/49 (55.1%) patients, while type B was observed in 22/49 (44.9%) patients. No patient of type C IAA was encountered in the current series. The most commonly associated cardiovascular anomaly was patent ductus arteriosus (PDA) seen in 45/49 (91.8%) patients. Atrial septal defect was seen in 15/49 (30.6%) patients, while 39/49 (79.6%) patients had a ventricular septal defect (VSD). Common arterial trunk was the most common abnormal ventriculo‐arterial connection seen in 13/49 (26.5%) patients. Aorto‐pulmonary window was associated with 7/49 (14.3%) patients. Conclusion. IAA is a rare congenital heart defect associated with an array of cardiovascular anomalies, most common being PDA and VSD. CT angiography is useful not only in diagnosing and characterizing the IAA but also in identifying associated anomalies which may have a bearing on the clinical presentation, prognosis, and surgical management. [ABSTRACT FROM AUTHOR]

Introduction: Sustained lung inflation (SLI) right after birth to decrease the use of mechanical ventilation of preterm infants is controversial because of potential harm. This randomized controlled trial was conducted to evaluate the effectiveness and safety of delayed SLI in neonatal intensive care unit (NICU). Methods: Preterm neonates requiring continuous positive airway pressure after birth were eligible for enrollment. In the experimental group, SLI with 20 cm H2O for 15 s was conducted by experienced staff in the NICU between 30 min and 24 h after birth. Results: A total of 45 neonates were enrolled into this study, including 24 in the experimental group and 21 in the control group. There was no significant difference in the birth condition between the experimental and control groups, including gestational age (p = 0.151), birth weight (p = 0.692), and Apgar score at 1 min (p = 0.410) and 5 min (p = 0.518). The results showed the duration of respiratory support was shorter in the experimental group than the control group (p = 0.044). In addition, there was no significant difference in the other outcomes, such as pneumothorax, patent ductus arteriosus, and bronchopulmonary dysplasia. Conclusion: Our findings indicate that sustained inflation conducted by experienced staff in the NICU is safe. The data suggest that SLI conducted by experienced staff in the NICU after stabilization could serve as an alternative management for preterm infants with respiratory distress. However, the reduction in use of respiratory support should be interpreted cautiously as a result of limited sample size. Trial registration: University hospital Medical Information Network (UMIN) Clinical Trials Registry: UMIN000052797 (retrospectively registered). [ABSTRACT FROM AUTHOR]

Aim Methods Results Conclusion Concerns exist regarding potential adverse neurodevelopmental outcomes associated with paracetamol exposure during pregnancy and early infancy. This review evaluates the evidence for the impact of paracetamol use for patent ductus arteriosus (PDA) treatment on neurodevelopmental outcomes in preterm infants.A literature search was performed via Medline, Ovid Embase and Cumulative Index to Nursing and Allied Health Literature (CINAHL) databases. The search details are below: (‘Infant, Newborn’ [MeSH] OR ‘neonate*’ [Title/Abstract]) AND (‘Paracetamol’ [MeSH] OR ‘Acetaminophen’ [Title/Abstract]) AND (‘Ductus Arteriosus, Patent/drug therapy’ [MeSH] OR ‘patent ductus arteriosus’ [Title/Abstract]) AND (‘Neurodevelopmental Disorders’ [MeSH] OR ‘neurodevelopment*’ [Title/Abstract] OR ‘Child Development’ [MeSH] OR ‘Developmental Disabilities’ [MeSH]). All studies were critically appraised and synthesised.Seven studies reported neurodevelopmental outcomes after paracetamol use for PDA treatment in preterm infants <32 weeks gestation. The studies varied in dosage, route, and duration of paracetamol administration and in the methods used to assess neurodevelopmental outcomes. None of the studies revealed different outcomes between paracetamol‐exposed preterm infants and controls.Current low‐to‐moderate quality evidence suggests no association between paracetamol used for PDA treatment and adverse neurodevelopmental outcomes in preterm infants. Future well‐powered studies with standardised neurodevelopmental assessments are warranted to strengthen the current evidence base. [ABSTRACT FROM AUTHOR]

Aim Method Results Conclusion To investigate predictors of language and reading outcomes in 12‐year‐old Swedish children born very preterm (<32 gestational weeks) in 2004–2007.Children born very preterm (n = 78, 43 girls), and term‐born controls (n = 50, 32 girls), were examined on verbal IQ, semantic and phonemic fluency, sentence recall, reading fluency, word and phonological decoding at 12 years of age. The results were related to neonatal characteristics, language development, measured with Bayley‐III, at 2.5 years corrected age, and concurrent non‐verbal IQ.Preterm children showed language and reading difficulties that were not completely accounted for by level of concurrent non‐verbal IQ. Extremely preterm born children (<28 gestational weeks) demonstrated specific linguistic weaknesses. Administration of antenatal steroids, retinopathy of prematurity and persistent ductus arteriosus explained unique variance in language and reading outcomes. Language assessments at 2.5 years had low predictive value for language and reading outcomes at age 12.Language and reading difficulties in 12‐year‐old children born preterm were not fully explained by concurrent non‐verbal IQ, and were not reliably predicted by language assessments at 2.5 years. Renewed language assessments at school age are warranted for identifying children with persisting linguistic difficulties. [ABSTRACT FROM AUTHOR]

Neonates with critical congenital heart disease of the ductal-dependent pulmonary circulation type (CCHD-DDPC) require prostaglandin E1 (PGE1) to maintain oxygen saturation until surgery. However, the factors contributing to the maintenance doses of PGE1 remain unclear. This study aimed to determine the predictors of high maintenance PGE1 doses in these neonates. This retrospective cohort study included neonates with CCHD-DDPC at Songklanagarind Hospital between January 1, 2006, and December 31, 2021. Factors associated with high maintenance PGE1 doses (> 0.01 mcg/kg/min) were analyzed to identify predictors. Odds ratios were calculated using tabulation and logistic regression analysis. A prediction score was developed for high maintenance PGE1 doses. Among 96 neonates with CCHD-DDPC, 55 % required high maintenance doses of PGE1. Three factors significantly associated with high maintenance PGE1 doses were patent ductus arteriosus (PDA) size-to-birthweight ratio ≤1.3 mm/kg, initial PGE1 dose >0.03 mcg/kg/min, and preoperative invasive mechanical ventilation. The area under the receiver operating characteristic curve for these three predictors was 0.7409. A predictive score of 0–3 was created based on these factors. The probabilities of receiving a high maintenance dose of PGE1 for patients with overall scores of 0, 1, 2, and 3 were 0.19 (95 % CI: 0.04–0.33), 0.42 (95 % CI: 0.30–0.54), 0.69 (95 % CI: 0.57–0.81), and 0.87 (95 % CI: 0.76–0.99), respectively. In neonates with CCHD-DDPC, a PDA size-to-birth weight ratio ≤1.3 mm/kg, an initial dose of PGE1 > 0.03 mcg/kg/min, and preoperative invasive mechanical ventilation were predictors of high maintenance PGE1 doses during the preoperative period. [ABSTRACT FROM AUTHOR]

Objective: Neurocristopathies play a role as pathogenesis of Hirschsprung's disease and congenital heart diseases (CHDs). This study seeks to identify concomitant deformities, syndromes, and/or associations associated with Hirschsprung's disease warrant evaluation for CHDs through echocardiography. Materials and Methods: A retrospective analysis was conducted on Hirschsprung's disease patients at Siriraj Hospital between January 2006 and December 2022. Echocardiograms were performed when clinical symptoms, abnormal chest X-rays (CXR), desaturation, heart murmurs raised suspicions of cardiovascular anomalies. Results: Among 299 Hirschsprung's disease patients, 43 (14.4%) exhibited CHDs. The sensitivity of CXR (n=268) and echocardiograms (n=51) in diagnosing CHDs was 48.8% and 100%, respectively. Predominant CHD presentations included patent ductus arteriosus (n=29), atrial septal defects (n=18), and ventricular septal defects (n=15). The presence of concomitant deformities, syndromes and/or associations associated with Hirschsprung's disease significantly heightened the likelihood of concurrent CHDs (Odds ratio = 23.56, p < 0.001). Patients with Hirschsprung's disease and concomitant deformities (n=28) (excluding syndromic or chromosomal abnormalities) had 1.73 times the odds of CHDs (p = 0.262) compared to those without concomitant deformities. Patients with Hirschsprung's disease and Down syndrome (n=34) exhibited 77.78 times higher odds of CHDs (p < 0.001), while those with other syndromes and/or associations (n=6) had 13.03 times higher odds of CHDs (p = 0.005) compared to patients lacking these conditions. Conclusion: CHDs were identified in 14.4% of Hirschsprung's disease patients. Echocardiograms should be selectively employed in Hirschsprung's disease associated with Down syndrome, other syndromes, or concomitant deformities. [ABSTRACT FROM AUTHOR]

This document describes a case study presented at a medical conference about a preterm neonate with a congenital hepatic hemangioma (CHH). The neonate had right heart failure and pulmonary hypertension, as well as a patent ductus arteriosus (PDA) and atrial septal defect (ASD). Due to the neonate's small femoral arteries and bleeding diathesis, a trans-umbilical venous approach was used to access the arterial system via the PDA. The embolization procedure was successful, and the neonate's condition improved in the following weeks. This approach offers an alternative for neonates with difficult arterial access and coagulopathy. [Extracted from the article]

Background: Cardiac catheterization is an invasive diagnostic and treatment tool for congenital heart disease (CHD) with potential complications. Objective: To describe the immediate outcomes of patients who underwent cardiac catheterization for CHD at the Uganda Heart Institute (UHI). Methods: The study was a retrospective chart review of 857 patients who underwent cardiac catheterization for CHD at UHI from 1st February 2012 to 30th June 2023. Precardiac catheterization clinical data, procedure details, and post-procedure data were recorded. The statistical software SPSS was used for data analysis. Results: We studied 857 patients who underwent cardiac catheterization for CHD at UHI. Females comprised 62.8% (n = 528). The age range was 3 days to 64 years, with a mean of 5.1 years (SD 7.4). Advanced heart failure was present in 24(2.8%) of the study participants. The most common procedures were patent ductus arteriosus device closure (n = 500, 58.3%), diagnostic catheterization (n = 194, 22.5%), and balloon pulmonary valvuloplasty (n = 114, 13.0%). PDA device closure had 89.4% optimal results while BPV had 75.9% optimal performance outcome. Adverse events occurred in 52 out of 857 study participants (6.1%). Clinically meaningful adverse events (CMAES) occurred in 3.9%, (n = 33), high severity adverse events in 2.9% (n = 25) and mortality in 1.5% (n = 13). Advanced heart failure at the time of cardiac catheterization, was significantly associated with clinically meaningful adverse events (OR 52 p-value < 0.001) and mortality (OR 564, p value < 0.001). Conclusion: Many patients with CHD have benefited from the cardiac catheterization program at UHI with high optimal procedure outcome results. Patients with advanced heart failure at the time of cardiac catheterization have less favorable outcomes emphasizing the need for early detection and early intervention. [ABSTRACT FROM AUTHOR]

Background: PPHN is a common cause of neonatal respiratory failure and is still a serious condition and associated with high mortality. Objectives: To compare the demographic variables, clinical characteristics, and treatment outcomes in neonates with PHHN who underwent ECMO and survived compared to neonates with PHHN who underwent ECMO and died. Methods: We adhered to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline and searched ProQuest, Medline, Embase, PubMed, CINAHL, Wiley online library, Scopus and Nature for studies on the development of PPHN in neonates who underwent ECMO, published from January 1, 2010 to May 31, 2023, with English language restriction. Results: Of the 5689 papers that were identified, 134 articles were included in the systematic review. Studies involving 1814 neonates with PPHN who were placed on ECMO were analyzed (1218 survived and 594 died). Neonates in the PPHN group who died had lower proportion of normal spontaneous vaginal delivery (6.4% vs 1.8%; p value > 0.05) and lower Apgar scores at 1 min and 5 min [i.e., low Apgar score: 1.5% vs 0.5%, moderately abnormal Apgar score: 10.3% vs 1.2% and reassuring Apgar score: 4% vs 2.3%; p value = 0.039] compared to those who survived. Neonates who had PPHN and died had higher proportion of medical comorbidities such as omphalocele (0.7% vs 4.7%), systemic hypotension (1% vs 2.5%), infection with Herpes simplex virus (0.4% vs 2.2%) or Bordetella pertussis (0.7% vs 2%); p = 0.042. Neonates with PPHN in the death group were more likely to present due to congenital diaphragmatic hernia (25.5% vs 47.3%), neonatal respiratory distress syndrome (4.2% vs 13.5%), meconium aspiration syndrome (8% vs 12.1%), pneumonia (1.6% vs 8.4%), sepsis (1.5% vs 8.2%) and alveolar capillary dysplasia with misalignment of pulmonary veins (0.1% vs 4.4%); p = 0.019. Neonates with PPHN who died needed a longer median time of mechanical ventilation (15 days, IQR 10 to 27 vs. 10 days, IQR 7 to 28; p = 0.024) and ECMO use (9.2 days, IQR 3.9 to 13.5 vs. 6 days, IQR 3 to 12.5; p = 0.033), and a shorter median duration of hospital stay (23 days, IQR 12.5 to 46 vs. 58.5 days, IQR 28.2 to 60.7; p = 0.000) compared to the neonates with PPHN who survived. ECMO-related complications such as chylothorax (1% vs 2.7%), intracranial bleeding (1.2% vs 1.7%) and catheter-related infections (0% vs 0.3%) were more frequent in the group of neonates with PPHN who died (p = 0.031). Conclusion: ECMO in the neonates with PPHN who failed supportive cardiorespiratory care and conventional therapies has been successfully utilized with a neonatal survival rate of 67.1%. Mortality in neonates with PPHN who underwent ECMO was highest in cases born via the caesarean delivery mode or neonates who had lower Apgar scores at birth. Fatality rate in neonates with PPHN who underwent ECMO was the highest in patients with higher rate of specific medical comorbidities (omphalocele, systemic hypotension and infection with Herpes simplex virus or Bordetella pertussis) or cases who had PPHN due to higher rate of specific etiologies (congenital diaphragmatic hernia, neonatal respiratory distress syndrome and meconium aspiration syndrome). Neonates with PPHN who died may need a longer time of mechanical ventilation and ECMO use and a shorter duration of hospital stay; and may experience higher frequency of ECMO-related complications (chylothorax, intracranial bleeding and catheter-related infections) in comparison with the neonates with PPHN who survived. [ABSTRACT FROM AUTHOR]

Dextro-transposition of the great arteries (D-TGA) is the second most common cyanotic heart disease, accounting for 5–7% of all congenital heart defects (CHDs). It is characterized by ventriculoarterial (VA) connection discordance, atrioventricular (AV) concordance, and a parallel relationship with D-TGA. As a result, the pulmonary and systemic circulations are separated [the morphological right ventricle (RV) is connected to the aorta and the morphological left ventricle (LV) is connected to the pulmonary artery]. This anomaly is included in the group of developmental disorders of embryonic heart conotruncal irregularities, and their pathogenesis is multifactorial. The anomaly's development is influenced by genetic, epigenetic, and environmental factors. It can occur either as an isolated anomaly, or in association with other cardiac defects. The typical concomitant cardiac anomalies that may occur in patients with D-TGA include ventriculoseptal defects, patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), mitral and tricuspid valve abnormalities, and coronary artery variations. Correction of the defect during infancy is the preferred treatment for D-TGA. Balloon atrial septostomy (BAS) is necessary prior to the operation. The recommended surgical correction methods include arterial switch operation (ASO) and atrial switch operation (AtrSR), as well as the Rastelli and Nikaidoh procedures. The most common postoperative complications include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency and neopulmonic stenosis, right ventricular (RV) outflow tract obstruction (RVOTO), left ventricular (LV) dysfunction, arrhythmias, and heart failure. Early diagnosis and treatment of D-TGA is paramount to the prognosis of the patient. Improved surgical techniques have made it possible for patients with D-TGA to survive into adulthood. [ABSTRACT FROM AUTHOR]

Background: Preterm infants are at high risk for retinopathy of prematurity (ROP), with potential life-long visual impairment. Low fetal hemoglobin (HbF) levels predict ROP. It is unknown if preventing the HbF decrease also reduces ROP. Methods: BORN is an ongoing multicenter double-blinded randomized controlled trial investigating whether transfusing HbF-enriched cord blood-red blood cells (CB-RBCs) instead of adult donor-RBC units (A-RBCs) reduces the incidence of severe ROP (NCT05100212). Neonates born between 24 and 27 + 6 weeks of gestation are enrolled and randomized 1:1 to receive adult donor-RBCs (A-RBCs, arm A) or allogeneic CB-RBCs (arm B) from birth to the postmenstrual age (PMA) of 31 + 6 weeks. Primary outcome is the rate of severe ROP at 40 weeks of PMA or discharge, with a sample size of 146 patients. A prespecified interim analysis was scheduled after the first 58 patients were enrolled, with the main purpose to evaluate the safety of CB-RBC transfusions. Results: Results in the intention-to-treat and per-protocol analysis are reported. Twenty-eight patients were in arm A and 30 in arm B. Overall, 104 A-RBC units and 49 CB-RBC units were transfused, with a high rate of protocol deviations. A total of 336 adverse events were recorded, with similar incidence and severity in the two arms. By per-protocol analysis, patients receiving A-RBCs or both RBC types experienced more adverse events than non-transfused patients or those transfused exclusively with CB-RBCs, and suffered from more severe forms of bradycardia, pulmonary hypertension, and hemodynamically significant patent ductus arteriosus. Serum potassium, lactate, and pH were similar after CB-RBCs or A-RBCs. Fourteen patients died and 44 were evaluated for ROP. Ten of them developed severe ROP, with no differences between arms. At per-protocol analysis each A-RBC transfusion carried a relative risk for severe ROP of 1.66 (95% CI 1.06–2.20) in comparison with CB-RBCs. The area under the curve of HbF suggested that HbF decrement before 30 weeks PMA is critical for severe ROP development. Subsequent CB-RBC transfusions do not lessen the ROP risk. Conclusions: The interim analysis shows that CB-RBC transfusion strategy in preterm neonates is safe and, if early adopted, might protect them from severe ROP. Trial registration: Prospectively registered at ClinicalTrials.gov on October 29, 2021. Identifier number NCT05100212. [ABSTRACT FROM AUTHOR]

Purpose: Left atrium to aortic root ratio (LA/Ao) is an echocardiographic marker of hemodynamically significant patent ductus arteriosus (PDA). Since 2‐dimensional measurement of the ratio is geometrically limited, left atrial volume (LAV) which has 3‐dimensional characteristics was investigated. The aim of this study was to determine a correlation between LA/Ao ratio and LAV as well as holodiastolic flow reversal in preterm neonates with and without a PDA. Methods: A retrospective evaluation of neonates with and without PDA was performed. Targeted neonatal echocardiography evaluation of LA/Ao and LAV was measured from parasternal long‐axis view and the apical 4 and 2‐chamber views, respectively. Univariate and linear regression analysis were performed. Results: 200 patients were included of whom 158 (79.0%) had a PDA shunt. The median gestational age at the time of echo was 27.4 weeks (IQR: 25.7–29.4 weeks). The median LA/Ao ratio was 1.51 (IQR: 1.26–1.83) and median LAV indexed to weight was.91 mL/kg (IQR:.65–1.18 mL/kg). There was a significant correlation between LA/Ao and LAV indexed to weight in the PDA group (r2 =.080, p =.0003). LA/Ao ratio and LAV indexed to weight differed significantly between those with diastolic flow reversal versus no‐flow reversal (LA/Ao, p =.003; LAV, p =.001). Conclusions: This study demonstrated a significant correlation between LA/Ao and LAV in preterm infants with PDA, with greater magnitude of discordance for LAV. The power of LAV versus LA/Ao in monitoring hemodynamically significant PDA requires prospective evaluation. [ABSTRACT FROM AUTHOR]

Aim Methods Results Conclusion In extremely low birth weight infants, fluid overload has been associated with bronchopulmonary dysplasia and death. Excessive weight loss may increase the risk of meconium obstruction and intestinal perforation. As these infants display oliguria followed by polyuria, we embarked on a diuresis‐led volume replacement strategy as of January 2020.This single‐centre analysis presents data of infants <1000 g birth weight surviving for more than 3 days admitted 2017–2019 (n = 217, daily volume increase) versus 2020–2022 (n = 2022, diuresis‐led volume replacement).The second cohort had lower gestational age (median [interquartile range]: 253/7 [243/7–264/7] vs. 263/7 [251/7–282/7] weeks), less antenatal steroids (58% vs. 69%), more indomethacin (66% vs. 47%) and higher initial diuresis (5.6 [4.9–6.8] vs. 4.8 [4.2–5.5] mL/kg/h) but did not differ by relative weight loss at Day 7 of life. Employing binary logistic regression with gestational age, antenatal steroids and indomethacin as covariates, the cohorts did not differ by rates of patent ductus arteriosus, abdominal surgery or severe retinopathy of prematurity, while there were significant declines in sepsis (from 32% to 19%), bronchopulmonary dysplasia (from 26% to 23%) and mortality (from 13% to 7%).Diuresis‐led volume replacement appears to be safe and maybe beneficial. [ABSTRACT FROM AUTHOR]

BACKGROUND AND OBJECTIVE: The optimal patent ductus arteriosus (PDA) closure method in very low birth weight (VLBW) infants is uncertain. In 2019, the first transcatheter occlusion device was approved in the United States for infants ≥700 g. We described survival and short-term outcomes among VLBW infants who underwent transcatheter or surgical PDA closure (2018-2022). METHODS: Vermont Oxford Network members submitted data on infants born from 401 to 1500 g or 22 to 29 weeks' gestational age. Adjusted risk ratios (aRR) for survival, length of stay (LOS), prematurity complications, and discharge support were used to compare transcatheter versus surgical closure. Subgroup analyses were conducted for infants with birth weight ≥700 g and born in 2020-2022. RESULTS: Overall, 6410 of 216 267 infants at 726 hospitals received invasive PDA treatment. Transcatheter closure increased from 29.8% in 2018 to 71.7% in 2022. VLBW infants undergoing transcatheter closure had higher survival (adjusted rate ratio [aRR] 1.03; 1.02-1.04) with similar LOS (aRR 1.00; 0.97-1.03), neonatal complications (aRR 1.00; 0.98-1.01), and receipt of discharge support (aRR 0.94; 0.89-1.01). In subgroup analyses, survival (aRR 1.02; 1.00-1.04) and discharge support (aRR 0.90; 0.81-1.01) were similar between groups, whereas selected neonatal complications (aRR 0.95; 0.93-0.98) and LOS (aRR 0.95; 0.90-0.99) were lower after transcatheter closure. CONCLUSIONS: Transcatheter PDA closure in VLBW infants was increasingly used after 2018. Selected short-term outcomes for infants receiving transcatheter closure may be more favorable, compared with surgical, and warrants further clinical investigation. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Emerging data indicate that acetaminophen may adversely affect lung health.We examined whether acetaminophen compared with cyclooxygenase (COX) inhibitor alone for patent ductus arteriosus (PDA) is associated with mortality or respiratory morbidity in extremely preterm infants. METHODS: This is a retrospective cohort study using data from the National Institute of Child Health and Human Development Neonatal Research Network. Infants were born at 22 to 28 weeks' gestation or weighing 401 to 1000 g between 2016 and 2020 and received acetaminophen, ibuprofen, and/or indomethacin for PDA closure. The primary outcome was death or grade 2 to 3 bronchopulmonary dysplasia (BPD) at 36 weeks' postmenstrual age. Secondary outcomes included predischarge mortality and respiratory morbidities. Risk ratios were adjusted for baseline and early postnatal factors. Additional exploratory analyses were adjusted for later postnatal covariates. RESULTS: Of 1921 infants, 627 (32.6%) received acetaminophen and 1294 (67.3%) received COX inhibitor only. Multidrug therapy (42.9% vs 4.7%) and surgical or catheter PDA closure (26.5% vs 19.9%) were more common among acetaminophen-exposed infants. Death or grade 2 to 3 BPD at 36 weeks' postmenstrual age was similar between infants treated with acetaminophen versus COX inhibitor only (57.1% vs 58.3%; adjusted relative risk [aRR] 0.96, 95% confidence interval [CI] 0.87-1.06). Acetaminophen was associated with increased risk of predischarge mortality (13.3% vs 10.0%) when adjusting for perinatal and early postnatal factors (aRR 1.42, 95% CI 1.02-1.93), but not in exploratory analyses that included later postnatal factors (aRR 1.28, 95% CI 0.91-1.82). CONCLUSIONS: Treatment with acetaminophen versus COX inhibitor alone for PDA was not associated with the composite outcome of death or BPD in extremely preterm infants. Our results support further evaluation of whether acetaminophen for PDA increases mortality. [ABSTRACT FROM AUTHOR]

Congenital heart disease (CHD) and patent ductus arteriosus (PDA) are risk factors of necrotizing enterocolitis (NEC) in infants. However, it is unclear whether the prognosis of NEC is different between very preterm infants (VPIs) with and without heart diseases. This was an observational cohort study that enrolled VPIs (born between 24+0 and 31+6 weeks) admitted to 79 tertiary neonatal intensive care units (NICU) in the Chinese Neonatal Network (CHNN) between 2019 and 2021. The exposure was CHD or isolated PDA, and VPIs with NEC were divided into three groups: complicated with CHD, with isolated PDA, and without heart diseases. The primary outcomes were NEC-related adverse outcomes (death or extrauterine growth restriction (EUGR)). Logistic regression models were used to adjust potential confounders and calculate the odds ratios (ORs) and 95% confidential intervals (CIs) for each outcome. A total of 1335 VPIs with NEC were enrolled in this study, including 65 VPIs with CHD and 406 VPIs with isolated PDA. The VPIs with heart diseases had smaller gestational ages and lower body weights at birth, more antenatal steroids use, and requiring inotrope prior to the onset of NEC. While suffering from NEC, there was no significant increased risks in NEC-related death in VPIs with either CHD (adjusted OR [aOR]: 1.10; 95% CI: 0.41–2.50) or isolated PDA (aOR: 1.25; 95% CI 0.82–1.87), and increased risks in EUGR were identified in either survival VPIs with CHD (aOR: 2.35; 95% CI: 1.31–4.20) or isolated PDA (aOR: 1.53; 95% CI: 1.16–2.01) in survivors. The composite outcome (death or EUGR) was also more often observed in VPIs with either CHD (aOR: 2.07; 95% confidence interval [CI]: 1.20–3.60) or isolated PDA (aOR: 1.51; 95% CI: 1.17–1.94) than that without heart diseases. VPIs with either CHD or isolated PDA were associated with significantly prolonged duration of fasting, extended time to achieve full enteral feeding, and longer ventilation duration and hospitalization duration. Similar characteristics were also seen in VPIs with isolated PDA, with the exception that VPIs with CHD are more likely to undergo surgical intervention and maintain a prolonged fast after NEC. Conclusion: In VPIs with NEC, CHD and isolated PDA are associated with an increased risk in worse outcomes. We recommend that VPIs with cardiac NEC be managed with aggressive treatment and nutrition strategies to prevent EUGR. What is Known: • CHD and PDA are risk factors for NEC in infants, which can lead to adverse outcomes such as death and EUGR. • NEC in infants with heart disease differs clinically from that in infants without heart disease and should be recognized as a separate disease process. What is New: • CHD and isolated PDA are associated with increased risks of EUGR in VPIs with NEC. • Risk factors associated with VPIs with cardiac NEC suggested these patients should be managed with aggressive treatment and nutrition strategies to adverse outcomes. [ABSTRACT FROM AUTHOR]

The Atrial Flow Regulator (AFR) is a self-expandable double-disk device with a central fenestration, intended to maintain a calibrated communication across the interatrial septum. Only case reports and small case series have been published about its use in the pediatric and congenital heart disease (CHD) population. We described AFR implantation in three congenital patients with different anatomies and indications. In the first case, the AFR was deployed to create a stable fenestration in a Fontan conduit, while in the second, it was used to reduce a Fontan fenestration. In the third case, we implanted an AFR to decompress the left atrium of an adolescent with complex CHD in natural history, with complete mixing, ductal-dependent systemic circulation and combined pulmonary hypertension. This case series demonstrates the great potential of the AFR device in the CHD field, showing versatility, efficacy, and safety in establishing a calibrated and stable shunt, with promising hemodynamic and symptomatic benefits. [ABSTRACT FROM AUTHOR]

Patients born with obstructed total anomalous pulmonary venous return have a high risk of morbidity and mortality in the neonatal period, which only increases when combined with single ventricle physiology and non-cardiac congenital anomalies such as heterotaxy syndrome. Despite advances in management of congenital heart disease, surgery within the first weeks of life to repair the pulmonary venous connection and establish pulmonary blood flow with a systemic-to-pulmonary shunt has historically led to disappointing outcomes. A multidisciplinary approach with pediatric interventional cardiology and cardiac surgery is required to reduce morbidity and mortality in this extremely high-risk patient population. Extending the time between birth and cardiac surgery can lessen postoperative complications and mortality risk, especially in patients with abnormal thoracoabdominal relationships. Our team was able to successfully utilize transcatheter stent placement in a vertical vein and patent ductus arteriosus to delay and stage cardiac surgeries in an infant born with obstructed total anomalous pulmonary venous return, unbalanced atrioventricular septal defect with pulmonary atresia and heterotaxy, thus reducing the morbidity and mortality associated with this diagnosis. [ABSTRACT FROM AUTHOR]

Aim: Progressive respiratory deterioration in infants at high risk of bronchopulmonary dysplasia (BPD) is associated with patent ductus arteriosus (PDA) exposure. This study aimed to design an early predictive model for BPD or death in preterm infants using early echocardiographic markers and clinical data. Methods: Infants born with gestational age (GA) ≤ 29 weeks and/or birth weight (BW) < 1500 g at Cork University Maternity Hospital, Ireland were retrospectively evaluated. Those with echocardiography performed between 36 h and 7 days of life were eligible for inclusion. Exclusion criteria were pulmonary hypertension and major congenital anomalies. The primary outcome was a composite of BPD and death before discharge. Results: The study included 99 infants. A predictive model for the primary outcome was developed, which included three variables (BW, Respiratory Severity Score and flow pattern across the PDA), and yielding an area under the curve of 0.98 (95% CI 0.96–1.00, p < 0.001). Higher scores were predictive of the primary outcome. A cut‐off of −1.0 had positive and negative predictive values of 89% and 98%, and sensitivity and specificity of 98% and 88%, respectively. Conclusion: Our prediction model is an accessible bedside tool that predicts BPD or death in premature infants. [ABSTRACT FROM AUTHOR]

Aim: To describe sodium and potassium intake, their sources and plasma concentrations, and the association between intake and morbidity in very‐low‐birthweight (VLBW, <1500 g) infants during the first week of life. Methods: This retrospective cohort study comprised 951 VLBW infants born at <32 weeks. Infants were divided into three groups according to gestational age: 23–26 (n = 275), 27–29 (n = 433) and 30–31 (n = 243) weeks. Data on fluid management and laboratory findings were acquired from an electronic patient information system. Results: The median sodium intake was highest in the 23–26 week group, peaking at 6.4 mmol/kg/day. A significant proportion of sodium derived from intravascular flushes; it reached 27% on day 1 in the 23–26 week group. High cumulative sodium intake in the first postnatal week was associated with weight gain from birth to day 8 in the 23–26 week group. High intake of sodium associated with an increased risk of surgically ligated patent ductus arteriosus (PDA), bronchopulmonary dysplasia and intraventricular haemorrhage, whereas low intake of potassium associated with an increased risk of PDA. Conclusion: Sodium intake in the most premature infants exceeded recommendations during the first postnatal week. Saline flushes accounted for a significant proportion of the sodium load. [ABSTRACT FROM AUTHOR]

The purpose of this randomized controlled trial was to investigate the influence of stateof- the-art pulse oximetry technology on the health outcomes of preterm newborns in a Neonatal Intensive Care Unit (NICU). We included preterm infants born between 24 and 36 weeks of gestation in our analysis, excluding those with significant congenital anomalies, lung malformations, congenital heart defects, or early neonatal mortality. Our study involved 52 newborns, divided into two groups: one cohort was monitored using conventional oximetry, while the other was monitored with advanced pulse oximetry technology. The baseline characteristics of both cohorts, such as gender, gestational age, birth weight, prenatal care received, and infection risk, were comparable. Both groups received standardized care based on established clinical guidelines, including feeding assistance, infection prevention techniques, thermoregulation, respiratory distress management, and parental involvement through kangaroo care. We assessed the outcomes for common neonatal issues, such as chronic lung disease, sepsis, intraventricular hemorrhage, patent ductus arteriosus, and air leak syndromes. Our results indicate that there were no statistically significant differences in the primary outcomes between the two groups, suggesting that the randomization process was effective in creating balanced comparison groups. We utilized independent samples t-tests and chi-square tests as part of our statistical framework, with a p-value of less than 0.05 indicating statistical significance. Overall, our findings suggest that the implementation of next-generation pulse oximetry equipment in the NICU for monitoring preterm infants does not significantly affect clinical outcomes compared to conventional monitoring techniques. Further research is necessary to fully evaluate the benefits and feasibility of incorporating cutting-edge oximetry technologies into newborn care procedures. [ABSTRACT FROM AUTHOR]

Background In cases of atrial septal defect with pulmonary arterial hypertension (PAH), a treat-and-repair strategy that adopts pulmonary vasodilator therapy and subsequent defect closure is postulated to be effective. However, this strategy has not been applied to the large patent ductus arteriosus (PDA) with PAH. Case summary A 10-year-old girl with trisomy 21 was referred to our hospital for the treatment of a large PDA with PAH. Cardiac catheterization and angiography revealed a type C tubular PDA with a minimal diameter of 8.1 mm, an increase in mean pulmonary artery pressure (mPAP) of 60 mmHg, a ratio of pulmonary to systemic blood flow (Qp/Qs) of 2.7, and pulmonary artery resistance (Rp) of 7.1 U/m2. Because she was categorized in the grey zone for operability, we adopted a hybrid treat-and-repair strategy in which palliative surgical duct banding was performed before pulmonary vasodilator therapy to prevent excessive pulmonary blood flow and was followed by transcatheter closure of the PDA. Postoperatively, we confirmed the flow-restricted duct with a minimal diameter of 3.3 mm, decreased Qp/Qs 1.38, high mPAP 40 mmHg, and Rp 7.3 U/m2. Six months after treatment with macitentan and tadalafil, we confirmed a decrease in Rp 4.1 U/m2 as well as low Qp/Qs 1.12, which was low enough for the duct occlusion. The transcatheter occlusion of the surgically created type A conical duct was easily and safely performed. In the mid-term follow-up, favourable haemodynamics and improved exercise were confirmed. Discussion This is the first proof-of-concept case report to show the successful hybrid treat-and-repair strategy for large PDA, which warrants further investigation. [ABSTRACT FROM AUTHOR]

Bronchopulmonary dysplasia (BPD) is a lung complication of premature births. The leading causes of BPD are oxidative stress (OS) from oxygen treatment, infection or inflammation, and mechanical ventilation. OS activates alveolar myeloid cells with subsequent myeloperoxidase (MPO)-mediated OS. Premature human neonates lack sufficient antioxidative capacity and are susceptible to OS. Unopposed OS elicits inflammation, endoplasmic reticulum (ER) stress, and cellular senescence, culminating in a BPD phenotype. Poor nutrition, patent ductus arteriosus, and infection further aggravate OS. BPD survivors frequently suffer from reactive airway disease, neurodevelopmental deficits, and inadequate exercise performance and are prone to developing early-onset chronic obstructive pulmonary disease. Rats and mice are commonly used to study BPD, as they are born at the saccular stage, comparable to human neonates at 22–36 weeks of gestation. The alveolar stage in rats and mice starts at the postnatal age of 5 days. Because of their well-established antioxidative capacities, a higher oxygen concentration (hyperoxia, HOX) is required to elicit OS lung damage in rats and mice. Neutrophil infiltration and ER stress occur shortly after HOX, while cellular senescence is seen later. Studies have shown that MPO plays a critical role in the process. A novel tripeptide, N-acetyl-lysyltyrosylcysteine amide (KYC), a reversible MPO inhibitor, attenuates BPD effectively. In contrast, the irreversible MPO inhibitor—AZD4831—failed to provide similar efficacy. Interestingly, KYC cannot offer its effectiveness without the existence of MPO. We review the mechanisms by which this anti-MPO agent attenuates BPD. [ABSTRACT FROM AUTHOR]

Rationale: Hemodynamically significant patent ductus arteriosus (hsPDA) in premature infants has been associated with bronchopulmonary dysplasia (BPD) and pulmonary hypertension (PH). However, these associations remain incompletely understood. Objectives: To assess the associations between hsPDA duration and clinical outcomes, PH, and phenotypic differences on lung magnetic resonance imaging (MRI). Methods: In this retrospective cohort study, we identified all infants with BPD at <32 weeks' gestation who also underwent research lung MRI at <48 weeks' postmenstrual age (PMA) from 2014 to 2022. Clinical echocardiograms were reviewed for hsPDA and categorized as no hsPDA, hsPDA 1–60 days, and hsPDA >60 days. Outcome variables included BPD severity, PH at 36 weeks' PMA, PH after 36 weeks' PMA in the absence of shunt (PH–pulmonary vascular disease [PVD]), tracheostomy or death, and lung phenotype by MRI via modified Ochiai score, indexed total lung volume, and whole-lung hyperdensity. Logistic regression and ANOVA were used. Measurements and Main Results: In total, 133 infants born at 26.2 ± 1.9 weeks, weighing 776 ± 276 g, were reviewed (47 with no hsPDA, 44 with hsPDA 1–60 days, and 42 with hsPDA >60 d). hsPDA duration > 60 days was associated with BPD severity (P < 0.01), PH at 36 weeks' PMA (adjusted odds ratio [aOR], 9.7 [95% confidence interval (CI), 3.3–28.4]), PH–PVD (aOR, 6.5 [95% CI, 2.3–18.3]), and tracheostomy or death (aOR, 3.0 [95% CI, 1.0–8.8]). Duration of hsPDA > 60 days was associated with higher Ochiai score (P = 0.03) and indexed total lung volume (P = 0.01) but not whole-lung hyperdensity (P = 0.91). Conclusions: In infants with moderate or severe BPD, prolonged exposure to hsPDA is associated with BPD severity, PH–PVD, and increased parenchymal lung disease by MRI. [ABSTRACT FROM AUTHOR]

A patent ductus arteriosus is a common entity in preterm infants. Literature is lacking regarding physiological effects on preductal circulation. This report describes 3 patients with abnormal flow Doppler pattern in brachiocephalic artery. Further research is warranted to better understand the impact of PDA on preductal circulation. [ABSTRACT FROM AUTHOR]

\nIntroduction: Providing adequate nutrition in the management of preterm infants has been challenging. The objective of this secondary analysis of data from the randomized trial comparing “less invasive surfactant therapy (LISA) with InSurE method of surfactant administration” is to demonstrate the feasibility of early total enteral feeding (ETEF) in hemodynamically stable preterm neonates on respiratory support and to examine the factors associated with failure of ETEF. Methods: Secondary analysis of a randomized controlled trial comparing “LISA versus InSurE among preterm infants between 26 and 34 weeks of gestation” enrolled 150 infants with 117 being hemodynamically stable. ETEF without any parenteral supplementation was started on day 1 of life using the mother’s own milk (MoM) or donor human milk (<32 weeks of GA) and MoM or preterm formula (33–34 weeks of GA). The data were analyzed to assess the proportion of babies developing feed intolerance and/or necrotizing enterocolitis (NEC) and factors associated with failure of ETEF. All Infants were assessed for the day of attainment of full enteral feeding defined as receiving and tolerating 150 mL/kg of enteral feeds per day. Results: Out of these 117 babies, 102 tolerated ETEF, and 15 had one or more episodes of FI requiring total parenteral nutrition, but none developed NEC till discharge or death. On the assessment of possible factors associated with ETEF failure, there were no differences in baseline characteristics but statistically significantly increased incidence of culture-positive sepsis as well as the requirement of antibiotic therapy for possible sepsis (early as well as late-onset sepsis) in babies with failure of ETEF. The babies who tolerated ETEF achieved full enteral feeding (150 mL/kg/day) significantly earlier (5.48 ± 1.1 days) compared to those with ETEF failure (7 ± 3.4 days) (p 0.001). The time to regain birth weight was earlier in the ETEF group without significant differences in growth parameters. There was also a reduction in the duration of hospital stay in babies who tolerated ETEF, but both these results were not statistically significant. Conclusion: ETEF is feasible in preterm neonates with respiratory distress syndrome who are on respiratory support. It resulted in earlier attainment of full enteral feeds and decreased the incidence of sepsis with reduced antibiotic usage. The objective of this secondary analysis of data from the randomized trial comparing less invasive surfactant therapy (LISA) with InSurE method of surfactant administration among preterm infants with respiratory distress syndrome (RDS) is to demonstrate the feasibility of ETEF in hemodynamically stable preterm neonates on respiratory support.In this randomized control trial comparing LISA versus InSurE among preterm infants with RDS between 26 and 34 weeks of gestation, 150 infants were enrolled with 117 being hemodynamically stable. In 33 patients, early total enteral feeding could not be started due to various reasons – 7 babies had absence/reversal of end-diastolic flow (A/REDF) on antenatal Doppler, 15 babies had shock and required inotropic support on day 1 of life, 8 babies had higher ventilatory settings at the time of admission, and in the 3 babies, the abdomen was not soft to start total enteral feeding. Full-volume enteral feeding without any parenteral supplementation was started on day 1 of life using the mother’s own milk (MoM) or donor human milk (≤32 weeks of GA) and MoM or preterm formula (33–34 weeks of GA). The data were analyzed to assess the proportion of babies developing feed intolerance and/or NEC and factors associated with failure of ETEF. Out of these 117 babies, 102 tolerated ETEF, and 15 had one or more episodes of FI requiring parenteral supplementation, but none developed NEC. There was a statistically significantly increased incidence of culture-positive sepsis as well as the requirement of antibiotic therapy for possible sepsis in babies with failure of ETEF. There was no difference in the morbidities noted such as intraventricular hemorrhage grade 2 or more, patent ductus arteriosus requiring medical or surgical management, retinopathy of prematurity requiring treatment, and NEC stage II or more. Our study suggests that hemodynamically stable preterm infants with RDS on respiratory support can be successfully fed with exclusive enteral feeds started immediately post-birth. ETEF results in early attainment of full feeding and reduces the incidence of sepsis and the duration of hospital stay without increasing the risk of NEC. [ABSTRACT FROM AUTHOR]

Objective The aim of the study is to determine clinical correlates of moderate to severe bronchopulmonary dysplasia (BPD) in preterm infants following surgical necrotizing enterocolitis (NEC). Study Design This is a retrospective, single-center cohort study comparing patients with moderate to severe BPD to patients with non/mild BPD among surgical NEC infants. BPD was defined by NIH 2001 consensus definition. Results Of 92 consecutive neonates with surgical NEC, 77% (71/92) had moderate/severe BPD and 22% (21/92) had non/mild BPD. The patent ductus arteriosus (PDA) was significantly higher in those developing moderate/severe BPD (67.6% [48/71]) than non/mild BPD (28.6% [6/21]; p = 0.001). Postoperatively, infants with moderate/severe BPD had more severe acute kidney injury (AKI; 67.6 [48/71] vs. 28.6% [6/21]; p = 0.001), were intubated longer (40.5 [interquartile (IQR): 12, 59] vs. 6 days [IQR: 2, 13]; p <0.001), received more parenteral nutrition (109 [IQR: 77, 147] vs. 55 days [IQR: 19, 70]; p <0.001), developed higher surgical morbidity (46.5 [33/71] vs. 14.3% [3/21]; p = 0.008), had more intestinal failure (62.5 vs. 13.3%; p <0.001), required a longer hospital stay (161 [IQR: 112, 186] vs. 64 days [IQR: 20, 91]; p <0.001), and were more likely to need home oxygen. In a multivariable analysis, lower birth weight (OR = 0.3, [95% confidence interval (CI): 0.1–0.5]; p = 0.001), PDA (OR = 10.3, [95% CI: 1.6–65.4]; p = 0.014), and longer parenteral nutritional days (OR = 8.8; [95% CI: 2.0–43.0]; p = 0.005) were significantly and independently associated with higher odds of moderate/severe versus non-/mild BPD. Conclusion Development of moderate/severe BPD occurred in the majority of preterm infants with surgical NEC in this consecutive series. Preterm infants with moderate/severe BPD were more likely to have a PDA before NEC. Development of moderate/severe BPD was associated with significantly greater burden and duration of postoperative morbidity following surgical NEC. Identifying surgical NEC infants at increased risk of moderate/severe BPD and developing lung protection strategies may improve surgical NEC outcomes. Key Points Three-fourths of preterm infants experienced severe lung injury following surgical NEC. The infants with severe moderate/severe BPD were most likely associated with greater duration of postoperative morbidity. There is need to understand and develop lung protective strategies in infants with surgical NEC. [ABSTRACT FROM AUTHOR]

Objectives: To standardize the diagnosis of patent ductus arteriosus (PDA) and report its association with adverse neonatal outcomes in very low birth weight infants (VLBW, birth weight < 1500 g). Study design: A multicenter prospective observational study was conducted in Emilia Romagna from March 2018 to October 2019. The association between ultrasound grading of PDA and adverse neonatal outcomes was evaluated after correction for gestational age. A diagnosis of hemodynamically significant PDA (hsPDA) was established when the PDA diameter was ≥ 1.6 mm at the pulmonary end with growing or pulsatile flow pattern, and at least 2 of 3 indexes of pulmonary overcirculation and/or systemic hypoperfusion were present. Results: 218 VLBW infants were included. Among infants treated for PDA closure in the first postnatal week, up to 40% did not have hsPDA on ultrasound, but experienced clinical worsening. The risk of death was 15 times higher among neonates with non-hemodynamically significant PDA (non-hsPDA) compared to neonates with no PDA. In contrast, the risk of death was similar between neonates with hsPDA and neonates with no PDA. The occurrence of BPD was 6-fold higher among neonates with hsPDA, with no apparent beneficial role of early treatment for PDA closure. The risk of IVH (grade ≥ 3) and ROP (grade ≥ 3) increased by 8.7-fold and 18-fold, respectively, when both systemic hypoperfusion and pulmonary overcirculation were present in hsPDA. Conclusions: The increased risk of mortality in neonates with non-hsPDA underscores the potential inadequacy of criteria for defining hsPDA within the first 3 postnatal days (as they may be adversely affected by other clinically severe factors, i.e. persistent pulmonary hypertension and mechanical ventilation). Parameters such as length, diameter, and morphology may serve as more suitable ultrasound indicators during this period, to be combined with clinical data for individualized management. Additionally, BPD, IVH (grade ≥ 3) and ROP (grade ≥ 3) are associated with hsPDA. The existence of an optimal timeframe for closing PDA to minimize these adverse neonatal outcomes remains uncertain. [ABSTRACT FROM AUTHOR]

Background: Mitral valvuloplasty (MVP) is a surgical procedure for treating severe mitral regurgitation in dogs. Although MVP is considered highly invasive, the extent of myocardial injury, postoperative complications, and recovery has not been evaluated. Aim: This study examined the degree of MVP invasiveness, the extent of myocardial damage, postoperative complications, cardiomyocyte recovery, and timing of hospital discharge. Methods: Cardiac troponin I (cTnI) was used to investigate the myocardial damage caused by cardiac arrest associated with a surgical approach to the myocardium in 13 patients with MVP and five controls with patent ductus arteriosus (PDA) who underwent similar anesthesia and thoracotomy. Results: The level of cTnI peaked 1 day after surgery and was significantly higher in the MVP group (median, 19.90 ng/ml) than in the control group (median, 1.50 ng/ml p < 0.001). At day 7, the cTnI level was significantly higher in the MVP group (1.9 ng/ml) than in the control group (0.1 ng/ml) (p < 0.001), and recovery to the preoperative level took 10 days in the MVP group but returned to the preoperative level at day 7 in the control group. Although the mean arterial pressure of cardiopulmonary bypass (CPB) at the time of use was 42.92 mmHg, the peak cTnI levels in the two patients who exhibited a temporary decrease of 20 mmHg or less (46.03 ng/ml) were significantly higher than in the other 11 patients (19.70 ng/ml) (p < 0.05). Preoperative cTnI levels were correlated with the severity of postoperative complications (P = 0.03, F = 0.71). Conclusion: The results showed that MVP caused temporary greater myocardial tissue damage than thoracotomy, but postoperative recovery was smoother. A high preoperative cTnI level requires relatively more careful postoperative management, and measuring the level of cTnI over time after surgery can provide information about the extent of myocardial damage and recovery from surgery and help determine the time of discharge. [ABSTRACT FROM AUTHOR]

OBJECTIVES In patients with borderline left hearts or a severe left ventricular outflow tract obstruction, hybrid palliation can be used to stabilize the patient and postpone biventricular repair (BVR). In this study, we analysed growth of left-sided structures and outcomes of these patients. METHODS We conducted a retrospective cohort study including patients who received hybrid palliation between January 2010 and September 2023. Echo measurements were collected at hybrid palliation, BVR and last follow-up. Growth of left ventricular structures were analysed. RESULTS In 38 patients, hybrid palliation was used to promote growth of left ventricular structures. In total, 15 patients received a Ross–Konno/Yasui procedure, while 23 patients received conventional BVR. In patients with a conventional BVR, a significant increase was found in left ventricular volume indexed by body surface area, Z-score of aortic valve and left ventricular outflow tract between hybrid palliation and BVR. Mitral valve Z-score did not increase significantly. After BVR until follow-up, only increase of the aortic valve Z-scores and left ventricular volume indexed by body surface area was found significant. Of all included patients (n  = 38), additional surgical procedures were necessary in 8 patients during the interstage period and 15 patients after BVR. Additional catheter interventions were needed in 14 patients in the interstage period and 15 after BVR. Six patients died, with no mortality in the conventional BVR group. CONCLUSIONS Hybrid palliation as part of a staged BVR is a safe and effective initial step and promotes the growth of left ventricular structures in patients with small left-sided heart structures. Close follow-up is mandatory because extra catheter or surgical interventions are frequently needed. [ABSTRACT FROM AUTHOR]

Objective To explore the relationship between maternal childbearing age and congenital heart disease (CHD) in offspring. Methods Based on the CHD screening data of live births born in midwifery institutions in Liuzhou area of Guangxi Zhuang Autonomous Region from January 1, 2020 to December 31, 2022, they were divided into CHD group (n=1 117) and non-CHD group (n=107 458). The data of the two groups were matched by propensity score matching method. The childbearing age of mothers was divided into five groups: < 20 years old, 20 years old, 24 years old, 25 years old, 29 years old, 30 years old, 34 years old, and ≥ 35 years old. The multivariate logistic regression model was used to analyze the relationship between mothers and their offspring with congenital heart disease in different childbirth age groups. The restricted cubic spline curve was used to further analyze the nonlinear relationship between maternal childbearing age and offspring CHD. Results There were 1 117 cases in CHD group and 3 148 cases in control group without CHD. The corrected Logistic regression model analysis showed that the risk of atrial septal defect (ASD) in offspring of < 20 years old group and ≥ 35 years old group was significantly higher than that of mothers aged 25 to 29 years old (OR=5.38, 95%CI: 2.96-9.79; OR=1.43, 95%CI: 1.01-2.03). The risk of ventricular septal defect (VSD) was significantly increased in the offspring ≥ 35 years old (OR=1.51, 95%CI: 1.04-2.19). Restricted cubic spline and threshold analysis showed that there was a curvilinear relationship between maternal childbearing age and the risk of CHD, ASD, and VSD in offspring. The risk of CHD in offspring increased with childbirth age (OR=1.04, 95%CI: 1.02-1.06) when childbirth age was more than 25.43 years old, and VSD risk increased with childbirth age when childbirth age > 32.04 years old (OR=1.16, 95%CI: 1.09-1.24). When the delivery age was less than 23.75 years old, the risk of ASD in offspring decreased with the increase of delivery age (OR=0.69, 95%CI: 0.58-0.82). Conclusion Maternal childbearing age is an independent risk factor for CHD in offspring. Women of appropriate age should be encouraged to marry and give birth, and screening policies should be more reasonably specified for young and older women, and prenatal screening should be strengthened. [ABSTRACT FROM AUTHOR]

Key Clinical Message: In this study, we introduced one of the rarest concomitants of the absence of left pulmonary artery (LPA), which was seen in our patient along with patent ductus arteriosus (PDA) and ventricular septal defect (VSD). Unilateral absence of pulmonary artery (UAPA) is a congenital heart disease in association with other abnormalities such as tetralogy of Fallot and septal defects or isolated in 30% of cases and occurs in the right lung in two thirds of cases. Our case is a 33‐year‐old man who was hospitalized with symptoms of cough, shortness of breath, and hemoptysis. The echocardiography revealed a large ventricular septal defect, absent left pulmonary artery, and severe pulmonary hypertension (PH) along with patent ductus arteriosus. These findings were confirmed by CT angiography. This association has rarely been found in past studies. Due to PH and pulmonary infection, the patient was treated with intravenous prostaglandin and antibiotics. However, in cases of timely diagnosis and treatment of UAPA, fatal complications such as pulmonary hypertension, morbidity, and mortality are reduced. This case emphasizes the importance of awareness of this abnormality and its associated anomalies to enable early diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Objective Little is known about the hospital outcomes of moderately preterm (MPT; 29 0/7–33 6/7 weeks gestational age) infants born to insulin-dependent diabetic mothers (IDDMs). We evaluated characteristics and outcomes of MPT infants born to IDDMs compared with those without IDDM (non-IDDM). Study Design Cohort study of infants from 18 centers included in the MPT infant database from 2012 to 2013. We compared characteristics and outcomes of infants born to IDDMs and non-IDDMs. Results Of 7,036 infants, 527 (7.5%) were born to IDDMs. Infants of IDDMs were larger at birth, more often received continuous positive pressure ventilation in the delivery room, and had higher risk of patent ductus arteriosus (adjusted relative risk or aRR: 1.49, 95% confidence interval [CI]: 1.20–1.85) and continued hospitalization at 40 weeks postmenstrual age (aRR: 1.55, 95% CI: 1.18–2.05). Conclusion MPT infants of IDDM received more respiratory support and prolonged hospitalizations, providing further evidence of the important neonatal health consequences of maternal diabetes. Key Points Little data are available on moderate preterm infants of IDDMs. MPT infants of IDDMs need more respiratory support. Longer neonatal intensive care unit stays among MPT infants of IDDMs. [ABSTRACT FROM AUTHOR]

Objective: This study aims to report the experience and outcomes in patients undergoing endovascular closure of small to medium-size PDA with a Nit-Occlud® device in a tertiary referral hospital in Colombia. Methods: Longitudinal descriptive study, which included all patients under 18 years of age who underwent percutaneous ductal closure with Nit-Occlud® device between January 1, 2011, and February 1, 2023. Patients with associated complex congenital heart disease requiring surgical management, pregnant patients, and patients with incomplete data regarding studied variables were excluded from the study. Results: Eighty-seven patients were documented, with a mean age, weight, and height at closure of 51 months, 14 kg, and 95.83 cm, respectively. About 70% of the patients (n = 61) were female, 76% were under 6-years-old and only one patient was over 15. The average size of the ductus at the pulmonary end was 2 mm. Four of the total number of patients did not achieve PDA closure during the procedure. Of the remaining 83, complete immediate closure was achieved in 81 patients. A device exchange for a larger device was required during the same procedure in one of the cases. Two patients presented residual shunt of 0.5 mm during follow-up, and one required a new procedure for device closure 10 months later. Only one device presented repeatedly embolization to the aorta, requiring surgical removal. As a technical difficulty, one device presented repeated passage into the aorta, so it was decided to remove it before releasing it to avoid complications, and given the complex anatomy of the ductus, surgical closure was indicated. Among the complications, one patient presented a hematoma of the subcutaneous tissue in the right thigh, which improved with medical management, and no deaths related to the procedure were registered. Conclusions: Using the Nit-Occlud® device to close small to moderate-sized ductus remains a safe and effective strategy with successful closure rates at 1-year follow-up irrespective of age, weight, height, or whether it involves a small or medium-sized duct. Despite our limitations, results concerning adverse effects are comparable to those observed in multicentric studies conducted in other regions. [ABSTRACT FROM AUTHOR]