Ductus Arteriosus Research Articles

Diagnosis of Congenital Heart Disease in Adulthood: How Often, How Relevant?

Congenital heart disease (CHD) is typically detected during fetal life, infancy, or early childhood. However, there is no published data regarding the proportion of congenital heart defects that are diagnosed in adulthood or the impact of these defects. Retrospective analyses of all consecutive patients (n = 1,010) referred to an adult CHD unit between 2018 and 2023. We analyzed the proportion of cases diagnosed in adulthood, defining the type of defect, reasons for diagnosis, complications, and need for intervention. In total, 26.5% of patients were diagnosed in adulthood (mean age 47 ± 16 years). Overall, 75% were in New York Heart Association class I/IV. Most were mild complexity lesions (57.5%). The most common diagnoses were pre-tricuspid shunts, including ostium secundum atrial septal defect (ASD, 23.9%), partial anomalous pulmonary vein drainage (18.3%), and other types of ASD (5.9%). Bicuspid aortic valve (16.8%) and aortic coarctation (8.2%) were common. Other diagnoses included Ebstein's anomaly (5.6%), ventricular septal defect (4.5%), patent ductus arteriosus (2.6%), or congenitally corrected transposition of the great arteries (2.6%). The main reason for diagnostic work-up was cardiac symptoms (28.4%) such as dyspnea (19%) and palpitations (7.1%), followed by incidental findings on imaging (25.4%). A total of 47.4% had some complications, the most common being pulmonary hypertension (24.3%). Surgical repair was required in 27.2% and 25.4% underwent percutaneous intervention. About one-fourth of patients with CHD were diagnosed in adulthood, and up to 42.5% had moderate or severe complexity lesions. A significant proportion had developed complications at the time of diagnosis and half of them required intervention.

Clinical correlates of cerebellar injury in preterm infants with surgical necrotizing enterocolitis.

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.

Delayed sustained lung inflation for preterm neonates in neonatal intensive care unit: a randomized controlled trial

IntroductionSustained 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).MethodsPreterm 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.ResultsA 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.ConclusionOur 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 registrationUniversity hospital Medical Information Network (UMIN) Clinical Trials Registry: UMIN000052797 (retrospectively registered).

Percutaneous correction of structural congenital heart diseases during the early puerperium. Experience of a center

Congenital heart diseases (CHD) can be found in pregnant women. Although cardiac interventions in the catheterization laboratory are considered safe and effective, it is preferable to wait 3-6 months after delivery to correct simple, uncomplicated CHD; however, this may result in follow-up losses. The objective is to present our experience in correcting CHD during the early puerperium (EP). All cases of pregnant women with CHD, including atrial septal defect (ASD), patent ductus arteriosus (PDA), and aortic coarctation (CoA) between 2017-2023, who underwent percutaneous defect correction during the EP were collected. Fifteen pregnant women were included, diagnosed with ASD (5), PDA (6), and CoA (4). Five patients (33.3%) were classified as WHO risk class IV; the procedure was successful in 80% of the cases, and only 1 patient presented complications. In our experience, the closure of uncomplicated congenital defects during the EP did not present major complications and could be a treatment strategy to prevent follow-up losses after delivery in pregnant women with CHD.

Neurodevelopmental outcomes following paracetamol use for treatment of patent ductus arteriosus: A review.

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.

MULTI-SYSTEM INFLAMMATORY SYNDROME IN PATIENTS ADMITTED AT NICU OF A TERTIARY CARE HOSPITAL

Multi-inflammatory syndrome in neonates (MIS-N) associated with COVID-19 is an emerging concern in neonatal intensive care units (NICUs). Understanding its clinical spectrum and outcomes is crucial for developing management strategies and improving neonatal care. Objective: To explore the clinical spectrum, associated laboratory markers, and outcomes of neonates diagnosed with MIS-N and COVID-19 admitted to the NICU. Methods: This case series was conducted at the NICU of Shifa International Hospital, Islamabad, from January 2023 to June 2023. It included 47 infants diagnosed with COVID-19 who developed MIS-N. Data collected encompassed clinical presentations, laboratory markers, treatments administered in the ICU, and clinical outcomes. Descriptive statistics were used to analyze the data, focusing on symptoms, treatment efficacy, and complications. Results: The neonates had a mean age of 20.65 ± 16.60 days and a mean gestational age of 36.40 ± 2.62 weeks. Elevated inflammatory markers were noted, with D-Dimers elevated in 46.8%, serum ferritin in 66%, and C-reactive protein (CRP) in 48.9% of patients. The most common symptoms included respiratory distress (74.5%), poor feeding (38.3%), and fever (29.8%). Significant findings included pulmonary hypertension in 27.7% of the patients, patent ductus arteriosus in 23.4%, and other cardiovascular anomalies. Complications such as sepsis (70.2%), oxygen dependency (21.3%), and renal failure (29.8%) were prevalent. The mortality rate was 12.8%. Conclusion: MIS-N presents a severe threat to neonates with COVID-19, demonstrating high rates of serious complications and a notable mortality rate. Echocardiography is essential for early detection of cardiac dysfunctions. These findings underscore the urgent need for specific guidelines for the early recognition and management of MIS-N in neonates.

Predictors of language and reading outcomes in 12-year-old children born very preterm.

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.

Impact of Age and of the Patent Ductus Arteriosus on Pulmonary Hemodynamics in Children with Complete Atrioventricular Septal Defect.

Complete atrioventricular septal defect (CAVSD) can lead to the development of pulmonary obstructive vascular disease due to high pulmonary blood flow and pressures. This study aimed to evaluate the changes in pulmonary hemodynamics with aging and with patent ductus arteriosus (PDA) in children with CAVSD. We retrospectively evaluated 137 children (94% with trisomy 21, median age of 195 (25-2963) days, 58.4% female) with CASVD referred to cardiac catheterization from January 2000 to December 2020. Those with associated congenital heart disease, except PDA, had been excluded. They were divided into three age terciles (T1, T2, and T3). Aging was directly associated with higher mean (T1: 34.2 ± 9.1; T2: 37.1 ± 5.8; T3: 42 ± 10.6 mmHg, p < 0.001) and diastolic (T1: 19.4 ± 5.3; T2 21.6 ± 5.0; T3: 26.0 ± 9.5 mmHg, P < 0.001) pulmonary arterial pressures, and with higher pulmonary vascular resistance (T1: 3.24 ± 1.69, T2: 3.47 ± 1.19; T3: 4.49 ± 3.91 Wu.m2, p = 0.023). This resulted in a loss of eligibility for anatomical correction, which became evident only after 300 days of age. PDA was associated with a higher mean (37.2 [35.9; 38.5] vs. 41.3 [37.5; 45.0] mmHg, p = 0.049) and diastolic (21.7 [20.7; 22.6] vs. 26.4 [24.1; 29.0] mmHg, p = 0.001) pulmonary pressure, and resistor-compliance time (0.28 [0.26; 0.29] vs. 0.36 [0.31; 0.40], p = 0.001) after adjusting for age and sex. In children with CAVSD, aging was associated with worsening of pulmonary vascular hemodynamics, particularly when PDA was associated, resulting in loss of eligibility for anatomical correction after 10 months of age as the first surgical option.

Prevalence and Association of Congenital Heart Disease with Hirschsprung’s Disease

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 &lt; 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 &lt; 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.

A retrospective study of the incidence, patterns, and risk factors of retinopathy of prematurity in infants with birth weight &gt;1500 g in a South Indian tertiary care hospital

Abstract: OBJECTIVE: The objective of the study was to determine the incidence, patterns, and risk factors for the development of retinopathy of prematurity (ROP) in infants weighing &gt;1500 g at birth. MATERIALS AND METHODS: A retrospective analysis was done of the ROP screening records between April 2019 and March 2022. All the infants with birth weight &gt;1500 g who underwent ROP screening were included. Data collected included demographic details, details of the risk factors (respiratory distress syndrome [RDS], hypoxia, anemia, septicemia, patent ductus arteriosus, congenital heart disease [CHD], i.e., atrial septal defects or ventricular septal defects, and multiple gestations), and ocular examination findings. Statistical analyses included calculation of mean, range, Chi-square test, and bivariate logistic regression for associations of risk factors with ROP. RESULTS: ROP was noted in 16.6% of the 336 eyes of 168 infants. The mean birth weight and mean gestational age (GA) of the infants were 1850 ± 33 g and 33.33 weeks ± 1.71 weeks, respectively. Eighteen eyes (32.1%) had Stage I, 17 eyes (30.35%) had Stage II, and 11 eyes (19.64%) had Stage III ROP in Zone II. Aggressive ROP was seen in 8 eyes (14.28%) and stage 5 ROP in two eyes (0.59%). Male gender, GA, CHD, and RDS showed a statistically significant association with the development of ROP in these infants. CONCLUSION: ROP was seen in 16.66% of our subjects. Treatment was required in 6.25% of infants. Risk factors for the development of ROP were low gestational age, male gender, the presence of RDS, and congenital heart disease.

Establishing a nomogram for predicting necrotizing enterocolitis in premature infants: A retrospective multicenter cohort study

BackgroundTo construct a nomogram for predicting necrotizing enterocolitis (NEC) in preterm infants. MethodsA total of 4,724 preterm infants who were admitted into 8 hospitals between April 2019 and September 2020 were initially enrolled this retrospective multicenter cohort study. Finally, 1,092 eligible cases were divided into training set and test set based on a 7:3 ratio. A univariate logistic regression analysis was performed to compare the variables between the two groups. Stepwise backward regression, LASSO regression, and Boruta feature selection were utilized in the multivariate analysis to identify independent risk factors. Then a nomogram model was constructed based on the identified risk factors. ResultsRisk factors for NEC included gestational diabetes mellitus, gestational age, small for gestational age, patent ductus arteriosus, septicemia, red blood cell transfusion, intravenous immunoglobulin, severe feeding intolerance, and absence of breastfeeding. The nomogram model developed based on these factors showed well discriminative ability. Calibration and decision curve analysis curves confirmed the good consistency and clinical utility of the model. ConclusionsWe developed a nomogram model with strong discriminative ability, consistency, and clinical utility for predicting NEC. This model could be valuable for the early prediction of preterm infants at risk of developing NEC.

Erratum to: Use of paracetamol for treatment of patent ductus arteriosus in preterm neonates: A 5-year experience from a tertiary hospital in India

Erratum to: Use of paracetamol for treatment of patent ductus arteriosus in preterm neonates: A 5-year experience from a tertiary hospital in India

Стентування відкритої протоки як перший етап хірургічного лікування тетради Фалло з незливними гілками легеневої артерії

The aim – to show the results of patent ductus arteriosus (PDA) stenting as the first stage of palliative treatment in patients with tetralogy of Fallot with non-confluent branches of the pulmonary artery.Materials and methods. This retrospective, single-center study included 10 consecutive patients who underwent PDA stenting since 2013 to 2023. The average age at the time of surgery was 96.00 ± 82.92 days (range 4 to 411 days), the average weight was 5.10 ± 1.78 kg (range 2.8 to 11 kg). The arterial oxygen saturation level (SaO2) before the intervention was 72.0 ± 6.7 % (range 60 to 87 %).Results. There were no intraoperative complications in all patients of this group. The length of the stay in the intensive care unit after surgery was 5.00 ± 1.36 days. SaO2 level increased to 89.00 ± 4.08 %. The duration of artificial lung ventilation after surgery was 36.5 ± 18.00 hours, and the duration of inotropic support was 81.1 ± 33.8 hours. In the long term, all patients showed significant growth of the native pulmonary artery branches. All patients of this group achieved total repair of this pathology after 192.00 ± 60.28 days, on average. At the moment of total repair, the size of the left branch of the pulmonary artery increased from 3.9 ± 0.8 mm to 7.8 ± 1.6 mm, and the right branch increased from 5.8 ± 1.2 mm to 7.70 ± 1.04 mm; the Nakata index increased from 133.0 ± 29.1 mm2/m2 to 241.2 ± 97.8 mm2/m2; end-diastolic index of the left ventricle increased from 31.4 ± 10.8 ml/m2 to 43.1 ± 11.4 ml/m2.Conclusion. PDA stenting in patients with tetralogy of Fallot combined with non-confluent branches of the pulmonary artery is an effective palliative procedure. This method allows to postpone the total repair to an older age, ensures sufficient and symmetric growth of the branches of pulmonary artery, and helps to avoid risks associated with repeat open heart surgeries.

Comparative transcatheter occlusion of patent ductus arteriosus: multicenter collaborative study across pediatric and veterinary cardiology centers

Introduction/ObjectiveTranscatheter therapeutics have revolutionized treatment of patent ductus arteriosus (PDA). Greater understanding of corrective interventions across species can advance best practices, protocols, and outcomes while minimizing adverse events. The objective of this study was to describe characteristics and outcomes in children and dogs undergoing transcatheter PDA occlusion. Animals, Materials and MethodsThis was a multicenter, retrospective cohort study from two pediatric and three veterinary centers. Demographics, procedural characteristics, and outcomes were assessed. ResultsData included 202 children and 106 dogs treated from July 2019 to June 2021. Forty-five (23%) children and 19 (18%) dogs had congestive heart failure prior to catheterization. Transvenous and transarterial approaches for deployment were most used in children and dogs, respectively. All children had percutaneous vascular access compared to 17 (16%) dogs. Intraprocedural anticoagulation was standard for children (100% of 165 reported), but not for dogs (2/103). The median (interquartile range) pulmonary ostium diameter in children was 2.2 mm (1.5–3.0 mm) and 3.0 mm (2.0–4.2 mm) in dogs when all body sizes were considered (P<0.001). Amplatzer™ Duct Occluder was most commonly used in children (64/202, 32%); the Amplatz® Canine Duct Occluder was used in dogs (96/100, 96%). Closure was manual compression in all children, whereas vessel ligation was most used in dogs (74/104, 73%). Successful device deployment was achieved in 197 (98%) children and 98 (93%) dogs (P=0.03). Major reasons for failure included device embolization in six (2.9%) children and PDA morphology concerns in four (3.8%) dogs. ConclusionsTranscatheter PDA occlusion is successful in children and dogs. Study data might be useful for optimizing transcatheter therapeutics and animal models for interventional cardiology.

Congenital heart disease cardiac catheterization at Uganda Heart Institute, a 12-year retrospective study of immediate outcomes

BackgroundCardiac catheterization is an invasive diagnostic and treatment tool for congenital heart disease (CHD) with potential complications.ObjectiveTo describe the immediate outcomes of patients who underwent cardiac catheterization for CHD at the Uganda Heart Institute (UHI).MethodsThe 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.ResultsWe 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).ConclusionMany 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.

Impact of a Multidisciplinary Service Line and Program for Transcatheter Device Closure of the Neonatal Ductus Arteriosus.

Outline a quality initiative establishing an institutional service line for neonatal transcatheter device closure of the patent ductus arteriosus (TDC-PDA). A retrospective descriptive observational study surrounds programmatic approach to TDC-PDA in premature neonates with process measure spanning education, implementation, referral, and post-procedural care. Metrics tracked pre- and post-program creation with statistical analyses performed. Neonatal TDC-PDA referrals increased exponentially since program inception (n = 13 in year prior; n = 42year 1; n = 74year 2), especially in patients weighing less than 1.3kg (12.5%; 55%; 50%), and were associated with an increased procedural success rate (81%; 95%; 99%). Procedural checklist creation decreased procedural "out of isolette" time (median 93min; 59; 52), and procedural-related complication or clinical sequelae (19%; 12%; 4%). A multidisciplinary service line and program dedicated to neonatal TDC-PDA can result in a significant increase in referrals as well as procedural efficacy and safety for this medically fragile population.

Health comorbidities in children with down syndrome (DS) at Dr. Sardjito General Hospital, Yogyakarta

Down syndrome (DS) is a disease caused by trisomy of chromosome 21. The phenotype in DS leads to manifestations in several organ systems. This study aimed to identify the pattern of comorbidities in DS patients. It was a single-center, cross-sectional study at Dr. Sardjito General Hospital, Yogyakarta. Medical records of pediatric patients with DS from a period of January 2022 to May 2023 were included. Descriptive analysis was performed to demonstrate demographic and clinical characteristics. A total of 355 pediatric patients with DS were found at Dr. Sardjito General Hospital and the majority were male (196 children or 55.2%). As much as 339 children (95.49%) had comorbidities. The highest comorbidity was congenital heart disease (230 patients or 67.84%) in specifics were atrial septal defect (41 patients or 12.39%), atrioventricular septal defect (29 patients or 8.17%), and patent ductus arteriosus (28 patients or 7.88%). The second highest comorbidity was endocrine system disorders (102 patients or 30.09%), with 100 patients (28.16%) children suffering hypothyroidism. The number of children who had one comorbidity was 248 patients (69.86%), 74 patients (20.48%) had two comorbidities, and 17 patients (4.79%) had three or more comorbidities. The highest co-prevalence of the two comorbidities was congenital heart disease and endocrine system disorders (36 patients or 10.14%). The highest co-prevalence of 3 or more comorbidities was a combination of congenital heart disease, visual impairment, and hearing impairment (6 patients or 1.69%). In conclusion, 95.49% of children with DS have comorbidities. The most common comorbidity was heart defects. About 25.63% of patients had more than one comorbidity. Children with DS who have comorbidities require more attention to prevent complications and to reduce morbidity.

The Potential of Ambroxol as a Panacea for Neonatal Diseases: A Scoping Review.

Ambroxol, a commonly used mucolytic agent, has been extensively studied for its clinical effectiveness in managing respiratory conditions in pediatric and adult patients. The existing body of research on ambroxol demonstrates its safety and efficacy. However, its potential role in preventing and treating neonatal diseases still needs to be explored. This scoping review aims to shed light on the unexplored potential of ambroxol, particularly its applications in perinatal and neonatal care. We aim to offer valuable insights for healthcare professionals, researchers, and academics, thus presenting a positive perspective. Key scientific databases such as Google Scholar, PubMed, Cochrane Library, and Europe PMC were meticulously searched for relevant literature on ambroxol in perinatal and neonatal medicine. Gray literature was also surveyed, and the search encompassed all study designs and languages up to June 2024. Furthermore, citations and reference lists of relevant articles were scrutinized to identify additional pertinent literature. Ambroxol has demonstrated promising effects in preventing and managing respiratory distress syndrome (RDS). It can enter the placental circulation and rapidly build up in human lung tissue to a much greater extent than in plasma. It promotes fetal lung maturation, surfactant production, and alveolar expansion. Numerous studies have demonstrated the efficacy of antenatal and postnatal ambroxol in the prevention and treatment of RDS. Ambroxol has the potential to be administered intravenously or through nebulization, offering the hopeful possibility of reducing the high failure rate typically associated with non-invasive ventilation in extremely preterm infants, instilling a sense of hope and optimism about the potential of ambroxol. It also shows potential in treating bronchopulmonary dysplasia, meconium aspiration syndrome, and neonatal infections. Ambroxol has been observed to assist in the closure of patent ductus arteriosus in preterm infants by inhibiting vasodilator agents such as nitric oxide and exerting vasoconstrictive properties. However, these biological actions may raise concerns regarding the potential induction of pulmonary hypertension and an increased risk of necrotizing enterocolitis. The present scoping review also examines the clinical evidence and the potential of ambroxol in reducing the incidence of intraventricular hemorrhage in preterm infants. Ambroxol may have potential analgesic properties in managing neonatal pain, and as it can penetrate the blood-brain barrier, it suggests potential neuroprotective properties. These properties may encompass the modulation of microglial activation and the antagonistic impact on glutamate receptors. Ambroxol's attributes could contribute to a decreased susceptibility to neurological complications and have demonstrated anticonvulsant effects in preclinical studies. While low-to-moderate-quality evidence indicates potential applications of ambroxol in neonatal care, further research is needed to determine the drug's optimal dosing, timing, and safety profiles in this patient population. We need to investigate ambroxol's potential synergistic effects with antenatal steroids. Exploration is required to assess ambroxol's potential in reducing the high failure rate associated with non-invasive respiratory support for RDS. Lastly, comprehensive studies on the long-term neurodevelopmental outcomes of neonates exposed to ambroxol are essential.

Use of cardiac gated computed tomography in the diagnosis of a rare congenital cardiovascular anomaly in an adult alpaca.

A 5-year-old female alpaca was presented with respiratory distress and lethargy. Thoracic radiographs revealed a cranioventrally distributed alveolar pattern, caudodorsal bronchial pattern, cardiomegaly, increased soft tissue opaque content in the ventral thorax, and rounded soft tissue opaque structures craniodorsal to the carina. Cardiac gated CT demonstrated a patent ductus arteriosus, ventricular septal defect, complete left atrioventricular valve atresia, partial anomalous venous connections from the cranial pulmonary veins to the azygous and cranial vena cava, severe right-sided cardiomegaly, pleural and peritoneal fluid, and severe hepatic congestion. These findings were confirmed with necropsy.

Facial dysmorphism and Limb abnormalities of Fetal Valproate Syndrome.

A neonate born to nonconsanguineous parents was evaluated for dysmorphic features. The neonate was born at term by normal vaginal delivery. The mother has had epilepsy for 12 years and has been on sodium valproate (700 mg/day) since conception and throughout pregnancy. Examination revealed facial dysmorphism, including triangular forehead, sparse arched eyebrows, telecanthus, flat nasal bridge, long thin upper vermilion border, smooth philtrum, and low-set ears. The limb anomalies observed were arachnodactyly, wrist and elbow contractures, clinodactyly, hypoplastic toenails, and overlapping toes. The other dysmorphisms noted were widely spaced nipples and hypospadias. Ultrasonogram (USG) cranium showed bilateral choroid plexus cysts, and USG abdomen revealed bilateral mild hydronephrosis. 2D-Echocardiography revealed a small patent ductus arteriosus (PDA). A diagnosis of fetal valproate syndrome (FVS) was considered, and other differentials, including fetal alcohol syndrome and genetic conditions, were ruled out by a clinical geneticist review. The index neonate is currently on multidisciplinary follow-up. The index neonate had common features of FVS as described in the literature, in addition to wrist and elbow contractures. Apart from the anomalies, there is a significant risk for neurocognitive delay and neurodevelopmental disorders. Postnatally, these babies need multidisciplinary care and neurodevelopmental follow-up. Valproate use for treating epilepsy in pregnant women and women of childbearing age may be restricted, and alternative choices of ASMs with better safety profiles should be preferred. Facial dysmorphism and limb anomalies in fetal valproate syndrome may occur irrespective of the dose of sodium valproate. Fetal valproate syndrome may present with wrist and elbow contractures.