Ductal-dependent Lesions Research Articles

Outcomes and characteristics in term infants with necrotising enterocolitis and CHD.

CHD is a significant risk factor for the development of necrotising enterocolitis. Existing literature does not differentiate between term and preterm populations. Long-term outcomes of these patients are not well understood. The aim was to investigate the baseline characteristics and outcomes of term normal birth weight infants with CHD who developed necrotising enterocolitis. A retrospective review was performed of infants from a single tertiary centre with CHD who developed necrotising enterocolitis of Bell's Stage 1-3, over a ten-year period. Inclusion criteria was those born greater than 36 weeks' gestation and birth weight over 2500g. Exclusion criteria included congenital gastro-intestinal abnormalities. Sub-group analysis was performed using Fisher's exact test. Twenty-five patients were identified, with a median gestational age of 38 weeks. Patients with univentricular physiology accounted for 32% (n = 8) and 52% of patients (n = 13) had a duct-dependent lesion. Atrioventricular septal defect was the most common cardiac diagnosis (n = 6, 24%). Patients with trisomy 21 accounted for 20% of cases. Mortality within 30 days of necrotising enterocolitis was 20%. Long-term mortality was 40%, which increased with increasing Bell's Stage. In total, 36% (n = 9) required surgical management of necrotising enterocolitis, the rate of which was significantly higher in trisomy 21 cases (p < 0.05). Not previously described in term infants is the high rate of trisomy 21 and atrioventricular septal defect. This may reflect higher baseline incidence in our population. Infants with trisomy 21 were more likely to develop surgical necrotising enterocolitis. Mortality at long-term follow-up was high in patients with Bell's Stage 2-3.

Cumulative Dose of Prostaglandin E1 Determines Gastrointestinal Adverse Effects in Term and Near-Term Neonates Awaiting Cardiac Surgery: A Retrospective Cohort Study.

This study aimed to assess the association between treatment characteristics of prostaglandin E1 including initiation time and duration, maximal and cumulative doses, and adverse effects. A retrospective cohort study in which medical records of neonates with duct-dependent lesions were studied for treatment parameters and adverse effects. Multivariable logistic regression model was applied for testing the effect PGE1 variables on outcomes. The primary outcomes of this study were association of adverse effects of PGE1 treatment with maximal dose, cumulative dose, and treatment duration. The secondary outcomes included safety of feeding in infants treated with PGE1. Eighty-two infants with duct-dependent lesions receiving PGE1 were included. Several infants who received early PGE1 treatment required ventilation support. Feeds were ceased more often as the cumulative dose and duration of PGE1 treatment increased. Gastrointestinal adverse effects were significantly associated with the cumulative dose of PGE1 and treatment duration. Apneas, hyperthermia, and tachycardia were associated with maximal dose. Our data did not demonstrate a difference in the incidence of NEC associated with characteristics of PGE1 treatment. Cumulative PGE1 dose is associated with gastrointestinal adverse effects in neonates. Lower doses should be considered in neonates expecting prolonged PGE1 treatment.

Outcomes of neonatal critical congenital heart disease: results of a prospective registry-based study from South India

ObjectivesCongenital heart disease (CHD) is now a leading contributor of infant and neonatal mortality in many low/middle-income countries including India. We established a prospective neonatal heart disease registry in Kerala...

Multicenter Evaluation of Pre-operative Feeding in Infants with Ductal Dependent Circulation.

Enteral feeds are often withheld from neonates with ductal dependent cardiac lesions who are receiving prostaglandins. This is despite positive benefits of enteral feeding. We describe a multicenter cohort of these neonates who were fed pre-operatively. We also give a granular description of vital sign measurements and other risk factors prior to feeding. A retrospective chart review was performed at seven centers. Inclusion criteria were full-term neonates under one month of age with ductal dependent lesions receiving prostaglandins. These neonates were fed for at least 24h during the pre-operative period. Premature neonates were excluded. Using the inclusion criteria, 127 neonates were identified. While being fed, 20.5% of the neonates were intubated, 10.2% were on inotropes, and 55.9% had an umbilical arterial catheter in place. Median oxygen saturations in the six hours prior to feeding were 92.5% in patients with cyanotic lesions, median diastolic blood pressure was 38mmHg and median somatic NIRS were 66.5%. The median peak daily feeding volume reached was 29ml/kg/day (IQ range 15.5-96.8ml/kg/day). One patient developed suspected necrotizing enterocolitis (NEC) in this cohort. Only one adverse event occurred, which was an aspiration thought to be related to feeding, but did not result in intubation or cessation of feeds. NEC was rare among neonates with ductal dependent lesions while receiving enteral nutrition pre-operatively. Umbilical arterial catheters were in place in the majority of these patients. Hemodynamic measures demonstrated a high median oxygen saturation prior to initiation of feeds.

Inhaled Nitric Oxide and Higher Necrotizing Enterocolitis Rates in Congenital Heart Disease Patients

IntroductionCertain congenital cardiac lesions are at increased risk for the development of necrotizing enterocolitis (NEC). These patients are often reliant on pulmonary and systemic vasomodulators to maintain adequate perfusion and oxygenation. This study sought to determine whether pulmonary or systemic vasodilator treatment is protective against the development of NEC in this population. MethodsWe utilized International Classification of Diseases (ICD) codes to identify high risk congenital cardiac disease patients ≤6 mo of age, cared for at a tertiary children's hospital between January 2011 and January 2021. Cardiac anomalies were stratified into ductal dependent (pulmonary DD-P or systemic DD-S) or independent lesions. The rate of NEC development in those who received vasodilators (inhaled nitric oxide [iNO], pulmonary vasodilators, systemic vasodilators) was compared to controls in a multivariate analysis. ResultsOf the 352 patients, who met inclusion criteria, 77.6% had ductal dependent lesions (DD-S 41.9%, DD-P 35.7%), 19.5% received iNO, and 37.5% received other vasodilatory drugs. The overall NEC rate was 15.1%. On univariate analysis, DD-S, iNO use, and systemic vasodilators was associated with a significantly higher risk of NEC, while DD-P was associated with lower NEC risk. On multivariate analysis, only iNO (odds ratio 2.725, confidence interval [1.36-5.44]) and DD-S (odds ratio 2.279, confidence interval [1.02-5.11]) were independent risk factors for NEC. ConclusionsIn patients with at-risk congenital cardiac disease lesions, a ductus dependent systemic circulation or iNO treatment is associated with an increased risk of developing NEC. The presence of iNO or DD-S should be utilized as markers of increased risk both in the prevention and workup of suspected NEC.

Safety and Feasibility of Skin-to-Skin Contact in the Delivery Room for High-Risk Cardiac Neonates.

Early skin-to-skin contact (SSC), beginning in the delivery room, provides myriad health benefits for mother and baby. Early SSC in the delivery room is the standard of care for healthy neonates following both vaginal and cesarean delivery. However, there is little published evidence on the safety of this practice in infants with congenital anomalies requiring immediate postnatal evaluation, including critical congenital heart disease (CCHD). Currently, the standard practice following delivery of infants with CCHD in many delivery centers has been immediate separation of mother and baby for neonatal stabilization and transfer to a different hospital unit or a different hospital altogether. However, most neonates with prenatally diagnosed congenital heart disease, even those with ductal-dependent lesions, are clinically stable in the immediate newborn period. Therefore, we sought to increase the percentage of newborns with prenatally diagnosed CCHD who are born in our regional level II-III delivery hospitals who receive mother-baby SSC in the delivery room. Using quality improvement methodology, through a series of Plan-Do-Study-Act cycles we successfully increased mother-baby skin-to-skin contact in the delivery room for eligible cardiac patients born across our city-wide delivery hospitals from a baseline 15% to greater than 50%.

Inhaled Nitric Oxide Treatment Is Associated with Higher Rates of Necrotizing Enterocolitis in At-risk Congenital Cardiac Disease Patients

INTRODUCTION: Certain congenital cardiac lesions are at increased risk for the development of necrotizing enterocolitis (NEC). These patients with these lesions are often reliant on pulmonary and systemic vasomodulators to maintain adequate perfusion and oxygenation. This study sought to determine whether pulmonary or systemic vasodilator treatment is protective against the development of NEC in this population. METHODS: We used ICD codes to identify high-risk congenital cardiac disease patients ≤6 months of age, cared for at a tertiary children’s hospital between January 2011 and January 2021. Cardiac anomalies were stratified into ductal-dependent (pulmonary DD-P or systemic DD-S) or independent lesions. The rate of NEC development in those who received vasodilators (inhaled nitric oxide [iNO], pulmonary vasodilators, systemic vasodilators) was compared with controls in a multivariate analysis. RESULTS: Of the 353 patients who met inclusion criteria, 77.6% had ductal-dependent lesions (DD-S 41.9%, DD-P 35.7%), 19.5% received iNO, and 51.8% received other vasodilatory drugs. The overall NEC rate was 15.3%. On univariate analysis, DD-S, iNO use, and systemic vasodilators had a significantly higher risk of NEC, and DD-P was associated with lower NEC risk (Figure). On multivariate analysis, only iNO (odds ratio 2.725 [CI 1.36 to 5.44]) and DD-S (odds ratio 2.279 [CI 1.02 to 5.11]) were independent risk factors for NEC.FigureCONCLUSION: In patients with at-risk congenital cardiac disease lesions, a ductus-dependent systemic circulation and/or iNO treatment increases the risk of developing NEC. The presence of iNO or DD-S should be used as a marker of increased risk both in the prevention and workup of suspected NEC.

Effect of standardized vs. local preoperative enteral feeding practice on the incidence of NEC in infants with duct dependent lesions: Protocol for a randomized control trial.

BackgroundInfants with duct dependent heart lesions often require invasive procedures during the neonatal or early infancy period. These patients remain a challenge for pediatric cardiologists, neonatologists, and intensive care unit personnel. A relevant portion of these infant suffer from respiratory, cardiac failure and may develop NEC, which leads to inadequate growth and nutrition, causing delayed or complicated cardiac surgery.MethodsThis randomized control trial will recruit term infants diagnosed with a duct dependant lesion within the first 72 h of life. After obtaining written parental consent patients will be randomized to either the physician led enteral feeding or protocol-based feeding group. The intervention will continue up to 28 days of life or day of cardiosurgical treatment, whichever comes first. The primary outcomes include NEC and death related to NEC. Secondary outcomes include among others, number of interrupted feedings, growth velocity, daily protein and caloric intake, days to reach full enteral feeding and on mechanical ventilation.DiscussionOur study will be the first randomized control trial to evaluate if standard (as in healthy newborns) initiation and advancement of enteral feeding is safe, improves short term outcomes and does not increase the risk of NEC. If the studied feeding regime proves to be intact, swift implementation and advancement of enteral nutrition may become a recommendation.Trial registrationThe study protocol has been approved by the local ethical board. It is registered at ClinicalTrials.gov NCT05117164.

Analysis of clinical features of neonates with congenital heart disease who develop necrotizing enterocolitis: a retrospective case-control study.

BackgroundInfants with congenital heart disease (CHD) are known to have higher rates of necrotizing enterocolitis (NEC). Although the etiology is recognized as distinct from the premature neonatal population, there is not a universal consensus regarding etiology or specific risk factors. To analyze the clinical features of neonates with CHD who develop NEC.MethodsA retrospective study of neonates with CHD in the cardiac intensive care unit (ICU) between 2015 and 2018 was performed, and modified Bell’s criteria were used to diagnose NEC. Patients were divided into 2 groups according to ductal-dependent (DD) lesions, and were further stratified by Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1) score and Aristotle score, to compare the differences.ResultsAmong 412 patients with CHD, 69 (16%) developed NEC. The incidence of NEC was notably higher among DD patients than among non-DD (nDD) patients (18.7% vs. 11.1%; P=0.04). Patients with RACHS-1 >2 also had a higher rate of NEC than did those with RACHS-1 ≤2 (19.49% vs. 9.29%; P=0.01). nDD patients who developed NEC were younger, had a lower gestational age (36.25±1.88 vs. 38.10±1.28 weeks; P=0.00), a lower weight (2.86±0.85 vs. 3.33±0.55 kg; P=0.01), and a lower birth weight (2.79±0.79 vs. 3.26±0.55; P=0.01) compared to the DD group. All nDD patients developed NEC after congenital heart surgery, while only 38 cases (76%), NEC occurred after heart surgery in the DD group. Four patients needed surgery for NEC in the DD group and RACHS-1 >2 group. Presence of NEC was not associated with an increased risk of mortality in any group.ConclusionsNEC is a common complication in neonates with CHD and can occur both before and after CHD operations. Likely there are varying mechanism for NEC in different forms of CHD. While NEC is more common in patients with DD CHD and those with more complex forms of CHD, there was no significant difference observed in weight-for-age Z-score (WAZ) between the DD group during follow-up.

Role of Echocardiography in Ductal Stenting

Patent ductus arteriosus stenting has been acknowledged as a reliable alternative to palliative shunt surgeries in neonates with duct-dependent circulation. This procedure is technically challenging and can have serious complications; hence, a thorough evaluation of the case and preprocedural planning is required. Transthoracic echocardiography (TTE) remains the first diagnostic modality for all congenital heart defects. This article focuses on the role of TTE in diagnosis and transcatheter management of duct-dependent lesions.

Patent ductus arteriosus stenting versus modified Blalock-Taussig shunt for palliation of duct-dependent pulmonary blood flow lesions.

Patent ductus arteriosus (PDA) stenting is an alternative to modified Blalock-Taussig shunt (MBTS) as first-stage palliation of duct-dependent lesions. The superiority of one approach over the other is still controversial. Our objective was to compare PDAstent versusMBTS for palliation in regard to safety, efficacy, and efficiency. From 2010 to 2021, 134 patients had first-stage palliation with either PDAstent (n = 83) or MBTS (n = 51). Twenty-seven patients failed the primary treatment and were converted to the other group. The study endpoints were hospital outcomes, interstage reintervention, and concomitant procedures at the second-stage palliation. Patients with PDAstent were significantly younger. The prevalence of antegrade pulmonary blood flow (PBF) was higher in patients who had MBTS and graft thrombosis was higher in the PDAstent. Hospital stay was significantly longer in patients who had MBTS. Predictors of prolonged mechanical ventilation were low-weight, MBTS, and conversion. Intensive careunit stay significantly increased with conversion, low-weight, and antegrade PBF. The interstage intervention was required more frequently in PDA-stent group. Predictors of reintervention were conversion and pulmonary atresia with the intact interventricular septum. Pulmonary artery plasty was required more frequently during the second-stage palliation in PDA-stent group. PDAstent is an alternative to MBTS for first-stage palliation. It is associated with shorter hospital stays and avoidance of surgery at the expense of a high rate of stent thrombosis and interstage reintervention. Conversion increased the risk of the procedure. More studies are needed to determine factors that affect PDA-stent outcomes and patient selection criteria.

Conception by assisted reproductive technology in infants with critical congenital heart disease in Japan

Research questionWhat is the proportion of infants born as a result of assisted reproductive technology ART across different types of neonatal critical congenital heart disease (CCHD) in a Japanese population? DesignA retrospective analysis of 418 consecutive infants with CCHD that required catheter treatment or surgery within the first 28 days of life or ductal-dependent lesions, in two paediatric centres in Japan, between January 2014 and December 2019. The proportion of ART in infants with each type of CCHD was evaluated. The proportion of ART in infants with univentricular heart defect (UVH) compared with those with biventricular heart defect (BVH) was evaluated. ResultsThe study group included 229 boys and 189 girls, with a gestational age of 38 ± 2 weeks. Overall, 61 infants (14.6%) were conceived by fertility treatment with 46 (11.0%) conceived by ART. Univentricular heart defect and BVH were identified in 111 infants (26.6%) and 307 infants (73.4%), respectively. The proportion of infants conceived by ART was significantly higher in UVH (16.2%) than in BVH (9.1%) (OR 2.28, 95% CI 1.11 to 4.68, P = 0.025), regardless of maternal age and maternal history of miscarriage. ConclusionsThe proportion of ART in infants with CCHD, especially UVH, was high. These findings could form the basis of a rationale for carrying out fetal echocardiography in fetuses conceived by ART.

Duct-dependent cyanotic neonates with patent ductus arteriosus stenting pose a unique challenge for the next stage of surgical intervention

Objective To study the role of patent ductus arteriosus (PDA) stenting in the management of single-ventricle patients and the surgical difficulties facing the surgeons in the next stages of surgical procedures. Background Thirty consecutive neonates with cyanotic duct-dependent lesions were initially palliated with (PDA) stenting in our center. Eighteen of them had undergone one or more surgical procedures. We retrospectively assessed the indexed growth of pulmonary arteries (PA) over a period of time and the requirement of further rehabilitation of PAs in these patients. Patients and methods From January 2014 to December 2018, 30 neonates with cyanotic congenital heart diseases underwent PDA stenting as initial palliation; 60% were ventilated before the procedure, which resulted in 100% success. All patients were followed up regularly by monitoring of PA size imaging. Eighteen patients of this group underwent further surgical procedures. Results Ninety percent of the neonates had stenosis of the PA branches before PDA stenting. The indexed PA branch size was significantly smaller, which increased after stenting of PDA. At the time of the next surgical procedures (Glenn, etc.), stent division/ligation was required in addition to PA plasty in 90% of patients. Conclusions PDA stenting provides efficient palliation in cyanotic neonates. Despite the origin of stenosis, the stent allowed growth in the size of both PAs. However, the majority required surgical augmentation and further stenting of PAs in the mid-term follow-up.

CARDIAC EVALUATION IN NEWBORNS: AN ECHOCARDIOGRAPHY BASED STUDY

Objective: To evaluate the cardiac status of newborns in terms of structural and functional heart diseases.&#x0D; Methodology: This descriptive study was conducted at Paediatric Cardiology Department of “The Children’s Hospital and The Institute of Child Health”, Multan, Pakistan, from January 2017 to December 2019. Neonates aged 0-28 days and referred for echocardiographic evaluation were enrolled. Diagnostic findings were confirmed with the assistance of echocardiography. We were focused on analyzing total number and types of heart diseases along with gender distribution during the study period.&#x0D; Results: During the study period, a total of 2729 newborns were evaluated. We noted 1523 (55.8%) newborns as structurally and functionally normal, 866 (31.7%) had congenital heart disease, 69 (2.5%) duct dependent lesions, 248 (9.1%) persistent pulmonary hypertension and 23 (0.8%) left ventricular (LV) dysfunction but with structurally normal heart. Newborns with LV dysfunction might have hypoxic myocardial damage, metabolic derangements or acute myocarditis. Ventricular Septal Defect (VSD) followed by Patent Ductus Arteriosus (PDA) and Atrial Septal Defect (ASD) were the most frequent types of acyanotic CHD observed in 248 (28.2%), 171 (19.7%) and 100 (11.5%) neonates respectively while Tetralogy of Fallot (TOF) was the most common type of cyanotic CHD 74 (8.5%).&#x0D; Conclusion: VSD followed by PDA, ASD and TOF were the most common types of CHD among neonates. Diagnosis of congenital heart defects in the early age is pointing towards improvement in healthcare facilities.

CARDIAC EVALUATION IN NEWBORNS: AN ECHOCARDIOGRAPHY BASED STUDY

Objective: To evaluate the cardiac status of newborns in terms of structural and functional heart diseases. Methodology: This descriptive study was conducted at Paediatric Cardiology Department of “The Children’s Hospital and The Institute of Child Health”, Multan, Pakistan, from January 2017 to December 2019. Neonates aged 0-28 days and referred for echocardiographic evaluation were enrolled. Diagnostic findings were confirmed with the assistance of echocardiography. We were focused on analyzing total number and types of heart diseases along with gender distribution during the study period. Results: During the study period, a total of 2729 newborns were evaluated. We noted 1523 (55.8%) newborns as structurally and functionally normal, 866 (31.7%) had congenital heart disease, 69 (2.5%) duct dependent lesions, 248 (9.1%) persistent pulmonary hypertension and 23 (0.8%) left ventricular (LV) dysfunction but with structurally normal heart. Newborns with LV dysfunction might have hypoxic myocardial damage, metabolic derangements or acute myocarditis. Ventricular Septal Defect (VSD) followed by Patent Ductus Arteriosus (PDA) and Atrial Septal Defect (ASD) were the most frequent types of acyanotic CHD observed in 248 (28.2%), 171 (19.7%) and 100 (11.5%) neonates respectively while Tetralogy of Fallot (TOF) was the most common type of cyanotic CHD 74 (8.5%). Conclusion: VSD followed by PDA, ASD and TOF were the most common types of CHD among neonates. Diagnosis of congenital heart defects in the early age is pointing towards improvement in healthcare facilities.

P0173 / #868: CAN A TELEPHONE TRIAGE TOOL AID DECISION MAKING AND PRIORITISATION OF CRITICALLY UNWELL CHILDREN NEEDING TRANSFER TO PICU?

Aims & Objectives: To assess whether a telephone triage tool can be developed to aid decision making and prioritisation of critically unwell children requiring transfer to a paediatric intensive care unit (PICU) by a retrieval team. Methods: Using paediatric early warning (PEWS) scores and goal directed therapy parameters; a draft severity/triaging scoring system was developed by a group of clinicians at a well-established regional paediatric transport service. 100 telephone referrals were retrospectively triaged by 2 blinded clinicians independently using the new triage tool. Notes were selected by electronic randomisation and each referral scored on parameters such as fast track need for intensive care (e.g. duct dependent lesion, need for ECMO etc), PEWS, acid-base status, fluid resuscitation and level of consciousness. Scores were compared with set patient outcomes previously identified as need for transport to PICU, number of organ support required and length of stay on PICU. Further analysis into association with inotrope score and PIM3 score are ongoing. Results: showed that the scores between the two clinicans correlated but it did not show any association between the triage score and identified patient outcome. Patients requiring advice only and no transfer scored higher than those requiring transfers to PICU, only 32% of the referral required transfer (23% to PICU and 9% for tertiary opinions). 51% improved in the DGH with advice only and 17% excluded. Conclusions: At current the triage tool does not predict or help prioritise need for transfer but further analysis is needed using parameters such as inotrope scores and PIM3 scores

Early detection of significant congenital heart disease: The contribution of fetal cardiac ultrasound and newborn pulse oximetry screening.

Fetal cardiac and newborn pulse oximetry screening has greatly facilitated the detection of cardiac abnormalities, which may be serious with potentially dire neonatal consequences. The prenatal diagnosis of a serious cardiac abnormality allows the attending obstetrician to organise the much safer in-utero transfer of the fetus for delivery at a tertiary centre, particularly if there is evidence of a duct-dependent lesion that may require the infusion of Prostaglandin E1 to maintain duct patency pending surgical intervention. Newborn pulse oximetry alerts the paediatrician that the baby may have a significant cardiac abnormality, which warrants further elucidation prior to discharge, rather than for the baby to represent unwell a few days later. Despite these advances, serious cardiac abnormalities may be missed on screening. Their detection then falls back onto the clinical acumen of the attending paediatrician/family physician to review the history, carefully elicit and evaluate the clinical signs further aided by whatever investigations that may be available at the birthing hospital, frequently less resourced than the tertiary centres. At the outset, a brief synopsis is provided of the clinical findings that may point to a cardiac abnormality. That is followed by a critical review of the accuracy of prenatal and newborn pulse oximetry screening with emphasis on the lesions that may be missed. Suggestions are made as to how to improve the diagnostic accuracy.

Anatomic Concordance of Neonatologist-Performed Echocardiography as Part of Hemodynamics Consultation and Pediatric Cardiology

Targeted neonatal echocardiography (TnECHO) performed by neonatologists as part of a hemodynamics consultation is increasingly being used in neonatal intensive care units. To minimize delays in obtaining physiologic data, first echocardiograms may be obtained by the neonatal hemodynamics team and reviewed afterward by a pediatric cardiologist. This practice has not been systematically evaluated. The aim of this study was to compare concordance between anatomic findings on TnECHO and pediatric cardiology reports. This was a retrospective evaluation of 339 infants at low risk for congenital heart disease (CHD) admitted to two large referral centers with established neonatal hemodynamics programs who underwent comprehensive TnECHO as their first postnatal echocardiographic examinations. The protocol included comprehensive imaging of intracardiac anatomy, outflow tract concordance and integrity, aortic arch anatomy, pulmonary vein location and flow, and transitional shunts. The hemodynamics consultation note was compared with the cardiology report to determine anatomic concordance or major or minor discrepancies in all first studies. Anatomic concordance occurred in 97.9% (κ=0.862; 95% CI, 0.762-0.962; P<.001). There were seven minor discrepancies (small muscular ventricular septal defects and coronary fistulas). The index population included 23 infants (6.7%) with CHD, of whom only one (0.3%) had a ductal-dependent lesion (coarctation of the aorta) which was correctly identified by both teams. The rate of major CHD in patients considered eligible for hemodynamics consultation was low, and there was high diagnostic concordance between trained neonatal hemodynamics specialists and pediatric cardiology. First echocardiograms obtained by subspecialty neonatologists may provide imaging of sufficient quality to evaluate a critically unwell neonate with low suspicion for critical CHD lesions. These results should not be extrapolated to infants in whom CHD is suspected. This study highlights the importance of formalized, rigorous, and standardized training for neonatologists with hemodynamics expertise who perform timely assessments using TnECHO.

Abstract 12526: The Risk of Critical Congenital Heart Disease in Children Born After Assisted Reproductive Technology

Introduction: Assisted reproductive technology (ART), which began 40 years ago, has spread all over the world. And 1 in 18 Japanese infants is conceived through ART. But it is known that infants conceived through ART have a high frequency of congenital anomalies and a high birth rate of congenital heart disease (CHD). Because of the high elderly birth rate and advanced medical system in Japan, Japan has the highest number of ART implementations in the world. However, there are no reports on the frequency of CHD in infants conceived through ART in Japan, so we performed this study. Methods: Four hundred eighteen infants diagnosed with critical CHD (lesions requiring catheter treatment or surgery within 28 days of life, or ductal-dependent lesions; CCHD) at the two pediatric heart centers from January 2014 to December 2019, were reviewed retrospectively. Results: In total, 229 males and 189 females were included in this study. The gestational age was 38± 2weeks. Maternal age was 32±5 years and paternal age was 34±6 years. Seventy-eight patients (19%) had chromosome abnormalities, gene abnormalities or syndrome. Sixty-one patients (15%) were conceived through fertility treatment and 46 patients (11%) were conceived through ART. One hundred eleven patients (27%) had univentricular heart defect (UVH). Compared with the proportion of ART (6%) in all births in Japan, the proportion of ART (11%) in CCHD was high. In addition, the proportion of ART in UVH was 20/111 (18%), while the proportion of ART in biventricular heart defect was 30/307 (10%) (p=0.0218). Among them, the highest proportion of ART in hypoplastic left heart syndrome and pulmonary atresia with intact ventricular septum was 7/23 (30%) and 5/19 (26%), respectively. Conclusions: The number of ART implementations in Japan is expected to increase in the future. At present, there are many cases in which infants conceived through ART cannot be diagnosed with CCHD before birth. Because infants conceived through ART might have a high incidence of complex CHD and UVH, they need more careful fetal follow-up than infants conceived naturally.

Serum Sirolimus Levels After Implantation of Third Generation Drug Eluting Cobalt Chromium Coronary Stent in Ductus Arteriosus in Neonates with Duct-Dependent Pulmonary Circulation.

Ductal stenting (DS) palliates duct-dependent lesions using coronary stents. Sirolimus-eluting stents have replaced bare-metal stents in coronary interventions. Concerns exist about sirolimus levels in neonates. Therapeutic immunosuppressive sirolimus level is 5-15ng/ml. After neonatal DS, drug levels were assessed at 24 h, 7days and monthly thereafter till they were undetectable. Clinical course, ductal patency till their final corrective surgery was analyzed. The exact quantity of sirolimus in each stent was known. Twelve neonates with median age of 5.5days received sirolimus-eluting stents, one stent in nine and two in the rest. The lesions were pulmonary atresia intact ventricular septum(PAIVS) in four, univentricular lesions with pulmonary atresia in four, biventricular lesions with pulmonary atresia in three and right ventricular rhabdomyoma in one neonate. If single stents up to 22mm length, 24-h drug levels were less than 5ng/ml. Even though 24-h levels were above 5ng/ml in patients with single longer stent or two stents, it reduced to very low levels by seventh day. Two hospital deaths included rhabdomyoma with complete heart block and post-valvotomy cardiac failure for PAIVS. Stent patency after valvotomy for PAIVS exceeded three years. Patency was retained for 8-27months till their elective corrective surgery in others. Sirolimus levels were acceptable at 24 h in all neonates receiving single stent under 22mm length. In patients needing two stents, drug levels were in immunosuppressive range at 24 h but reduced rapidly within 7days. The palliation provided by sirolimus-eluting DS was sufficiently long to provide clinical benefit.