Ductal-dependent Congenital Heart Disease Research Articles

Comparing Neurodevelopmental Outcomes in Infants With Patent Ductus Arteriosus Stenting Versus Blalock-Taussig-Thomas Shunt: A Pilot Study

BackgroundPatent ductus arteriosus stenting (PDAS) is a nonsurgical alternative to Blalock-Taussig-Thomas shunt (BTTS) for infants with ductal-dependent congenital heart disease. In this single-center study, we aimed to compare neurodevelopmental outcomes in children who underwent BTTS as initial palliation versus PDAS. MethodsBayley Scales of Infant and Toddler Development Screening Test (Bayley-III) reports and mode of feeding data were collected for any patient who underwent PDAS or BTTS at Rady Children’s Hospital from 2013 to 2021. We also prospectively administered the Parents’ Evaluation of Development Status questionnaire (PEDS) to parents of children aged 2-8 years in this patient population. ResultsOf the 99 patients, 64 received a Bayley-III assessment and/or PEDS screen. Of the 35 who had a Bayley-III, there was a higher proportion of patients with PDAS who scored as developmentally appropriate compared with BTTS. PEDS screen showed that a higher proportion of patients with PDAS had no parental concern for delay than that of patients with BTTS (63% vs 30%). Patients with BTTS were more likely to undergo gastrostomy tube placement than patients with PDAS. ConclusionsOur study suggests that neurodevelopmental measures are feasible, clinically relevant, and should be included in comparative effectiveness studies of infant congenital interventions. Whether PDAS offers neurodevelopmental benefit over BTTS should be confirmed in a prospective powered randomized controlled clinical trial.

Effectiveness of Alprostadil for Ductal Patency

OBJECTIVES This study aims to describe the effectiveness of low initial alprostadil dosages to maintain a patent ductus arteriosus (PDA) in infants with ductal-dependent congenital heart disease (DDCHD). Secondary objectives were to describe any adverse drug events, describe prescribing trends, describe ductus arteriosus diameter changes, and compare the safety and efficacy of very low and low initial alprostadil dosage regimens. METHODS This retrospective observational cohort study at the British Columbia’s Women’s and Children’s Hospital neonatal intensive care unit and pediatric intensive care unit examined neonates admitted with DDCHD who received alprostadil to maintain ductal patency. Very low–dose alprostadil (less than 0.01 mcg/kg/min) versus low-dose alprostadil (equal to or greater than 0.01 mcg/kg/min) was examined. Effectiveness was defined as survival and infants not requiring a resuscitation event (cardiac arrest, cardiogenic shock, code blue, extracorporeal life support, requirement for emergent cardiac surgery, and respiratory acidosis). Adverse drug events with a Naranjo score of 3 or more were included. RESULTS Alprostadil was effective for 88% of patients, with no difference between the very low–dose and low-dose groups. Of the 75 patients included, 25 received very low–dose alprostadil. Adverse drug events were common (51%) with neonates in the low-dose group experiencing more apnea and pyrexia than neonates in the very low–dose group. CONCLUSIONS Alprostadil therapy was effective in maintaining the PDA in neonates with DDCHD with low-dosage regimens. Adverse drug events were common with both dosage regimens; however, the very low dosage appeared to have less apnea and pyrexia.

Abstract 13368: Availability of PGE1 in Northern California Hospitals: Before and After Routine CCHD Screening

Introduction: Fetal echocardiogram and critical congenital heart disease (CCHD) screening has improved early detection of CCHD. CCHD screening has been recommended for all infants since 2011 and mandated in California since July 2013. In patients with suspected ductal dependent congenital heart disease, early intervention with prostaglandin E1 (PGE1) is the standard of care to reduce morbidity and mortality. Hypothesis: In 2004, we assessed the availability of PGE1 in Northern California hospitals that care for newborn infants and found that 32% of hospitals with obstetric services did not have the ability to institute PGE1 infusion. As California has adopted CCHD screening for all newborn infants prior to hospital discharge, our aim was to assess the percentage of hospitals that perform CCHD screening as well as reassess PGE1 availability. Our hypothesis was that CCHD screening would increase the availability of PGE1 at hospitals providing obstetric services. Methods: The initial study contacted 117 Northern California hospitals to determine PGE1 availability. We contacted the same hospitals, ending up with 106 hospitals due to closures and mergers. The newborn nursery and/or NICU for each of these hospitals was contacted to determine CCHD screening practices and availability of PGE1. Mileage to the nearest level IV NICU was used as a gross estimate of distance to a tertiary care center. Results: In 2004, 92 of 117 hospitals had obstetric service, and 30 (32.6%) of those did not have PGE1. In this study, 74 of the 106 provided obstetric services. All performed CCHD screening, but 15 (20.3%) did not have ability to provide PGE1 infusion. 8 of the 15 hospitals were more than 30 miles (range 1.5 - 192 miles) from the closest level IV NICU. Conclusions: All hospitals in Northern California providing obstetric service are performing CCHD screening. However, 20.3% of these hospitals still do not have the ability to administer PGE1 infusion. Of these, 53% were in suburban and rural areas of Northern California, with significant transportation distance to a tertiary care center. Delay in initiating PGE1 infusion in patients with ductal dependent CCHD may lead to morbidity or mortality, and increasing PGE1 availability is an opportunity to improve access to care.

A multicenter study on two-stage transfer learning model for duct-dependent CHDs screening in fetal echocardiography

Duct-dependent congenital heart diseases (CHDs) are a serious form of CHD with a low detection rate, especially in underdeveloped countries and areas. Although existing studies have developed models for fetal heart structure identification, there is a lack of comprehensive evaluation of the long axis of the aorta. In this study, a total of 6698 images and 48 videos are collected to develop and test a two-stage deep transfer learning model named DDCHD-DenseNet for screening critical duct-dependent CHDs. The model achieves a sensitivity of 0.973, 0.843, 0.769, and 0.759, and a specificity of 0.985, 0.967, 0.956, and 0.759, respectively, on the four multicenter test sets. It is expected to be employed as a potential automatic screening tool for hierarchical care and computer-aided diagnosis. Our two-stage strategy effectively improves the robustness of the model and can be extended to screen for other fetal heart development defects.

GATA4 rs61277615, rs73203482, and rs35813172 in Newborns with Transposition of the Great Arteries.

Congenital heart disease is the most common malformative pathology in newborns, with a worldwide incidence at 0.4-5%. We investigated the possible relationship between variations in nucleotide sequences and specific cardiac malformations in the GATA-binding factor 4 (GATA4) exon 1 region by using Sanger sequencing. Forty-four newborns from a third-level neonatal intensive care unit who were diagnosed with nonsyndromic, ductal-dependent congenital heart disease (i.e., transposition of the great arteries or ductal-dependent coarctation of the aorta) were enrolled. Their DNA was extracted using commercial methods and tested using the multiplex ligation-dependent probe amplification (MLPA) technique. The Sanger sequencing for GATA4 exon 1 in the newborns' DNA identified rs61277615, rs73203482, and rs35813172 variants not reported in the ClinVar archive of human variations in newborns previously diagnosed with transposition of the great arteries (n=5) and coarctation of the aorta (n=1). The identification of these novel variants in newborns with transposition of the great arteries or ductal-dependent coarctation of the aorta may be the first step in determining the variants' contribution to the occurrence of congenital heart disease. However, these results may be inconclusive, since the observed variants within GATA4 gene were not previously reported.

Adaptive Growth of the Ductus Arteriosus and Aortic Isthmus in Various Ductus-Dependent Complex Congenital Heart Diseases.

The ductus arteriosus (DA) is critical in maintaining postnatal circulation in neonates with obstructed systemic circulation (OSC) and pulmonary circulation (OPC). We hypothesized that the size of the DA and aortic isthmus (AoI) undergoes adaptive growth in utero to counteract the hemodynamic challenges in these congenital heart diseases (CHD). Postnatal echocardiograms of neonates diagnosed prenatally with ductal-dependent CHD who were started on prostaglandins within 24h of birth were reviewed. We assessed the cross-sectional area of the aortic valve opening, pulmonary valve opening, AoI, and DA by calculating (diameter)2/body surface area. Neonates were classified into OSC or OPC then subgrouped depending upon the patency of semilunar valves: OSC with and without aortic atresia (OSC-AA and OSC-nAA, respectively) and OPC with and without pulmonary atresia (OPC-PA and OPC-nPA, respectively). Ninety-four cases were studied. The DA in OSC was significantly larger than OPC, and the DA in OSC-AA was significantly larger than OSC-nAA. The size of the AoI was significantly larger in OPC than OSC and larger in OSC-AA than OSC-nAA. Within the OSC-nAA group, there was no significant difference in the size of the DA, AoI, or pulmonary valve opening between those with retrograde flow (RF) at the AoI and without (nRF) except the aortic valve opening was significantly larger in nRF. All groups had comparable cross-sectional areas of systemic output. Our findings suggest that DA and AoI show compensatory growth to maintain critical blood flow to vital organs against primary anatomical abnormalities in ductus-dependent CHD. (249 words).

Diagnostic and prognostic role of troponin i in neonates with critical duct-dependent congenital heart diseases

Background Cardiac troponins (cTn) are markers of myocardial injury with well established diagnostic value in adults. However, the benefits of using troponins in neonates with critical duct-dependent congenital cardiac diseases (cDD-CHDs) is questionable. Aim The study aimed to investigate the diagnostic role of cTnI in cDD-CHDs as a marker of myocardial injury and to explore its role in patient’s outcome. Methods A case-control study, conducted over 80 neonates; Group I: Forty neonates with cDD-CHDs and Group II: Forty healthy neonates. CBC, CRP, electrolytes, liver and kidney functions, cTnI and echocardiography were done for both groups in first week of life. Then serum level of cTnI were re-evaluated after 3 weeks from initial sampling. Results The mean value of serum cTnI was significantly higher among cases when compared to controls (0.91±1.88 ng/ml versus 0.003±0.006 ng/ml, P<0.001*). The diagnostic performance of cTn I for cardiac injury was at cut-off 0.03 ng/ml with sensitivity 65% and specificity 97.5%. However, the prognostic role of cTnI in predicting death was at serum level of 0.7 ng/ml with 61% sensitivity and 81% specificity. cTn I in cDD-CHD was positively correlated to the severity of heart failure (HF) based on Modified Ross Score. The mean value of survival of the cDD-CHDs neonates was 28.11 days when cTnI is >0.6 ng/ml. Conclusion Initial serum levels of cTnI of cDD-CHD neonates in first week of life might be a useful marker in predicting the 40-days-survival and outcome.

Особенности микробиома кишечника при некротизирующим энтероколите у недоношенных новорожденных и доношенных новорожденных с врожденными пороками сердца по данным метагеномного секвенирования

Necrotizing enterocolitis (NEC) is a serious disease that predominantly affects premature infants with a birth weight of less than 1500 g. Newborns with ductus-dependent congenital heart disease (CHD) are also at risk of developing NEC, which is associated with impaired mesenteric perfusion and hypoxemia. Objective. To identify features of the intestinal microbiome composition of preterm neonates and full-term neonates with ductusdependent CHD, depending on the development of NEC. Patients and methods. A prospective observation of neonates and a retrospective “case-control” analysis (1:1 ratio) of the intestinal microbiome composition depending on the development of NEC were performed. In neonates with NEC, specimens were collected at the onset of NEC (±2 days), in patients without NEC – on the day of life, corresponding to the onset of the disease in newborns with NEC (±3 days) matched by gestational age. The intestinal composition was analyzed in 31 preterm newborns with stage IB–III NEC (subgroup N1 with early-onset NEC (days 1–7 of life) (n = 19); subgroup N2 with late-onset NEC (days 13–34 of life) (n = 12)), as well as in 31 preterm neonates without NEC (subgroup C1 of patients with specimens collected on days 2–7 of life (n = 19), subgroup C2 of patients with specimens collected on days 11–36 of life (n = 12)). Also, an analysis of the microbiome composition was performed in 14 full-term newborns with ductus-dependent CHD who developed NEC (group H1), and 14 full-term newborns with ductus-dependent CHD without NEC (group H2). Fecal specimens were collected from diapers into sterile tubes and stored at -40°C. Analysis of the intestinal microbiome was performed on Illumina MiSeq by prokaryotic 16S rRNA sequencing of amplicons with primers F515/R806 to the V4 16S rRNA variable region. A Naive Bayes classifier trained on the NCBI 16S RefSeq database was used for ASV taxonomic classification. Results. NEC stages of preterm neonates: IB – 5 children, IIA – 19, IIB – 4, III – 3. Stages of NEC in newborns with ductusdependent congenital heart disease: IB – 3 children; IIA – 11. There were no statistically significant differences in the Shannon alpha diversity index between groups (N1 and C1, N2 and C2, H1 and H2). Neonates with antibiotic exposure (ABT) at specimen collection had different beta diversity (PERMANOVA F-value: 24.534; R-squared: 0.21997; p = 0.001) and had a higher alpha diversity (Shannon index: 2.0 (0.8–2.8) vs. 1.2 (0.8–1.5), p = 0.004), primarily due to representatives of opportunistic flora. Analysis of the intestinal microbiome did not reveal bacteria, which could be interpreted as a universal marker for the development of NEC, but some features were identified. Among neonates from the “early” groups (N1 and C1), the dominant genera were more often Staphylococcus, Serratia, and Corynebacterium. Neonates with early-onset NEC (N1) had a significantly lower relative abundance of Enterococcus, Lacticaseibacillus, Clostridium, Fusobacterium, Limosilactobacillus, Agrococcus, Alkalibacterium, Ancrocorticia, Blautia, Brachybacterium, Brevibacterium, Carnobacterium, Citricoccus, Flaviflexus, Garicola, Lactococcus, Microbacterium, Phocaeicola, Propionimicrobium, Ruoffia, Stenotrophomonas compared to subgroup C1. Preterm neonates with late-onset NEC (N2) had a lower relative abundance of Bifidobacterium (p = 0.027) and Streptococcus (p = 0.037) than the comparison subgroup (C2) had. Neonates with late-onset NEC (N2) had a significantly higher relative abundance of Enterococcus than in early-onset NEC (N1) (7.83 (0.09–46.15) % vs. 0 (0–0.18) % resp., p = 0.0004), no differences were found between the comparison subgroups (C2 and C1). Preterm neonates with a longer NEC duration (n = 16, nil per os 20 (16–27) days) compared to children with a shorter duration (nil per os 10 (9.5–11) days) had several pathobionts/pathogens in fecal specimens at the NEC onset: Alkalibacterium, Brevibacterium, Flaviflexus and Ruo (Facklamia). Full-term neonates with ductus-dependent CHD and NEC (H1) had a higher relative abundance of Staphylococcus (p = 0.029), Serratia ( p = 0.011; OR = 14 (95% CI 1.43–137.3)), Pelomonas ( Roseateles) (p = 0.017), Methylobacterium (p = 0.017), and Nitrincola (p = 0.017) than neonates with CHD without NEC (H2). Conclusion. Intestinal dysbiosis may act as a predisposing background for the development of NEC, but not its main cause. The dysbiosis by antibiotic exposure is not a sufficient factor for NEC development; the mother's health, fetal development status, and the early postnatal period play a significant role. Early correction of antibiotic-induced dysbiosis may be a reasonable approach to preventing NEC. Probiotics, prebiotics, postbiotics/metabolites, live biotherapeutic products, and phages may be potential microbiome-modulating agents in neonates. Key words: necrotizing enterocolitis, ductus-dependent congenital heart disease, metagenomics, 16S rRNA sequencing, intestinal microbiome

Comparison of Patent Ductus Arteriosus Stenting and Blalock-Taussig Shunt in Ductal Dependent Blood Flow Congenital Heart Disease and Decreased Pulmonary Blood Flow.

Congenital ductal-dependent cyanotic congenital heart disease (CHD) is a group of diseases that require early intervention during early infancy or the neonatal period. In this study, we compared the effectiveness, safety, and side effects of stenting patent ductus arteriosus versus a modified Blalock-Taussig (BT) shunt. Thirty-six neonates and infants with cyanotic CHD who were <6 months old and were not suitable for complete surgery were admitted to Chamran Hospital in Isfahan and enrolled in this prospective longitudinal cross-sectional study. Ductal stenting (DS) was performed in 18 patients and BT shunt in 18 patients. Data were collected and compared in these patients. Age, height, and weight were lower in the DS group compared to the shunt group (1.28 ± 0.46 vs. 2.50 ± 0.51 months, 53.22 ± 1.80 vs. 56.11 ± 1.67 cm, 3.38 ± 0.45 vs. 4.98 ± 0.57 kg, respectively P < 0.001). The duration of the intensive care unit stay as well as hospital stay in patients in the DS group was much shorter than the shunt group (1.33 ± 0.68 vs. 2.83 ± 0.70 days, 3.33 ± 0.68 vs. 9.55 ± 0.51, P < 0.001, respectively). In both groups, the diameter of the pulmonary branches increased after the procedure compared to before the procedure (P < 0.001). Furthermore, the patient's hemoglobin was significantly decreased compared to before the procedure (P < 0.001). In both groups, one patient died in the postprocedure period. In ductal stent group one patient died in catheterization lab and in shunt group died 48 hours after surgery. There was no significant difference in postprocedural complications between the two groups. Duct stents can be used as an effective and safe method in duct-dependent cyanotic CHD patients who are not suitable candidates for complete surgery. As operators become proficient, this method can be a good alternative to BT shunts with fewer complications.

Standardizing Prostaglandin Initiation in Prenatally Diagnosed Ductal-Dependent Neonates; A Quality Initiative.

Prostaglandin E1 (PGE) is used in patients with ductal-dependent congenital heart disease (CHD). Side effects of apnea and fever are often dose dependent and occur within 48h after initiation. We initiated a standardized approach to PGE initiation after our institution recognized a high incidence of side effects and a wide variety of starting doses of PGE. Neonates with prenatally diagnosed ductal-dependent CHD were identified, started on a standardized protocol that started PGE at 0.01 mcg/kg/min, and evaluated for PGE related side effects. Compliance, outcomes and dose adjustments during the first 48h post-PGE initiation were evaluated. Fifty patients were identified (25 pre-intervention; 25 post-intervention). After intervention, compliance with the protocol was 96%, and apnea or fever occurred in 28% (compared to 63% pre-intervention, p = 0.015). Dose adjustments (either increase or decrease) prior to cardiac surgery were similar in both cohorts (60%, 52%, p = 0.569). There were no mortalities or emergent procedures performed due to ductus arteriosus closure. Standardizing a protocol for initiating PGE in prenatally diagnosed ductal-dependent CHD was successful and reduced the incidence of apnea, fever, and sepsis evaluations. A starting dose of 0.01 mcg/kg/min did not cause increased adverse effects.

The ductus arteriosus: a review of embryology to intervention.

This article reviews the physiology of the ductus arteriosus, the pathophysiology of the patent ductus arteriosus (PDA), and the role advanced imaging such as computed tomography (CT) and magnetic resonance imaging (MRI) can play in guiding diagnosis and percutaneous or surgical intervention. A PDA can have variable clinical and radiologic presentations and can be important to characterize in patients with vascular rings, aortic maldevelopment and congenital heart disease. An understanding of the PDA and the application of CT and MRI can allow the radiologist to provide key information to physicians who plan to close a PDA or maintain PDA patency in the setting of ductal-dependent congenital heart disease.

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.

Development of necrotizing enterocolitis in full-term infants with duct dependent congenital heart disease

BackgroundAlthough many studies have described an increased risk of necrotizing enterocolitis in duct dependent congenital heart diseases, very few have investigated its occurrence in full-term infants with duct dependent congenital heart diseases.MethodsTo evaluate the characteristics and risk factors of necrotizing enterocolitis, we performed a retrospective review of 355 full-term infants with duct dependent congenital heart diseases who received prostaglandin E1 therapy from April 2000 to May 2020.ResultsNecrotizing enterocolitis was observed in 10 patients (3.0%). Their average gestational age and birth weight were 38.2 weeks and 2783.5 g, respectively. The median age at diagnosis was 8.0 days (2–70 days). One patient was diagnosed with necrotizing enterocolitis stage IIA, five with stage IIB, two with stage IIIA, and two with stage IIIB; two (20%) received surgical treatment. The duct dependent pulmonary circulation group had higher frequencies of necrotizing enterocolitis (4.4%) than the duct dependent systemic circulation (2.0%) and parallel circulation (1.3%) groups. The necrotizing enterocolitis and the other groups had significantly different birth weight (2783.5 g vs 3170.9 g, respectively) and gestational age (38.2 weeks vs 39.1 weeks, respectively). Gestational age under 38 weeks (OR 8.87, p = 0.002), birth weight of < 2500 g (OR 5.1, p = 0.042), need for mechanical ventilation (OR 4.6, p = 0.021), parenteral nutrition (OR 107.7, p < 0.001), and functional single ventricle (OR 5.8, p = 0.009) were significant risk factors. The case-fatality rate was higher in the necrotizing enterocolitis (40.0%) than in the other group (8.3%, p = 0.009).ConclusionsThree percent of full-term infants with duct dependent congenital heart diseases developed necrotizing enterocolitis. Neonates with low birth weight, gestational age less than 38 weeks, functional single ventricle, or receiving assisted mechanical ventilation or parenteral nutrition are at increased risk.

Screening for Duct-Dependent Congenital Heart Disease from Fetal Echocardiography with Two-Stage Deep Transfer Learning Model: A Multicenter, Retrospective Study

Screening for Duct-Dependent Congenital Heart Disease from Fetal Echocardiography with Two-Stage Deep Transfer Learning Model: A Multicenter, Retrospective Study

Percutaneous Transcatheter Closure of a Stented Patent Ductus Arteriosus in a Patient With Critical Pulmonary Stenosis: A Case Series

INTRODUCTION: TStenting of the ductus is an alternative to surgical shunt in patients with critical pulmonary stenosis who remain ductal dependent following valvuloplasty. Stents close spontaneously after improvement of right ventricular function; however, in rare cases, stents fail to completely endothelialize, resulting in pulmonary overcirculation. CASE PRESENTATION: The first case was a 3-year-old who had critical pulmonary stenosis at birth, and the second case was a 3-year-old boy who at 5 months of age was managed for severe pulmonary valve stenosis. Both underwent stenting of the duct and percutaneous pulmonary balloon valvuloplasty. On follow-up, the patent ductus arteriosus (PDA) stent of both cases remained patent even after an adequate growth and improvement of the right ventricle, resulting in significant left-to-right shunting and development of increased pulmonary blood flow; hence, percutaneous transcatheter closure of the stented PDA was done. DISCUSSION: Stenting of the ductus allows significant and balanced growth of the pulmonary artery in patients with duct-dependent congenital heart diseases leading to adequate growth of the right ventricle. Persistence of patency of stent causing significant left-to-right shunting warrants closure of the PDA stent. Closure of the stent is challenging and requires a high degree of technical skill because of the difficulty to enter the partially closed stent. CONCLUSION: Percutaneous transcatheter closure of a stented PDA in patients with critical pulmonary stenosis can be a technically challenging procedure because of the difficulty to enter the stent due to the presence of intrastent endothelial proliferation. KEYWORDS: congenital heart disease, critical pulmonary stenosis, patent ductus arteriosus, transcatheter device closure, stent

Diagnostic performance of 320 cardiac MDCT angiography in assessment of PDA either isolated or associated with duct dependent congenital heart disease

BackgroundPatent ductus arteriosus (PDA) is one of most common congenital heart defects, it's a unique vascular structure that provides direct communication between pulmonary and systemic circulation. MDCT angiography is a good imaging modality for evaluation of the PDAs and detection of their exact morphological type; course and diameters, which is important before percutaneous closure or stenting procedure of the PDA, also for selection of closure hardware. The aim of this study was to assess the role of MDCT angiography in qualitative and quantitative evaluation of PDA and associated cardiac and\\or extracardiac anomalies.ResultsEchocardiography detected PDA in 28\\30 cases while cardiac MDCT detected PDA in all studied 30 cases confirmed by cardiac catheterization and/or operation. MDCT angiography had sensitivity 100% and specificity 100% for PDA detection. PDA originated from aortic isthmus in 15 cases, inferior surface of aortic arch in 11 cases and innominate artery in 4 cases. The most common morphological type of PDA was type A (cone\\46.67%) followed by type C (tubular\\23.3%), type D (complex\\10%), type E (elongated\\13.33%) and type B (window\\6.67%). The spearman correlation coefficient test demonstrated poor correlation between size of aortic end and MPA (P = 0.75), and between size of pulmonary end and diameter of MPA (P = 0.99) and also demonstrated fair correlation between length of PDA and MPA (P = 0.018). PDA was isolated in 4\\30 cases and associated with cardiac and\\or extra cardiac anomalies in 26\\30 cases included; ASD (n = 18), VSD (n = 16), pulmonary atresia (n = 7), transposition of great arteries (n = 5), teratology of Fallot (n = 4), aortic coarctation (n = 4), persistent truncus arteriosus (n = 3), tricuspid atresia (n = 3), anomalous of pulmonary venous return (n = 3), hypoplastic segment of aorta (n = 2), Ebstein's anomaly (n = 1), bicuspid aortic arch (n = 1) and left hypoplastic heart syndrome (n = 1).ConclusionCardiac MDCT angiography was superior to Echocardiography in detection, quantitative and qualitative evaluation of PDA either isolated or associated with congenital cardiac and\\or extracardiac anomalies and was superior to Echocardiography in detection of associated extracardiac anomalies rather than associated intra cardiac anomalies.

Emergency atrial septostomy with nasogastric tube through umbilical venous route in neonates with critical congenital heart defects

Neonates born with critical duct dependent congenital heart diseases acutely deteriorate in first few days after birth when physiological conduits which allow for mixing blood close. Balloon Atrial septostomy is performed on emergency basis in such neonates to create a right to left intracardiac shunt, until a definitive surgery is performed, but requires a cardiac intervention setup. In settings where advanced cardiac interventions are not available, we propose an alternative approach for atrial septostomy using nasogastric feeding tube through umbilical venous route, under echocardiographic guidance. Feeding tube gradually inserted into the umbilical vein, reaches the right atrium, following which it is thrust into the atrial septum at the location of foramen ovale, perforating it to create an emergency conduit between the atria. We performed the above procedure in a neonate with dTGA at our NICU setup, with partially successful outcome. We propose this method as an alternative, safe, simple and cost effective procedure, though not definitive, in resource limited settings for emergency stabilisation of neonates with critical duct dependent heart defects.

Comparative analysis of enteral feeding practices for prevention of gastrointestinal complications in infants with ductus-dependent congenital heart diseases

Mesenterial hypoperfusion is one of the main pathogenetic factors of necrotizing enterocolitis (NEC) in infants with ductus-dependent congenital heart diseases. NEC in infants undergoing congenital heart surgery increases mortality and length of hospital stay. NEC is also associated with adverse neurodevelopmental outcome. Optimization of enteral feeding can reduce the risk of gastrointestinal complications. NEC risk factors in infants with congenital heart are of special interest in the literature. This article discusses criteria for enteral feeding initiation and increasing preoperatively and after heart surgery. Enteral feeding protocols of leading cardiac surgical centers are reviewed. Practices to provide high energy and nutrient consumption in infants with congenital heart disease are described.

Effect of a Dual-Strain Probiotic on Necrotizing Enterocolitis in Neonates with Ductal-Dependent Congenital Heart Disease: A Retrospective Cohort Study

Background: Newborns with ductal-dependent congenital heart disease (CHD) are at increased risk for developing necrotizing enterocolitis (NEC). Objectives: To investigate whether the use of dual-strain probiotics is beneficial for prevention of NEC in CHD patients, as demonstrated for premature infants. Study Design: Single-center retrospective cohort study of newborns with ductal-dependent CHD before and after implementation of oral dual-strain probiotics containing Bifidobacterium infantis and Lactobacillus acidophilus, on each day of exposure to prostaglandin E1 (PGE1). Results: Birth weight, gestational age, and distribution of heart defects were similar in both cohorts. NEC occurred in 6 of 247 (2.4%) patients without probiotics, and in 3 of 242 (1.2%) patients who received probiotics (p = 0.504). NEC-related mortality (0.4 vs. 0.4%, p = 1.000) and overall mortality (11.0 vs. 8.7%, p = 0.448) were likewise not different. PGE1 exposure was 1,788 and 2,455 days, respectively. In subgroup analysis of 152 infants with aortic arch malformations, such as coarctation of the aorta and interrupted aortic arch, we observed a significant reduction of NEC frequency (5.6 vs. 0.0%, p = 0.048). Conclusions: This is the first study to investigate the effect of a dual-strain probiotic on NEC in CHD patients. Infants with aortic arch malformations appear to benefit from dual-strain probiotics. Due to the scarcity of concurrence of ductal-dependent CHD and NEC, a clinical trial on probiotics to decrease risk of NEC in infants with ductal-dependent CHD would require several thousand infants.

Impact of ductal stenting on pulmonary artery reconstruction in patients with duct-dependent congenital heart diseases-an institutional experience.

This study aims to review our institutional experience of ductal stenting (DS) on the growth of pulmonary arteries (PAs) and surgical outcomes of PA reconstruction in this subset of patients. This is a retrospective study done in neonates and infants up to 3 months of age with duct-dependent pulmonary circulation who underwent DS from January 2014 to December 2015. Post-stenting PA growth, surgical outcomes of PA reconstruction, post-surgical re-interventions, morbidity and mortality were analysed. During the study period, 46 patients underwent successful DS, of whom 38 underwent presurgery catheterization and definite surgery. There was significant growth of PAs in these patients. Biventricular repair was done in 31 patients while 7 had univentricular palliation. Left PA augmentation was required in 13 patients, and 10 required central PA augmentation during surgery. The mean follow-up period post-surgery was 4.5 ± 1.5 years. No significant postoperative complications were seen. No early or follow-up post-surgery mortality was seen. Four patients required re-interventions in the form of left PA stenting based on the echocardiography or computed tomography evidence of significant stenosis. DS provides good short-term palliation and the growth of PAs. However, a significant number of stented patients require reparative procedure on PAs at the time of surgical intervention. Acquired changes in the PAs following DS may be the reason for reintervention following PA reconstruction.