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Interventional Cardiology Clinics Jul 2024Pulmonary vein stenosis (PVS) is a rare and unique disease of infants and young children. PVS is attended by high morbidity and mortality, and for many decades,... (Review)
Review
Pulmonary vein stenosis (PVS) is a rare and unique disease of infants and young children. PVS is attended by high morbidity and mortality, and for many decades, effective therapy eluded the practitioner. However, in the most recent era, interventional techniques when employed in combination with systemic (primary) therapy have had a remarkable impact on outcomes in these at-risk children. Despite apparent complete relief of PVS in a discrete region of a pulmonary vein, stenosis reliably recurs and progresses. In this review, we discuss the current state-of-the-art interventional techniques, through the lens of our collective experiences and practices.
Topics: Humans; Stenosis, Pulmonary Vein; Pulmonary Veins; Stents; Infant
PubMed: 38839175
DOI: 10.1016/j.iccl.2024.03.003 -
Interventional Cardiology Clinics Jul 2024Since PDA stenting was first attempted in the early 1990s, significant technical advancements have improved outcomes and some centers have even transitioned to exclusive... (Review)
Review
Since PDA stenting was first attempted in the early 1990s, significant technical advancements have improved outcomes and some centers have even transitioned to exclusive PDA stenting for all infants with duct-dependent pulmonary circulation. In addition to its use in infants with duct-dependent pulmonary circulation, PDA stenting has also been adapted as a percutaneous palliative option for suprasystemic pulmonary arterial hypertension and as a component of the hybrid procedure. In this article, the authors aim to review indications and outcomes for PDA stenting, describe the procedure, and discuss future directions.
Topics: Humans; Ductus Arteriosus, Patent; Stents; Cardiac Catheterization; Palliative Care; Treatment Outcome
PubMed: 38839174
DOI: 10.1016/j.iccl.2024.02.002 -
Interventional Cardiology Clinics Jul 2024Transcatheter ductal closure has proven to be safe and effective to occlude the patent ductus arteriosus (PDA) in premature infants. We have developed a robust... (Review)
Review
Transcatheter ductal closure has proven to be safe and effective to occlude the patent ductus arteriosus (PDA) in premature infants. We have developed a robust multidisciplinary PDA program. After gaining experience performing transcatheter ductal closure in the cardiac catheterization laboratory, we transitioned to performing the procedure at the patient's bedside in the neonatal intensive care unit using echocardiographic guidance. The bedside approach has the potential to expand this therapy to patients who currently undergo expectant PDA management because of lack of availability of this therapy, and allow for earlier referral, which will reduce time of exposure to this pathologic shunt.
Topics: Humans; Ductus Arteriosus, Patent; Infant, Newborn; Infant, Premature; Cardiac Catheterization; Septal Occluder Device; Echocardiography
PubMed: 38839169
DOI: 10.1016/j.iccl.2024.02.001 -
Journal of Veterinary Cardiology : the... May 2024A 6-month-old male intact miniature Australian Shepherd presented for surgical consultation for a previously diagnosed patent ductus arteriosus. Echocardiogram revealed...
A 6-month-old male intact miniature Australian Shepherd presented for surgical consultation for a previously diagnosed patent ductus arteriosus. Echocardiogram revealed a patent ductus arteriosus and a hyperechoic oscillating lesion within the main pulmonary artery. Blood cultures and eventual post-mortem examination revealed Candida tropicalis endocarditis. This case report highlights a rare case of fungal endocarditis with both echocardiographic and post-mortem findings.
PubMed: 38838576
DOI: 10.1016/j.jvc.2024.04.004 -
Cardiology in Review Jun 2024Robert Gross performed the first repair of a patent ductus arteriosus in 1938. His life and career were dedicated to the advancement of vascular, cardiac, pediatric...
Robert Gross performed the first repair of a patent ductus arteriosus in 1938. His life and career were dedicated to the advancement of vascular, cardiac, pediatric surgery, and the unrelenting care of his patients.
PubMed: 38833498
DOI: 10.1097/CRD.0000000000000731 -
Oral docosahexaenoic acid supplementation and retinopathy of prematurity: The JoiDHA Clinical Trial.The British Journal of Nutrition Jun 2024Retinopathy of prematurity (ROP) is a leading cause of blindness in premature infants. The condition is associated with docosahexaenoic acid (DHA) deficiency. This study...
Retinopathy of prematurity (ROP) is a leading cause of blindness in premature infants. The condition is associated with docosahexaenoic acid (DHA) deficiency. This study aimed to investigate the effect of DHA supplementation on the occurrence of ROP in infants receiving oral oil drops. It is part of the Joinville DHA study (JoiDHA Study), a non-parallel-group cohort study conducted from March 2020 to January 2023 at a public maternity hospital in Brazil. Infants born before 33 weeks of gestational age or with a birth weight ≤1,500 g were recruited. Among 155 infants, 81 did not receive and 74 received DHA supplementation until complete vascularization of the peripheral retina. There was a higher incidence of infants with ROP in the unsupplemented group (58·6%) compared to the DHA group (41·4%), but this difference was not significant (=0·22). Unadjusted logistic regression analysis showed that patent ductus arteriosus and neonatal corticosteroids were significantly (<0·05) associated with ROP in both groups. In the DHA group, surfactant use was also associated with ROP (=0·003). After adjusting for important covariates, patent ductus arteriosus and neonatal corticosteroids continued to be significant for infants in the unsupplemented group (OR=3·99; =0·022 and OR=5·64; =0·019, respectively). In the DHA group, only surfactant use continued to be associated with ROP (OR=4·84; =0·015). In summary, DHA supplementation was not associated with ROP. Further studies are necessary to better understand the relationship between DHA supplementation, ROP, and associated comorbidities.
PubMed: 38826072
DOI: 10.1017/S0007114524001120 -
European Journal of Pediatrics Jun 2024Congenital heart disease (CHD) and patent ductus arteriosus (PDA) are risk factors of necrotizing enterocolitis (NEC) in infants. However, it is unclear whether the...
Congenital heart disease (CHD) and patent ductus arteriosus (PDA) are risk factors of necrotizing enterocolitis (NEC) in infants. However, it is unclear whether the prognosis of NEC is different between very preterm infants (VPIs) with and without heart diseases. This was an observational cohort study that enrolled VPIs (born between 24 and 31 weeks) admitted to 79 tertiary neonatal intensive care units (NICU) in the Chinese Neonatal Network (CHNN) between 2019 and 2021. The exposure was CHD or isolated PDA, and VPIs with NEC were divided into three groups: complicated with CHD, with isolated PDA, and without heart diseases. The primary outcomes were NEC-related adverse outcomes (death or extrauterine growth restriction (EUGR)). Logistic regression models were used to adjust potential confounders and calculate the odds ratios (ORs) and 95% confidential intervals (CIs) for each outcome. A total of 1335 VPIs with NEC were enrolled in this study, including 65 VPIs with CHD and 406 VPIs with isolated PDA. The VPIs with heart diseases had smaller gestational ages and lower body weights at birth, more antenatal steroids use, and requiring inotrope prior to the onset of NEC. While suffering from NEC, there was no significant increased risks in NEC-related death in VPIs with either CHD (adjusted OR [aOR]: 1.10; 95% CI: 0.41-2.50) or isolated PDA (aOR: 1.25; 95% CI 0.82-1.87), and increased risks in EUGR were identified in either survival VPIs with CHD (aOR: 2.35; 95% CI: 1.31-4.20) or isolated PDA (aOR: 1.53; 95% CI: 1.16-2.01) in survivors. The composite outcome (death or EUGR) was also more often observed in VPIs with either CHD (aOR: 2.07; 95% confidence interval [CI]: 1.20-3.60) or isolated PDA (aOR: 1.51; 95% CI: 1.17-1.94) than that without heart diseases. VPIs with either CHD or isolated PDA were associated with significantly prolonged duration of fasting, extended time to achieve full enteral feeding, and longer ventilation duration and hospitalization duration. Similar characteristics were also seen in VPIs with isolated PDA, with the exception that VPIs with CHD are more likely to undergo surgical intervention and maintain a prolonged fast after NEC. Conclusion: In VPIs with NEC, CHD and isolated PDA are associated with an increased risk in worse outcomes. We recommend that VPIs with cardiac NEC be managed with aggressive treatment and nutrition strategies to prevent EUGR. What is Known: • CHD and PDA are risk factors for NEC in infants, which can lead to adverse outcomes such as death and EUGR. • NEC in infants with heart disease differs clinically from that in infants without heart disease and should be recognized as a separate disease process. What is New: • CHD and isolated PDA are associated with increased risks of EUGR in VPIs with NEC. • Risk factors associated with VPIs with cardiac NEC suggested these patients should be managed with aggressive treatment and nutrition strategies to adverse outcomes.
PubMed: 38822834
DOI: 10.1007/s00431-024-05599-z -
BMC Pediatrics May 2024Patent ductus arteriosus (PDA) is commonly encountered morbidity which often occurs as up to 60% of extremely preterm infants born at < 29 weeks gestational age (GA).
BACKGROUND
Patent ductus arteriosus (PDA) is commonly encountered morbidity which often occurs as up to 60% of extremely preterm infants born at < 29 weeks gestational age (GA).
PURPOSE
This study aims to assess the clinical risk factors associated with PDA ligation among very low birth weight infants (VLBWI) and evaluate the neurodevelopmental outcomes of those who underwent PDA ligation.
METHODS
A total of 540 VLBWI were initially diagnosed with PDA in our 50-bed level IV NICU at Seoul St. Mary's Hospital, The Catholic University of Korea, between January 2015 and June 2023. Among these 540 VLBWI with PDA, only 221 had consistent hemodynamically significant (hs) PDA which required intervention including fluid restriction, medical treatment, or surgical ligation. In this study, only those 221 VLBWI with hsPDA who underwent neurodevelopmental assessment at corrected 18-24 months of age were included in this study analysis.
RESULTS
Out of 221 VLBWI diagnosed with hemodynamically significant (hs) PDA, 133 (60.2%) underwent PDA ligation, while the remaining 88 (39.8%) had their hs PDAs closed either medically or with fluid restriction. The mean gestational age and birth weight were significantly lower in PDA ligation group compared to no PDA ligation group (27.02 ± 2.17 vs. 27.98 ± 2.36, 907.31 ± 258.36 vs. 1006.07 ± 283.65, p = 0.001, 0.008). Resuscitation including intubation at delivery and intraventricular hemorrhage (IVH) (grade ≥ 3) were significantly higher in PDA ligation group. The clinical outcomes in the PDA ligation group revealed significantly worse oucomes compared to the no PDA ligation group. Both resuscitation, including intubation at delivery, and IVH (grade ≥ 3), consistently exhibited an increased risk for PDA ligation in a multivariable logistic regression analysis. Concerning neurodevelopmental outcomes, infants who underwent PDA ligation demonstrated significantly lower cognitive scores. However, only IVH (grade ≥ 3) and PVL were consistently associated with an increased risk of abnormal neurodevelopment at the corrected age of 18-24 months.
CONCLUSION
In our study, despite the consistent association between cognitive developmental delays in VLBWI who underwent PDA ligation, we observed that sicker and more vulnerable VLBWIs, specifically those experincing severe IVH, consistently exhibited an increased risk for both PDA ligation and abnormal neurodevelopment at the corrected age of 18-24 months.
Topics: Humans; Ductus Arteriosus, Patent; Ligation; Infant, Very Low Birth Weight; Female; Male; Risk Factors; Infant, Newborn; Infant; Retrospective Studies; Child, Preschool; Neurodevelopmental Disorders; Gestational Age
PubMed: 38822298
DOI: 10.1186/s12887-024-04860-6 -
American Journal of Perinatology May 2024Prostaglandins (PGs) play a major role in maintaining patency of the ductal arteriosus (DA). Pulmonary 15-hydroxyprostaglandin dehydrogenase (PGDH), which is ecoded...
OBJECTIVE
Prostaglandins (PGs) play a major role in maintaining patency of the ductal arteriosus (DA). Pulmonary 15-hydroxyprostaglandin dehydrogenase (PGDH), which is ecoded by the hydroxyprostaglandin dehydrogenase () gene, is the primary enzyme responsible for PG breakdown. Animal studies have shown -knockout mice have significantly higher prostaglandin E2 levels and no ductal remodeling. Functional variants of the gene that alter PG breakdown have not been studied in preterm infants with patent ductus arteriosus (PDA).
STUDY DESIGN
This was an observational cohort study including extreme low birth weight (ELBW) infants classified as having spontaneous, medical, or procedural (transcatheter or surgical ligation) closure of their DA. Urine prostaglandin E metabolite (PGEM) levels were measured in ELBW infants following ibuprofen treatment using competitive ELISA. genetic variants rs8752, rs2612656, and rs9312555 were analyzed. Kruskal-Wallis, Fisher's exact, chi square, logistic regression, and Wilcoxon signed-rank tests were used; < 0.05 was considered significant.
RESULTS
Infants in the procedural closure group had a younger gestational age (GA). The incidence of spontaneous closure or medical closure was higher compared to procedural closure in the presence of any minor allele of rs8752 (67 and 27%, respectively; = 0.01), when adjusted for GA and gender. Haplotype analysis of three variants of revealed differences when comparing the spontaneous and medical closure group to the procedural group ( < 0.05). Urinary PGEM levels dropped significantly in those ELBW infants who responded to ibuprofen ( = 0.003) in contrast to those who did not respond ( = 0.5).
CONCLUSION
There was a different genotype distribution for the rs8752 genetic variant of the gene-as it relates to the mode of treatment for ELBW infants with PDA. We speculate that medical management in the presence of this variant facilitated additional PG breakdown, significantly abrogating the need for procedural closure. Additionally, differences in genotype and haplotype distributions implicate a specific genetic foundation for DA closure in ELBW infants.
KEY POINTS
· PGs and their metabolism play a major role in PDA patency or closure.. · Genetic variants of the HPGD gene influence mode of treatment of PDA in ELBW infants.. · ELBW infants with PDA that responded to medical closure had significantly decreased urine PGEM levels..
PubMed: 38821070
DOI: 10.1055/s-0044-1787172 -
Zhonghua Yi Xue Yi Chuan Xue Za Zhi =... Jun 2024Char syndrome is a rare autosomal dominant genetic disorder characterized by patent ductus arteriosus, facial dysmorphism, and dysplasia of fingers/toes. It may also be... (Review)
Review
Char syndrome is a rare autosomal dominant genetic disorder characterized by patent ductus arteriosus, facial dysmorphism, and dysplasia of fingers/toes. It may also be associated with multiple papillae, dental dysplasia, and sleep disorders. TFAP2B has proven to be a pathogenic gene for neural crest derivation and development, and several variants of this gene have been identified. Bone morphogenetic protein signaling plays an important role in embryonic development by participating in limb growth and patterning, and regulation of neural crest cell development. TFAP2B is an upstream regulatory gene for bone morphogenetic proteins 2 and 4. Variants of the TFAP2B gene may lead to abnormal proliferation of neural crest cells by affecting the expression of bone morphogenetic proteins, resulting in multiple organ dysplasia syndrome. In addition, TFAP2B variants may only lead to patent ductus arteriosus instead of typical Char syndrome.
Topics: Humans; Ductus Arteriosus, Patent; Transcription Factor AP-2; Abnormalities, Multiple; Bone Morphogenetic Proteins; Neural Crest; Face; Fingers
PubMed: 38818565
DOI: 10.3760/cma.j.cn511374-20210607-00478