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Journal of Clinical Medicine Apr 2024: Patent ductus arteriosus (PDA) is a prevalent congenital heart defect in premature infants, associated with significant morbidity and mortality. Accurate and timely...
: Patent ductus arteriosus (PDA) is a prevalent congenital heart defect in premature infants, associated with significant morbidity and mortality. Accurate and timely diagnosis of PDA is crucial, given the vulnerability of this population. : We introduce an artificial intelligence (AI)-based PDA diagnostic support system designed to assist medical professionals in diagnosing PDA in premature infants. This study utilized electronic health record (EHR) data from 409 premature infants spanning a decade at Severance Children's Hospital. Our system integrates a data viewer, data analyzer, and AI-based diagnosis supporter, facilitating comprehensive data presentation, analysis, and early symptom detection. : The system's performance was evaluated through diagnostic tests involving medical professionals. This early detection model achieved an accuracy rate of up to 84%, enabling detection up to 3.3 days in advance. In diagnostic tests, medical professionals using the system with the AI-based diagnosis supporter outperformed those using the system without the supporter. : Our AI-based PDA diagnostic support system offers a comprehensive solution for medical professionals to accurately diagnose PDA in a timely manner in premature infants. The collaborative integration of medical expertise and technological innovation demonstrated in this study underscores the potential of AI-driven tools in advancing neonatal diagnosis and care.
PubMed: 38610854
DOI: 10.3390/jcm13072089 -
International Journal of General... 2024Children with DS are at higher risk of developing congenital anomalies, particularly cardiac anomalies.
BACKGROUND
Children with DS are at higher risk of developing congenital anomalies, particularly cardiac anomalies.
METHODS
Medical records of 502 DS patients were reviewed. The logistic regression analyses were performed to determine independent predictors.
RESULTS
Of the total 502 study subjects, 53.4% were males. Only 1.4% of the DS case diagnosis were confirmed by karyotyping. All cases were diagnosed postnatally. The median age at DS diagnosis was 5 months. About 13% were born preterm; 50.2% of the subjects maternal age at conception were thirty-five years and above. Over three-quarters (75.1%) had at least one structural congenital anomaly. Multiple anomalies were diagnosed in 12.8% of the subjects. At least one cardiac congenital anomaly was diagnosed in 67.3% of the study subjects, and 32.8% of them were diagnosed with multiple cardiac anomalies. Patent ductus arteriosus (28.5%), Ventricular septal defect (23.2%), and AVSD (21.9%) were the three common lesions. At least one genitourinary system anomaly was identified in 32 (6.4%) of them. Roughly, 8% of study participants exhibited congenital anomaly of the head, eye, nose, and throat. Anorectal malformation was found as the most common gastrointestinal anomaly. Maternal age at conception was found as independent predictor for presence of structural congenital anomaly (AOR 2.59; 95% CI 1.58-4.23, p-value < 0.01). Advanced maternal age is also found increasing the risk of developing congenital heart defect (AOR 2.37; 95% CI 1.52-3.7, p-value < 0.01).
CONCLUSION
High prevalence of congenital anomalies has been noted in the current study compared to previous studies. Predictive factors increasing risk of congenital anomalies in DS patients have been identified. The current findings may help in developing strategies and more targeted preventive and therapeutic interventions.
PubMed: 38596643
DOI: 10.2147/IJGM.S453181 -
SAGE Open Medical Case Reports 2024Reversed flow in the basilar artery can be acquired or congenital. Acquired reversed flow in the basilar artery can result from acute thrombosis of the basilar artery or...
Reversed flow in the basilar artery can be acquired or congenital. Acquired reversed flow in the basilar artery can result from acute thrombosis of the basilar artery or retrograde vertebral artery flow. Congenital continuous retrograde basilar artery flow has not been described. We report a 2-day-old male presenting with hypocalcemic seizures which led us to obtain a Duplex echoencephalogram. An echocardiogram was subsequently ordered. In the coronal plane through the anterior fontanelle, retrograde flow was seen in the basilar artery and the right vertebral artery. In the axial plane through the temporal window, the flow was anteroposterior in both posterior communicating arteries. In the posterior cerebral arteries, the flow was retrograde in the P1 segment and anterograde in the P2 and P3 segments. An interrupted aortic arch was suspected. The echocardiogram showed a large perimembranous ventricular septal defect with bidirectional shunting, a hypoplastic and bicuspid aortic valve, an aortic arch interrupted between the left common carotid artery and the left subclavian artery (type B interrupted aortic arch), and a 5 mm patent ductus arteriosus with predominant right to left flow. Because of the patency of the large patent ductus arteriosus, our patient showed no sign of posterior circulation insufficiency. Prostaglandin E1 therapy was initiated immediately. Diagnosis of DiGeorge syndrome was proven. The infant underwent interrupted aortic arch repair and anterograde flow was established in the basilar artery. We conclude that congenital asymptomatic continuous retrograde flow in the basilar artery and left vertebral artery is a medical emergency as it implies the presence of type B interrupted aortic arch with large patent ductus arteriosus in a neonate.
PubMed: 38595944
DOI: 10.1177/2050313X241245281 -
JPMA. the Journal of the Pakistan... Mar 2024Pulmonary Artery Aneur ysm (PAA), whether congenital or acquired, is a rare diagnostic find ing com pare d to aor tic aneur ysms. There have been fe w cases where PA As...
Pulmonary Artery Aneur ysm (PAA), whether congenital or acquired, is a rare diagnostic find ing com pare d to aor tic aneur ysms. There have been fe w cases where PA As were documented as a complication of untreated Patent Ductus Ar teriosus (PDA) due to long-standing Pulmonary Arterial H ypertension (PAH). However, it is quite rare for a case of PAA to be reported with co-existing PDA without PAH. This report highlights a case of a five -year-old girl who was presented with palpitations, easy fatigability, fever, c yanos is, and vomiting. A Chest X-ray s howed mo derate cardiomega ly. A PDA of 6 mm was diagnosed on Transthoracic E chocardiog rap hy ( TTE ) and a large cavity con necte d with LPA raised suspicion of a possible LPA aneur ysm. A Chest CT scan confirm ed the diagnosis of a saccular aneurysm, originating from the distal part of the main Left Pulmonary Artery (LPA) just proximal to the point of bifurcation into lobar branches, measuring 7.5x6.5 cm. During surgery, the aneurysm was opened, emptied with suction and closed without resecting the aneur ysmal walls. The patient had an uneventful post-op course and is doing well during regular interval follow up visits.
Topics: Child, Preschool; Female; Humans; Aneurysm; Ductus Arteriosus, Patent; Pulmonary Artery; Tomography, X-Ray Computed; Vascular Malformations
PubMed: 38591305
DOI: 10.47391/JPMA.9952 -
Heliyon Apr 2024We aimed to evaluate the safety and efficacy of radiation dose reduction with a new-generation biplane angiocardiography system in patients undergoing transcatheter...
OBJECTIVE
We aimed to evaluate the safety and efficacy of radiation dose reduction with a new-generation biplane angiocardiography system in patients undergoing transcatheter isolated patent ductus arteriosus (PDA) closure.
MATERIALS AND METHODS
Fifty pediatric patients who underwent transcatheter PDA closure were randomly divided into two groups as normal radiation dose and low dose. Patients who required additional procedures other than PDA closure were excluded. PDA closure was performed according to the angiographic measurement of the defect. After the procedure, age, weight, sex, PDA measurements, and radiation measurements such as dose-area product (DAP, Gy.cm) and air kerma (AK, mGy) were compared between the groups.
RESULTS
There was no statistically significant difference between the groups in age, sex, weight, PDA diameter, PDA type, device used, and device diameter (p > 0.05). While there was no statistically significant difference between the groups in terms of cine recording, number of recorded images, and fluoroscopy time (p > 0.05), there was a statistically significant difference between the total DAP, cine and fluoroscopy DAP, total AK, frontal and lateral tube AK, and DAP/kg (mGy.m/kg) measurements (p < 0.05).
CONCLUSION
Transcatheter PDA closure with a low radiation dose is as effective as that with a normal radiation dose. The radiation dose received by the patient during the procedure was significantly reduced. With the vision provided by this study, it seems possible to work with a low radiation dose in other groups of patients.
PubMed: 38590841
DOI: 10.1016/j.heliyon.2024.e28138 -
Turk Gogus Kalp Damar Cerrahisi Dergisi Jan 2024In this study, the flip technique was compared with the classical method in terms of procedural success and procedure time during ductal stent implantation (DSI) via the...
BACKGROUND
In this study, the flip technique was compared with the classical method in terms of procedural success and procedure time during ductal stent implantation (DSI) via the carotid artery in patients whose pulmonary blood flow is dependent on the vertical type of ductus arteriosus (DA).
METHODS
Between January 2019 and June 2023, 40 patients (24 males, 16 females; mean age: 15.9±15.4 days; range, 1 to 68 days) with vertical ductus-dependent pulmonary circulation who underwent patent DA stent implantation via the carotid artery were included in the study. Patients were divided into two groups: those who underwent the flip technique (Group 1) and those who did not undergo the flip technique (Group 2). Data were retospectively compiled by reviewing patient files and catheter images.
RESULTS
Demographic findings were similar in the groups. The distribution of the DA in terms of tortuosity index was also similar in the groups. The procedure was successful in 18 (90%) patients in Group 1 and 20 (100%) patients in Group 2. There was no procedure-related mortality in both groups. The frequency of procedure-related complications was similar. Procedure duration was 53.6±18.4 min in Group 1 and 41.5±9.1 min in Group 2; the difference was significantly lower in Group 2 (p=0.028). The shorter follow-up in Group 2 was attributed to the flip method starting to be used later in the clinic. During follow-up, stent dilatation was required in two patients in Group 1 and one patient in Group 2, and a second stent implantation was needed in one patient in Group 2. No significant difference was observed between the two groups in terms of reintervention.
CONCLUSION
The success rate of DSI using the carotid artery access is high with both the classical and the flip method in patients with vertical DA. However, the use of the flip technique could be preferred in terms of operator habituation, ergonomic use of the catheter, guidewires, and shorter procedure time.
PubMed: 38545363
DOI: 10.5606/tgkdc.dergisi.2024.25813 -
CJC Pediatric and Congenital Heart... Feb 2024Congenital malformations of the trachea are rare but often life-threatening. Limited data have been published on the outcomes of tracheal reconstruction for congenital...
BACKGROUND
Congenital malformations of the trachea are rare but often life-threatening. Limited data have been published on the outcomes of tracheal reconstruction for congenital tracheal stenosis. We sought to describe the outcomes of patients undergoing tracheal reconstruction over 10 years at our centre.
METHODS
All paediatric patients who underwent long-segment tracheal or bronchial reconstruction from January 1, 2012, to August 31, 2022, were included. The primary outcome was mortality, and secondary outcomes included reoperation and postoperative morbidity. Patients were followed up to 10 years.
RESULTS
Thirty-three patients with ages ranging from 1 day to 12 years (mean 8.5 months) at the time of tracheoplasty or bronchoplasty were included, with 5 patients undergoing off-pump tracheal reconstruction. The most common preoperative comorbidities included patent ductus arteriosus (30.3%), atrial septal defect (27.3%), and prematurity (24.2%). There were no deaths postoperatively within the follow-up period. All patients experienced successful reconstruction with no patients requiring reoperation of the trachea. A total of 14 patients (42.4%) required postoperative balloon dilation, 3 (9.1%) required bronchial repair after tracheal repair, and 2 (6.1%) required bronchoscopic tracheal debridement.
CONCLUSIONS
This single-centre retrospective study provides a large cohort of congenital tracheal reconstruction patients with a survival rate of 100%, experiencing no mortality during follow-up. The majority of patients had preoperative comorbidities and concomitant congenital cardiac defects. Although tracheal reconstruction continues to be complex with significant postoperative morbidity and mortality, the results of our single-centre study demonstrate the continual advancement of this field and the evolving improvement of postoperative outcomes for these patients.
PubMed: 38544878
DOI: 10.1016/j.cjcpc.2023.10.003 -
Diagnostics (Basel, Switzerland) Mar 2024Sotos syndrome is an autosomal dominant condition characterized by overgrowth with advanced bone age, macrodolicocephaly, motor developmental delays and learning...
Sotos syndrome is an autosomal dominant condition characterized by overgrowth with advanced bone age, macrodolicocephaly, motor developmental delays and learning difficulties, and characteristic facial features caused by heterozygous pathogenetic variants in the gene located on chromosome 5q35. The prevalence of heart defects (HDs) in individuals with Sotos syndrome is estimated to be around 15-40%. Septal defects and patent ductus arteriosus are the most commonly diagnosed malformations, but complex defects have also been reported. The aim of our study was to analyze the prevalence of HD, the anatomic types, and the genetic characteristics of 45 patients with Sotos syndrome carrying pathogenetic variants of or a 5q35 deletion encompassing , who were followed at Bambino Gesù Children's Hospital in Rome. Thirty-nine of the forty-five patients (86.7%) had a mutation in , while six of the forty-five (13.3%) had a deletion. Most of the patients (62.2%, 28/45) were male, with a mean age of 14 ± 7 years (range 0.2-37 years). A total of 27/45 (60.0%) of the patients had heart defects, isolated or combined with other defects, including septal defects (12 patients), aortic anomalies (9 patients), mitral valve and/or tricuspid valve dysplasia/insufficiency (1 patient), patent ductus arteriosus (3 patients), left ventricular non-compaction/hypertrabeculated left ventricle (LV) (4 patients), aortic coarctation (1 patient), aortopulmonary window (1 patient), and pulmonary valve anomalies (3 patients). The prevalences of HD in the two subgroups (deletion versus intragenic mutation) were similar (66.7% (4/6) in the deletion group versus 58.91% (23/39) in the intragenic variant group). Our results showed a higher prevalence of HD in patients with Sotos syndrome in comparison to that described in the literature, with similar distributions of patients with mutated and deleted genes. An accurate and detailed echocardiogram should be performed in patients with Sotos syndrome at diagnosis, and a specific cardiological follow-up program is needed.
PubMed: 38535015
DOI: 10.3390/diagnostics14060594 -
CASE (Philadelphia, Pa.) Mar 2024• Large uncorrected PDA may first be diagnosed during pregnancy. • Multimodality imaging may be safely utilized in these patients. • Multimodality imaging is...
• Large uncorrected PDA may first be diagnosed during pregnancy. • Multimodality imaging may be safely utilized in these patients. • Multimodality imaging is safely utilized in these patients. • Multidisciplinary team approach is key for percutaneous closure of PDA in pregnancy.
PubMed: 38524975
DOI: 10.1016/j.case.2023.12.008 -
Frontiers in Pediatrics 2024Hyperglycemia in pregnancy (HGP) has generally been considered a risk factor associated with adverse outcomes in offspring, but its impact on the short-term outcomes of...
BACKGROUND
Hyperglycemia in pregnancy (HGP) has generally been considered a risk factor associated with adverse outcomes in offspring, but its impact on the short-term outcomes of very preterm infants remains unclear.
METHODS
A secondary analysis was performed based on clinical data collected prospectively from 28 hospitals in seven regions of China from September 2019 to December 2020. According to maternal HGP, all infants were divided into the HGP group or the non-HGP group. A propensity score matching analysis was used to adjust for confounding factors, including gestational age, twin or multiple births, sex, antenatal steroid administration, delivery mode and hypertensive disorders of pregnancy. The main complications and the short-term growth status during hospitalization were evaluated in the HGP and non-HGP groups.
RESULTS
A total of 2,514 infants were eligible for analysis. After matching, there were 437 infants in the HGP group and 874 infants in the non-HGP group. There was no significant difference between the two groups in main complications including respiratory distress syndrome, bronchopulmonary dysplasia, necrotizing enterocolitis, retinopathy of prematurity, patent ductus arteriosus, culture positive sepsis, intraventricular hemorrhage, periventricular leukomalacia, anemia, feeding intolerance, metabolic bone disease of prematurity, or parenteral nutrition-associated cholestasis. The incidences of extrauterine growth retardation and increased growth retardation for weight and head circumference in the non-HGP group were all higher than those in the HGP group after matching ( < 0.05).
CONCLUSIONS
HGP did not worsen the short-term outcomes of the surviving very preterm infants, as it did not lead to a higher risk of the main neonatal complications, and the infants' growth improved during hospitalization.
PubMed: 38510082
DOI: 10.3389/fped.2024.1341221