Arterial Duct Research Articles

The Effect of Oral Acetaminophen on Open Arterial Duct Closure in Premature Infants Less Than 32 Weeks

Abstract Background In most term neonates, the ductus arteriosus closes functionally on the first day of life, and a permanently open ductus arteriosus is abnormal. Objectives To evaluate the effect of oral acetaminophen on patent arterial duct (PDA) closure in premature infants less than 32 weeks. Methods This randomized clinical trial was performed on 140 preterm neonates with gestational age less than 32 weeks and birth weight less than 1,500 g, admitted to the neonatal intensive care unit of Ali Enbe Abitalib Hospital in Zahedan between 2019 and 2021. The neonates were randomly divided into two groups, with and without acetaminophen administration. In the intervention group, acetaminophen drops were started during the first 24 hours after birth and were administered for five days. In both groups, echocardiography was performed five days after birth. Finally, the data were analyzed in SPSS software. Results About 140 neonates with gestational age of ≤32 weeks and weight at birth ≤1,500 g were admitted for the study. The neonates were equally divided into two groups and acetaminophen medication assigned for one of the groups. Of the 70 neonates who did not receive acetaminophen administration, 75.7% had PDA closure spontaneously; this pattern was 94.3% in patients who had treatment with acetaminophen. This effect of treatment was significant (X2=9.468, p=0.002). Conclusion The study showed significant improvement in PDA in neonates receiving paracetamol. This can have a significant impact on the treatment approach to preterm neonates.

Treatment of Patent Ductus Arteriosus in Premature Infants: Intravenous Paracetamol or Oral Ibuprofen?

The similarity in the mechanism of action between paracetamol and ibuprofen can cause similar side effects. However, in preterm neonates with feeding intolerance, intravenous (IV) paracetamol has replaced oral ibuprofen. Therefore, a comparison of the effectiveness and side effects is essential. In this retrospective cohort study, the data of 118 preterm infants with a definite diagnosis of patent ductus arteriosus (PDA), including 59 patients who received oral ibuprofen and 59 patients who received IV paracetamol were analyzed. Laboratory evaluations of serum total and direct bilirubin, hemoglobin, and creatinine levels before and seven days after treatment were made. Using analysis of covariance (ANCOVA) and multiple multinomial logistic regression models, the effect of two treatment groups on the post-treatment variables as well as their efficacy comparison were evaluated. In both pre- and post-treatment periods, there was no significant association between echocardiography variables with treatment groups. The results from the ANCOVA model showed that the paracetamol and ibuprofen were followed by a significant decrease in the mean total bilirubin and Hct variables after treatment by 1.38 and 1.65 units, respectively. In addition, results from the Mann-Whitney U test revealed that the median Hb and K differences after and before treatment had a significant difference between the two treatment groups. Furthermore, based on the multiple multinomial logistic model results, the odds of complete arterial duct closure with IV paracetamol was 1.27 times higher than with oral ibuprofen, while in the oral ibuprofen group, the odds of closing was 1.44 times higher than the IV paracetamol group, but there was no statistically significant difference between the two groups. Intravenous paracetamol has equal efficacy compared to oral ibuprofen in the treatment of PDA. Also, it seems to be associated with a lower risk of hyperbilirubinemia following the treatment.

Combination chromosome abnormaliries in girl with malformations of the large intestine

The purpose of the clinical case was to describe combination chromosome abnormaliries in girl with malformations of the large intestine.Girl A., 1, 9 year old was born prematurely. From birth, multiple stigmas of dysembriogenesis: poor weight gain, absence of an independent stool, marked delayed physical and psychomotor development attracted attention. During doppler echocardiography, a left-sided right- formed heart (chest), an open arterial duct, hemodynamically insignificant, with a diameter of about 1 mm is determined. In the second year of life, cholelithiasis was diagnosed (a single gallbladder concretion of 1.5 mm), erosive- hemorrhagic gastritis, subatrophic duodenoejunitis, an anomaly of the development of the large intestine (dolichosigma, dolichocolon) with hypomotor-type kolodiskinesia. During the examination, multiple stigmas of dysembriogenesis (oblique occiput, ocular hypotelorism, large, protruding auricles, strabismus, umbilical hernia), marked muscular hypotension, marked delayed physical and psychomotor development attract attention. Physical development: body length 70 cm, body weight 6650 g, teeth 8. SDS height –4.5, BMI 13.57 kg/m2, SDS –6.12. A molecular cytogenetic study revealed a normal female karyotype in proband’s mother, an abnormal karyotype 46, XX, der (21) t(4;21)(q31; q22) pat in proband’s mother, and a normal male karyotype with a balanced translocation 46, XX, t(4:21) in the patient’s father(q31: q22). The child has a chromosomal anomaly: partial trisomy of the q-arm of chromosome 4, as a result of translocation between chromosomes 4 and 21 of paternal inherited. Thus, the patient has an unbalanced karyotype, which causes a clinical features characterized by the marked delay in psychomotor and physical development, multiple stigmas of dysembriogenesis and malformations of the large intestine.

Impact on the trachea in children with prenatally diagnosed vascular ring formed by a right aortic arch.

There has been a rise in the prenatal detection of right sided aortic arch (RAA). When associated with a left-sided arterial duct (LD), this forms a vascular ring encircling the trachea. Infants may have symptoms or signs suggestive of tracheoesophageal compression but many are asymptomatic. The objective of this study was to investigate the relationship between symptoms and severity of tracheobronchial compression assessed by bronchoscopy. Retrospective review of all cases of prenatally diagnosed RAA-LD in the absence of associated congenital heart disease at Evelina London Children's Hospital and Kings College Hospital over a 4-year period between April 2015-2019. Clinical records, fetal echocardiograms, and free-breathing flexible bronchoscopy (FB) data were reviewed. One hundred and twelve cases of isolated RAA-LD were identified of whom 82 cases (73%) underwent FB. FB was performed median age of 11 months (range 1-36 months), no complications occurred. Aberrant left subclavian artery (ALSA) was present in 86% (96/112) and mirror image branching (MIB) in 13% (15/112). 34/112 (30%) reported symptoms during follow-up. 36/77 (47%) with ALSA who underwent FB showed moderate-severe compression mostly at distal tracheal and carinal level of whom 38% had parent-reported symptoms. Moderate-severe compression was seen in 3/5 (60%) with MIB mostly at mid tracheal level; 3 were symptomatic but only 2 had tracheal compression. In total 36% (18/50) of investigated asymptomatic patients showed moderate-severe compression. Respiratory symptoms were poorly predictive of moderate-severe tracheal compression (positive predictive value 66%, negative predictive value 64%). The absence of symptoms did not exclude significant tracheal compression. The anatomical effect of the vascular ring is under appreciated when symptoms alone are used as a marker of tracheal compression.

Echocardiographic score to predict neonatal surgery for aortic coarctation in newborns with prenatal suspicion and patent ductus arteriosus

Introduction The evaluation of upcoming Aortic Coarctation (CoA) in new-borns with prenatal suspicion entails a close echocardiographic monitor until Arterial Duct (AD) closure, in a department with pediatric cardiological and surgical expertise. The significant number of false-positive prenatal diagnoses causes parental stress and healthcare costs. Aim The aim of this study was to elaborate an echocardiographic prediction model to be employed at birth when PDA is still present, in patients suspected of CoA during fetal life in order to foretell CoA requiring neonatal surgical intervention. Methods This retrospective monocentric study included consecutive full-term and late preterm neonates with prenatal suspicion of CoA born from 01 January 2007 to 31 December 2020. Patients were divided into two groups according to the need for aortic surgery (CoA - NoCoA). All patients underwent a comprehensive transthoracic echocardiographic exam in presence of PDA. Multivariable logistic regression was used to create a coarctation probability model (CoMOD) including isthmal (D4), transverse arch (D3) diameters, the distance between a left common carotid artery (LCA) and left subclavian artery (LSA), presence/absence of ventricular septal defect (VSD) and bicuspid aortic valve (BAV). Results We enrolled 87 neonates (49 male, 56%). 44 patients developed CoA in need of surgical repair. Our index CoMOD showed an AUC = 0.9382, high sensitivity (91%) and specificity (86%) in the prediction of CoA in neonates with prenatal suspicion. We classified neonates with CoMOD > 0 to be at high risk for surgical correction of CoA, with good PPV (86.9%) and NPV (90.9%). Conclusions CoMOD > 0 is highly suggestive of the need for CoA corrective surgery in newborns with prenatal suspicion.

Predictive value of a nomogram model for adverse outcomes in very low birth weight infants with patent ductus arteriosus: A prospective study.

To establish a nomogram model incorporating markers of echocardiography and N-terminal pro brain natriuretic peptide (NT-proBNP) for predicting adverse outcomes of patent ductus arteriosus (PDAao) in very low birth weight infants and to evaluate the predictive values of the model. A prospective study was conducted for very low birth weight infants who were admitted from May 2019 to September 2020. An echocardiogram and blood NT-proBNP test were carried out in the first 48 h after birth, and the arterial duct remained open in all patients. Other data collected included clinical symptoms and infant characteristics. A nomogram model was established to predict the risk of PDAao (including severe BPD, IVH, NEC or death). Internal verifications were performed for the nomogram, and the discrimination and calibration of the model were evaluated by the C-index and calibration curve. Eighty-two infants were enrolled and divided into an adverse outcome (AO) group and normal outcome (NO) group with 41 patients in each group. PDA diameter, PDA maximum flow velocity, left atrium diameter/aortic diameter (LA/AO) ratio and NT-proBNP level were independent risk factors for PDAao and were included in the nomogram model. The model presented good discrimination with a C-index of 0.917 (95% CI 0.859-0.975). The calibration curves in showed high consistency and indicated good Correspondence: between the event incidence predicted by the nomogram model and the true incidence of PDAao. The nomogram model incorporating the PDA diameter, PDA maximum flow velocity, LA/AO ratio and NT-proBNP level in the first 48 h could early predict the later occurrence of PDAao in very low birth weight infants.

Early spontaneous closure of large arterial ducts in two term neonates with Ebstein anomaly after failed attempts of transcatheter closure.

In neonatal Ebstein's anomaly of the tricuspid valve, prolonged ductal patency in patients without anatomic pulmonary valve atresia can be deleterious. Circular shunts may develop in patients with different degrees of pulmonary and tricuspid insufficiency. Closure of the arterial duct may result in haemodynamic improvement in particular scenarios. The transcatheter approach is a valuable closure alternative despite some technical difficulties in large-sized arterial ducts and low birth weight neonates. Herein, we report on two consecutive term newborns with Ebstein's anomaly and large arterial ducts in whom mechanical stimulus of the arterial duct during failed attempts of transcatheter closure led after two days to definitive closure followed by good clinical outcomes.

Interventional treatment and early-stage follow-up of pulmonary atresia with intact ventricular septum

Objective: To explore the application value of percutaneous peripheral interventional therapy in pulmonary atresia with intact ventricular septal (PA-IVS). Methods: Retrospective case summary. The data was collected from 25 children who were hospitalized at the Children's Hospital,Zhejiang University School of Medicine from August 2019 to August 2022, had been diagnosed with PA-IVS by echocardiography, and underwent interventional treatment. The sex, age, weight, operation time, radiation exposure time, and radiation dose of the patients were collected. The patients were divided into the arterial duct stenting group and the non-stenting group. Preoperative tricuspid annular diameters and Z scores, right ventricular length diameters, and right ventricular/left ventricular length-diameter ratios were compared by paired t-tests. Right ventricular systolic pressure difference, oxygen saturation, lactic acid before and after the surgery were compared for 24 children who received percutaneous balloon pulmonary valvuloplasty. Right ventricular improvement in 25 children after operation was analyzed. The correlation between postoperative oxygen saturation and postoperative right ventricular systolic blood pressure difference, the degree of pulmonary valve opening and the Z value of tricuspid valve ring in the non-stenting group were analyzed. Results: A total of 25 patients with PA-IVS were enrolled in the study, of whom 19 were males and 6 females, with an age at surgery of 12 (6, 28) days and a weight of (3.7±0.5) kg. One of them underwent only stenting of the arterial duct; 20 children underwent only percutaneous pulmonary valve perforation and balloon angioplasty; 4 children underwent both procedures. The Z-value of the tricuspid ring was -1.5±1.2 in the group with arterial duct stenting, and -0.1±0.4 in the group without stenting (t=2.77, P=0.010). The tricuspid regurgitant flow rate 1 month after surgery was significantly lower than the preoperative ((3.4±0.6) vs. (4.8±0.9) m/s, t=6.62,P<0.001). In the 24 children with percutaneous pulmonary valve perforation and balloon angioplasty, the preoperative right ventricular systolic blood pressure was (110±32) mmHg, and the postoperative systolic blood pressure was (52±19) mmHg (1 mmHg=0.133 kPa) (F=59.55, P<0.001). The factors that may affect postoperative oxygen saturation in 20 cases of non-stenting group were analyzed. The results suggested that the pre and post-operative right ventricular systolic blood pressure differences (r=-0.11, P=0.649), and the pulmonary valve orifice opening (r=-0.31, P=0.201) and tricuspid annulus Z value (r=-0.18, P=0.452) at 1 month after the operation were not significantly correlated with the postoperative oxygen saturation. Conclusions: Interventional therapy can be used as the first choice for one-stage operation of PA-IVS. Percutaneous pulmonary valve perforation and balloon angioplasty are more suitable for children with well-developed right ventricles, tricuspid annulus, and pulmonary arteries. While the smaller the tricuspid annulus, the more dependent it is on the ductus arteriosus and thus patients are more suitable for arterial duct stenting.

Evaluation of the Relationship Between Perfusion Index and the Improvement of Patent Ductus Arteriosus

Background: Patent ductus arteriosus (PDA) is a disease presenting on the first days of birth, associated with unpleasant complications such as exaggerated respiratory distress and increased mortality, requiring prompt treatment. Objectives: This study evaluated the relationship between perfusion index and the recovery of patients diagnosed with PDA. Methods: In this cross-sectional study, 22 neonates with PDA (as the case group) and 22 healthy neonates (as the control group) were selected. The difference in perfusion index (PI) before and after arterial duct closing was determined, and also the relationship between pre-ductal and post-ductal PI in the case and control groups was evaluated. Data were analyzed by SPSS version 21. Results: The results showed a significant difference in the mean scores of pre-ductal and post-ductal PI before and after arterial duct closure (P = 0.004). Also, pre-ductal and post-ductal PI scores were significantly lower in the case group than in the control group (P &lt; 0.001) Conclusions: According to our findings, PI in infants with PDA is low at presentation and increases after treatment. Therefore, PI can be used as an indicator to evaluate response to treatment during follow-up, particularly when repeated echocardiography is not accessible.

Patent ductus arteriosus (review): history, anatomy and physiology of a functioning duct

Fetal communication in the vascular system of the fetus is necessary for fetal blood circulation. Metabolic support is provided through the placenta and vessels of the umbilical cord. The function of the arterial duct (ductus arteriosus) is the main component of the fetal blood circulation, which acts as a link between the pulmonary artery and aorta when the babys lungs are not functioning and the placenta is the gas exchange organ. Disorders occurring in the physiological processes of the cardiovascular system, especially in the intranatal period, can lead to the development of severe pathology. One of the most important defects in the structure and, consequently, in the nature of the blood flow, is the failure of the arterial duct. The patent ductus arteriosus is a necessary anatomical structure at the heart of the fetal circulatory system. Normally, the patent ductus arteriosus (PDA) must close soon after birth, turning into a ligamentum arteriosum. If there is no closure of the duct or its partial obliteration occurs, then it means there is the presence of the defect. The references to it go far into antiquity. This review presents the main stages of study, anatomical and physiological features of the ductus arteriosus.

Surgical treatment for patent ductus arteriosus in adult patients: a single center experience.

In contemporary practice, percutaneous closure of the patent ductus arteriosus (PDA) has become the procedure of choice. While the surgical ligation of ductus arteriosus ensures an immediate and definitive obliteration of the ductus, this therapeutic option is rarely used and reserved for situations when percutaneous solution is unsuitable. In this manuscript, we summarize the clinical and intraoperative findings of consecutive adult patients referred to our Institution in an interval of 10 years for surgical treatment of PDA. A total of five cases of surgical closure for PDA were performed in our Center. Four subjects were not suitable for percutaneous closure, and one was discovered intraoperatively, during surgery for another cardiac condition. In all patients, the closure of the PDA was carried out by means of a suture with reinforced patch threads, in a double layer. The intervention was performed in total cardiopulmonary bypass and mild or moderate hypothermia, through a transpulmonary approach. Total circulatory arrest was not required, in any of the cases. The occlusive balloon technique was applied to all patients. All patients survived the intervention and did not suffer perioperative complications. Postoperative follow-up at 36 months did not show repermeabilization of the arterial duct or aneurysmal dilation of the adjacent aorta. Moreover, all patients showed postoperative improvement in the performance of the left ventricle. In adult patients with PDA and contraindication to percutaneous closure or in those who require surgical sanction for other cardiac diseases, surgical closure of the duct is safe and associated with favorable clinical evolution.

Outcomes of different types of pulmonary atresia in neonates treated by ductus arteriosus stenting

Objective: To summarize the outcomes of different types of pulmonary atresia in neonates treated by ductus arteriosus stenting. Methods: This study was a retrospective cohort study. A total of 19 neonates who had pulmonary atresia treated by ductus arteriosus stenting in Xinhua Hospital, Shanghai Jiao Tong University School of Medicine from April 2014 to June 2021 were included. They were divided into the intact ventricular septum (PA-IVS) group and the ventricular septal defect (PA-VSD) group. Ductus arteriosus stents were implanted by different approaches. These children were followed up regularly at the 1, 3, 6, and 12 months after the surgery and annually since then to evaluate the outcome. Independent sample t-test was used for the statistical analysis. Results: There were 12 children in PA-IVS group and 7 in PA-VSD group. All of them were full term in fants. The gestational age of the PA-IVS group and the PA-VSD group was (38.8±1.1) and (37.7±1.8) weeks, the birth weights were (3.2±0.4) and (3.4±1.1) kg, and the age at operation was (10±9) and (12±7) days, respectively, without significant difference (all P>0.05). Among the 12 children with PA-IVS, 9 had stents successfully implanted through the femoral artery and 3 through the femoral vein. Of the 7 children with PA-VSD, 2 had the stents successfully implanted via the femoral artery and 2 failed, and the remaining 3 had stents successfully implanted via the left carotid artery. There was no postoperative thromboembolism, arteriovenous fistula, pseudoaneurysm or other vascular complications. Five children with PA-VSD who had successful operations were followed up at 6 months of age. They all had the operation for pulmonary atresia, repair of the ventricular septal defect, removal of arterial duct stents, and ligation of the arterial duct. All children survived without any stent displacement or stenosis and biventricular circulation was achieved during the follow-up. Conclusions: Ductus arteriosous stenting can be the first-stage treatment for children with PA-IVS and PA-VSD. In addition to the traditional femoral vein and femoral artery approach, the carotid artery can be used as a route for stent placement.

Trisomy 18: Anti-Congestive Drug Treatment Combined with Transcatheter Occlusion of an Arterial Duct and Misaligned Perimembranous VSD

<i>Background </i>Trisomy 18 syndrome, the second most common autosomal numerical chromosomal disorder, is associated with multiple-organ abnormalities. Cardiovascular malformations are just one of those affecting prenatal and postnatal life expectancy. In general, the type and scope of treatment are still controversial and are handled very differently. Palliative care usually focuses on improving quality of life or life-threatening conditions. <i>Objective</i> Based on parental treatment preferences for their child, we report the anticongestive drug treatment and consecutive transcatheter therapy of cardiac shunt lesions in a girl with trisomy 18 who was born small-for-date. <i>Methods </i>Anticongestive drug treatment was switched form diuretic-directed drug treatment to our standard care for chronic heart failure in infants, which consists of a ß1-specific beta-blocker (bisoprolol) in combination with an ACE-inhibitor (lisinopril) and a low-dose mineralocorticoid-blocker (spironolactone). In addition, the strategy and technique of semi-invasive transcatheter treatment of significant cardiovascular shunt lesions is reported. At 3 months of age and weighing 2.4kg, a significant arterial duct was first occluded percutaneously with an MVP-5Q device, followed by transcatheter closure of a slightly misaligned perimembranous VSD six weeks later. Both interventions were performed on a spontaneously breathing only sedated baby. <i>Results </i>The device strategy for treating the hemodynamically significant cardiovascular shunt lesions was technically feasible and effective in combination with medication. However, the medium- or long-term outcome cannot be defined due to the limitations caused by the syndrome. <i>Conclusion</i> As part of a medical risk-benefit assessment, the parents should make the decision as to whether their child should be treated or not; the responsible physicians have individually to offer the best treatment option.

Comparison of treatment strategies for neonates with tetralogy of Fallot and pulmonary atresia

ObjectiveNeonates with tetralogy of Fallot and pulmonary atresia (TOF/PA) but no major aorta-pulmonary collaterals are dependent on the arterial duct for pulmonary blood flow and require early intervention, either by primary (PR) or staged repair (SR) with initial palliation (IP) followed by complete repair (CR). The optimal approach has not been established. MethodsNeonates with TOF/PA who underwent PR or SR were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Outcomes were compared between PR and SR (IP + CR) strategies. Propensity scoring was used to adjust for baseline differences. The primary outcome was mortality. Secondary outcomes included complications, length of stay, cardiopulmonary bypass and anesthesia times, reintervention (RI), and pulmonary artery (PA) growth. ResultsOf 282 neonates, 106 underwent PR and 176 underwent SR (IP: 144 surgical, 32 transcatheter). Patients who underwent SR were more likely to have DiGeorge syndrome and greater rates of mechanical ventilation before the initial intervention. Mortality was not significantly different. Duration of mechanical ventilation, inotrope use, and complication rates were similar. Cumulative length of stay, cardiopulmonary bypass, and anesthesia times favored PR (P ≤ .001). Early RI was more common in patients who underwent SR (rate ratio, 1.42; P = .003) but was similar after CR (P = .837). Conduit size at the time of CR was larger with SR. Right PA growth was greater with PR. ConclusionsIn neonates with TOF/PA, SR is more common in greater-risk patients. Accounting for this, SR and PR strategies have similar mortality. Perioperative morbidities, RI, and right PA growth generally favor PR, whereas SR allows for larger initial conduit implantation.

Haemodynamic and clinical variables after surgical systemic to pulmonary artery shunt placement versus arterial ductal stenting.

Transcatheter stenting of the arterial duct is an alternative to surgical systemic to pulmonary artery shunt in neonates with parallel circulation. The current study compares haemodynamic and laboratory values in these patients for the first 48 hours after either intervention. Neonates with ductal dependent pulmonary blood flow who underwent surgical shunt placement or catheter-based arterial ductal stent placement between January 2013 and January 2022 were identified. Haemodynamic variables included heart rate, blood pressure, near infrared spectroscopy, central venous pressure, vasoactive inotropic score, and arterial saturation. Laboratory variables collected included blood urea nitrogen, serum creatinine, and serum lactate. Variables were collected at baseline, upon post-procedural admission, 6 hours after admission, 12 hours after admission, and 48 hours after admission. Secondary outcomes included post-procedural mechanical ventilation duration, post-procedural hospital length of stay, need for reintervention, need for extracorporeal membrane oxygenation, cardiac arrest, and inpatient mortality. Of the 52 patients included, 38 (73%) underwent shunt placement while 14 (27%) underwent a stent placement. Heart rates, renal oxygen extraction ratio, and cerebral oxygen extraction ratio were significantly lower in the stent group (p = <0.01, 0.01, and < 0.01, respectively).Haemoglobin and vasoactive inotropic scores were significantly lower in the stent group (p = <0.01, <0.01, respectively). The stent group had increased risk for cardiac arrest (p = 0.04). Patients who undergo arterial ductal stent placement have lower heart rates, haemoglobin, renal oxygen extraction ratio, cerebral oxygen extraction ratio, and vasoactive inotropic score in the first 48 hours post-procedure compared to patients with shunt placement.

Retracted: Cases of Ultrasound-Diagnosed Right Aortic Arch with Right Arterial Duct and the Treatment.

[This retracts the article DOI: 10.1155/2022/8252808.].

Clinical and morphological features of arterial duct in premature newborns

The open arterial duct has a pathological significance in premature newborns. Closure of the ductus arteriosus is a complex process that includes functional and anatomical closure.Purpose. To identify the features of arterial duct remodeling in premature newborns.Material and methods. We conducted a retrospective clinical and morphological analysis of 35 deceased premature newborns. During their lifetime, all children were examined using clinical and laboratory-instrumental methods. In all cases, autopsies of deceased children were performed with a complex of morphological studies of the arterial duct.Results. It has been established that the open arterial duct in premature newborns plays an important role in increasing respiratory failure. It also necessitates the appointment and conduct of invasive respiratory therapy. The functioning of the arterial duct is facilitated by low partial pressure and blood oxygen saturation levels. Morphofunctional features of arterial duct remodeling were revealed. A positive correlation was established between the indicators of the gas composition of the child’s blood and the thickness of the duct wall.Conclusion. Risk factors for an open arterial duct in premature newborns are the age of the mother over 35 years, threatened miscarriage, prenatal discharge of amniotic fluid, gestational diabetes mellitus, fetoplacental insufficiency, and severe asphyxia at birth. Morphological changes in the form of thickening of the endothelial layer and the formation of intimal pillows, fragmentation and focal necrosis of the internal elastic membrane with the accumulation of mucoid matter, proliferation and migration of smooth muscle cells into the subendothelial space reflect the processes of remodeling of the arterial duct.

Long-term outcomes of transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension.

Transcatheter Potts shunt (TPS) is a palliation alternative for children with severe pulmonary arterial hypertension (PAH). Debates on the long-term outcomes remain unsolved. To evaluate long-term clinical and procedural outcomes of TPS intervention. Single-center retrospective data review of children with severe PAH who had TPS between 2009 and 2018. Patients who died per-operatively and early post-procedure were excluded. Long-term outcomes of survivors were evaluated. Out of 13 identified patients (53.8% males), 7 had endovascular stenting of probe/patent arterial ducts, while 6 individuals had aorta-to-pulmonary radiofrequency perforation and covered stent placement. Compared to baseline, the overall clinical condition significantly improved at discharge (p < 0.001) and stayed better at the last visit (p < 0.05) despite frequent clinical worsening events across follow-up. Improvement in functional class across follow-up was significant (p < 0.001). There was, however, no significant improvement in other disease markers (TPASE, 6MWD z-scores, and NT-proBNP levels) or reduction in PAH medications. The median follow-up was 77.4 months (IQR, 70.7-113.4). Survival was 100% at 1 year and 92.3% at 6 years. Freedom from reinterventions was 77% at 1 year and 21% at 6 years. Nine (69.2%) patients had stent reinterventions at a median of 25 months (IQR, 9.5-56) postoperative. Balloon dilatation and restenting were performed in 53.8% and 46.2% of patients, respectively. High-pressure post-dilatation of implanted stents was performed in 53.8% of patients during TPS intervention for incomplete stent expansion and/or residual pressure gradient and was associated with higher rates of reinterventions (p = 0.021). Stent malfunctioning was present in 46.2% of patients at last follow-up. Two patients are listed for heart-lung transplantation. Survivors of TPS procedures experience significant improvement in functional class that can be durable. Clinical worsening and stent malfunctioning are frequent morbid events indicating recurrent transcatheter reinterventions throughout follow-up. Six-year survival is, however, satisfactory.

Prenatal Diagnosis of Chromosome 22q11.21 Microduplication Syndrome in a Fetus with Congenital Malformation of the Cardiovascular System: Case Report

A clinical case of microduplication of chromosome 22q11.21 in a fetus with a malformation of the cardiovascular system (vascular ring) is presented. This anatomical variant of the vascular ring is extremely rare and is formed by the pathological circumflex retroesophageal left-sided cervical aortic arch with the left arterial ductus arteriosus. The anatomical features of the circumflex cervical aortic arch are the left-sided pathological curved aortic arch in combination with the right-sided descending aorta. This vascular pathology can cause compression of the trachea and esophagus. This variant of the vascular ring is extremely unfavorable for surgical correction. Chromosome 22q11.21 microduplication syndrome is associated with the clinical presentation of “cat eye syndrome”. The clinical case description shows unique data from instrumental and genetic studies. All stages of examination of a pregnant woman from primary to tertiary medical care are described. The features and importance of a multidisciplinary approach are also presented.&#x0D; Every case of congenital heart defect in fetus, confirmed by echocardiographic method in a specialized institution, requires medical and genetic counseling. Using of the highly sensitive specific molecular cytogenetic method, comparative genomic hybridization, in prenatal diagnosis allows for timely diagnosis of microstructural disorders of chromosomes in fetus. Follow-up of a pregnant woman with congenital heart defect in the fetus requires a multidisciplinary approach not only to improve diagnosis and treatment, but also to calculate genetic risk.

Long-term outcomes of Transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension

Transcatheter Potts shunt (TPS) is a palliation alternative for children with severe pulmonary arterial hypertension (PAH). Debates on the long-term outcomes remain unsolved. Single-center retrospective data review of children with severe PAH who had TPS between 2009 and 2018. Patients who died per-operatively and early post-procedure were excluded. Long-term outcomes of survivors were evaluated. Out of 13 identified patients (53.8% males), 7 had endovascular stenting of probe/patent arterial ducts, while 6 individuals had aorta-to-pulmonary radiofrequency perforation and covered stent placement. Compared to baseline, the overall clinical condition significantly improved at discharge ( P < 0.001) and stayed better at the last visit ( P < 0.05) despite frequent clinical worsening events across follow-up. Improvement in functional status across follow-up was significant ( P < 0.001). There was no significant improvement in other disease markers (TPASE, 6MWD z-scores, and NT-proBNP levels) or reduction in PAH medications. The Median follow-up was 62.7 months (IQR, 32.2–96.5). Survival was 100% at 1 year and 92.3% at 5 years. Freedom from reinterventions was 77% at 1 year and 17% at 5 years. Eight (61.5%) patients had stent reinterventions at a median of 22 months (IQR, 8.8–49.8) postoperative. Balloon dilatation and restenting were performed in 46.1% and 38.5% of patients, respectively. Stent malfunctioning was present in 46.2% of patients at the last follow-up. One patient is listed for heart-lung transplantation. Survivors of TPS procedures experience significant improvement in functional class that can be durable. Clinical worsening and stent malfunctioning are frequent morbid events indicating recurrent transcatheter reinterventions throughout follow-up. Five-year survival is however satisfactory.