Arterial Duct Research Articles

Embolization of vascular abnormalities in children with congenital heart diseases using Medtronic micro-vascular plugs

Micro-vascular plug (MVP) is one of the most recent addition to the armamentarium for peripheral embolization. Data on its use in congenital cardiology is scarce. We aim to evaluate our experience with MVP for embolization of vascular abnormalities in children with congenital heart diseases (CHDs). Medical records of children with CHDs who had embolizations with MVPs between April 2015 and September 2020 were reviewed. Immediate, and follow-up data were comprehensively assessed. A total of 153 patients underwent 172 procedures during which 240 embolizations using 259 MVPs were attempted. Median age and weight were 34.9 months (IQR: 4–75 months) and 12.5 kg (IQR: 4.8–19.4 kg). Targets were abnormal systemic arteries ( n = 163), patent arterial ducts ( n = 26), venous ( n = 45), and coronary-cameral fistulas ( n = 6). The median vessel diameter was 3.3 mm (IQR: 2.5–4 mm). Veins were larger than arteries (2.3 mm > 2.1 mm, P < 0.01). MVPs were implanted in narrower diameters compared to the manufacturer's recommendations. Compared to veins, solitary implanted MVPs in arteries were additionally oversized at 12.4%, 5.1%, and 7.9% for MVP-3Q, 5Q, and 7Q, respectively. Additional occlusion material (16.7%) and 2 MVPs (5.8%) were needed at the same site for complete closure. Implantation and procedure success rates were 99.2% and 96.7%, respectively. Five complications were treated percutaneously ( n = 4), and surgically ( n = 1). The safety and efficacy of vascular embolization using MVPs were demonstrated in the largest cohort of children with CHDs and in a variety of clinical settings. Immediate and stable closure is obtained with a single device in most cases. A detailed device selection chart according to vessel type and diameter is proposed to achieve intended outcomes.

Case report: A rare combination of right aortic arch with right patent ductus arteriosus and right tracheal bronchus causing impaired respiratory function

A right aortic arch with concomitant right patent ductus arteriosus and right tracheal bronchus is a rare congenital anomaly. Herein, the respiratory and circulatory functions of the child were normal at early ages, and imaging examination indicated that conservative treatment was suitable. However, with the growth and development of the child, the right tracheal bronchus was oppressed by the right arterial duct. We performed a cut and ligation of the right patent ductus arteriosus to relieve the pressure on the right tracheal bronchus. At the 6-month follow-up, the child had recovered well and exhibited no symptoms of respiratory restriction. Therefore, we believe that early interventions should be considered for this rare anatomic presentation to benefit the patient’s respiratory and circulatory systems. Our experience provides a foundational reference for future cases.

Impact of patent ductus arteriosus shunt size and duration on risk of death or severe respiratory morbidity in preterm infants born in China

The purpose of this study is to assess whether duration and size of the arterial duct were associated with severe respiratory morbidity and mortality in preterm infants. All echocardiography evaluations for patent ductus arteriosus (PDA) in a cohort of preterm infants, born at a gestational age less than 28 weeks, from birth up to 36 weeks of postconceptional age or final ductal closure were reviewed. Ductal size was measured at the pulmonary end. PDA was classified as small (E1: ductal diameter (DD) ≤ 1.5 mm), moderate (E2: 1.5 mm < DD ≤ 2.5 mm), or large (E3) (DD > 2.5 mm). The primary outcome was adverse outcome defined by the composite outcome of bronchopulmonary dysplasia (BPD) or death. Infants in whom the primary outcome occurred were classified as “high-risk” whereas patients who did not satisfy this outcome were classified as “low-risk”. Intergroup comparison (high vs. low risk) was performed using univariate and multivariate analyses. A total of 135 infants, born between 2010 and 2020, were evaluated. The primary outcome was satisfied in 46 (34.1%) patients. The high-risk group was characterized by increased duration of exposure to PDA of any (E1/E2/E3) grade (44 vs. 25.5 days, p = .0004), moderate or large (E2/E3) PDA (30.5 vs. 11.5 days, p < .0001), moderate (E2) PDA (10.8 vs.6 days, p = 0.05), and large (E3) PDA (11.5 vs.0 days, p < .0001) compared with low-risk group. Lower gestational age, prolonged duration of mechanical ventilation, higher rate of inotrope use, pharmacological therapy, and PDA ligation were also associated with development of BPD or death (high-risk group). After adjusting for confounders, the rate of inotrope use [OR 2.688, 95% CI (1.011–7.142), p = 0.047], duration of large (E3) PDA [OR 1.060, 95% CI (1.005–1.118), p = 0.03], and mechanical ventilation [OR 1.130, 95% CI (1.064–1.200), p = 0.0001] were independently associated with the composite of BPD or death. Among infants who developed BPD, 27 were classified as grade I and 18 as grade II BPD, respectively. Infants with grade II BPD had prolonged MV (20.0 vs. 9.0 days, p = 0.024), prolonged exposure to PDA of any grade (55.8 vs. 36.0 days, p = 0.03), and prolonged exposure to large (E3) PDA compared with infants with grade I BPD. Conclusion: Prolonged exposure to a large PDA was associated with severe respiratory morbidity and mortality in preterm infants. The modulator role of early intervention, in the most pathologic shunts, on severe respiratory morbidity in preterm infants should be tested in well-designed clinical trials.What is Known:• Current guidelines recommended against accelerating PDA closure of preterm infants within 2 weeks of life, with low certainty evidence indicating improved long-term outcomes.• Recent studies suggest that conservative approach regarding PDA management has detrimental effects on the respiratory outcomes in a subgroup population.What is New:• Persistent patency of significant PDA is associated with increased risk of BPD/death in extremely preterm infants.• Targeted intervention of PDA is beneficial for the at-risk preterm infants with increased PDA hemodynamic significance.

Re; Dilemma in the management of disconnected pulmonary arteries with double arterial ducts.

Re; Dilemma in the management of disconnected pulmonary arteries with double arterial ducts.

Aneurysms of the fetal arterial duct are usually benign.

Fetal arterial duct aneurysm, saccular, or fusiform enlargement of the arterial duct affect > 8% of pregnancies. It is uncommonly associated with serious sequelae postnatally, including thromboembolic events such as stroke and left pulmonary artery obstruction, rupture with demise, and vocal cord compression. Risk factors include maternal diabetes, late maternal age, maternal blood type A, large size for gestational age, and connective tissue disorders. The clinical importance remains unknown, making it difficult to determine how to monitor this finding postnatally. This is a retrospective echocardiogram study assessing the outcomes of fetally diagnosed arterial duct aneurysm. Images and records were reviewed to confirm the diagnosis and assess risk factors and outcomes. Descriptive statistics were performed. Fifty-three affected fetuses were identified. The median gestational age at diagnosis was 34.9 weeks (IQR 32.6, 36.6). The median maternal age was 31 years (IQR 27.3-34.1). Eight (15%) had maternal diabetes. The most common blood type was type O. The median maximal dimension of the aneurysm was 7.6 mm (IQR 6.1, 8.7). The aortic end was the maximal dimension in 67.9%. Median postnatal follow-up period was 76 days (IQR 7.5, 368). No patients sustained postnatal demise related to the duct, rupture of the ductal aneurysm, cerebral infarction, or other sequelae. No newborn had associated connective tissue disorders. No patients underwent ductal intervention. In our experience, no adverse outcomes related to the ductal aneurysm were identified. This should be considered when counselling families about the need for postnatal follow-up.

Platelet counts and C-reactive protein in preterm infants with patent ductus arteriosus

Introduction: Patent ductus arteriosus (PDA) is a common disorder in premature infants which causes heart failure. Platelets and C-reactive protein (CRP) play an important role in closure. Objectives: The purpose of this study was to evaluate serum CRP and plasma platelet count in preterm infants with PDA compared to infants without PDA. Patients and Methods: This case-control study was conducted on premature infants with PDA admitted to Imam Khomeini hospital in Ahvaz, Iran (2020-2021). A group of 120 infants with inclusion criteria was selected and divided into two groups of 60 subjects. The preterm infant with PDA and without PDA was defined as the case and control group, respectively. Platelet count, serum CRP, and an echocardiogram were assessed in all infants. The subjects were matched by gender, gestational age, and birth weight. Results: The mean platelet count was 194.67±74.03 (×103 /mm3 ) in the neonate with PDA, and it was significantly lower than in neonate without PDA (P=0.04). The mean of serum CRP was significantly different in neonates with PDA (11.62±5.96 mg/L) compared to neonates with closed arterial ducts (8.52±3.97 mg/L; P=0.002). Additionally, PDA was associated with high platelet distribution width (PDW). Conclusion: The findings of this study revealed that PDA is associated with a low-number of platelets and high serum levels of CRP in preterm neonates. It is suggested that further studies with a higher sample size on platelet count and/or function be performed in PDA patients to understanding more about the cause of PDA and to discover novel and beneficial aims in these cases

P163 BOTALLO ARTERIAL DUCT SURGICALLY TREATED IN URGENCY

Abstract The Botallo arterial duct connects the pulmonary artery and the aorta during fetal life; after birth there is a progressive fall in pulmonary resistance mediated by the increase in oxygen concentration and the decrease in prostaglandins with a vasodilating action. In these physiological conditions, spontaneous closure of the duct is observed within the first 48–72 hours of life. In the preterm infant the patency of the Botallo duct is very frequent due to the peculiar anatomical and physiological characteristics of the preterm infant. The incidence of PDA is higher the lower the birth weight and gestational age are. We report a case of Manuel, a baby born at 32 weeks of gestational age from elective CT due to transverse presentation with difficulty in extraction and anhydrosis. 37–year–old mother hospitalized for reduction of amniotic fluid (AFI 85 index) with no apparent rupture of the membranes, for which she began a cycle of glucocorticoids 12mg twice a day. At birth, the weight of the child was 1,780 g, length 42 cm, skull circumference 28 cm, absence of valid respiratory activity, bradycardia (78 bpm) APGAR index of 4. At 5 ‘of life he was intubated with a 3 Fi rush tube fixed to 8 cm from the buccal rim with neopuff with PIP 20 cmH2O, PEEP 5 cmH2O, FiO2 0.40, transferred to the NICU, placed in the incubator, connected to the ventilator and ventilated with A/C mode with PIP 23 cmH2O, PEEP 5 cmH2O, FiO2 0, 40 –&amp;gt; 0.60, given surfactant alfa 200 mg/kg. Anemic, plasma and red blood cells were administered multiple times; infectious disease tests (aerobic–anaerobic haemocolutre, umbilical culture, culture of OT tubes, BAL, ear and throat swabs) were negative. After 3 days, systolic heart murmur appeared 2–3/6 radiated to the axilla. The echocardium showed the persistence of the Duct of Botallo, with a mosaic jet in the pulmonary artery and a “growing” ductal flow pattern. He started treatment with indomethacin 0.2 mg/kg every 12 hours x 3 doses, but on echocardiographic control the picture remained unchanged. At 16 days of age, the newborn was transferred to the Bambin Gesù in Rome and underwent cardiac surgery via the left posterolateral thoracotomy to close the arterial duct with a Weck clip. The post–operative course was regular. Manuel was discharged 2.5 months after birth in good general conditions, good growth–weight, with a weight of 2460 g.

Critical coarctation of the aorta in newborn - A case presentation

Background: Heart defects are the most popular congenital disease noticed among infants. Aortic Coarctation (CoA) forms 5 - 10 % of congenital heart diseases. The heart defect consist on aortic coarctation between inlet of the left subclavian artery and origin of the arterial ligament. In the during neonatal and early infantile period, in case of delayed diagnosis, it may lead to acute heart failure and the death of the child. &#x0D; Case report: A female infant born at 37 weeks of gestation, by caesarean section, in good condition, was admitted to the Neonatal Department. There was a suspicion of CoA in the fetus in the pregnancy history. The Echocardiography showed a narrowing of the carpal tunel, prostin was included in the treatment. In the second day of life, the child was transferred to the Cardiology Department. Four-extremity blood pressures test showed higher values in the upper limbs than in the lower limbs. Echocardiography revealed hypoplasia of the aortic arch in the section below the exit of the second cephalic vessel with coarctation at the isthmus site. Laboratory tests showed a high level of pro-BNP. The control echocardiography showed the anatomy of the defect as before, an unobstructed wide arterial duct with a dominant R-L flow, a flow in the descending aorta of 2.7 m/s, with a coartable flow spectrum. The child was transported to the Department of Cardiac Surgery, Heart Transplantation and Mechanical Circulatory Support for Children in Zabrze for surgical treatment. &#x0D; Conclusions: In neonates and infants with critical aortic stenosis, ensure the patency of the ductus arteriosus with continuous intravenous infusion of prostaglandin E1. Currently, echocardiography is the basic method in the diagnosis of aortic stenosis. &#x0D;

Timing of surgical repair of the stented aortic arch and coarctation in neonates.

We report a neonate with aortic arch hypoplasia and coarctation, in whom patency of the arterial duct could not be re-established and who was too ill to undergo primary surgical correction safely. This patient was treated in two stages: 1) angioplasty and stenting, 2) surgical correction. The safe period for surgical correction may be 2-3 weeks after angioplasty and stenting.

A Comprehensive Approach to the Management of Patients With HLHS and Related Malformations: An Analysis of 83 Patients (2015-2021).

Background: Some patients with hypoplastic left heart syndrome (HLHS) and HLHS-related malformations with ductal-dependent systemic circulation are extremely high-risk for Norwood palliation. We report our comprehensive approach to the management of these patients designed to maximize survival and optimize the utilization of donor hearts. Methods: We reviewed our entire current single center experience with 83 neonates and infants with HLHS and HLHS-related malformations (2015-2021). Standard-risk patients (n = 62) underwent initial Norwood (Stage 1) palliation. High-risk patients with risk factors other than major cardiac risk factors (n = 9) underwent initial Hybrid Stage 1 palliation, consisting of application of bilateral pulmonary bands, stent placement in the patent arterial duct, and atrial septectomy if needed. High-risk patients with major cardiac risk factors (n = 9) were bridged to transplantation with initial combined Hybrid Stage 1 palliation and pulsatile ventricular assist device (VAD) insertion (HYBRID + VAD). Three patients were bridged to transplantation with prostaglandin. Results: Overall survival at 1 year = 90.4% (75/83). Operative Mortality for standard-risk patients undergoing initial Norwood (Stage 1) Operation was 2/62 (3.2%). Of 60 survivors: 57 underwent Glenn, 2 underwent biventricular repair, and 1 underwent cardiac transplantation. Operative Mortality for high-risk patients with risk factors other than major cardiac risk factors undergoing initial Hybrid Stage 1 palliation without VAD was 0/9: 4 underwent transplantation, 1 awaits transplantation, 3 underwent Comprehensive Stage 2 (with 1 death), and 1 underwent biventricular repair. Of 9 HYBRID + VAD patients, 6 (67%) underwent successful cardiac transplantation and are alive today and 3 (33%) died while awaiting transplantation on VAD. Median length of VAD support was 134 days (mean = 134, range = 56-226). Conclusion: A comprehensive approach to the management of patients with HLHS or HLHS-related malformations is associated with Operative Mortality after Norwood of 2/62 = 3.2% and a one-year survival of 75/83 = 90.4%. A subset of 9/83 patients (11%) were stabilized with HYBRID + VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged wait times.

Rapid Two-Stage Norwood Procedure Using an Auto-Pericardial Patch Fixed with an Arch-Shaped Mold

While the classical 1-stage Norwood procedure is still performed, there are several types of “hybrid” procedures for the management of hypoplastic left heart syndrome. These hybrid approaches consist of bilateral pulmonary artery banding with ductal stenting or prostaglandin infusion as the first-stage palliation, followed by a second-stage Norwood procedure or comprehensive stage II procedure. Since 2012, we have adopted a rapid 2-stage Norwood procedure as a routine strategy, where bilateral pulmonary artery banding is performed within 5 days of age with balloon atrial septectomy, if needed, before the development of hemodynamic instability. The second-stage Norwood procedure is performed within 1 month of age. The arterial duct is kept open by continuing prostaglandin administration. In addition, an important improvement of our Norwood procedure is the use of an auto-pericardial patch fixed on an arch-shaped metal mold. The pericardium is wrapped around the lesser curvature of the mold and treated with 0.6% glutaraldehyde for 15 min. This makes it easier to imagine the final shape of the arch and helps to enlarge the retroaortic space significantly, which could reduce the risk of bronchus or central pulmonary artery stenosis and facilitate hemostasis. These developments in strategy and procedure could improve our surgical results. While the classical 1-stage Norwood procedure is still performed, there are several types of “hybrid” procedures for the management of hypoplastic left heart syndrome. These hybrid approaches consist of bilateral pulmonary artery banding with ductal stenting or prostaglandin infusion as the first-stage palliation, followed by a second-stage Norwood procedure or comprehensive stage II procedure. Since 2012, we have adopted a rapid 2-stage Norwood procedure as a routine strategy, where bilateral pulmonary artery banding is performed within 5 days of age with balloon atrial septectomy, if needed, before the development of hemodynamic instability. The second-stage Norwood procedure is performed within 1 month of age. The arterial duct is kept open by continuing prostaglandin administration. In addition, an important improvement of our Norwood procedure is the use of an auto-pericardial patch fixed on an arch-shaped metal mold. The pericardium is wrapped around the lesser curvature of the mold and treated with 0.6% glutaraldehyde for 15 min. This makes it easier to imagine the final shape of the arch and helps to enlarge the retroaortic space significantly, which could reduce the risk of bronchus or central pulmonary artery stenosis and facilitate hemostasis. These developments in strategy and procedure could improve our surgical results.

Imaging characteristics and associations of left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers on multidetector CT.

To study the imaging characteristics and the associations of left atrioventricular valve atresia with patent aortic root and lateralized atrial chambers on multidetector computed tomography (CT) angiography. We retrospectively evaluated all the CT angiography studies performed for the evaluation of various congenital heart diseases at our center from January 2014 to December 2021. Cases with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers were identified. The types of cardiovascular involvement as identifiable on CT angiography were studied and compiled. A total of 38 patients with left atrioventricular valve atresia and patent aortic root were included (median age: 5 months, males: 31). A total of 89.5% had usual atrial arrangement and 10.5% had mirror imagery of atria. Normal drainage of right and left superior caval veins and inferior caval vein into systemic venous atrium was seen in 77.8%, 61.5%, and 86.6%, respectively. Anomalous pulmonary venous drainage was seen in 3 (7.9%) patients. Atrial and ventricular septal defects were the most common associations. Imperforate left atrioventricular membrane was seen in 9 (23.7%) patients and absent left atrioventricular connection in 27 (71.1%). Discordant right atrioventricular connection was seen in 5 (13.1%) patients. While a double outlet right ventricle (76.3%) was the most common ventriculoarterial connection, discordant ventriculo-arterial connection (transposition) in 4 (10.5%). Most patients had good sized pulmonary arteries. Patent arterial duct and right aortic arch were seen in 29% and 21%, respectively. Aberrant right subclavian artery was the most common arch vessel anomaly. Coronary anomalies were seen in 7 patients (19%); single coronary artery being the most common. Majority of the patients with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers have usual atrial arrangement, normal right atrioventricular connections and a double outlet configuration of the right ventricle. Absent left atrioventricular connection is a more common than imperforate left atrioventricular valve membrane. Atrial and ventricular septal defects are the most common associations. Comprehensive CT based evaluation of vascular and nonvascular thoracic structures is imperative before surgery or interventions in patients with mitral atresia.

Palliation Plus Ventricular Assist Device Insertion in 15 Neonates and Infants With Functionally Univentricular Circulation

BackgroundWe report 15 high-risk neonates and infants with functionally univentricular circulation stabilized with initial surgical palliation plus ventricular assist device (VAD) insertion (PALLIATION+VAD) in preparation for transplantation. MethodsFifteen functionally univentricular patients with ductal-dependent systemic circulation (8 hypoplastic left heart syndrome, 1 hypoplastic left heart syndrome-related malformation: 7 neonates, 2 infants) or ductal-dependent pulmonary circulation (6 hypoplastic right heart syndrome: 5 neonates, 1 infant) presented with anatomical and/or physiological features associated with increased risk for conventional univentricular palliation (large coronary sinusoids with ventricular-dependent coronary circulation, severe systemic atrioventricular valvar regurgitation, cardiogenic shock, or restrictive atrial septum). PALLIATION+VAD for patients with ductal-dependent systemic circulation was: VAD insertion plus application of bilateral pulmonary bands, stent placement in the arterial duct, and atrial septectomy, if needed. PALLIATION+VAD for patients with ductal-dependent pulmonary circulation was: VAD insertion plus stent placement in the arterial duct or systemic-to-pulmonary artery shunt with pulmonary arterioplasty, if needed. ResultsAt PALLIATION+VAD, median age was 20 days (range, 4-143 days) and median weight was 3.47 kg (range, 2.43-4.86 kg). Ten patients (67%) survived and 5 patients (33%) died. All ten survivors are at home doing well after successful transplantation. Only 2 of 10 survivors (20%) required intubation >10 days after PALLIATION+VAD. Median length of VAD support for all 15 patients was 138 days (range, 56-226 days). ConclusionsHigh-risk neonates with functionally univentricular hearts who are suboptimal candidates for conventional palliation can be successfully stabilized with pulsatile VAD insertion along with initial palliation while awaiting cardiac transplantation; these patients may be extubated, enterally nourished, and optimized for transplantation while on VAD.

Unexpected death in a newborn due to a congenital partial pericardial defect: a case report

BackgroundPericardial defects are rare anatomical variations that can present as an isolated variation or be associated with other conditions. They are usually asymptomatic and misdiagnosed conditions, and given their rarity, partial pericardial defects can have devastating outcomes. The sudden death of an apparently healthy newborn certainly raises concerns, and a medico-legal investigation is crucial in establishing the cause of death. This case report highlights the importance of awareness on the part of obstetric professionals of the lethal outcomes of pericardial partial congenital defects. This case also demonstrates the difficulty of establishing a correct diagnosis.Case presentationThe autopsy of a 15-h-old neonate revealed a partial pericardial defect ending in a biventricular strangulation by the defective pericardium. Other findings, such as the patency of the arterial ductus, a subarachnoid hemorrhage, and aspiration of amniotic fluid, were also reported.ConclusionsAlthough imaging techniques have evolved, fetal detection of cardiac abnormalities can be tricky, especially when occurring as an isolated variation.

Transcatheter closure of tubular patent ductus arteriosus using muscular ventricular septal defect devices in infants and small children with congestive heart failure

Transcatheter closure of a patent ductus arteriosus in children is widely performed to reduce symptoms and decrease the risk of endocarditis. Most arterial ducts are closed successfully with dedicated devices. However, in a tubular or "type C" patent ductus arteriosus with congestive heart failure, the occlusion is more challenging with these devices, with a higher risk of complications, such as aortic or left pulmonary stenosis and device embolization. To report our experience with muscular ventricular septal defect devices for patent ductus arteriosus occlusion in small children. Retrospective observational series of patients weighing<10kg, with a tubular patent ductus arteriosus (typeC) and congestive heart failure, who underwent transcatheter closure with a muscular ventricular septal defect device between 2017 and 2019. Eight patients were included. The mean age and weight at closure were 6.3 months (range 1-18 months) and 5.3kg (range 2.4-8.2kg), respectively. All patent ductus arteriosus were occluded successfully using Occlutech® (N=3) or Amplatzer® (N=5) muscular ventricular septal defect devices. In four cases, the muscular ventricular septal defect device was used after failure to close the patent ductus arteriosus with a dedicated patent ductus arteriosus device. Two patients had mild left pulmonary artery stenosis, with a maximum velocity on continuous Doppler of 3m/s and 2.7m/s, respectively. After a mean follow-up of 28 months (range 14-41 months), all patients were asymptomatic with excellent results. The mild pulmonary stenosis improved, with a maximum velocity of 2.3m/s in both patients. Closure of tubular patent ductus arteriosus in small children with congestive heart failure using a muscular ventricular septal defect device is safe in this preliminary experience. Further studies with more patients are warranted.

ЗАБОЛЕВАНИЯ, АССОЦИИРОВАННЫЕ С МУТАЦИЯМИ В ГЕНЕ ФИЛАМИНА А: ТРУДНЫЙ ПУТЬ К ДИАГНОЗУ (ДВА КЛИНИЧЕСКИХ НАБЛЮДЕНИЯ)

The article presents the first descriptions of observations in the Russian Federation, the difficult path to diagnosis in two young girls with diseases associated with mutations in the filamin A gene, characterized by damage to the central nervous system (periventricular nodular heterotopy), lungs (interstitial emphysematous lesion due to impaired growth), cardiovascular system (open arterial duct, high pulmonary hypertension, insufficiency of heart valves, large vessels). Clinical and anamnestic data, the results of laboratory and instrumental diagnostic methods, including molecular genetic ones, which allowed to identify unique, previously undescribed mutations, the characteristics of the course of the disease, the treatment being carried out, are presented.

Current trends in the treatment of patent ductus arteriosus

The experience of work of the cardiosurgical department of the Children medical centre on the diagnosis, treatment and postoperative dispensary examination of patients with open arterial duct is generalized. The Botallos duct was closed by surgical method in 92 patients making up 18,4% of the total number of operated patients, catheterization of heart cavities was performed in 88 children. For closing the open arterial duct the various operation techniques were carried out: the dressing of the duct from aorta out of the anterolateral access (1%), out of the posteriorlateral access from aorta (85,8%), as to recanalization out of the anterolateral access from aorta and pulmonary artery intrapericardially (1%), clipping of the duct out of the minithoracotomic access (8,7%), videothoracoscopic clipping (3%). The operated children are under examination of the cardiologist throughout the year with visits every month, three months, 6 months and one year.

Use of the Medtronic Microvascular Plug 7Q for transcatheter closure of large patent ductus arteriosus in infants weighing less than 2.5 kg.

BackgroundThe sole Food and Drug Administration‐approved device for transcatheter closure of the patent arterial duct in premature infants is indicated for patent ductus arteriosus (PDAs) ≤ 4 mm in diameter. We report a two‐center experience with transcatheter closure of large PDAs (>4 mm) in infants weighing <2.5 kg using the Microvascular Plug 7Q (MVP‐7Q) device.MethodsThis is a retrospective review of departmental databases and medical charts to define patient cohort and report demographic, procedural, and follow‐up data.ResultsTwenty‐two patients (12 male) with a median gestational age and birthweight of 25.5 weeks (interquartile range [IQR] = 24–28) and 800 g (572–1075), respectively, underwent attempted PDA occlusion with the MVP‐7Q using a transvenous approach. The median age and weight at the time of PDA occlusion was 32 days (IQR = 24–28) and 1100 g (IQR = 960–1700), respectively. The median PDA length was 12 mm (IQR = 11–12.65). The median PDA diameters at the aortic and pulmonary ends were 5.1 (IQR = 4.9–5.5) and 4.8 mm (IQR = 4.6–5.3), respectively. Successful device occlusion was achieved in 20 patients (91%). There were two failed attempts: One due to inappropriate sizing, and the other secondary to left pulmonary artery stenosis. There were no procedural complications and no residual shunting on follow‐up.ConclusionsThe MVP‐7Q is safe and effective for transcatheter closure of large (>4 mm) PDAs in infants <2.5 kg. The lack of retention disks may help with avoiding impingement on surrounding vessels.

Embolization of vascular abnormalities in children with congenital heart diseases using medtronic micro vascular plugs.

This study aimed at evaluating our experience with Micro Vascular Plug (MVP) for embolization of vascular abnormalities in children with congenital heart diseases (CHDs). MVP is one of the most recent additions to the armamentarium for peripheral embolization. Data on its use in congenital cardiology are scarce. Medical records of children with CHDs who had embolizations with MVPs between April 2015 and September 2020 were reviewed. Immediate and follow-up data were assessed. A total of153 patients underwent 172 procedures during which 240 embolizations using 259 MVPs were attempted. Median age and weight were 34.9months (IQR, 4-75months) and 12.5kg (IQR, 4.8-19.4kg), respectively. Targets were abnormal systemic arteries (n = 163), patent arterial ducts (n = 26), venous (n = 45), and coronary-cameral fistulas (n = 6). Median vessel diameter was 3.3mm (IQR, 2.5-4mm). Veins were larger than arteries (2.3mm > 2.1mm, p < 0.01). MVPs were implanted in narrower diameters compared to manufacturer's recommendations. Compared to veins, solitary implanted MVPs in arteries were additionally oversized at 12.4%, 5.1%, and 7.9% for MVP-3Q, 5Q, and 7Q, respectively. Additional occlusion material (16.7%) and 2 MVPs (5.8%) were needed at the same site for complete closure. Implantation and procedure success rates were 99.2% and 96.7%, respectively. Five complications were treated percutaneously (n = 4) and surgically (n = 1). Efficacy of vascular embolization using MVPs was demonstrated in the largest cohort of children with CHDs and a variety of clinical settings. Immediate and stable closure is obtained with a single device in most cases. Detailed device selection chart according to vessel type and diameter is proposed to achieve intended outcomes.

"Absent" unilateral pulmonary arteries aren't absent, but disconnected. Review of the current literature based on 4 cases.

Disconnected unilateral pulmonary arteries are frequently misdiagnosed as "absent". They typically arise from the base of the innominate artery and are fed by an aberrant arterial duct. If diagnosed early enough, they can be reconnected with catheter techniques even after closure of this aberrant duct. Consecutive surgical anatomical correction at a later stage is possible. Four cases illustrate the anatomical findings on computed tomography and angiography, all show an outpouching at the base of the brachiocephalic artery. The therapeutic approach consisted of stenting of the aberrant ductus and consecutive surgery. In the oldest patient, 13 years, such an approach was impossible. If identified early in life, disconnected pulmonary arteries can be recruited with catheter techniques, and reconnected surgically at a later stage. It is not yet known if this approach prevents pulmonary damage, which is frequently seen in older untreated patients.