Search

An asymptomatic woman aged 24 years was informed about the presence of a heart murmur by her attending obstetrician while she was hospitalised for a spontaneous abortion 6 months ago and was subsequently referred to us to rule out underlying heart disease. After clinical examination and relevant laboratory evaluation, a diagnosis of severe valvular pulmonary stenosis (PS) was established. Interestingly, our patient had undergone an open thoracotomy for surgical closure of a patent ductus arteriosus (PDA) under general anaesthesia 12 years ago in the absence of any appreciable shunt across the ligated ductus at present. Considering the severe gradient across her pulmonary valve, she underwent a successful percutaneous balloon pulmonary valvuloplasty (PBPV) procedure with excellent haemodynamic outcome. The pertinent literature concerning the rare combination of PDA in association with PS as well as the technicalities of PBPV procedure in an adult are discussed.

We describe a 9-day-old baby with coarctation of the aorta who required urgent resuscitation including intubation and cardiac compressions. Despite the commencement of prostaglandin E1 (PGE1) to reopen the ductus arteriosus via the intraosseous route, postductal saturations remained unrecordable for a further 45 min. Within 3 min of administration of PGE1 via an umbilical venous catheter (UVC), saturations were recordable at 92%. UVC access was the sentinel intervention that irrevocably altered the clinical prognosis. This baby boy has survived with excellent neurodevelopmental outcome. Clinicians are less familiar with UVCs outside of the newborn period. Our data demonstrate successful placement in neonates up to 28 days of age. We hope this case encourages clinicians to consider the UVC as first-line central venous access in collapsed neonates. In cases of suspected left heart obstruction, we argue that UVCs are the optimal route.

A 20-year-old woman was referred to the diabetes clinic with type 2 diabetes diagnosed at the age of 19. Her body mass index was 31.4 kg/m2, HbA1C was 76 mmol/mol, GAD antibodies were negative with a detectable C-peptide. She had a characteristic facial appearance with widespread eyes, posterior hairline suggesting a facial gestalt and abnormal dentition. She also had hypothyroidism, mild intellectual disability, primary amenorrhoea and patent ductus arteriosus. Karyotyping reported normal 46XX karyotype. Genetic testing revealed a pathogenic variant in the gene encoding the HIST1H1E protein which confirmed her diagnosis of HIST1H1E syndrome. Type 2 diabetes has not been reported in previous cases of HIST1H1E and so this is the first reported case of type 2 diabetes with HIST1H1E syndrome.

OBJECTIVEFailure to close the ductus arteriosus immediately post-birth, patent ductus arteriosus (PDA), accounts for up to 10% of all congenital heart defects. Despite significant advances in PDA management options, including pharmacological treatment targeting the prostaglandin pathway, a proportion of patients fail to respond and must undergo surgical intervention. Thus, further refinement of the cellular and molecular mechanisms that govern vascular remodeling of this vessel is required.APPROACH AND RESULTSAs anticipated, single-cell RNA sequencing on the ductus arteriosus in mouse embryos at E18.5, P0.5, and P5, revealed broad transcriptional alterations in the endothelial, smooth muscle, and fibroblast cell compartments. Making use of these data sets, vimentin emerged as an interesting candidate for further investigation. Subsequent studies demonstrated that, in fact, mice with genetic deletion of vimentin fail to complete vascular remodeling of the ductus arteriosus, as per presence of a functional lumen.CONCLUSIONSThrough single-cell RNA-sequencing and by tracking closure of the ductus arteriosus postnatally in mice, we uncovered the unexpected contribution of vimentin in driving complete closure of the ductus arteriosus potentially through regulation of the Notch signaling pathway.HIGHLIGHTSSingle-cell RNA-sequencing on the ductus arteriosus at E18.5, P0.5, and P5 reveals how the ductus arteriosus undergoes drastic transcriptional changes at the single-cell level.Endothelial cells increase levels of Vimentin, Notch1 and Jag1 transcripts soon after birth (P0.5), concurrent with ductus arteriosus closure.Loss of vimentin, the major intermediate filament protein of endothelial cells, prevents proper permanent closure of the ductus arteriosus.

A male term neonate was admitted to the neonatal intensive care unit in the first hours of life with central cyanosis. Echocardiogram showed severe biventricular hypertrophy, markedly right-sided, tricuspid regurgitation, a patent foramen ovale and a closed ductus arteriosus (CDA). The mother recalled being treated with a single dose of intravenous diclofenac for low back pain 2 weeks earlier. The newborn was started on propranolol with symptomatic improvement and was discharged on day 10. At 1-month follow-up, he showed complete resolution of ventricular hypertrophy and suspended propranolol. In the literature, of the 22 cases of CDA after intrauterine exposure to diclofenac, 11 resolved in utero, 3 required ventilatory and inotropic support and 1 evolved to persistent pulmonary hypertension. In this case, a thorough anamnesis was key to identify the probable cause of an otherwise unexplained transient ventricular hypertrophy. This case also alerts to the fetal risks of non-steroidal anti-inflammatory drugs during the third trimester, requiring close monitoring.

Ductal origin of one distal pulmonary artery (DODPA) is a rare congenital cardiovascular anomaly, with an incidence of approximately 1 in 200,000 people1-3 . It refers to the proximal interruption of one pulmonary artery branch, with the distal part arising from the base of the brachiocephalic artery(BA) via the ipsilateral ductus arteriosus (DA) and being commonly intact in the lungs. If timely treatment is not provided after birth, severe hypoplasia is highly likely to occur in the lungs supplied by the abnormal pulmonary artery due to DA closure. Hence, the prenatal diagnosis of fetal DODPA is important to help initiate prostaglandin treatment and the early rehabilitation of the affected lung. Herein, we report three cases of fetal DODPA diagnosed via two-dimensional (2D) ultrasonography combined with the four-dimensional (4D) spatiotemporal image correlation (STIC) technique. Moreover, a literature review was performed, and ultrasonographic findings and the importance of 2D and 4D ultrasonography in obtaining an accurate prenatal diagnosis of DODPA were explored. This article is protected by copyright. All rights reserved.

We present a case of an extreme preterm infant (Baby X) born at 24-week gestation. The echocardiogram showed evidence of hypertrophic cardiomyopathy (HCM) and a patent ductus arteriosus (PDA). There are a number of well-known causes of neonatal HCM including genetic, metabolic and endocrine. PDA is commonly present in preterm infants, and this can contribute to cardiac remodelling and result in cardiac changes mimicking HCM. Furthermore, medications such as steroids can also cause HCM through various mechanisms. A careful consideration of all the different aetiologies for HCM is important for appropriate management of such cases. This report examines the evidence in the literature for the above differential diagnoses and highlights the challenges in diagnosing the underlying cause of HCM in a preterm infant.

Pulmonary artery coarctation may pose a risk for pulmonary stenosis and subsequent failure to achieve definitive repair. We sought to assess the impact of pulmonary artery coarctation on pulmonary artery growth.A retrospective chart review was performed in 130 patients, including 37 single ventricles with a modified Blalock-Taussig shunt as first palliation. Pulmonary artery coarctation was defined as discrete stenosis of the pulmonary artery, with a diameter of less than 3 mm and with the ductus arteriosus connected. Preoperative echocardiography showed pulmonary artery coarctation in 29 patients (22%). Concomitant pulmonary artery plasty was performed in 14 patients with discrete stenosis having a diameter of less than 2 mm.Pre-modified Blalock-Taussig shunt left pulmonary artery z-scores were lower in patients with pulmonary artery coarctation than in those without (-4.0 [-5.8, -2.1] vs -1.7 [-2.6, -0.8], P .001), and this remained the same even after modified Blalock-Taussig shunt (-2.5 [-5.1, -0.5] vs -0.5 [-2.4, 0.8], P = .010). Concomitant pulmonary artery plasty did not result in catch-up growth of the left pulmonary artery (post-modified Blalock-Taussig shunt left pulmonary artery z-score in patients with pulmonary artery plasty: -3.0 (-6.5, -2.0) versus those without: -1.8 (-3.3, -0.3), P = .279). Definitive repair/Fontan completion was achieved in 111 patients (85%), and this was not affected by the presence of pulmonary artery coarctation.Pulmonary artery coarctation affected disproportionate pulmonary artery growth throughout the staged repair, but did not result in failure of definitive repair/Fontan completion. Pulmonary artery plasty during the neonatal period did not contribute to catch-up growth of the left pulmonary artery; therefore, surgical indications and timing should be carefully considered.

BACKGROUND: There remains controversy regarding the outcomes resulting from treatment versus conservative management of patent ductus arteriosus (PDA) among preterm infants. The effects of extreme prematurity, hemodynamic status of the PDA, and age at treatment remain poorly defined. STUDY DESIGN: This retrospective case-control study including infants

The impact of fetal echocardiography on the diagnosis and outcomes of vascular ring has not been well examined. We hypothesized that prenatal detection of vascular ring has improved over time and that prenatal diagnosis of vascular ring is associated with earlier intervention and favorable outcomes.This is a single-center, retrospective study of the evolution and outcomes of prenatal diagnosis of vascular ring from 2000 to 2020. We compared clinical presentation, timing of surgical intervention, and outcomes between the prenatally and postnatally diagnosed cases during the same study period.A total of 170 patients were included: 50 with prenatal and 120 with postnatal diagnosis of vascular ring. Prenatal diagnoses included 42 patients (84%) with right aortic arch (RAA), aberrant left subclavian artery (ALSCA), and a left-sided ductus arteriosus and eight (16%) patients with double aortic arch (DAA). The postnatal cohort consisted mainly of 90 patients (75%) with DAA and 22 (18%) with RAA-ALSCA. None of the postnatally diagnosed cases had undergone a fetal echocardiogram. Numbers (percentage) of prenatally diagnosed cases of vascular ring compared with the postnatal cases improved from 4/31 (13%), to 10/29 (34%), to 14/25 (56%), and to 22/35 (69%), respectively, during 2000-2005, 2005-10, 2010-15, and 2015-20 (P = .032). Vascular ring was an isolated abnormality in 84% and 85% of the prenatal and postnatal cohorts, respectively. Compared with the prenatal cohort, postnatally diagnosed patients with an isolated vascular ring were more frequently symptomatic (66% vs 48%, P .03) and underwent cross-sectional imaging (69% vs 44%, P = .009) and surgery more frequently (79% vs 48%, P = .003). Surgery was performed at a later patient age (18 [2-147] months vs 4.8 [0.5-42] months, P = .01) and was more often associated with residual symptoms (27/81 [33%] vs 1/20 [5%], P = .01) in the postnatal cohort than in the prenatal cohort.The diagnosis of vascular ring by fetal echocardiography has improved over time. A significantly higher incidence of RAA-ALSCA in the prenatal compared with the postnatal cohort suggests that patients with this form of vascular ring often do not present to medical attention with significant symptoms postnatally. Prenatal diagnosis of vascular ring was associated with a lower incidence of symptoms, less frequent use of cross-sectional imaging, earlier age at surgical intervention, and lower likelihood of residual symptoms.

The correlation between the Z-score of the left ventricular (LV) diameter and the LV volume-overload due to pulmonary over-circulation in children with ventricular septal defect (VSD) or patent ductus arteriosus (PDA) remains unclear.Methods and Results:The present, retrospective study enrolled 70 children (aged 0.3-16.8 years; 33 males, 37 females) with a diagnosis of isolated VSD and/or PDA who underwent cardiac catheterization (CC) between 2015 and 2019. Patients with chromosomal/genetic anomalies, growth disorder, right-ventricular enlargement or other conditions causing LV enlargement were excluded. Echocardiographic parameters were retrospectively evaluated from the medical records, converted to a Z-score, then compared with CC data. The pulmonary-systemic flow ratio on CC (cQp/Qs) correlated significantly with the Z-score of both the LV end-diastolic diameter (Zd) (r=0.698, P0.0001) and LV end-systolic diameter (r=0.593, P0.0001). Regression analysis and curve-fitting were used to predict the cQp/Qs based on the Zd, and a significant regression equation was found on cubic regression (RThe Z-score of the LV diameter can be a useful, non-invasive marker for evaluating LV volume overload and determining the surgical indications in children with VSD or PDA because of its strong correlation with the cQp/Qs.

A five-month-old, intact female domestic shorthair cat was presented to a specialty referral hospital for evaluation of a patent ductus arteriosus. Transvenous embolization of the defect was achieved with a commercially available peripheral vascular plug. The use of vascular plugs for the closure of patent ductus arteriosus has been validated in dogs, yet literature for its use in cats is lacking. The product and procedural details of the device are described.

The procedure of stenting the patent ductus arteriosus (PDA) is a palliative procedure applied as an alternative to surgery in newborns with ductus-dependent pulmonary circulation. However, it is still a very challenging method in patients with aortic arch anomalies. We describe our experience with a newborn with right atrial isomerism and dextrocardia, complete atrioventricular septal defect, aortic outlet right ventricle with pulmonary atresia, right aortic arch, and a PDA from the left innominate artery. Because the PDA was long and tortuous, we preferred placing three short stents instead of a single long stent. The procedure applied the femoral artery approach with a Glidesheath Slender to decrease arterial injuries. PDA stenting in challenging morphologies can be performed successfully using multiple short stents and via Glidesheath Slenders.

To investigate fetal pulmonary hemodynamics in normal pregnancy using pulsed and color Doppler ultrasonography and to define Doppler reference ranges values in the whole fetal pulmonary circulation during different gestational ages.A prospective observational study was conducted to evaluate 190 healthy singleton pregnancies between 20 and 38 weeks of gestation, including 10 cases for each gestational age. The main pulmonary artery, right pulmonary artery, left pulmonary artery, ductus arteriosus and pulmonary veins waveforms were evaluated. The waveform profile was studied for each pulmonary vessel investigated. We calculated the normal distribution of data of 12 Doppler parameters, their values were expressed as 5th, 25th, 50th, 75th and 95th centile.We report several pulmonary hemodynamic changes with the progression of pregnancy. We focused on the hemodynamic values of the parameters most useful in clinical practice and that would better describe the hemodynamic events of this vascular district.This study reports a complete description of the fetal pulmonary hemodynamics at different gestational ages. Many hemodynamic parameters show a typical change during gestation. The reference ranges described in this study may help in situations where is indicated to evaluate the pulmonary hemodynamics and to identify healthy fetuses from those affected by pathological conditions related mainly to fetal cardiovascular anomalies and/or feto-maternal pathologic conditions.

Objective To summarize experience in the treatment of pentalogy of Cantrell (POC) in our hospital and explore the effect of artificial materials in repairing sternal defects. Materials and Methods A retrospective analysis was performed on treatment of five children with POC treated by using the Gore-Tex patch and titanium mesh in the Department of Cardio-Thoracic Surgery, Children's Hospital of Chongqing Medical University, from January 2010 to January 2019. Results The concurrent conditions included double outlet of right ventricle (n = 2), ventricular septal defect (VSD) and atrial septal defect (ASD) (n = 1), VSD and ASD and patent ductus arteriosus (n = 1), and VSD and left ventricular diverticulum (n = 1) in five cases with POC. Color Doppler echocardiography and computed tomography (CT) + three-dimensional (3D) reconstruction of the thorax and abdomen were performed preoperatively. The cardiac malformation was corrected according to color Doppler echocardiography, and a Gore-Tex patch was used to repair the pericardial defect. Titanium mesh was made according to CT 3D reconstruction with a 3D printing mold to repair sternal defects. All patients underwent a one-stage operation, all hearts were eventually repositioned, no deaths occurred after the operation, and follow-up was performed for 6 months to 2 years. The patients recovered well, and the exterior thorax was normal. Conclusion The diagnosis of POC is not difficult. The priority of surgical treatment for POC is to obtain satisfactory corrections of cardiac malformation. The repair of the pericardial defect with the Gore-Tex patch and the sternal defect with the titanium mesh can make the heart return to the mediastinum, reduce the pressure on the heart, reduce the surgical trauma, reduce the difficulty of repairing the sternal defect, and optimally restore the exterior thorax.

To evaluate the effect of bilirubin levels in the first week of life on the frequency of oxidative-stress related morbidity. We included all preterm infants with a gestational age less than 32 weeks. The mean total serum bilirubin of the first week of life was measured and compared between infants with and without oxidative stress related morbidity. A total of 116 preterm infants were included. Univariate analysis showed that mean ± SD TSB levels were statistically significantly lower in infants with chronic lung disease (95 ± 31.4micromole/l vs 119 ± 31micromole/l, p = 0.019), necrotizing enterocolitis (94.4 ± 29micromole/l vs 118 ± 31micromole/l p = 0.044) and patent ductus arteriosus (104 ± 33micromole/l vs 120 ± 30micromole/l p = 0.018). However, when adjusted for gestational age, there were no longer statistically significant differences observed. Elevated bilirubin levels in the first week of life are not protective against the oxidative stress related morbidity in very preterm infants.

Background and Aims: Two dimensional transthoracic echocardiography (2DE) is widely used for detecting congenital heart disease and is possible to obtain precise measurement of Patent ductus arteriosus (PDA) for device selection required for transcatheter closure. Primary aim of the study is to determine whether echocardiographic assessment alone can be used for selection of device for transcatheter closure of PDA. Methods: Children with PDA and planned for transcatheter intervention were included in this cross-sectional study of one year. PDA was assessed with 2DE and prediction of device size was made. Results: The results were obtained from 107 children. The median age and weight at intervention were 3.8 years (ranging from 6 months to14 years) and 12 kg (ranging from 3.5 to 60 kg). Type A (Conical) PDA was the commonest PDA morphology accounting for 87.8% and 85% in 2DE and angiography respectively. There was no difference (p < 0.05) in the narrowest diameter at pulmonary end measured by 2DE and angiography, however ampulla diameter and ductal length were statistically different (p = 0.95). The predicted size of device by 2DE was discordant (p < 0.05) to the actual device used in a total study population, however when patients with severe pulmonary hypertension, non-type A and larger PDA (narrowest diameter > 6mm) were excluded, the predicted size of device by 2DE was statistically concordant (p = 0.1) to the actual device used in 89 (83%) patients. Conclusion: Two dimensional Transthoracic echocardiography alone may be helpful in choosing the device during transcatheter closure of PDA in selective group of patients.

An infant was referred for evaluation of congenital glaucoma and corneal clouding. In addition, he had a pelvic kidney, hypotonia, patent ductus arteriosus, abnormal pinnae, and developmental delay. Exome sequencing identified a previously unpublished de novo single nucleotide insertion in PBX1 c.400dupG (NM_002585.3), predicted to cause a frameshift resulting in a truncated protein with loss of function (p.Ala134Glyfs*65). Identification of this loss of function variant supports the diagnosis of congenital anomalies of the kidney and urinary tract syndrome with or without hearing loss, abnormal ears, or developmental delay (CAKUTHED). Here, we propose glaucoma as an extra-renal manifestation associated with PBX1-related disease due to the relationship of PBX1 with MEIS1, MEIS2, and FOXC1 transcription factors associated with eye development.

Hemodynamically significant patent ductus arteriosus (hsPDA) is a severe condition in newborns. Ibuprofen is an effective treatment to reduce the severe complications and the need for surgical treatment. Several single-nucleotide polymorphisms (SNPs) were related to the ibuprofen metabolism, treatment effects, and the onset of side effects. The effects of SNPs on hsPDA response after ibuprofen treatment are unknown. Therefore, in this study, we recruited hsPDA patients with standard ibuprofen treatment. Those patients had participated in China Neonatal Genomes Project (CNGP, ClinicalTrials.gov Identifier: NCT03931707) with next-generation sequencing data. We reanalyzed the sequencing data and compared the allele frequencies of known ibuprofen-related SNPs between ibuprofen Responder and Non-responder groups. In total, 185 hsPDA patients were recruited with gestational age (GA) ranging from 24 to 40 weeks. No significant differences were detected in the basic information, period of ibuprofen treatment, rate of conservative treatment, complications, and side effects between ibuprofen Responder group and Non-responder group. Totally, 17 hsPDA carried CYP2C9*3 and one with CYP2C9*2 were detected. In the GA group of more than 30 GA weeks (GA > 30 wks group), we found higher allele frequency of CYP2C9*3 in Responder group than in Non-responder group (16% vs. 0, p = 0.0391). In the GA group of less than 30 GA weeks (GA ≤ 30 wks group), the sum allele frequency of CYP2C9*3 and CYP2C9*2 had no stastical difference between two groups (Responder group vs. Non-responder group, 13% vs. 11%, p = 0.768). Therefore, we came to conclude that genetic tests of CYP2C9*3 site may benefit the prediction of ibuprofen treatment outcome for hsPDA patients with gestational age of more than 30 weeks. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s43657-021-00028-9.

Monitoring patent ductus arteriosus (PDA) in premature infants is currently performed intermittently using echocardiography which requires considerable expertise. The aim of this pilot study was to investigate whether PDA status could be assessed from standard neonatal intensive care monitoring. Electrocardiography (ECG) and blood pressure (BP) waveforms were acquired from extremely preterm infants using standard neonatal monitors. We developed software using MATLAB to analyse ECG and BP waveforms and their interrelationships in terms of pulse transit time (PTT) and pulse wave velocity (PWV). The times from peak systolic BP to diastolic trough (BPFt) and from the diastolic trough to peak systolic BP (BPRt) were also calculated. PTT, BPFt and BPRt were normalised for heart rate (HR) termed NPTT, NBPFt and NBPRt, respectively. ECG, invasive aortic BP monitoring and echocardiography were performed in 14 preterm infants r = 0.69, P = 0.007) as well as NBPFt (r = 0.65, P = 0.012) and NBPRt (r = 0.71, P = 0.005). No relationship was found between PDA diameter and pulse pressure.Conclusions: Interrelationships between ECG and BP traces as well as BP waveform time analysis are straightforward to measure and associated with PDA diameter. The results of this pilot study suggest that this approach may help provide biomarkers for continuous monitoring PDA diameter and function. What is Known:• Patent ductus arteriosus (PDA) in premature infants is associated with increased risk of developing chronic lung disease, necrotising enterocolitis and cerebral injury.• Currently PDA is assessed intermittently using echocardiography which requires considerable expertise and sometimes is not well tolerated by critically ill preterm infants. What is New:• Blood pressure (BP) and ECG waveform interrelation and BP trace time analysis, taking account of heart rate, relate to PDA diameter.• ECG and BP waveform phase difference as well as BP waveform time analysis may be useful in the continuous assessment of PDA function.

OBJECTIVE: Infectious endarteritis associated with patent ductus arteriosus (PDA-IE) is an uncommon complication in the era of antibiotics. However, it implies a clinical challenge in patients with a fever of undetermined origin. Two-dimensional transthoracic echocardiography (TTE) plays a fundamental role in diagnosis and follow-up. METHODS: A retrospective analysis of the data of all patients admitted to our center with PDA-IE within 15 years was conducted, and a review of the literature regarding diagnosis, TTE findings, and treatment was performed. RESULTS: A total of 17 patients were identified with a mean age of 17.8 years. TTE done in all patients confirmed the diagnosis of PDA and pulmonary artery vegetation. In five patients, one vegetation was present; in three patients, two vegetations were found; and in the nine remaining patients, three or more vegetations were observed. In two-thirds of the patients, the size of the vegetation was three to 28 mm, and the principal morphology was filiform. In all patients, at least one vegetation developed on the lateral wall of the ductus arteriosus. Pulmonary valve was affected in 41% of the patients and caused low to moderate valvular regurgitation. Pulmonary embolism was present in seven patients and pulmonary aneurysm in one patient. CONCLUSION: The incidence of PDA-IE has decreased in the recent years with early antibiotic therapy. However, today, this complication carries a significant risk of valve damage and other cardiac structures’ involvement.

A neonate is admitted to the NICU for a late preterm birth at 34 6/7 weeks’ gestation. The pregnancy was complicated by premature rupture of membranes and maternal intrapartum fever. The neonate’s initial course is unremarkable except for a requirement of supplemental oxygen by nasal cannula to maintain oxygen saturation above 94% and nasogastric feedings. On the 10th postnatal day, the neonate is noted to have episodic desaturations, with oxygen saturations decreasing to the low 80s. Physical examination findings are notable for a new grade 2/6 soft systolic heart murmur heard best at the apex. Clips of the infant’s echocardiogram are shown in Video 1 and Video 2. The cardiologist is concerned about left ventricular systolic dysfunction with significant regional wall motion abnormalities. The study also shows that the neonate has moderate mitral regurgitation, a large patent ductus arteriosus, and left atrial enlargement. The coronary artery origins are noted to be normal. The neonate is started on a milrinone infusion to manage the cardiac dysfunction. On the following day, he is noted to have multiple episodes of supraventricular tachycardia with heart rates up to 230 beats/min. Because of concerns about cardiac dysfunction, the cardiology team recommends starting digoxin for an antiarrhythmic effect. Two days later, the rhythm shown in Fig 1 is noted on the bedside monitor. Question 1. Based on this information, what is the likely diagnosis in this neonate? 1. Ebstein anomaly 2. Intrauterine viral infection 3. Total anomalous pulmonary venous return 4. Vascular ring 5. Ventricular septal defect Question 2. What is the most appropriate next management step for this neonate? 1. Administer digoxin-specific antibody fragments 2. Feel for a brachial or femoral pulse and if absent, perform emergent defibrillation 3. Feel for a brachial or femoral pulse and if present, perform …

Background: In full term newborns, the Ductus arteriosus closes functionally within of birth, and it normally closes structurally between 2 to 8 weeks. Complete conclusion of the ductus in untimely babies may not happen until a half year old enough, and in exceptional cases may not happen until adulthood. Is medical term that afterwards birth, the ductus arteriosus fails to close, allowing oxygenated blood to travel back to the lungs from the left heart Changed Word Structural Changes Thesaurus via the pulmonary artery, which has a higher pressure. Increased laboured breathing and failure to gain weight at a normal rate are frequent later in the first year of life. A patent ductus arteriosus (PDA) can lead to pulmonary hypertension and right-sided heart failure if left untreated. Case Report Information: A 2 years 6 months old child brought by the parents in Aacharya Vinoba Bhave Rural Hospital on 22/12/2020. An patients parent’s verbalization, the child was apparently alright at 8 months of age. When he started having fever, cold and cough and decreased feed intake. Then they took the child to local doctor where 2D ECHO was done. Suggestive of patent ductus arteriosus. Patient is referred to Aacharya Vinoba Bhave hospital for further management. Conclusion: The patient was admitted in AVBRH with complaints of fever, cold and cough, decreased feed intake. Then the report mainly focused on surgical management and quality nursing care due to which patient was discharged without any further complication and satisfaction.

BACKGROUND Transcatheter ductus arteriosus stenting (DS) is emerging as an alternative method to modified Blalock-Taussig shunt (MBTS) in providing pulmonary blood flow in cyanotic congenital heart disease (CCHD) with duct-dependent pulmonary circulation. OBJECTIVE To evaluate post-procedural outcomes and survival between patients undergoing DS and MBTS. METHODS All infants ≤ 60 days of age having CCHD with diminished pulmonary blood flow who underwent palliative procedure either with MBTS or DS at King Chulalongkorn Memorial Hospital during January 1st, 2013 and December 31th, 2017 were retrospectively reviewed. RESULTS 98 patients were included; 34 patients underwent a transcatheter DS and 64 patients underwent MBTS. There was no significant difference in post-procedural outcomes and overall mortality rate between two groups (17.6% in MBTS group and 6.1% in DS group, p = 0.09). Single ventricle morphology was the major risk factor associated with increased mortality compared with biventricular morphology (aHR 3.9, 95% CI 1.49-10.2, p = 0.01). There was similar number of early and pre-repair additional interventions focusing on MBTS/DS patency between two groups. The MBTS group had a greater number of early interventions on PA branch stenosis related to baseline diagnosis. Risk factors associated with additional intervention were pre-existing pulmonary branch stenosis (aHR 2.54, 95% CI 1.3-4.97, p = 0.006) and body weight less than 2.5 kg (aHR 3.33, 95% CI 1.57-7.08, p = 0.003). Having pulmonic valve perforation or balloon pulmonary valvuloplasty to promote antegrade pulmonary blood flow could result in a lower number of additional interventions required before definitive repair. CONCLUSION Duct stenting is a feasible and safe alternative to MBTS in cyanotic infants with duct-dependent pulmonary circulation. However, mortality rate was significantly higher in patients with single ventricle that required careful follow-up after procedure.

Quadricuspid aortic valve is a remarkably rare congenital cardiac anomaly that predominantly becomes regurgitant with the passage of time. Aortic valve stenosis and aortic root dilatation are less common in quadricuspid aortic valve as compared to bicuspid aortic valve. The clinical presentation depends upon the functional status of the aortic valve, left ventricular function, and associated cardiac or coronary anomalies. The quadricuspid aortic valve is easily visualised during transthoracic echocardiogram with a characteristic X pattern of aortic valve in diastole. The association of quadricuspid aortic valve with patent ductus arteriosus is exceedingly rare. We are reporting a case of young girl with mildly regurgitant quadricuspid aortic valve, large patent ductus arteriosus, and volume-loaded left heart who underwent a successful device closure of her patent ductus arteriosus. To the best of our knowledge, such a case is being reported from Pakistan for the first time.

Extremely low birth weight (ELBW) infants weighing less than 1 kilogram are at a high-risk for delayed patent ductus arteriosus (PDA) closure. Percutaneous PDA closure offers a less invasive approach when compared with surgical PDA closure, which may provide faster recovery times and less transfusion requirements. However, this procedure involves transporting tenuous, unstable patients from the neonatal intensive care unit (NICU) to the catheterization laboratory which introduces many potential risks for the neonate. Performing percutaneous PDA closure at the bedside offers a successful alternative to performing the procedure in the catheterization laboratory and avoiding risk associated with transporting ELBW neonates.

Heterotaxy is an abnormal arrangement of viscera across the left-right axis of the body often associated with a variety of complex cardiac lesions. We present a case of left isomeric heterotaxy, a right aortic arch, and a left brachiocephalic artery arising from a left ductus arteriosus.

To identify risk factors associated with high-frequency ventilation (HFV) following definitive closure of the patent ductus arteriosus (PDA). We performed a retrospective study of premature infants (

Aortopulmonary (AP) window is exactly a rare congenital anomaly that represents approximately 0.2 and 0.5% of all congenital heart abnormalities. It consists of communication between the aorta and the pulmonary artery or its branches. Although closely related to AP window, the pulmonary artery origin from the ascending aorta (also termed “hemitruncus”) is classified as a separate defect. AP window was first described by Elliotson in 1830 in an autopsy study. In 1948, Robert Gross successfully ligated an AP window in a patient undergoing a thoracotomy for closure of a patent ductus arteriosus (PDA). In 1957, Cooley and associates described the first successful repair of AP window using cardiopulmonary bypass. Aortopulmonary window occurs due to the abnormal development of the heart’s major blood vessels during early fetal growth. In most cases, this heart defect occurs by chance, with no clear reason. This condition can occur on its own or with other heart defects such as: 1. Tetralogy of Fallot, 2. Pulmonary atresia, 3. Truncus arteriosus, 4. Atrial septal defect, 5. Patent ductus arteriosus, 6. Interrupted aortic arch. Treatment for aortopulmonary window involves surgery to close the hole between the aorta and the pulmonary artery with a patch or device. This surgery is normally done as soon as possible after the diagnosis is made, usually when the child is a newborn. Associated lesions are usually repaired during the same surgery. Associated lesions are usually repaired during the same surgery. More complex repairs and myocardial protection strategies are required in patients with associated lesions, increasing the morbidity and mortality associated with the operation.

Minimal data exist about the incidence and risk factors for arch intervention after comprehensive stage II (CSII). Goal of this study was to document incidence of arch interventions after CSII and determine if any differences existed between those who underwent an arch intervention (aiCSII) versus those did not have an intervention. Single-center retrospective chart review of all hypoplastic left heart syndrome patients who underwent a CSII between 6/1/2005 and 2/1/2020 was performed. Univariate analysis was conducted in addition to principal components analysis (PCA). One hundred patients were evaluated. Sixteen patients underwent 24 arch interventions. Age at initial arch reintervention was 1.3 ± 1.2 years (median 1.0 years, range 0.5-2.2 years). Univariate analysis showed that the aiCSII group were more likely to be female, to have had a retrograde arch intervention post-hybrid procedure, and to be younger at time of CSII. On echocardiograms, aiCSII group had significantly higher pre-CSII patent ductus arteriosus velocities, arch velocities on their 1st post-operative and discharge study post-CSII, and arch velocities pre-Fontan. Gradients were higher in the aiCSII via pre-Fontan catheterization. With PCA, echocardiographic and catheterization data remained significantly associated with aiCSII versus those who did not undergo an arch intervention (OR = 4.5 (1.9, 19.8), p = 0.008). Incidence of arch intervention post-CSII was 16%. Echocardiographic arch velocities during the CSII hospitalization were the strongest predictors for subsequent aortic arch interventions. Further studies are needed to determine any modifiable variables that may reduce the incidence of arch interventions.

BACKGROUND: To analyze the change and clinical significance of cortisol, B-type brain natriuretic peptide (BNP), and prostacyclin-2 (PGE-2) levels in premature infants with patent ductus arteriosus (PDA). METHODS: A total of 67 cases of premature infants admitted to our hospital from January 2018 to April 2020 were included, all of whom developed PDA (PDA group). According to the presence or absence of symptoms, they were divided into the symptomatic group (28 cases) and the asymptomatic group (39 cases). In addition, 62 premature infants without PDA who were born in our hospital during the same period were selected as the control group. The expression levels of cortisol, BNP, and PGE-2 in infants in different groups and between infants with symptoms and without symptoms were analyzed, along with the risk factors leading to PDA in preterm infants. The value of cortisol, BNP, and PGE-2 in the diagnosis of PDA in premature infants was also analyzed. RESULTS: Compared with the control group, cortisol in the PDA group was significantly decreased (P

Background Pulmonary hypertension (PH) is a common complication of bronchopulmonary dysplasia (BPD) in very-low-birth-weight infants (VLBWIs). Although recent studies have increased awareness that PH contributes significantly to the high morbidity and mortality of BPD, the risk factors and clinical characteristics for PH in VLBWIs are little known. Objectives To investigate the risk factors and clinical characteristics for BPD-associated pulmonary hypertension (BPD-PH) in VLBWIs. Methods A retrospective case–control observational study of VLBWIs with BPD admitted to a neonatal intensive care unit (NICU) over 4 years. According to echocardiograms confirming elevated pulmonary artery pressure after 28 days after birth, we divided BPD infants into PH group (n = 18) and non-PH group (n = 65). We compared pre- and postnatal characteristics between VLBWIs with or without PH. Multivariable logistic regression analysis was conducted with backward selection. Results A total of 83 infants with BPD were divided into PH group (n = 18) or non-PH group (n = 65). The average birth weight of the infants with BPD was 1078.1 g. Compared with those infants of the non-PH group, the birth weight of BPD-PH infants was significantly lower (968.1 ± 187.7 vs. 1108.5 ± 185.8, P = 0.006). Infants in the PH group had a higher incidence of patent ductus arteriosus (PDA) and underwent longer durations of oxygen therapy and mechanical ventilation compared to those in the non-PH group. In all subjects, birth weight (OR 0.995; 95% CI 0.991–0.999; P = 0.025) and PDA (OR 13.355; 95% CI 2.950–60.469; P = 0.001) were found to be specific risk factors for BPD-PH in this cohort. Conclusions The study shows PDA and birth weight are specific risk factors for BPD-PH in VLBWIs.

A 2.5-year-old intact female Marans domestic chicken was presented for lethargy, open beak breathing, and hyporexia. Echocardiography noted left atrial and left ventricular enlargement and computed tomography angiography revealed a type III left-sided patent ductus arteriosus. Retrograde catheterization of the ductus was performed via percutaneous access of the right external jugular vein, and transvenous ductal occlusion was achieved using an 8-mm Amplatzer™ Vascular Plug 4. Transient bradycardia and hypotension occurred during right heart catheterization, which were successfully treated with atropine and epinephrine. A two-week follow-up postoperative cardiac computed tomography scan confirmed appropriate placement of the occluder within the ductus, and echocardiography demonstrated reduced left heart size. The chicken showed an improvement in clinical signs and remains apparently well six months after the intervention. This report describes the computed tomographic findings of a patent ductus arteriosus in an avian species, minimally invasive transvenous closure of this congenital anomaly with a low-profile occlusion device, and the associated challenges and considerations specific to cardiac intervention in an avian patient.

The vein of Galen aneurysmal malformation (VGAM) is a rare cerebral arteriovenous malformation that can be life-threatening if not diagnosed and treated early. VGAM usually presents in the neonatal period with high-output cardiac failure. We report the case of a full-term male neonate who presented with respiratory distress, and a fontanel bruit soon after birth. A chest radiograph revealed marked cardiomegaly. Transthoracic echocardiography showed dilatation of all four cardiac chambers and a patent ductus arteriosus. Transfontanellar doppler ultrasound and brain computed tomography confirmed the diagnosis of a VGAM. Clinical worsening took place despite aggressive hemodynamic and ventilatory support. The patient's Bicêtre Neonatal Evaluation Score for embolization was 2. Endovascular treatment could not be performed. The patient regretfully passed away. VGAM should be considered in the differential diagnosis of neonatal congestive heart failure with a structurally normal heart. Early diagnosis and treatment improve prognosis considerably.

Transthoracic echocardiography (TTE) is increasingly utilized for guiding transcatheter closure of patent ductus arteriosus (PDA) in extremely low birth weight (ELBW) infants. The objectives of this study were to compare PDA size measurements by TTE with angiographic measurements and to describe TTE techniques used in guiding transcatheter PDA closure (TCPC) in ELBW infants.One hundred twenty-five consecutive ELBW infants (gestational age 27 weeks, birth weight 1 kg) who underwent TCPC before 8 weeks of age under TTE guidance were included. Patent ductus arteriosus sizes were measured from the procedural TTE and angiograms retrospectively by blinded observers. The TTE PDA diameters at the aortic (ED1) and pulmonary end (ED2) were compared with the corresponding angiographic diameters (CD1 and CD2). The TTE PDA lengths, obtained by two techniques (EL1, a straight line between ED1 and ED2; and EL2, a curvilinear line along the PDA), were compared with the PDA length by angiography (CL). Transthoracic echocardiography was used to guide accurate device positioning within the PDA.The procedure weight was 600-1,460 g. The TTE and angiographic PDA diameters were comparable (mean ED1 vs CD1 = 4.5 ± 0.68 vs 4.4 ± 0.85 mm, P = .26; and mean ED2 vs CD2 = 3.1 ± 0.72 vs 3.2 ± 0.94 mm, P = .14). The angiographic length was underestimated by EL1 by 2.6 ± 1.6 mm (P .0001), while EL2 estimated it better (mean EL2 vs CL = 11.0 ± 1.83 vs 10.8 ± 2.15 mm; P = .40). Transcatheter PDA closure was successful in 100% of the cases using TTE guidance. There were no intraprocedural complications.Transthoracic echocardiography guidance during TCPC in ELBW infants eliminates the need for aortograms via femoral arterial access, preventing the complications associated with it. Transthoracic echocardiography PDA measurements are comparable to angiographic measurements, thereby assisting in appropriate device size selection.

Transcatheter patent ductus arteriosus (PDA) closure under fluoroscopy imaging is the standardized method; however, it is imperative to utilize alternative methods due to cumulative damaging effects of radiation agents on operator and patient health. An 11-year-old boy with 9-mm-sized type A patent ductus arteriosus (PDA) had progressive limitation of his daily activities. During physical examination, he was hemodynamically stable, and continuous murmur could be heard in the second left intercostal space. The patient was sedated and intubated in the cathlab with endotracheal tube No. 6.0. Hemodynamic data was acquired with non-fluoroscopic technique combined with pressure tracking to observe the catheter position. The value of flow ratio (FR) was 2.2, pulmonary artery resistance index (PARi) was 2.3 W.U/m2, and the ratio of pulmonary vascular ratio to systemic vascular resistance (PVR/SVR) was 0.13. PDA closure was performed by antegrade transvenous approach using Heart-R PDA Occluder No. 18/16 mm which was inserted through 9F delivery sheath, with one of the discs inflated in the descending aorta and the other disc in the pulmonary artery with the help of pressure tracking. Evaluation of transthoracic echocardiography and transesophageal echocardiography showed good device position and the device was detached. No residual shunt observed in the patient with total procedural time of 53 min. Echocardiography-guided closure is safer for both interventionalist and patients due to avoidance of radiation agents, but must be performed by experienced teams familiar with the conventional method prior to its limited visualization.

Patent ductus arteriosus (PDA) stenting is an accepted method for securing pulmonary blood flow in cyanotic neonates. In neonates with pulmonary atresia and single source ductal-dependent pulmonary blood flow (SSPBF), PDA stenting remains controversial. We sought to evaluate outcomes in neonates with SSPBF, comparing PDA stenting and surgical Blalock–Taussig shunt (BTS). Neonates with SSPBF who underwent PDA stenting or BTS at the four centers of the Congenital Catheterization Research Collaborative from January 2008 to December 2015 were retrospectively reviewed. Reintervention on the BTS or PDA stent prior to planned surgical repair served as the primary endpoint. Additional analyses of peri-procedural complications, interventions, and pulmonary artery growth were performed. A propensity score was utilized to adjust for differences in factors. Thirty-five patients with PDA stents and 156 patients with BTS were included. The cohorts had similar baseline characteristics, procedural complications, and mortality. Interstage reintervention rates were higher in the PDA stent cohort (48.6% vs. 15.4%, p

INTRODUCCION: La coartacion de aorta es una cardiopatia congenita con una incidencia de 4 por cada 10 000 nacidos vivos y puede asociarse o no a ductus arterioso persistente u otras malformaciones. Suele ser asintomatico, normalmente se diagnostica mediante los signos clasicos; con gradiente de presion arterial entre extremidades, pulsos reducidos en extremidades inferiores, hipertension arterial en miembros superiores o, en casos graves, fallo cardiaco izquierdo. Su resolucion puede ser percutanea o quirurgica, dependiendo de la edad y sus caracteristicas. CASO CLINICO: Paciente masculino de 6 anos, asintomatico, con sospecha de coartacion aortica, por presentar una diferencia entre presiones arteriales de miembros superiores e inferiores, extremidades inferiores con pulsos reducidos y soplo sistolico en foco aortico. Se solicito ecocardiograma que informo valvula aortica bicuspide con rafe, insuficiencia ligera y coartacion de aorta; angiotomografia evidencio coartacion de aorta yuxtaductal; cateterismo cardiaco objetivo casi nulo paso de contraste a traves del defecto, por lo que se progra mo tratamiento quirurgico. EVOLUCION: La correccion quirurgica se realizo mediante coartectomia con anastomosis termino-terminal mas cierre del conducto arterioso. Posterior a la intervencion se logro objetivar una mejora relevante en el gradiente de presion entre las extremidades. En el postquirurgico el paciente presento hipertension que logro ser controlada, evoluciono favorablemente y recibio el alta a los 4 dias sin tratamiento antihipertensivo ambulatorio. CONCLUSION: La expectativa de vida en pacientes intervenidos para corregir su cardiopatia congenita es superior en relacion a aquellos que no son intervenidos, por lo que el diagnos tico oportuno supone una herramienta importante para mejorar la calidad y esperanza de vida. ABSTRACT Case Report: Surgical resolution of aortic coarctation in pediatric patient BACKGROUND: Coarctation of the aorta is a congenital heart disease with an incidence of 4 per 10 000 live births, it may or may not be associated with patent ductus arteriosus as well as other mal formations. It is usually asymptomatic and diagnosed by its classic signs such as; arterial pressure gradient between extremities, reduced pulses in the lower extremities, arterial hypertension in the upper extremities or, in severe cases, left heart failure. Its resolution can be percutaneous or surgical, depending on the patient’s age and the characteristics of the defect. CASE REPORTS: A 6-year-old male patient, asymptomatic, with suspected aortic coarctation, due to a difference between arterial pressures in the upper and lower limbs, lower limbs with reduced pulses, and a systolic murmur in the aortic focus. An echocardiogram was requested, which reported a bicuspid aortic valve with raphe, mild regurgitation, and coarctation of the aorta; CT angiography showed coarctation of the juxtaductal aorta; cardiac catheterization showed almost no passage of contrast through the defect, so surgical treatment was scheduled. EVOLUTION: Surgical correction was performed by coartectomy with end-to-end anastomosis and closure of the ductus arteriosus. After the intervention, a relevant improvement in the pressure gradient between the extremities was observed. In the postoperative period the patient presented hypertension, that we managed to control, the patient progressed favorably and was discharged after 4 days without antihypertensive treatment. CONCLUSION: Life expectancy in patients who underwent surgery to correct a congenital heart disease is higher than in those who don’t, so timely diagnosis is an important tool to improve life quality and life expectancy.

Pulmonary atresia with ventricular septal defect is a complex congenital cardiac anomaly. The blood is supplied to the lungs through a patent ductus arteriosus, a major aortopulmonary collateral artery, or in very rare cases from a coronary artery–pulmonary artery fistula. We present two cases with coronary artery–pulmonary artery fistula which underwent surgical intervention. In our first patient, the main pulmonary artery was supplied from the left main coronary artery. In the second patient, the right pulmonary artery originated from the left main coronary artery and continued to the right lung posteriorly to the aorta, while the left pulmonary artery originated from the patent ductus arteriosus. The difference in our cases is that the coronary artery pulmonary artery fistulas behave like major aortopulmonary collateral arteries originating from the coronary arteries. These fistulas were the main source of pulmonary blood flow.

Background Ductal stenting is the preferred method of securing adequate pulmonary blood flow in patients with duct-dependent pulmonary circulation. The main limitation in most centers is the difficult vertical tubular or convoluted ducts that represent real challenges to interventional pediatric cardiologists. We present our experience in patent ductus arteriosus (PDA) stenting with some technical tips to overcome difficulties, especially in stenting tortuous or long tubular ducts. This study was conducted on all patients with cyanotic congenital heart disease who underwent PDA stenting between January 2011 and December 2018. Results We attempted to stent the PDA in 43 patients, with a success rate of 93% (40 patients) and only one procedural mortality. There was also one stent migration that needed to be treated with urgent surgery. Three-fourths of the patients had difficult ductal morphology and origin. One stent was used to cover the PDA in 27 patients (62.8%), two stents were used in 13 (30.2%), and three stents were used in 2 patients (4.6%). In-stent stenosis rate was 12.5% (5 patients) and the development of progressive left pulmonary artery stenosis was seen in two patients (5%). Pulmonary artery growth was adequate in all patients. Conclusions PDA stenting is an effective method of palliation for patients with duct-dependent pulmonary circulation. It has low morbidity and mortality rates. Stenting difficult ducts have become more feasible with evolving materials and techniques.

Objectives To describe clinical and imaging characteristics of an isolated subclavian artery (ISA) in pediatric patients. Background ISA is a rare congenital aortic arch anomaly defined as a loss of connection between the subclavian artery and aorta. The clinical manifestations and complications of ISA in children are unclear. Methods This retrospective study included clinical and imaging data of ISA patients younger than 18 years whose data were recorded in the electronic radiology database during January 2006–August 2019. Results Of 102 enrolled patients, 59 had been diagnosed in the first year of life. The majority of the patients also had congenital heart diseases, of which tetralogy of Fallot was the most common. The vertebral artery and collateral branch of the descending aorta served as blood flow supplies in 94 and 8 patients with ISA, respectively, as confirmed using computed tomography or magnetic resonance imaging. However, the blood supply did not influence the development of ISA. Eleven patients exhibited mild or moderate stenosis of the ISA, although only two exhibited coldness or a blood pressure gradient in the upper extremities. These two symptomatic patients also presented with patent ductus arteriosus, and this association was significant (P = 0.008). Conclusion ISA management is often determined based on symptoms and associated congenital heart diseases. The ISA is prone to stenosis in patients with ipsilateral patent ductus arteriosus. We recommend early surgical ligation or percutaneous closure of the ductus arteriosus in patients with ISA.

A 36‐month‐old female Pembroke Welsh Corgi with a cardiac murmur weighing 12.6 kg was referred to the Matsubara Animal Hospital cardiology service. Echocardiography revealed a patent ductus arteriosus. The dog underwent ductus arteriosus closure using an Amplatz Canine Duct Occluder. After the operation, we suspected coagulation and a platelet disorder because of the slightly increased haemorrhage during the operation, postoperative purpura around the surgical wound inside of the thigh, and dog breed, which is known to be commonly affected with von Willebrand disease (vWD). Subsequently, type 1 vWD was confirmed. Complete occlusion was achieved 1 month after the operation; however, 2 months after the operation, recanalization appeared. Recanalization progressed gradually; cardiac redilation was not detected 6 years after the operation. The late recanalization was most likely associated with vWD. In canine breeds pre‐disposed to developing vWD, pre‐operative testing may be indicated prior to patent ductus arteriosus occlusion, though the prevalence of vWD is rare., This is the first report about late recanalization after complete occlusion of patent ductus arteriosus in a dog with von Willebrand disease.

Objectives: –This prospective study with a sizable cohort was undertaken to assess changes in left and right ventricle systolic and diastolic functions after percutaneous patent ductus arteriosus device closure with appropriate follow up evaluation. Methods: – It is an observational analytical prospective study. Ninety-eight patients were recruited out of which sixty-eight patients underwent percutaneous PDA device closure and were taken for final analysis. The primary objective was to study the left and right ventricular systolic and diastolic functions pre- and post-procedure at 48 h with follow up analysis at six months. Results: – The mean age of the patients was 7.88 ± 5.05 years with the female to male ratio was 3.85:1. Thirty-three (48.52%) of the patients had immediate post PDA device closure LV systolic dysfunction. It was more common in those having pre-procedure mean low LVEF and those having a significant reduction in mitral A velocity. It became normal at six months follow up. The study reported immediate decrease in mea/n LVEF from 63.55 ± 8.11% to 48.19 ± 7.9%. The changes in LVEDD, LVEF, LVFS and LVEDV were statistically significant (p

Background Percutaneous occlusion of ductus arteriosus is well established as the method of choice to treat this structural heart defect. A new generation of Amplatzer™ Duct Occluder II Aditional Sizes prostheses, with lower profile and greater flexibility, in addition to smaller retention discs, was developed for percutaneous treatment of patent ductus arteriosus. This study intended to demonstrate the experience of one center with the use of this device, evaluating technical aspects, immediate occlusion rates, and complications. Methods A retrospective study of a cohort of patients with patent ductus arteriosus treated with Amplatzer™ Duct Occluder II Aditional Sizes. Between October 2018 and March 2021, 27 patients with patent ductus arteriosus, types A and E according to the Krichenko classification, were treated with Amplatzer™ Duct Occluder II Aditional Sizes. The prosthesis was implanted by the usual anterograde approach in 25 patients, and retrograde in two cases. The prosthesis chosen were 2mm larger than the core of the defect, and the length of the prosthesis was 2mm for shorter lesions and smaller infants, and between 4 and 6mm for longer lesions. Results Out of 27 patients, 52% were female, with a mean age and weight of 44.2 months (1 to 135 months) and 15.8kg (2,0 to 29kg), respectively. In the sample, 11 patients presented type A patent ductus arteriosus, and 16 type E, with a mean ratio of 1.9:1 between the prosthesis waist and the central diameter. In all patients, the device was successfully implanted, and only one patient remained with residual shunt, presenting embolization of the prosthesis. This patient had the ductus arteriosus closed by another prosthesis. Conclusion Amplatzer™ Duct Occluder II Aditional Sizes demonstrated safety, versatility, and efficiency in cases of appropriate anatomy. The experience demonstrated here is from a single center, with the Amplatzer™ Duct Occluder II Aditional Sizes. Its increased flexibility and softness demand greater operator expertise.

Objective Evaluate the association of late treatment with acetaminophen vs. immediate surgical ligation with death or neurodevelopmental impairment (NDI) among extremely low gestational age neonates (ELGANs) with persistent patent ductus arteriosus (pPDA). Study design Retrospective comparative epoch study of ELGANs with pPDA being considered for surgical ligation. ELGANs in epoch 1 (2009-2012) were referred for ligation, while infants in epoch 2 (2012-2015) were treated with oral acetaminophen and referred for ligation in the absence of improvement. The primary outcome was a composite of death/NDI at 18-24 months. Results Ninety-two ELGANs with median[IQR] GA 25.2 weeks [24.4, 26.3] had pPDA (43 in epoch 1, 49 in -epoch 2) with acetaminophen-exposed neonates receiving 7 days [7, 7] of treatment. ELGANs in epoch 2 had reduced ligation (aOR 0.30; 95%CI: [0.11, 0.87]), but there was no difference in death/NDI (aOR 1.03; 95%CI: [0.30, 3.56]). Conclusions Late treatment with acetaminophen to avoid surgery for pPDA is associated with reduced ligation but no difference in death/NDI, supporting the safety and effectiveness of this approach.

The Potts shunt has been proposed as a strategy to alleviate symptoms and to delay lung transplantation in paediatric patients with pulmonary arterial hypertension (PAH) [1]. In a similar way to patients with Eisenmenger physiology with a patent ductus arteriosus, the Potts shunt may confer protection to the pressure-overloaded right ventricle in patients with suprasystemic pulmonary pressures. The results, in selected paediatric patients, have been encouraging in terms of survival, World Health Organization (WHO) functional classification and need for transplantation [1–4]. Recently, modifications to the classic Potts shunt have been reported by creating a unidirectional central valved conduit to protect the pulmonary circulation from reverse shunting [5, 6]. We report a second adult patient who underwent placement of a central unidirectional (valved) Potts shunt for severe suprasystemic PAH. After 1 year of follow up, the patient demonstrated overall clinical improvement with a significant increase in exercise tolerance., For young adults with end-stage idiopathic pulmonary hypertension, a valved graft connecting the central pulmonary artery to the distal aortic arch can provide substantial palliation of symptoms and may defer lung transplantation https://bit.ly/2TvMFFC