Search

Background: Interventional therapies for severe pulmonary arterial hypertension (PAH) can provide right ventricular (RV) decompression and preserve cardiac output. Transcatheter stent placement in a residual ductus arteriosus (PDA) is one potentially effective option in critically ill infants and young children with PAH. We sought to assess recovery of RV function by echocardiographic strain in infants and young children following PDA stenting for acute PAH., Methods: Retrospective review of patients < 2 years old who underwent PDA stenting for acute PAH. Clinical data were abstracted from the electronic medical record. RV strain (both total and free wall components) was assessed from echocardiographic images at baseline and 3, 6, and 12 months post-intervention, as well as at last echocardiogram., Results: Nine patients underwent attempted ductal stenting for PAH. The median age at intervention was 38 days and median weight 3.7 kg. One-third (3of 9) of patients had PAH associated with a congenital diaphragmatic hernia. PDA stents were successfully deployed in eight patients. Mean RV total strain was - 14.9 ± 5.6% at baseline and improved to - 23.8 ± 2.2% at 6 months post-procedure (p < 0.001). Mean free wall RV strain was - 19.5 ± 5.4% at baseline and improved to - 27.7 ± 4.1% at 6 months (p = 0.002). Five patients survived to discharge, and four patients survived 1 year post-discharge., Conclusion: PDA stenting for severe, acute PAH can improve RV function as assessed by strain echocardiography. The quantitative improvement is more prominent in the first 6 months post-procedure and stabilizes thereafter., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: The ductus arteriosus (DA) is critical in maintaining postnatal circulation in neonates with obstructed systemic circulation (OSC) and pulmonary circulation (OPC). We hypothesized that the size of the DA and aortic isthmus (AoI) undergoes adaptive growth in utero to counteract the hemodynamic challenges in these congenital heart diseases (CHD)., Methods: Postnatal echocardiograms of neonates diagnosed prenatally with ductal-dependent CHD who were started on prostaglandins within 24 h of birth were reviewed. We assessed the cross-sectional area of the aortic valve opening, pulmonary valve opening, AoI, and DA by calculating (diameter) 2 /body surface area. Neonates were classified into OSC or OPC then subgrouped depending upon the patency of semilunar valves: OSC with and without aortic atresia (OSC-AA and OSC-nAA, respectively) and OPC with and without pulmonary atresia (OPC-PA and OPC-nPA, respectively)., Results: Ninety-four cases were studied. The DA in OSC was significantly larger than OPC, and the DA in OSC-AA was significantly larger than OSC-nAA. The size of the AoI was significantly larger in OPC than OSC and larger in OSC-AA than OSC-nAA. Within the OSC-nAA group, there was no significant difference in the size of the DA, AoI, or pulmonary valve opening between those with retrograde flow (RF) at the AoI and without (nRF) except the aortic valve opening was significantly larger in nRF. All groups had comparable cross-sectional areas of systemic output., Conclusions: Our findings suggest that DA and AoI show compensatory growth to maintain critical blood flow to vital organs against primary anatomical abnormalities in ductus-dependent CHD. (249 words)., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Purpose: Fetal growth restriction (FGR) is a common pregnancy complication that can be associated with several adverse perinatal outcomes. One of these negative outcomes is ductus arteriosus, especially in preterm babies. In this study, intrauterine heart function and ductus diameter were evaluated in babies with FGR., Methods: Thirty-seven fetuses with FGR were compared with 37 normal-weight fetuses at the same gestational week. In our study, ventricular diameters, aorta, pulmonary artery, ductus arteriosus (DA), aortic arch diameter, and flow traces were examined. In addition, the aorta and aortic isthmus diameters were proportioned to the ductus diameter, and the left ventricular myocardial performance index (MPI) [(ICT + IRT)/ET] was evaluated., Results: There was no difference in DA diameters between the patient and control groups. The intragroup comparison of the cases with early- and late-onset FGR revealed no statistically significant difference between DA diameters. However, the ratios of the aortic annulus diameter/ductus diameter (AOD/DAD) and aortic isthmus diameter/ductus diameter (AID/DAD) were significantly lower in early-onset FGR because the diameter of the DA was greater. In addition, the mod-MPI values were higher in the patient group., Conclusions: In our study, although the ductal diameters did not change significantly in the patient group, the ductal diameter was greater in the early-onset intrauterine growth restriction (FGR) group compared with other cardiac measurements. The mod-MPI value, a cardiac function indicator, was higher in fetuses with FGR. These findings may be useful for evaluating postnatal cardiac functions in FGR., (© 2024 Wiley Periodicals LLC.)

There is limited experience in evaluating abnormal ductus arteriosus (DA) insertion pattern by contrast-enhanced cardiac computed tomography (cardiac CT) in patients with right-sided obstructive cardiac defects. Retrospective review of 38 infants with right-sided obstructive cardiac defects who underwent a preoperative cardiac CT between 2016 and 2021. We reviewed the types of cardiac lesions, patterns of ductal insertion, frequency of pulmonary artery (PA) stenosis requiring intervention, total dose length product (DLP), and effective radiation dose. Of 38 infants, 45% were female, the median gestational age and weight were 37 (range 34-40) weeks and 2.95 (range 2-4) kg. The most common pathologies were pulmonary atresia with ventricular septal defect (24%) and tetralogy of Fallot (24%). The abnormal ductal insertion patterns were DA inserting into the left PA in 39%, DA bifurcating into branch PA in 32%, and DA inserting into the right PA in 13%. Of the 38 infants, 76% developed branch PA stenosis requiring intervention. Among patients with abnormal DA insertion, 44% required branch PA arterioplasty during their index surgery compared to 17% without abnormal DA insertion. Regardless of the type of abnormal DA insertion, 67% developed bilateral branch PA stenosis over time. The mean DLP was 8 mGy-cm and the mean calculated effective radiation dose was 0.312 mSv. The utilization of contrast-enhanced cardiac CT in infants with right-sided obstructive heart defects can offer crucial insights into abnormal ductus arteriosus insertion patterns. This information is valuable for effective procedure planning and for monitoring the development of branch pulmonary artery stenosis., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: We describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus., Methods: We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair., Results: Median age at centralization was 9 days (minimum-maximum, 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n =20 [91%]; 2 after bilateral ductal stents) or bidirectional cavopulmonary connection (n = 2 [9%]) using pericardial roll (n = 14 [64%]), patch-augmented direct anastomosis (n = 7 [32%]), and interposition graft (n = 1 [5%]) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n = 11 [50%]) was associated with significantly inferior survival (P = .01). Five patients (23%) died at a median of 59 days (minimum-maximum, 6-257 days) after centralization, all with noncardiac TAPVC. At the latest follow-up for 17 survivors (median, 13.5 years; minimum-maximum, 0.5-25.1 years after centralization), 12 completed Fontan, 4 completed second-stage palliation, and 1 received a transplant before second-stage palliation. PA reintervention was required in 14 patients (64%), including 3 with reoperations independent of staged palliation. Echocardiography from baseline to before the second stage demonstrated branch PA growth with significantly increased diameters (left, P = .0006; right, P = .0002); z-scores significantly increased for right (P = .004) but not left (P = .11)., Conclusions: Successful single-ventricle palliation is possible, although high risk, for patients with bilateral discontinuous ductal PAs. Early postcentralization mortality remains substantial, particularly with associated noncardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation., Competing Interests: Disclosures Athar M. Qureshi reports a relationship with W. L. Gore & Associates, Inc. that includes: consulting or advisory; with Medtronic PLC that includes: consulting or advisory; and with B. Braun Medical Inc that includes: consulting or advisory. The other authors have no conflicts of interest to disclose., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF)., Study Design: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were as follows: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure., Results: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI: 1.07,5.27; P = .034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA)., Conclusions: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population., Competing Interests: Declaration of Competing Interest This research is supported, in part, by donations to the Congenital Cardiac Research Collaborative from the Kennedy Hammill Pediatric Cardiac Research Fund, Liam Sexton Foundation, and A Heart Like Ava. The authors declare no conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: To analyze the influence of RV dysfunction evaluated by Free-angle M-mode (FAM) TAPSE Z-score on retrograde ductus arteriosus flow (RDAF) in fetuses with Ebstein anomaly (EA)., Methods: A retrospective cohort study of 30 EA and 60 normal fetuses were enrolled. The EA group was divided into two groups: with RDAF (EA-RDAF group) and without RDAF (EA-NRDAF group). FAM was used to measure TAPSE of EA and normal fetuses, and Z-scores were calculated. The differences of FAM-TAPSE Z-score, gestational week (GW), maternal age (MA), and mitral valve-tricuspid valve distance (MTD) between three groups were compared. The correlation and binary logistic regression between FAM-TAPSE Z-score, GW, MA, MTD, and RDAF were analyzed., Results: FAM-TAPSE Z-score was significantly lower in EA-RDAF group compared to other groups (p < 0.05). FAM-TAPSE Z-score, GW, and MA were negatively correlated with RDAF (p < 0.05), but no correlation was found between TR, MDT, and RDAF (p > 0.05). Multivariate logistic regression showed that FAM-TAPSE Z-score was an independent influencing factor for RDAF (OR = 0.102, p < 0.05)., Conclusion: RV dysfunction is an independent factor leading to RDAF in EA fetus, which provides a feasible theoretical basis for further study on improvement of RV function through intrauterine treatment to delay and prevent the RDAF, to avoid death cycle and improve live-birth rate., (© 2024 Wiley Periodicals LLC.)

Objective: We aimed to analyze the echocardiographic characteristics and pregnancy outcomes for fetuses with premature complete closure of the fetal ductus arteriosus., Methods: A retrospective analysis was performed for eight cases of premature ductus arteriosus closure diagnosed by prenatal ultrasonography in the Hunan Maternal and Child Health Hospital from July 2019 to August 2022, and the characteristics of fetal echocardiography and pregnancy outcomes of the eight cases were analyzed and summarized., Results: In all cases, the intima of the ductus arteriosus was thickened and occluded, the ductus arteriosus could be seen with slightly hyperechogenic, and no blood flow signal was found in the ductus arteriosus by Doppler ultrasonography. The right heart was enlarged in seven cases, and the whole heart was enlarged in one case. Tricuspid valve regurgitation was observed to different degrees, of which seven cases were severe and one case was moderate. The pulmonary arteries of eight patients had varying degrees of widening. All eight cases were delivered by cesarean section, and one newborn died after follow-up. The prognosis of the other newborns was good., Conclusion: The parameters of prenatal echocardiography are helpful for the prognosis of fetuses with premature closure of the ductus arteriosus. Early prenatal detection, close observation, and clinical guidance can be used to select the right time of delivery., (© 2024 Wiley Periodicals LLC.)

We present the case of 60s male who underwent ductus arteriosus closure at the age of 10. He presented with hoarseness and a 25 mm-sized saccular aortic aneurysm was identified at the site of the closed ductus through the computed tomography( CT). The patient successfully underwent 1-debranch thoracic endovascular aortic repair resulting in improved hoarseness. While rare, several reports have documented aneurysm formation long after ductus arteriosus closure. Recent studies highlight favorable outcomes with endovascular repair. Despite its rarity, aneurysmal formation after ductus closure remains a serious complication. Given the increasing population of patients with prior ductus arteriosus closure and the discontinuation of long-term follow-up, awareness of the complication of aneurysmal formation is crucial. Not only congenital cardiologists but also general physicians should consider this differential diagnosis for patients presenting with symptoms such as hoarseness or back pain and a history of ductus closure.

Objective: In our center, we observed an increased frequency of right aortic arch (RAA) with an agenesis of the ductus arteriosus (ADA) in prenatally diagnosed tetralogy of Fallot (ToF) and its variations. This study aimed to determine whether there is an association of RAA and ADA in fetuses with ToF. Distribution of genetic anomalies and impact on postnatal outcome were further evaluated., Method: Single-center retrospective observational study including pregnancies with prenatal diagnosis of ToF from 2010 to 2023. All cases were subdivided into ToF with pulmonary stenosis (PS) and pulmonary atresia (PA). Clinical and echocardiographic databases were reviewed for pregnancy outcome, genetic anomalies, and postnatal course., Results: The cohort included 169 cases, 124 (73.4%) with ToF/PS and 45(26.6%) with ToF/PA. Agenesis of the ductus arteriosus was significantly associated with RAA in both subtypes of ToF (p = 0.001) compared to left aortic arch and found in 82.5% (33/40) versus 10.7% (9/84) of fetuses with ToF/PS and in 57.1% (8/14) versus 12.9% (4/31) of fetuses with ToF/PA. In both ToF/PS and ToF/PA, RAA/ADA versus RAA/patent DA revealed a significantly higher risk for the presence of genetic abnormalities, especially microdeletion 22q11.2, major aorto-pulmonary collateral arteries and a shorter time to complete surgical repair., Conclusion: We demonstrated a significantly increased frequency of RAA/ADA in patients with prenatally diagnosed ToF. Although this association revealed no significant impact on overall survival, the prenatal detection of RAA/ADA has implications for counseling, genetic evaluation and postnatal management., (© 2024 The Author(s). Prenatal Diagnosis published by John Wiley & Sons Ltd.)

Objective: Docosahexaenoic acid (DHA) is recommended routinely in pregnancy to promote fetal development. DHA has anti-inflammatory activity, but its effects on the fetal heart and circulation are unknown. This study aimed to investigate whether maternal DHA supplementation in the third trimester affects maternal prostaglandin levels and fetal ductus arteriosus flow dynamics., Methods: This was a double-blind randomized controlled trial with parallel groups conducted between 2018 and 2021. Pregnant women aged over 18 years with a normal fetus at 27-28 weeks' gestation showing no cardiac/extracardiac anomalies or ductal constriction were eligible for the trial. Women consuming substances with a known inhibitory effect on prostaglandin metabolism, such as non-steroidal anti-inflammatory drugs and polyphenol-rich foods, were excluded. The intervention group received oral supplementation of omega-3 with 450 mg/day of DHA for 8 weeks and the placebo group received capsules of soy lecithin for 8 weeks. Anthropometric measurements, assessment of polyphenol and omega-3 consumption, fetal morphological ultrasound examination, fetal Doppler echocardiographic examination and blood sample collection were performed at the start of the study and the latter two were repeated at follow-up. Prostaglandin E2 (PGE2) level and echocardiographic parameters were compared between the intervention and placebo groups and between baseline and follow-up., Results: A total of 24 participants were included in each group. After 8 weeks, there were no significant differences between the intervention and placebo groups in maternal serum PGE2 level or Doppler echocardiographic parameters of ductal flow. No case of ductus arteriosus constriction was observed. The expected intragroup changes in cardiac morphology, as a result of advancing gestation, were present., Conclusions: Maternal DHA supplementation in the third trimester at a clinically recommended dose did not result in inhibition of PGE2 or constriction of the ductus arteriosus. These findings should be confirmed in postmarket surveillance studies with larger patient numbers in order to test the full safety profile of DHA and provide robust clinical reassurance. © 2024 International Society of Ultrasound in Obstetrics and Gynecology., (© 2024 International Society of Ultrasound in Obstetrics and Gynecology.)

To evaluate the fetal ductus arteriosus anomalies diagnosed by fetal echocardiography. The perinatal outcomes and associated cardiac and genetic anomalies are also explored. The fetal echocardiography records of 2366 fetuses were evaluated retrospectively. Thirty-seven pregnancies prenatally diagnosed with ductus arteriosus anomalies and evaluated after delivery were enrolled in the study. Perinatal and obstetric outcomes were analyzed. The incidence of ductus arteriosus anomaly in our series was 1.5% (37/2366). The most frequent ductus arteriosus anomaly detected was right-sided ductus arteriosus followed by aneurysm, constriction and bilateral ductus arteriosus with an incidence of 51.3%, 27.1%, 18.9% and 2.7%, respectively. There were 19 fetuses with right-sided ductus arteriosus, of which 15 had tetralogy of Fallot. There were 2 chromosomal anomalies (22q11 microdeletion) in this group. Of the 7 fetuses with ductus arteriosus constriction, 3 (3/7, 42.9%) died in-utero. There were 2 (2/10, 20%) neonatal deaths due to hypoplastic left heart syndrome in the ductus arteriosus aneurysm group. Various types of ductus arteriosus anomalies can be diagnosed prenatally. Perinatal outcomes mostly dependent on the type of the ductus arteriosus anomaly and accompanying cardiac malformations., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

A circular shunt is a poor prognostic factor associated with Ebstein's anomaly. Targeting the constriction of the ductus arteriosus (DA) in order to limit or resolve the circular shunt, has been shown to improve fetal outcomes. Prenatal non-steroidal anti-inflammatory drugs (NSAIDs) have been known to constrict the DA. Recently, prenatal NSAIDs have been used for that purpose in the treatment of circular shunt. Limited research shows that it may be an effective treatment leading to improved fetal outcomes. In this article, we did an extensive review of literature to describe this therapy's effectiveness and outcomes. 82% of fetuses were able to achieve ductal constriction with prenatal NSAID therapy. For fetuses who achieved ductal constriction, fetal demise was less likely (6%) when compared to those who were unable to achieve the same (50%). Of all the fetuses with hydrops, 50% had resoluation of hydrops with prenatal NSAID treatment.

In utero idiopathic constriction of the arterial duct is a rare condition with only a handful reported cases. Ductal aneurysms with thrombus formations on the other hand are significantly more common. We report a case of a term infant who presented with right heart failure due to premature ductal closure and postnatal severe respiratory distress. Subsequent diagnostics revealed paresis of left laryngeal nerve and obstruction of the left pulmonary artery secondary to a ductal aneurysm. Consequently, surgical intervention was considered necessary. Post-operatively, right ventricular function and hoarseness resolved slowly.

Background: The objective was to study the ductus arteriosus morphology in duct-dependent pulmonary circulation and its pattern in different ventricle morphology using CT angiography., Method: From January 2013 to December 2015, patients aged 6 months and below with duct-dependent pulmonary circulation underwent CT angiography to delineate the ductus arteriosus origin, tortuosity, site of insertion, and pulmonary artery anatomy. The ductus arteriosus were classified into type I, IIa, IIb, and III based on its site of origin, either from descending aorta, distal arch, proximal arch, or subclavian artery, respectively., Results: A total of 114 patients and 116 ductus arteriosus (two had bilateral ductus arteriosus) were analysed. Type I, IIa, IIb, and III ductus arteriosus were seen in 13 (11.2 %), 71 (61.2%), 21 (18.1%), and 11 (9.5%), respectively. Tortuous ductus arteriosus was found in 38 (32.7%), which was commonly seen in single ventricular lesions. Ipsilateral and bilateral branch pulmonary artery stenosis was seen in 68 (59.6%) and 6 (5.3%) patients, respectively. The majority of patients with pulmonary atresia intact ventricular septum had type I (54.4%) and non-tortuous ductus arteriosus, while those with single and biventricular lesions had type II ductus arteriosus (84.9% and 89.7%, respectively). Type III ductus arteriosus was more common in biventricular lesions (77.8%)., Conclusions: Ductus arteriosus in duct-dependent pulmonary circulation has a diverse morphology with a distinct origin and tortuosity pattern in different types of ventricular morphology. CT may serve as an important tool in case selection and pre-procedural planning for ductal stenting.

Prenatal recognition of coarctation of the aorta (CoA) may improve neonatal survival and reduce morbidity. However, prenatal diagnosis of CoA remains challenging, with relatively high false-positive and false-negative rates. This study aimed to identify a novel formula based on fetal echocardiographic measures to predict prenatal identification of CoA. A retrospective comparison on the echocardiographic evaluation of 30 patients with suspected CoA between May 2016 and April 2021 was performed. The patients were divided into a postnatal surgical intervention group (n = 13) and a non-intervention group (n = 17). The measurements that showed significant differences were aortic isthmus diameter Z-score (p < 0.001), ductus arteriosus diameter/aortic isthmus diameter (p < 0.001), and distal aortic arch (DA) index (p < 0.001). In the receiver operating characteristic curves analysis, the DA index was the largest with an area under the curve of 0.941 and a cutoff value of 1.28, with a sensitivity of 85% and a specificity of 94%. Measurement of the DA index improved the diagnostic rate of fetal CoA and a DA index ≧ 1.28 indicated fetal CoA cases requiring surgical intervention., (© 2023. The Author(s).)

Double aortic arch (DAA) with subaortic left brachiocephalic vein (LBCV) and right-side ductus arteriosus (RDA) was not reported before delivery, only in adults with anatomy course findings. We present a case of fetal DAA with subaortic LBCV and RDA using high-definition (HD) flow render mode and spatiotemporal image correlation (STIC)., (© 2023 Wiley Periodicals LLC.)

Objectives: To describe prevalence, outcome, color Doppler, and HDlive Flow (Silhouette) with spatiotemporal image correlation (STIC) features of isolated tortuous ductus arteriosus in Japanese fetuses at 28-31 + 6 weeks of gestation., Methods: During a 37-month period, 3,150 fetal screenings were performed at 28-31 + 6 weeks of gestation. Isolated tortuous ductus arteriosus was evaluated using color Doppler and HDlive Flow (Silhouette) with STIC. Prevalence and outcomes were also assessed., Results: There were 22/3,150 fetuses (0.7%) had isolated tortuous ductus arteriosus at 28-31 + 6 weeks of gestation. According to color Doppler and HDlive Flow findings, two types of isolated tortuous ductus arteriosus (Right-angled and Loop shapes) were classified. There were 20 Right-angled and 2 Loop shapes. One fetus with a Right-angled shape was associated with aortic tortuosity. HDlive Flow with STIC revealed spatial relationships of tortuous ductus arteriosus, aortic arch, and descending aorta. All fetuses with isolated tortuous ductus arteriosus had good neonatal outcomes., Conclusions: The incidence of isolated tortuous ductus arteriosus in Japanese fetuses was low compared with those in previous reports. HDlive Flow with STIC provides precise information for spatial recognition and differentiation of isolated tortuous ductus arteriosus, resulting in two type classifications. Isolated tortuous ductus arteriosus in a fetus may be a transient, benign finding in utero ., (© 2023 Walter de Gruyter GmbH, Berlin/Boston.)

Background: There is no consensus on managing pregnancy when the fetus is diagnosed with idiopathic premature constriction or closure of the ductus arteriosus (PCDA). Knowing whether the ductus reopens is valuable information for managing idiopathic PCDA. We conducted a case-series study to investigate the natural perinatal course of idiopathic PCDA and examined factors associated with ductal reopening., Methods: We retrospectively collected information about the perinatal course and echocardiographic findings at our institution, which, on principle, does not determine delivery timing based on fetal echocardiographic results. We also examined perinatal factors related to the reopening of the ductus arteriosus., Results: Thirteen cases of idiopathic PCDA were included in the analysis. The ductus reopened in 38% of cases. Among cases diagnosed in < 37 weeks of gestation, 71% reopened, which was confirmed seven days after diagnosis (interquartile range 4-7). Diagnosis earlier in gestation was associated with ductal reopening (p = 0.006). Two cases (15%) developed persistent pulmonary hypertension. No fetal hydrops or death occurred., Conclusions: The ductus is likely to reopen when prenatally diagnosed before 37 weeks gestation. There were no complications due to our pregnancy management policy. In idiopathic PCDA, especially if the prenatal diagnosis is made before 37 weeks of gestational age, continuing the pregnancy with careful monitoring of the fetus's well-being is recommended.

Background: The ductus arteriosus is part of the fetal circulation. Normally, the vessel closes during the cardiac transition. Delayed closure is associated with complications. The aim of this study was to evaluate the age-related prevalence of open ductus arteriosus in full-term neonates., Methods: Echocardiograms were collected in the population study, the Copenhagen Baby Heart Study. The present study included full-term neonates with an echocardiogram performed within 28 days after birth. All echocardiograms were reviewed to assess ductus arteriosus patency., Results: A total of 21,649 neonates were included. In neonates examined at day zero and day seven, an open ductus arteriosus was found in 36% and 0.6%, respectively. Beyond day seven, the prevalence remained stable at 0.6%., Conclusion: More than one-third of full-term neonates had an open ductus arteriosus on the first day of life, declining rapidly within the first week and stabilizing below 1% after day seven., (© 2023 The Author(s). Published by S. Karger AG, Basel.)

Objective: 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., Materials and Methods: 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., Results: 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., Conclusion: 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.

The ductus arteriosus (DA) has a paradoxical biological role in neonates with congenital heart disease (CHD) and can present with significant management challenges. Critical congenital cardiac lesions rely on the patency of the DA to provide either systemic or pulmonary blood flow. A patent DA (PDA) that remains open can also have adverse consequences depending on the degree of systemic to-pulmonary shunting and volume of ductal steal. As such, the presence of a PDA may pose a challenge in the medical management and timing of surgical repair. In this perspective article, we provide an understanding of the role of the DA in the circulatory system in neonates with CHD and discuss traditional and emerging approaches to support the pulmonary and systemic circulations with manipulation of the DA., (© 2022. The Author(s), under exclusive licence to Springer Nature America, Inc.)

This study aimed to investigate the connections between the echocardiography indices of fetal ductus arteriosus premature constriction and newborn prognosis by analyzing 22 cases of spontaneous fetal ductus arteriosus premature constriction.An ultrasonic instrument was used to observe prenatal fetal heart state, combine clinical examination data and echocardiographic results after delivery, summarize the ultrasound manifestations and imaging characteristics, and analyze the prognosis of the fetus.In all cases, fetal ductus arteriosus premature constriction occurred in the third trimester of pregnancy (34 + 1 to 41 weeks), and no abnormality in extracardiac organs were observed. Seven neonates required respiratory support due to the accompanying severe tricuspid regurgitation. The remaining 15 neonates did not receive respiratory support, including 4 with severe tricuspid regurgitation, 5 with moderate regurgitation, and 6 with mild regurgitation. Significant differences were observed in the fetal right atrium size and tricuspid regurgitation severity between the neonatal respiratory support group and non-respiratory support group. Furthermore, there were statistical differences in the ductus arteriosus inner diameter and pulsation index between the two groups.The severity of fetal ductus arteriosus premature contraction accompanied by tricuspid regurgitation and right atrium enlargement can predict the immediate prognosis of the newborn and provide guidance for the clinical judgment of the timing of pregnancy termination.

The incidence of bilateral ductus arteriosus is sporadically reported in the uterus, and right aortic arch with aberrant of the left subclavian artery in association with bilateral ductus arteriosus is therefore extremely rare. We describe a case of right aortic arch and aberrant left subclavian artery associated with bilateral ductus arteriosus. Scanning around the three-vessel and trachea view to search for an "O" shape and a "U" shape vascular ring is essential for the diagnosis, which reveals vascular structures coursing around the trachea. Four-dimensional echocardiography with high-definition flow imaging and spatiotemporal image correlation technique illustrates the spatial relationships of these vessels and the trachea, which should be considered as a complementary modality in fetal cardiac examinations.

The ductus arteriosus is important to fetal circulation. Failure to close at birth is a common event. In this educational pictorial essay, we illustrate the association of the ductus arteriosus with a variety of congenital cardiac, vascular and pulmonary lesions. These lesions can impact the systemic circulation, the pulmonary circulation or the airway and include coarctation of the aorta, ductal origin of the pulmonary artery and vascular rings., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Branch pulmonary artery (PA) stenosis due to ductus arteriosus (DA) tissue (DA-PS) contributes to the morbidity associated with pulmonary atresia (PAtr). We sought to identify preoperative echocardiographic features predictive of DA-PS. Patients consecutively encountered with PAtr and a DA-dependent pulmonary circulation at birth who underwent intervention in our program over a 5-year period were identified and records reviewed. Preoperative echocardiograms were reviewed to identify features that predicted postoperative DA-PS. Seventy patients with PAtr met inclusion criteria and 36 (51%) had DA-PS. At preoperative echocardiography, the proximal diameter of the PA ipsilateral to the DA was smaller in those with versus without DA-PS (Z-score - 4.8 ± 1.7 vs - 1.1 ± 1.7, respectively p < 0.001). PA origins could not be imaged on the same axial plane in 21/36 (58%) with versus 2/34 (6%) without DA-PS. Patients with DA-PS had an obtuse posterior angle of the PA bifurcation compared to those without (128 ± 17° and 87 ± 21°, p < 0.001), and a posterior angle of > 100° best predicted DA-PS with a sensitivity of 97% and specificity of 76%. An abnormal PA relationship and/or an obtuse posterior bifurcation angle had a sensitivity, specificity, positive and negative predictive value for DA-PS of 78%, 94%, 90% and 86%, respectively. Finally, DA insertion was into the ipsilateral PA in 26/36 (72%) of cases with DA-PS. A smaller proximal ipsilateral PA diameter, inability to image the PAs in the same plane, a posterior PA bifurcation angle of > 100°, and insertion of the DA in the ipsilateral PA demonstrated by echo are useful in identifying patients at risk for DA-PS., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Device migration is one of serious complications in neonates and infants undergoing transcatheter closure of the patent ductus arteriosus (PDA). We hypothesize that neonates and young infants possess the distensibility of the ductus, which may be related to device migration. We retrospectively reviewed angiographic findings in 41 neonates and infants who underwent transcatheter closure of PDA. We measured diameters of the ductus at the pulmonary (PA) side, the center, and the aortic (AO) side before PDA closure, and the device center diameter after device closure. The distensibility index was defined as the ratio of the device center diameter after device deployment to the diameter at the center of the ductus before PDA closure. Age and weight at the procedure were 168 (117-260) days and 5.3 (4.3-6.9) kg, respectively. Thirty-seven subjects accomplished the successful device closure, and four subjects were declined because of the device instability or migration. Implanted devices included Amplatzer Duct Occluders in 33 subjects and Amplatzer Vascular Plug-2 in 8 subjects. The PDA diameters at PA side, at the center, AO side, and the device center diameter were 3.2 (2.2-4.3) mm, 4.7 (3.6-5.7) mm, 7.7 (6.3-9.4) mm, and 5.8 (4.2-6.9) mm, respectively. The PDA diameter before closure was not correlated age and weight. The distensibility index was 1.28 (1.06-1.64), which was significantly correlated to age (r = - 0.49, P = 0.001) and weight (r = - 0.53, P < 0.001). Infants with the younger age and the lower weight have the more distensible PDA, which may be a risk for device migration., (© 2021. Springer Japan KK, part of Springer Nature.)

Foetal ductus arteriosus (DA) constriction can be found in complex foetal heart malformations, but rarely as an isolated defect. Although many cases of DA constriction are usually related to Non-steroidal Anti-Inflammatory Drugs (NSAIDs) maternal intake, other causes remain without an established aetiology and are referred to as idiopathic. Recently, a wide range of risks factors or substances (polyphenol-rich foods intake, naphazoline, fluoxetine, caffeine and pesticides) showed a definitive effect upon the pathway of inflammation, causing DA constriction. We report a case of a premature DA constriction in a woman whose possible risk factor was identified in her maternal occupational exposure to solvents and a comprehensive literature review of 176 cases of NSAID-unrelated DA constriction. A 30-year-old Asian woman was referred to our institution at 33 gestational weeks and 0 days because of suspicion of premature DA constriction. The woman had no history of medication intake, including NSAIDs, alcohol, tobacco or polyphenol-rich-food consumption during pregnancy. A detailed foetal echocardiography revealed a normal cardiac anatomy with hypertrophic, hypokinetic and a dilated right ventricle due to right pressure overload, holosystolic tricuspid regurgitation, and, at the level of the DA, high systolic and diastolic velocities, indicating premature ductal restriction. The right outflow showed dilatation of the pulmonary artery with narrow DA. An urgent caesarean section was performed at 33 gestational weeks and 4 days due to worsening of DA PI and signs of right pressure overload, despite the interruption of exposure to solvents. We assume a relationship exists between premature DA constriction and a maternal occupational exposure to solvents. This hypothesis is reinforced by the presence of associated foetal malformations in in two of the patient's children. Further research is needed to confirm the role of exposure to solvents and toxic chemicals in the pathogenesis of DA constriction, also with experimental animal models.KEY MESSAGESMany cases of DA constriction are usually related to Nonsteroidal Anti-Inflammatory Drugs (NSAIDs) maternal intake.A wide range of risks factors or substances (polyphenol-rich foods intake, naphazoline, fluoxetine, caffeine and pesticides) can cause foetal DA constriction.Further investigation are needed to confirm the role of maternal exposure to solvents in the pathogenesis of DA constriction.

The ductus arteriosus (DA) functionally closes during the transition from fetal to postnatal life because of lung aeration and corresponding cardiovascular changes. The thorough and explicit measurement and visualization of the right and left heart output during this transition has not been previously accomplished. We combined 4D flow MRI (dynamic volumetric blood flow measurements) and T2 relaxometry (oxygen delivery quantification) in surgically instrumented newborn piglets to assess the DA. This was performed in Large White-Landrace-Duroc piglets (n = 34) spanning four age groups: term-9 days, term-3, term+1, and term+5. Subject's DA status was classified using 4D flow: closed DA, forward DA flow, reversed DA flow, and bidirectional DA flow. Over all states, vessel diameters and flows normalized to body weight increased with age (for example in the ascending aorta flow-term-9: 126.6 ± 45.4; term+5: 260.2 ± 80.0 ml/min per kg; p = 0.0005; ascending aorta diameter-term-9: 5.2 ± 0.8; term+5: 7.7 ± 0.4 mm; p = 0.0004). In subjects with reversed DA blood flow there was lower common carotid artery blood flow (forward: 37.5 ± 9.0; reversed: 20.0 ± 7.4 ml/min per kg; p = 0.032). Linear regression revealed that as net DA flow decreases, common carotid artery flow decreases (R 2  = 0.32, p = 0.004), and left (R 2  = 0.33, p = 0.003) and right (R 2  = 0.34, p = 0.003) pulmonary artery flow increases. Bidirectional DA blood flow changed oxygen saturation as determined by MRI between the ascending and descending aorta (ascending aorta: 90.1% ± 8.4%; descending aorta: 75.6% ± 14.2%; p < 0.05). Expanded use of these techniques in preterm animal models will aid in providing new understandings of normal versus abnormal DA transition, as well as to test the effectiveness of related clinical interventions., (© 2021 The Authors. Physiological Reports published by Wiley Periodicals LLC on behalf of The Physiological Society and the American Physiological Society.)

Objective: Ductus arteriosus (DA)-related branch pulmonary stenosis (PS), due to ductal tissue migration into the proximal pulmonary artery (PA) ipsilateral to the DA, is common in newborns with pulmonary atresia (PAtr) and contributes significantly to their mortality and morbidity. We sought to define fetal echocardiographic predictors of DA-PS in PAtr., Methods: This was a study of all neonates diagnosed prenatally with PAtr and a DA-dependent pulmonary circulation, with a DA that joined the underbelly of the arch, who had undergone surgical or catheter intervention in our hospital between 2009 and 2018. The postnatal echocardiograms and clinical records were reviewed to confirm the presence or absence of DA-PS based on the need for angioplasty at initial intervention and/or development of proximal PA stenosis post intervention. Fetal echocardiograms were examined for the features of DA-PS., Results: Of 53 fetuses with PAtr, 34 (64%) had analyzable images, including 20/34 (59%) with and 14/34 (41%) without DA-PS. An inability to visualize the branch PAs in the same plane, largely associated with abnormal DA insertion into the ipsilateral PA (85% of cases), had sensitivity, specificity and positive (PPV) and negative (NPV) predictive values of 75%, 100%, 100% and 74%, respectively, for the prediction of postnatal DA-PS. The mean branch PA posterior bifurcation angle was more obtuse in cases with DA-PS compared to cases without DA-PS (117° ± 17° vs 79° ± 17°, P < 0.001), and an angle of > 100°, the preoperative cut-off observed previously in affected newborns, had a sensitivity, specificity, PPV and NPV of 88%, 79%, 82% and 85%, respectively. The receiver-operating-characteristics curve revealed an angle of ≥ 105° to have a sensitivity and specificity of 88% and 93%, respectively, for prenatal prediction of DA-PS. The presence of one or both features (inability to image in the same plane and the posterior bifurcation angle of ≥ 105°) had a sensitivity, specificity, PPV and NPV of 100%, 93%, 95% and 100%, respectively., Conclusion: An inability to visualize the branch PAs in the same plane, associated with abnormal insertion of the DA in most cases, and/or the presence of a posterior PA bifurcation angle of ≥ 105° are predictive features of postnatal DA-PS in fetuses with PAtr. © 2020 International Society of Ultrasound in Obstetrics and Gynecology., (© 2020 International Society of Ultrasound in Obstetrics and Gynecology.)

The objective of this study was to explore a new method for the differential diagnosis between fetal double aortic arch (DAA) and right aortic arch with mirror-image branches (RAA-MB). Clinical data and prenatal echocardiographic features of the DAA (n = 22) and RAA-MB (n = 65) confirmed by postnatal or autopsy findings were analyzed retrospectively. The angles between the two aortic arches in the DAA group and between the right aortic arch and the mirror branch were measured. The differences between the two groups and differential diagnosis value of the angles were compared and analyzed based on the receiver operating characteristic curve. The proportion of left-sided ductal arteriosus (100%) was higher in the DAA group than that (32.3%) in the RAA-MB group, (P < 0.05). The proportion of conotruncal anomalies is higher in the RAA-MB group (64.6%) than in the DAA group (18.2%) (P < 0.05). There was a significant difference in the angles between the groups (DAA: 50.3° ± 8.3° vs. RAA-MB: 82.9° ± 13.8°) (P < 0.01). When the cutoff value was 62.8°, the sensitivity and specificity of the differential diagnosis were 95.5% and 96.9%, respectively. Distinguishing the angle measurement between DAA and RAA-MB is helpful in prenatal prognosis. We recommend a cutoff value of 62.8°., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Dextrocardia with a ductus arteriosus (DA) malformation is extremely rare. Here, we present a case of dextrocardia complicated with a right DA that is circuitously connecting the pulmonary artery and the right brachiocephalic artery. Coincidentally, the deformities of this fetus are almost symmetrical with those in another fetus (also presented here), which is astonishing and meaningful. Dextrocardia is rare, complicated, and not well understood. In this report, we carefully compared prenatal echocardiographic images and cardiovascular casts from two fetuses. Our report of these cases may provide new insights for cardiologists to better understand dextrocardia and its associated malformations., (© 2021 Wiley Periodicals LLC.)

The echogenic mass in the pulmonary artery is a rare finding in newborns. Differential diagnoses include pulmonary artery thrombosis, ductal aneurysm, and malignant tumor. We report a newborn case who presented with mild desaturation and was found to have a large echogenic mass at the pulmonary bifurcation on echocardiography, which caused partial flow obstruction in the proximal left pulmonary artery. Along with the findings of cardiac computed tomography and magnetic resonance imaging, the diagnosis of ductus arteriosus aneurysm (DAA) was made. This DAA spontaneously regressed without any intervention at 4 months follow-up., (© 2021 Wiley Periodicals LLC.)

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., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Stenting of the ductus arteriosus is part of the hybrid treatment of high-risk patients with hypoplastic left heart syndrome. Dislodgement of a ductal stent is a rare complication. We present challenges faced in successful surgical retrieval of a dislodged ductal stent in a high-risk infant undergoing hybrid palliation for hypoplastic left heart syndrome., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Congenital aneurysm of the ductus arteriosus is reported in 0.8% in neonatal autopsies; however, true incidence is unknown because of high rate of asymptomatic cases and spontaneous regression. Possible complications in symptomatic cases are thromboembolism, spontaneous rupture, erosion, infection, compression of airways, and death. In this report, we present a newborn with giant ductus arteriosus aneurysm (DAA) diagnosed in first day of life, surgically treated after thrombosis of aneurysm., (© 2021 Wiley Periodicals LLC.)