Diagnostic accuracy of prenatal ultrasound in coarctation of aorta: systematic review and individual participant data meta-analysis.

OBJECTIVE: To determine the diagnostic accuracy of prenatal ultrasound in detecting coarctation of the aorta (CoA). METHODS: An individual participant data meta-analysis was performed to report on the strength of association and diagnostic accuracy of different ultrasound signs in detecting CoA prenatally. MEDLINE, EMBASE and CINAHL were searched for studies published between January 2000 and November 2021. Inclusion criteria were fetuses with suspected isolated CoA, defined as ventricular and/or great vessel disproportion with right dominance on ultrasound assessment. Individual participant-level data were obtained by two leading teams. PRISMA-IPD and PRISMA-DTA guidelines were used for extracting data, and the QUADAS-2 tool was used for assessing quality and applicability. The reference standard was CoA, defined as narrowing of the aortic arch, diagnosed after birth. The most commonly evaluated parameters on ultrasound, both in B-mode and on Doppler, constituted the index test. Summary estimates of sensitivity, specificity, diagnostic odds ratio (DOR) and likelihood ratios were computed using the hierarchical summary receiver-operating-characteristics model. RESULTS: The initial search yielded 72 studies, of which 25 met the inclusion criteria. Seventeen studies (640 fetuses) were included. On random-effects logistic regression analysis, tricuspid valve/mitral valve diameter ratio > 1.4 and > 1.6, aortic isthmus/arterial duct diameter ratio < 0.7, hypoplastic aortic arch (all P < 0.001), aortic isthmus diameter Z-score of < -2 in the sagittal (P = 0.003) and three-vessel-and-trachea (P < 0.001) views, pulmonary artery/ascending aorta diameter ratio > 1.4 (P = 0.048) and bidirectional flow at the foramen ovale (P = 0.012) were independently associated with CoA. Redundant foramen ovale was inversely associated with CoA (P = 0.037). Regarding diagnostic accuracy, tricuspid valve/mitral valve diameter ratio > 1.4 had a sensitivity of 72.6% (95% CI, 48.2-88.3%), specificity of 65.4% (95% CI, 46.9-80.2%) and DOR of 5.02 (95% CI, 1.82-13.9). The sensitivity and specificity values were, respectively, 75.0% (95% CI, 61.1-86.0%) and 39.7% (95% CI, 27.0-53.4%) for pulmonary artery/ascending aorta diameter ratio > 1.4, 47.8% (95% CI, 14.6-83.0%) and 87.6% (95% CI, 27.3-99.3%) for aortic isthmus diameter Z-score of < -2 in the sagittal view and 74.1% (95% CI, 58.0-85.6%) and 62.0% (95% CI, 41.6-78.9%) for aortic isthmus diameter Z-score of < -2 in the three-vessel-and-trachea view. Hypoplastic aortic arch had a sensitivity of 70.0% (95% CI, 42.0-88.6%), specificity of 91.3% (95% CI, 78.6-96.8%) and DOR of 24.9 (95% CI, 6.18-100). The diagnostic yield of prenatal ultrasound in detecting CoA did not change significantly when considering multiple categorical parameters. Five of the 11 evaluated continuous parameters were independently associated with CoA (all P < 0.001) but all had low-to-moderate diagnostic yield. CONCLUSIONS: Several prenatal ultrasound parameters are associated with an increased risk for postnatal CoA. However, diagnostic accuracy is only moderate, even when combinations of parameters are considered. © 2024 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Fetal circular shunt in Ebstein's anomaly and non-steroidal anti-inflammatory treatment.

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.

Three- and Four-Dimensional Imaging of Ductus Arteriosus in Fetuses With Pulmonary Atresia and Aortic Arch Abnormalities.

Advances in fetal echocardiography including newer techniques like 4D spatio-temporal image correlation technology has improved our understanding of fetal cardiac and extracardiac abnormalities. High resolution ultrasound combined with color Doppler and 3D rendering have contributed to an improved understanding of the fetal vascular system and its anomalies. This pictorial essay including ultrasound images and videos alongside their respective clay models, provides precise information of duct anatomy in fetuses with pulmonary atresia and aortic arch abnormalities.

Aortic dissection in a young male with persistent ductus arteriosus and a novel variant in MYLK.

Pathogenic variants in several genes involved in the function or regulation of smooth muscle cells (SMC) are known to predispose to congenital heart disease and thoracic aortic aneurysm and dissection (TAAD). Variants in MYLK are primarily known to predispose to TAAD, but a growing body of evidence points toward MYLK also playing an essential role in the regulation of SMC contraction outside the aorta. In this case report, we present a patient with co-occurrence of persistent ductus arteriosus (PDA) and thoracic aortic dissection. Genetic analyses revealed a novel splice acceptor variant (c.3986-1G > A) in MYLK, which segregated with disease in the family. RNA-analyses on fibroblasts showed that the variant induced skipping of exon 24, which resulted in an in-frame deletion of 101 amino acids. These findings suggest that MYLK-associated disease could include a broader phenotypic spectrum than isolated TAAD, including PDA and obstructive pulmonary disease. Genetic analyses could be considered in families with TAAD and PDA or obstructive pulmonary disease.

Prenatal Diagnosis of Ductus Arteriosus Anomalies: A Single-Center Study.

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.

Confusion from prenatal diagnosis: Type C persistent fifth aortic arch or right-side ductus arteriosus.

We described a case of a double aortic arch (DAA) with a subaortic left brachiocephalic vein (LBCV) and right-side ductus arteriosus using high-definition (HD) flow render mode and spatiotemporal image correlation (STIC). We experienced uncertainty regarding this interesting case despite the diagnosis of right-sided ductus arteriosus. The ductus arteriosus originates from the right pulmonary artery (PA) and converges into the descending aorta (DAO), whereas the vessel originated from the PA and converged into the ascending aorta (AAO). Therefore, we assumed that the vessel connecting the PA to AAO may be a type-C persistent fifth aortic arch (PFAA).

Premature closure of the arterial duct presenting with right heart failure of the fetus and ductal aneurysm postnatally.

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.

Age-Related Prevalence of Open Ductus Arteriosus in Full-Term Newborns.

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.

Reopening of ductus arteriosus in idiopathic premature constriction or closure of ductus arteriosus: A case series.

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.

Utility of novel echocardiographic measurements to improve prenatal diagnosis of coarctation of the aorta.

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.

Prenatal diagnosis of double aortic arch with subaortic left brachiocephalic vein and right-side ductus arteriosus using high-definition flow render mode and spatiotemporal image correlation.

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).

Ductus arteriosus morphology in duct-dependent pulmonary circulation: CT classification and pattern in different ventricular morphology.

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.

Isolated tortuous ductus arteriosus in a fetus: HDlive Flow with spatiotemporal image correlation (STIC) study.

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.

The ductus arteriosus in neonates with critical congenital heart disease.

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.

Fetal Ductus Arteriosus Premature Constriction.

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.

Ductus arteriosus: more than just the patent ductus arteriosus.

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.

Echocardiographic Predictors of Ductal Tissue-Related Branch Pulmonary Artery Stenosis in Pulmonary Atresia.

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.

Prenatal diagnosis of ductal origin of distal pulmonary artery: presentation of three cases and literature review.

Ductal origin of distal pulmonary artery (DODPA) is a rare congenital cardiovascular anomaly, with an incidence of approximately 1 in 200 000 people. It involves the proximal interruption of one pulmonary artery branch, with the distal part arising from the base of the brachiocephalic artery, via the ipsilateral ductus arteriosus (DA) and the intrapulmonary branch of the pulmonary artery is usually intact. If timely treatment is not provided after birth, it is very likely that, due to DA closure, severe hypoplasia will occur in the lung supplied by the abnormal pulmonary artery. Hence, prenatal diagnosis of DODPA is important to enable initiation of prostaglandin treatment and early rehabilitation of the affected lung. Herein, we report three cases of fetal DODPA diagnosed via two-dimensional (2D) ultrasonography combined with four-dimensional (4D) spatiotemporal image correlation. We also present a literature review, and explore the ultrasonographic findings and the importance of 2D and 4D ultrasonography in obtaining an accurate prenatal diagnosis of DODPA. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Fetal pulmonary hemodynamics: Doppler reference values in low risk pregnancies.

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.