Bilateral Ductus Arteriosus and Discontinuity of the Pulmonary Branches and Pulmonary Atresia: An Unusual Anatomy Diagnosed by Echocardiography.
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ABSTRACT:
SUBMITTER: Rozema TK
PROVIDER: S-EPMC6058759 | biostudies-literature | 2018 Feb
REPOSITORIES: biostudies-literature
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Bilateral Ductus Arteriosus and Discontinuity of the Pulmonary Branches and Pulmonary Atresia: An Unusual Anatomy Diagnosed by Echocardiography.
Rozema Tamika K TK Ashwath Ravi R Snyder Christopher S CS
CASE (Philadelphia, Pa.) 20171208 1
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Project description:IntroductionPrenatal diagnosis of pulmonary atresia is difficult in relative, especially when the pulmonary artery is slim and hypoplastic in development. It is of great importance to search for the blood supply to the pulmonary artery in those fetuses while it challenges most screening sonographers, even fetal echocardiography specialists. We herein report a rare case of pulmonary atresia with ventricular septal defect, complicated with an aberrant ductus arteriosus which provides the blood supply to the pulmonary artery. Besides, the case was also accompanied by cardiac malposition, dextrocardia with situs solitus. The echocardiographic characteristics and autopsy findings are also presented to approach the skill of fetal diagnosis.Case presentationA 30-year-old primigravida woman was referred to our center at gestational age of (24 ± 3) weeks for further fetal cardiac examination for suspected fetal cardiac anomalies. Fetal echocardiography revealed dextrocardia, situs solitus of the atria, an L-ventricular loop, a ventricular septal defect, an enlarged coronary sinus, and pulmonary atresia by transverse scanning. The ductus arteriosus was not present at the three-vessel trachea view with the retrograde flow showing in the pulmonary artery trunk, which suggested the possibility of an aberrant ductus arteriosus. Sagittal and coronal scanning was attempted to find that the pulmonary artery connected with the innominate artery via the aberrant ductus arteriosus. Three-dimensional echocardiography with spatio-temporal image correlation and high-definition flow imaging technique was performed to obtain the three-dimensional rendered image, which clearly showed the malformation in space. The pregnancy was terminated and the gross findings confirmed the prenatal diagnosis.ConclusionA detailed evaluation of fetal cardiac anatomy and hemodynamics is crucial for the detection of an aberrant ductus arteriosus, which plays an important role in the diagnosis of pulmonary atresia with ventricular septal defect. Sagittal and coronal scanning is useful to find the course of this aberrant ductus arteriosus. The three-dimensional echocardiography with spatio-temporal image correlation technique could provide additional spatial information to show great arteries in detail, which can serve as a supplement to traditional two-dimensional modality and benefit examiners to make an accurate diagnosis.
| S-EPMC9283767 | biostudies-literature
Project description:IntroductionSince the first successful percutaneous closure under transesophageal echocardiographic (TEE) guidance, many centers explored transcatheter procedures without fluoroscopy. This single-center study is aimed to show the feasibility and safety of percutaneous patent ductus arteriosus (PDA) closure under echocardiography-only guidance during our 1-year experience.MethodsPatients with PDA were recruited for percutaneous PDA closure guided by either fluoroscopy or echocardiography-only in National Cardiovascular Center Harapan Kita (ClinicalTrials.gov Identifier: NCT05321849, clinicaltrials.gov/ct2/show/NCT05321849). Patients were evaluated clinically and radiologically using transthoracic echocardiography (TTE) at 6, 24, and 48 h after the procedure. The primary endpoint was the procedural success. Secondary endpoints were the procedural time and the rate of adverse events.ResultsA total of 60 patients underwent transcatheter PDA closure, 30 patients with fluoroscopy and 30 patients with echocardiography guidance. All patients had successful PDA closure. There were only residual shunts, which were disappeared after follow-up in both groups, but one patient with a fluoroscopy-guided procedure had moderate tricuspid regurgitation with suspected thrombus in the tricuspid valve. The procedural time was not significantly different between the fluoroscopy and echocardiography groups.
| S-EPMC9167953 | biostudies-literature
Project description:BackgroundPercutaneous occlusion under fluoroscopy guidance has become the preferred method for the treatment of patent ductus arteriosus (PDA). To avoid radiation exposure and contrast agent use, PDA occlusion under transthoracic echocardiography (TTE) guidance was conducted.ObjectivesWe assessed the hypothesis that the success rate of percutaneous PDA occlusion under TTE was noninferior to that under fluoroscopy guidance.MethodsIn this single-center trial, 100 patients were randomly assigned in a 1 : 1 ratio to the TTE group (n = 50) or to the fluoroscopy group (n = 50). The primary endpoint was the success rate of occlusion, with the noninferiority margin set at 8% for the between-group difference in intention-to-treat analysis. Secondary endpoints were hospitalization duration, cost, procedure time, and rate of adverse events including occluder migration, hemolysis, peripheral vascular complications, and residual shunt at 1-month and 12-month follow-up.ResultsPatient, defect, and device characteristics were similarly distributed between groups. The success rate of occlusion was 98% for the TTE group and 100% for the fluoroscopy group (absolute difference: -2%; 95% confidence interval: -5.9% to 1.9%). Cost and procedure duration were significantly lower in the TTE group, without adverse events in either group at a median of 12.0 months (range, 10.0-15.5 months) of follow-up.ConclusionPercutaneous PDA occlusion can be performed via TTE guidance safely and effectively, and the success rate of the TTE-guided procedure was noninferior to that under fluoroscopy guidance, with reduced cost and procedure time. The trial is registered with http://www.chictr.org.cn (ChiCTR-ICR-15006334).
| S-EPMC7556051 | biostudies-literature
Project description:Introduction: Echocardiography (ECHO) with color flow Doppler is considered as the gold standard to identify a hemodynamic patent ductus arteriosus (hs-PDA). However, the optimal diagnostic and therapeutic management for newborns with hs-PDA is still controversial. We aimed to investigate two clinical strategies: (1) targeted treatment based on ECHO criteria and (2) treatment based on ECHO criteria in addition to clinical signs and symptoms. Materials and Methods: This is a cohort study including all neonates consecutively admitted in the Neonatal Intensive Care Unit of University La Sapienza in Rome, with gestational age <32 weeks or body birth weight <1,500 g and with a diagnosis of hs-PDA as confirmed by ECHO evaluation performed within 72 h of life. We classified the babies in two cohorts: (A) pharmacological treatment immediately after ECHO screening and (B) pharmacological therapy for PDA was administered when the relevance of a hs-PDA was associated with clinical signs of hemodynamic instability. Results: We considered as primary outcome newborns who survived without any morbidities (A: 48.1% vs. B: 22.2%, p = 0.022). In particular, we found that the rate of intraventricular hemorrhage stage ≥2 was increased in cohort B (A: 3.7% vs. B 24.4%, p = 0.020). A multivariate analysis showed that assignment to cohort A independently influences the primary outcome. Conclusions: Adopting an hs-PDA management option based on ECHO-directed therapy regardless of symptoms may reduce the morbidity and improve the survival of very low birth weight infants.
| S-EPMC7779760 | biostudies-literature
Project description:Stenosis of systemic semilunar valve in cyanotic congenital heart defects is rare. It can happen in truncus arteriosus with truncal valve stenosis and the very rare anomaly of tetralogy of fallot with aortic valve stenosis. Here we describe a neonate with pulmonary atresia, ventricular septal defect and associated aortic valve stenosis and discuss the points of differentiation from truncus arteriosus.
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Project description: Not available
| S-EPMC10374387 | biostudies-literature
Project description:Disconnected branch pulmonary arteries are sparsely reported cases in prenatal diagnosis literature. We report a case of tetralogy of Fallot with disconnected branch pulmonary arteries, the left pulmonary artery (LPA) arising from an indirect ductus arteriosus (DA) from the base of the innominate artery in a right aortic arch, diagnosed by fetal echocardiography with 3D/4D spatiotemporal image correlation (STIC) imaging. Prenatal diagnosis led to early neonatal intervention to maintain blood flow to the LPA by stenting of the DA. Fetal echocardiographic evaluation (Voluson E10 systems, GE Healthcare, Zipf) with acquisition of images and volumes in the right ventricular outflow tract and three-vessel trachea view with rendering of 3D/4D STIC volume datasets to display images in high-definition color format. Prenatal evaluation was initially done at 17-week gestation in a 28-year-old pregnant female which showed tetralogy of Fallot (TOF). Subsequent evaluation at 34 weeks with 3D/4D STIC datasets showed a small main pulmonary artery (MPA) continuing into an adequately sized right pulmonary artery. The LPA was very small (Z-score -2.63), with no visible connection to MPA. Rendering of the 3D/4D STIC datasets revealed disconnected pulmonary arteries with the vertical DA from the base of the innominate artery in a right aortic arch, continuing as the LPA. Findings were confirmed on postnatal high-resolution CT pulmonary angiography and cardiac catheterization with subsequent stenting of the ductus. This report highlights the incremental benefit of advanced 3D/4D STIC rendering in accurate prenatal diagnosis of a rare anomaly of TOF with disconnected pulmonary arteries, leading to early neonatal intervention to preserve the blood supply to the left lung.
| S-EPMC10522146 | biostudies-literature
Project description:Background: Patent ductus arteriosus (PDA) treatment remains controversial. Modeling on the predictive capacity of early spontaneous PDA closure would help in decision-making. Aim: To design a predictive model of early spontaneous PDA closure. Methods: As part of a trial to assess efficacy and safety of two ibuprofen treatment schemes for PDA, infants below 29 weeks' gestation were scanned between 18 and 72 h of birth, and serially if indicated. PDA treatment was decided based on echocardiography signs of lung overflow or systemic hypoperfusion and clinical criteria. A PDA score that included the echocardiographic parameters significantly associated with treatment prescription was retrospectively applied. Perinatal variables and screening score were included in a backwards elimination model to predict early spontaneous closure. Results: Among 87 eligible infants (27 weeks' gestation; age at screening 45 h), 21 received ibuprofen at 69 h of life [screening score = 7 (IQR = 5-8.5); score at treatment = 9 (IQR = 8-9)], while 42 infants had conservative management, [screening score = 1 (IQR = 0-4)]. Twenty four infants were excluded (ibuprofen contraindication, declined consent or incomplete echocardiography). Screening score showed an AUC = 0.93 to predict early spontaneous PDA closure, [cut-off value = 4.5 (sensitivity = 0.90, specificity = 0.86)]. The predictive model for early spontaneous PDA closure followed the equation: Log (p/1-p) = -28.41 + 1.23* gestational age -0.87* PDA screening score. Conclusions: A predictive model of early spontaneous PDA closure that includes gestational age and the screening PDA score is proposed to help clinicians in the decision- making for PDA treatment. In addition, this model could be used in future intervention trials aimed to prevent PDA related morbidities to improve the eligibility criteria.
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Project description:Based upon our demonstration that the smooth muscle cell-selective (SMC-selective) putative methyltransferase, Prdm6, interacts with myocardin-related transcription factor-A, we examined Prdm6's role in SMCs in vivo using cell type-specific knockout mouse models. Although SMC-specific depletion of Prdm6 in adult mice was well tolerated, Prdm6 depletion in Wnt1-expressing cells during development resulted in perinatal lethality and a completely penetrant patent ductus arteriosus (DA) phenotype. Lineage tracing experiments in Wnt1Cre2 Prdm6fl/fl ROSA26LacZ mice revealed normal neural crest-derived SMC investment of the outflow tract. In contrast, myography measurements on DA segments isolated from E18.5 embryos indicated that Prdm6 depletion significantly reduced DA tone and contractility. RNA-Seq analyses on DA and ascending aorta samples at E18.5 identified a DA-enriched gene program that included many SMC-selective contractile associated proteins that was downregulated by Prdm6 depletion. Chromatin immunoprecipitation-sequencing experiments in outflow tract SMCs demonstrated that 50% of the genes Prdm6 depletion altered contained Prdm6 binding sites. Finally, using several genome-wide data sets, we identified an SMC-selective enhancer within the Prdm6 third intron that exhibited allele-specific activity, providing evidence that rs17149944 may be the causal SNP for a cardiovascular disease GWAS locus identified within the human PRDM6 gene.
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Project description:Pulmonary artery dissection is an exceedingly rare and highly lethal diagnosis that can result in arterial rupture; hence, it is most often identified postmortem. Moreover, pulmonary artery complications resulting from aortic dissection are uncommon occurrences that have seemingly only been reported in cases of Stanford type A aortic dissections. Due to the rarity of pulmonary artery dissections, there is no current established algorithm for treatment of these patients, unlike aortic dissections. We herein present a case of a 40-year-old male with history of uncontrolled hypertension who developed acute back and leg pain that was subsequently diagnosed with a Stanford type B aortic dissection that extended into the main pulmonary artery by way of the ductus arteriosus. Although the patient received appropriate care for his aortic dissection and hypertensive emergency, he eventually died due to development of extensive additional vascular insults: cerebrovascular accidents, compartment syndrome, and myocardial infarction. To our knowledge, this is the first case of combined pulmonary artery dissection and Stanford type B dissection in the literature, which unfortunately adds to the understanding that cases of pulmonary artery dissection tend to have a grim prognosis.
| S-EPMC7515976 | biostudies-literature