Merged bilateral arterial duct and circumflex retroesophageal right aortic arch in a fetus with normal intracardiac anatomy.

We report the case of a fetus with anamnios sequence and VACTERL syndrome, having a circumflex right aortic arch. Two arterial ducts join anteriorly to form a common vessel that connects to the pulmonary trunk with confluent pulmonary branches. Embryologically, the dorsal right 6th aortic arch did not disappear and the aortic arch development stopped in a symmetrical state with an exceptional "Y-shaped" merged bilateral arterial duct.

Prenatal echocardiographic assessment of right aortic arch.

OBJECTIVES: To report our experience with fetal diagnosis of right aortic arch (RAA) variants based on the ductus arteriosus (DA) anatomy and brachiocephalic vessel branching pattern in relation to the trachea, and to establish whether the echocardiographic 'V-shaped' or 'U-shaped' appearance of the junction between the DA and aortic arch (AA) in the fetal upper mediastinal view is sufficiently accurate for assessment of fetal AA anatomy. METHODS: This was a retrospective study of pregnancies with a prenatal diagnosis of fetal RAA that had postnatal confirmation of AA anatomy, referred to our tertiary center during 2011-2017. Prenatal and postnatal medical records, including echocardiographic and computed tomography (CT)/magnetic resonance imaging (MRI) scan reports, were reviewed, and cardiac and extracardiac abnormalities and the results of genetic testing were recorded. RESULTS: Of 55 consecutive pregnancies with a prenatal diagnosis of fetal RAA, six were lost to follow-up, one was terminated and three were excluded due to lack of postnatal confirmation of AA anatomy. Of the remaining 45 pregnancies, AA anatomy was assessed postnatally by CT in 39, by MRI in one and by direct examination at cardiac surgery in five. A U-shaped appearance was found in 37/45 (82.2%) patients, all of which had a complete vascular ring (CVR). Of these 37 patients, on postnatal confirmation, 21 (56.8%) had RAA with Kommerell's diverticulum, left posterior ductus arteriosus (LPDA) and aberrant left subclavian artery (ALSA) (RAA/LPDA/ALSA), 11 (29.7%) had a double AA (DAA), four (10.8%) had RAA with Kommerell's diverticulum, LPDA and mirror-image (MI) branching (RAA/LPDA/MI), and one (2.7%) had RAA with Kommerell's diverticulum, LPDA and aberrant left innominate artery (ALIA) (RAA/LPDA/ALIA). A V-shaped appearance was found in 3/45 (6.7%) patients, all of which had RAA with right DA not forming a CVR and MI branching. In the 5/45 (11.1%) fetuses with neither U- nor V-shaped appearance, RAA with left anterior DA arising from the left innominate artery and MI branching, not forming a CVR, was found. Twelve (26.7%) fetuses had a congenital heart defect (CHD). RAA forming a CVR (U-shaped appearance) was associated with a septal defect in 6/37 (16.2%) fetuses, while RAA not forming a CVR (V-shaped appearance or no U- or V-shaped appearance) was associated with major CHD in 6/8 (75.0%) fetuses. CONCLUSIONS: In fetuses with RAA, V-shaped appearance of the junction between the DA and AA indicates only that the transverse AA and DA run together on the same side of the thorax (trachea) while a U-shaped appearance is always a sign of a CVR. Among fetuses with a CVR, RAA/LPDA/MI is more frequent than described previously. Finally, RAA forming a CVR is not usually associated with complex CHD, as opposed to RAA not forming a CVR. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

[Prenatal diagnosis of right aortic arch. Types of presentation and postnatal evolution]. / Diagnóstico prenatal de arco aórtico derecho. Formas de presentación y evolución posnatal.

BACKGROUND: Right aortic arch is the most common abnormality of the aortic arch, excluding aberrant right subclavian artery. Its importance lies in its association with genetic syndromes, another important congenital heart defects, and that may cause clinical symptons from vascular rings. OBJECTIVE: Was to describe the prenatal clinical presentation, the correlation with postnatal findings and postnatal development of the fetus. METHODS: Observational retrospective analysis of 18 cases of right aortic arch diagnosed prenatally between the years 2005-2011. RESULTS: 5 pregnancies were interrupted because of ultrasound findings associated with poor prognosis. In 13 cases were term infants, with a good correlation between prenatal and postnatal findings, and the prognosis was excellent in cases in which the prenatal diagnosis of right aortic arch was isolated. CONCLUSIONS: In our experience, right aortic arch is a rare condition, but it can be associated with important congenital heart defects (heart disease or vascular rings) and/or other morphological anomalies in the fetus, in which case it should be ruled out chromosomal or genetic syndrome. If prenatally is considered an isolated finding, after careful ultrasound assessment by obstetricians and pediatric cardiologists, it is likely that evolution will be favorable postnatally.

Biomechanical characterization of ascending aortic aneurysm with concomitant bicuspid aortic valve and bovine aortic arch.

Studies have shown that patients harboring bicuspid aortic valve (BAV) or bovine aortic arch (BAA) are more likely than the general population to develop ascending aortic aneurysm (AsAA). A thorough quantification of the AsAA tissue properties for these patient groups may offer insights into the underlying mechanisms of AsAA development. Thus, the objective of this study was to investigate and compare the mechanical and microstructural properties of aortic tissues from AsAA patients with and without concomitant BAV or BAA. AsAA (n=20), BAV (n=20) and BAA (n=15) human tissues were obtained from patients who underwent elective AsAA surgery. Planar biaxial and uniaxial failure tests were used to characterize the mechanical and failure properties of the tissues, respectively. Histological analysis was performed to detect medial degenerative characteristics of aortic aneurysm. Individual layer thickness and composition were quantified for each patient group. The circumferential stress-strain response of the BAV samples was stiffer than both AsAA (p=0.473) and BAA (p=0.152) tissues at a low load. The BAV samples were nearly isotropic, while AsAA and BAA samples were anisotropic. The areal strain of BAV samples was significantly less than that of AsAA (p=0.041) and BAA (p=0.004) samples at a low load. The BAA samples were similar to the AsAA samples in both mechanical and failure properties. On the microstructural level, all samples displayed moderate medial degeneration, characterized by elastin fragmentation, cell loss, mucoid accumulation and fibrosis. The ultimate tensile strength of BAV and BAA sampleswere also found to decrease with age. Overall, the BAV samples were stiffer than both AsAA and BAA samples, and the BAA samples were similar to the AsAA samples. The BAV samples were thinnest, with less elastin than AsAA and BAA samples, which may be attributed to the loss of extensibility of these tissues at a low load. No apparent difference in failure mechanics among the tissue groups suggests that each of the patient groups may have a similar risk of rupture.

Two cases of a rare association: double aortic arch with tetralogy of Fallot.

Two cases of asymptomatic double aortic arch with tetralogy of Fallot are reported. One presented with a non-dominant left arch and left-sided descending thoracic aorta and the other with a non-dominant left arch, a right-sided descending thoracic aorta and a patent ductus arteriosus. One-stage operation was performed and both patients were discharged free of symptoms and signs related to the double aortic arch and tetralogy of Fallot after the operation. A preoperative recognition of DAA is important, especially in echocardiographic investigation.

4-D MRI flow analysis in the course of interrupted aortic arch reveals complex morphology and quantifies amount of collateral blood flow.

We present findings in a 17-year-old with interrupted aortic arch, in whom standard imaging techniques missed functional and morphological problems. Flow-sensitive four-dimensional magnetic resonance (4-D MR) enabled assessment of the complex anatomy and blood-flow characteristics in the entire aorta and direct quantification of blood flow in collateral vessels. Our findings highlight the entire morphological and functional problem of interrupted aortic arch and illustrate the potential of flow-sensitive 4-D MR for surgical planning in congenital heart disease.

Case report: airway and concurrent hemodynamic management in a neonate with oculo-auriculo-vertebral (Goldenhar) syndrome, severe cervical scoliosis, interrupted aortic arch, multiple ventricular septal defects, and an unstable cervical spine.

We report the challenging case of a 1-week-old, term, 2.4 kg neonate with Goldenhar syndrome (including microcephaly, left microtia, left facial palsy, dextro-scoliosis of the cervical spine, and cervico-thoracic levoscoliosis), multiple ventricular septal defects, a type B interrupted aortic arch, a large patent ductus arteriosis, and radiographic and clinical signs concerning for an unstable cervical spine. Our anesthesia team was consulted for perioperative management of this patient during her surgical repair. This case report describes the use of the Air-Q size 1 laryngeal airway (LA) to assist fiberoptic intubation in an ASA 4 neonate with cardiac disease, an anticipated difficult airway with the addition of an unstable cervical spine, as well as the anesthetic techniques used to maintain hemodynamic stability while the airway was secured.

Left cervical aortic arch associated with multiple vascular anomalies.

Cervical aortic arch is a rare anomaly occasionally associated with other cardiovascular abnormalities. We present a case of tortuous left cervical aortic arch associated with hypoplastic transverse arch, coarctation of the aorta, and right brachiocephalic arteries arising below the coarctation and stenotic origin of the left subclavian artery. These multiple anatomic anomalies, which are associated in our case, have not been described in a single patient previously.

Prenatal diagnosis of congenital vascular rings and slings: sonographic features and perinatal outcome in 81 consecutive cases.

OBJECTIVE: To describe the sonographic features and perinatal outcome of congenital vascular rings diagnosed prenatally at a single tertiary care institution. METHODS: All cases of congenital vascular rings diagnosed by prenatal ultrasound between July 2005 and December 2009 were identified at a single referral center using an established perinatal database. The diagnosis was confirmed by postnatal echocardiography for live-born infants or autopsy for terminated cases. RESULTS: The overall incidence of vascular ring during this 5-year period was 0.086% (81 cases among 94 321 women undergoing perinatal ultrasound examination). Forty-four cases had complete O-shaped (n = 3) or U-shaped (n = 41) vascular rings, and 37 cases had incomplete C-shaped vascular rings (vascular slings). The most common type of vascular ring seen in 50.6% (41/81) of cases was a right aortic arch with aberrant left subclavian artery. The second most common type was a left aortic arch with an aberrant right subclavian artery [39.5% (32/81)]. Chromosomal analysis was performed prenatally in 46 pregnancies, and six cases (13.0%) of fetal aneuploidy were identified. CONCLUSIONS: Congenital vascular rings can be diagnosed with prenatal ultrasound using the three-vessel trachea view and subsequent fetal echocardiography with particular attention to the relationship of the aortic arches, ductal arches, and the trachea.

Passive smoking and aortic arch calcification in older Chinese never smokers: the Guangzhou Biobank Cohort Study.

OBJECTIVE: To study whether passive smoking is a risk factor for aortic arch calcification (AAC) among never smokers. BACKGROUND: We have previously reported that active smoking increases the risk of AAC, but the effect of passive smoking has not been reported. METHODS: We used baseline data of the Phase 1 Guangzhou Biobank Cohort Study (GBCS). 7702 older Chinese never smokers from the Phase 1 GBCS were included. Information on passive smoking and potential confounders were collected by standardized interviews and laboratory assays. AAC was diagnosed from chest X-ray by two experienced radiologists. Unconditional logistic regression was used to estimate odds ratios of AAC for passive smoking with adjustment for potential confounders. RESULTS: In women, the risk for aortic arch calcification (AAC) increased significantly with increasing duration of adulthood passive smoking exposure at home, at work and total duration of adulthood home and work exposure [adjusted odds ratio 1.24 (95% confidence interval 1.09-1.41) for high level of total exposure] (P for trend from 0.012 to 0.001). For passive smoking at home, at work and total exposure, significant trends of increasing severity of AAC with increasing duration of exposure were observed in men and women combined (P for trend from 0.05 to 0.002). CONCLUSION: Passive smoking is a risk factor for aortic arch calcification. Studies of passive smoking and AAC, especially in developing countries can generate important local evidence to raise awareness and to support public health measures to protect non-smokers from second-hand smoke.

Unusual manifestations of vascular rings.

Vascular rings are a relatively rare entity caused by abnormal development of the fetal aortic arches. Most patients with vascular rings present in infancy or early childhood with respiratory (inspiratory) symptoms. The treatment of patients with symptomatic vascular rings is generally straight-forward, focusing on surgical division of the ring. The majority of patients are "cured" by this simple procedure. However, a small percentage of patients do not follow this typical course. The purpose of this manuscript is to review and discuss three patients who demonstrated unusual manifestations of vascular rings. Two patients required reoperation after double aortic arch repair and one had successful treatment of an aberrant right subclavian artery.

Open thoracic aortic repair for dysphagia in patients with Kommerell's diverticulum and right-sided aortic arch with aberrant left subclavian artery.

Kommerell's diverticulum of an aberrant left subclavian artery associated with a right-sided aortic arch is a rare congenital aortic anomaly. This communication reports two cases of this congenital anomaly leading to severe dysphagia. Both cases were successfully repaired with a staged open approach rather than an endovascular approach, which would not have addressed the presenting symptom of dysphagia.

Double aortic arch with dominant left arch and right ligamentum arteriosum.

This case report describes a rare example of double aortic arch with a dominant left aortic arch, patent minor right aortic arch, left descending aorta, and right ligamentum arteriosum causing tracheobronchial compression in a twin baby girl with DiGeorge syndrome. She also had large right subclavian artery arising from right-sided diverticulum of Kommerell, aplastic thymus, T cell lymphopenia with normal immunoglobulin, hypocalcemia, and hypomagnesemia. The diverticulum of Kommerell was resected and minor right aortic arch, right ligamentum arteriosum, and right subclavian artery were divided through right posterolateral thoracotomy. Aortopexy was performed under bronchoscopic guidance to relieve the airway compression. We strongly suggest a right-sided approach in this type of vascular arrangement for easy access and better outcome.

Interrupted aortic arch with anomalous origin of left pulmonary artery from aorta.

We report a case of interrupted aortic arch and origin of the left pulmonary artery from the aorta with bilateral ductus arteriosus and discuss its possible embryological basis. To the best of our knowledge, this combination of anomalies has not been reported in the English medical literature.

[Complete vascular rings]. / Anillos vasculares completos.

The complete vascular rings, embryological anomalies of the aortic arch and great vessels, are frequently incidental findings, although patients with significant anomalies can present with upper airway and oesophagus compression, resulting in non-cardiac morbidity. The diagnostic needs high clinical suspicion. We describe six cases, with a mean age of two and a half months at diagnosis. The objective of our review is to describe the type of complete vascular ring (double aortic arch, right circumflex aortic arch and right aortic arch with aberrant left subclavian artery and left ligamentum arteriosus), the most common symptoms (respiratory symptoms including constant high-pitched, aggravated crying) and invasive and non-invasive diagnostic procedures (esophagograms, CT, MRI).

Interrupted aortic arch: spectrum of MRI findings.

OBJECTIVE: The objective of this article is to review the types, pathogenesis, MRI appearance, treatment, and prognosis of interrupted aortic arch (IAA). CONCLUSION: IAA is a rare congenital vascular anomaly. Although this entity has been evaluated traditionally with echocardiography and angiography, MRI can accurately diagnose and characterize the various forms of IAA and associated congenital heart defects. MRI can also be used to evaluate for postoperative complications after repair.

Double aortic arch and nasogastric tubes: a fatal combination.

Double aortic arch is a common form of complete vascular ring that encircles both the trachea and the esophagus, and presents with various respiratory and esophageal symptoms, usually in the pediatric population. We present a case of double aortic arch in an adult patient that manifested as massive upper gastrointestinal bleeding after prolonged nasogastric intubation.