Aortic Arch Artery Research Articles

Prenatal cardiac biometry and flow assessment in fetuses with bicuspid aortic valve at 20 weeks' gestation: multicenter cohort study.

To investigate prenatal changes in cardiac biometric and flow parameters in fetuses with bicuspid aortic valve (BAV) diagnosed neonatally compared with controls with normal cardiac anatomy. This analysis was conducted as part of the Copenhagen Baby Heart Study, a multicenter cohort study of 25 556 neonates that underwent second-trimester anomaly scan at 18 + 0 to 22 + 6 weeks' gestation and neonatal echocardiography within 4 weeks after birth, in Copenhagen University Hospital Herlev, Hvidovre Hospital and Rigshospitalet in greater Copenhagen, between April 2016 and October 2018. From February 2017 (Rigshospitalet) and September 2017 (Herlev and Hvidovre hospitals), the protocol for second-trimester screening of the heart was extended to include evaluation of the four-chamber view, with assessment of flow across the atrioventricular valves, sagittal view of the aortic arch and midumbilical artery and ductus venosus pulsatility indices. All images were evaluated by two investigators, and cardiac biometric and flow parameters were measured and compared between cases with BAV and controls. All cases with neonatal BAV were assessed by a specialist. Maternal characteristics and first- and second-trimester biomarkers were also compared between the two groups. Fifty-five infants with BAV and 8316 controls with normal cardiac anatomy were identified during the study period and assessed using the extended prenatal cardiac imaging protocol. There were three times as many mothers who smoked before pregnancy in the group with BAV as in the control group (9.1% vs 2.7%; P = 0.003). All other baseline characteristics were similar between the two groups. Fetuses with BAV, compared with controls, had a significantly larger diameter of the aorta at the level of the aortic valve (3.1 mm vs 3.0 mm (mean difference, 0.12 mm (95% CI, 0.03-0.21 mm))) and the pulmonary artery at the level of the pulmonary valve (4.1 mm vs 3.9 mm (mean difference, 0.15 mm (95% CI, 0.03-0.28 mm))). Following conversion of the diameter measurements of the aorta and pulmonary artery to Z-scores and Bonferroni correction, the differences between the two groups were no longer statistically significant. Pregnancy-associated plasma protein-A (PAPP-A) multiples of the median (MoM) was significantly lower in the BAV group than in the control group (0.85 vs 1.03; P = 0.04). Our findings suggest that fetuses with BAV may have a larger aortic diameter at the level of the aortic valve, measured in the left-ventricular-outflow-tract view, and a larger pulmonary artery diameter at the level of the pulmonary valve, measured in the three-vessel view, at 20 weeks' gestation. Moreover, we found an association of maternal smoking and low PAPP-A MoM with BAV. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Msx1 haploinsufficiency modifies the Pax9-deficient cardiovascular phenotype

BackgroundSuccessful embryogenesis relies on the coordinated interaction between genes and tissues. The transcription factors Pax9 and Msx1 genetically interact during mouse craniofacial morphogenesis, and mice deficient for either gene display abnormal tooth and palate development. Pax9 is expressed specifically in the pharyngeal endoderm at mid-embryogenesis, and mice deficient for Pax9 on a C57Bl/6 genetic background also have cardiovascular defects affecting the outflow tract and aortic arch arteries giving double-outlet right ventricle, absent common carotid arteries and interruption of the aortic arch.ResultsIn this study we have investigated both the effect of a different genetic background and Msx1 haploinsufficiency on the presentation of the Pax9-deficient cardiovascular phenotype. Compared to mice on a C57Bl/6 background, congenic CD1-Pax9–/– mice displayed a significantly reduced incidence of outflow tract defects but aortic arch defects were unchanged. Pax9–/– mice with Msx1 haploinsufficiency, however, have a reduced incidence of interrupted aortic arch, but more cases with cervical origins of the right subclavian artery and aortic arch, than seen in Pax9–/– mice. This alteration in arch artery defects was accompanied by a rescue in third pharyngeal arch neural crest cell migration and smooth muscle cell coverage of the third pharyngeal arch arteries. Although this change in phenotype could theoretically be compatible with post-natal survival, using tissue-specific inactivation of Pax9 to maintain correct palate development whilst inducing the cardiovascular defects was unable to prevent postnatal death in the mutant mice. Hyoid bone and thyroid cartilage formation were abnormal in Pax9–/– mice.ConclusionsMsx1 haploinsufficiency mitigates the arch artery defects in Pax9–/– mice, potentially by maintaining the survival of the 3rd arch artery through unimpaired migration of neural crest cells to the third pharyngeal arches. With the neural crest cell derived hyoid bone and thyroid cartilage also being defective in Pax9–/– mice, we speculate that the pharyngeal endoderm is a key signalling centre that impacts on neural crest cell behaviour highlighting the ability of cells in different tissues to act synergistically or antagonistically during embryo development.

KOMMERELL DIVERTICULUM: A RARE BUT POTENTIALLY LIFE THREATENING ANOMALY

TOPIC: Cardiovascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Kommerell diverticulum and aberrant subclavian artery are rare anomalies of the aortic branches in the upper mediastinum. We are presenting a case of a patient incidentally diagnosed with Kommerell diverticulum and aberrant left subclavian artery. CASE PRESENTATION: A 61-year-old male with hypertension and heart failure with preserved ejection fraction, presented to the cardiology clinic after overnight hospitalization for one episode of short-lasting midline chest and left arm pain while resting. He had a history of multiple episodes of non-exertional chest pain and exertional dyspnea, dysphagia and occasional wheezing. Stress test was negative for reversible perfusion defect and showed normal left ventricular ejection fraction. A recently performed transthoracic echocardiogram showed dilated aortic root and possible bicuspid aortic valve. Computed tomography angiogram (CTA) of aorta was performed for further evaluation. CTA aorta revealed a right sided aortic arch and aberrant left subclavian artery with a diverticulum of Kommerell. The patient is being closely followed up outpatient to prevent any potential complications. DISCUSSION: Aberrant left subclavian artery (ALSA) is seen in 0.05-0.1% population and a right aortic arch (aortic arch traversing over the right bronchus) is seen in 0.5% population. These anomalies can be associated with DiGeorge syndrome (deletion of chromosome 22q11.2) and conotruncal congenital heart disease. They can incompletely encircle the trachea and esophagus, creating a partial vascular ring and causing dyspnea, wheezing and dysphagia. In many cases, the ALSA arises from a vascular structure called Kommerell diverticulum (KD), most often derived from a remnant of the fourth aortic arch. KD is similar to the ascending aorta in diameter, and can compress the trachea and esophagus, producing the symptoms mentioned above. It can also cause complications like recurrent pneumonia, aortic dissection or fatal mediastinal hemorrhage from aneurysmal rupture of the diverticulum. CONCLUSIONS: Aneurysms of aberrant subclavian arteries need surgical and/or endovascular resection to decrease mortality. Hence, early diagnosis with close monitoring is crucial in patients with Kommerell diverticulum and aberrant subclavian artery. REFERENCE #1: Adams MT, Wilhelm M, Singh N, Andersen C. Right Aortic Arch With Kommerell Diverticulum: A Rare Cause of Dysphagia. JAMA Otolaryngol Head Neck Surg. 2014;140(6):560-562. doi:10.1001/jamaoto.2014.417 REFERENCE #2: Bhatt TC, Muralidharan CG, Singh G, Jain NK. Kommerell's diverticulum: A rare aortic arch anomaly. Med J Armed Forces India. 2016;72(Suppl 1):S80-S83. doi:10.1016/j.mjafi.2016.09.003 DISCLOSURES: No relevant relationships by surya Aedma, source=Web Response No relevant relationships by Pranav Mahajan, source=Web Response No relevant relationships by Riddhi Upadhyay, source=Web Response No relevant relationships by Karen Wiarda, source=Web Response

Ascending aorta and aortic root replacement in an infant: a case report

We herein report the first clinical case of prosthetic replacement of the ascending aorta and aortic arch to repair a giant aneurysm in a 7-month-old child. The ascending aorta and arch replacement to the level of left subclavian artery was performed using a no. 16 Polymaille prosthesis, the brachiocephalic trunk was reimplanted into the vascular prosthesis and the kinked section of the left common carotid artery was removed, followed by reimplanting the left common carotid artery into the left subclavian artery. First, proximal anastomosis with the vascular prosthesis was created using a no. 16 Polymaille prosthesis and the vascular suture was strengthened with a Teflon strip. During circulatory arrest, the aortic arch was crossed between the orifice of the left common carotid artery and left subclavian artery, with the cut extended to the isthmus region along the small curvature of the arch. The brachiocephalic trunk was aligned and brought down, with subsequent implantation into the ascending aorta prosthesis 2 cm below the initial fixation point. In the final stage, the kinked section of the left common carotid artery was resected and the aligned left carotid artery was directly reimplanted into the left subclavian artery using end-to-side anastomosis. The patient developed tracheobronchitis and moderate heart failure during the postoperative period. The duration of mechanical ventilation was 16 hours. Infusion and antibacterial therapy were discontinued on postoperative day 8. On postoperative day 13, the patient was discharged and referred to the outpatient centre for further treatment and rehabilitation. A sufficiently large-sized prosthesis allows for further development in paediatric patients. This is facilitated by the preservation of the native aortic root with restored valve function and the formation of a bevelled distal anastomosis with a small unchanged aortic section in the isthmus region, which maintains growth potential. This first reported case of an infant demonstrates the possibility of combination interventions on the aortic arch and brachiocephalic artery during the first year of life.Received 30 January 2021. Revised 24 March 2021. Accepted 29 March 2021.Funding: The study did not have sponsorship.Conflict of interest: The authors declare no conflicts of interests.Contribution of the authors: The authors contributed equally to this article.

342 SURGICAL TREATMENT OF ESOPHAGEAL CANCER WITH VARIATIONS OF THE AORTIC ARCH AND ITS BRANCHES

Abstract The variations of the aortic arch and its branches are rare. The surgical treatment of esophageal cancer would be challenged for combination with this anomaly. This study intends to review significant group cases to analyze the influence of this variation on esophagectomy. Methods From 2013 to 2019, 19 patients with aortic arch and brachiocephalic vessels variations underwent esophagectomy, accounting for 0.3% during the same period. The abnormalities were distributed as follows: left-sided aortic arch combined with aberrant right subclavian artery (LAA + ARSA) occurred 13 cases, right-sided aortic arch anomalies with mirror image arch branches (RAA + MIAB) in 1 case, and right-sided aortic arch combined with aberrant left subclavian artery (RAA + ALSA) in 5 case. Of the patients with LAA + ARSA, 11 patients underwent the McKeown esophagectomy, and 2 patients via transhiatal. All patients with RAA underwent left thoracotomy approach.Perioperative outcomes and long-term survival were analyzed. Results The tumors were mostly located in the upper and middle thorax (47.4% and 42.1%). The R0 resection rate was 89.5% (17/19). Recurrent laryngeal nerve injury occurred in 2 patients, anastomotic fistula in 1 patient. Two patients died within 30 days postoperatively. The yield of lymph nodes was 21.8 ± 9.4. The sampling rate of lymph nodes along the recurrent laryngeal nerve was 61.5% in LAA + ARSA group, while 16.7% in RAA group. Recurrence happened in 5 cases (29.4%), including 2 regional relapse. Four patients died after recurrence, with a median time to death of 20 months (range: 10–48 months). Conclusion For aortic arch and brachiocephalic artery variations in esophageal cancer, aberrant right subclavian artery and right-sided aortic arch are the most common types. The surgical strategy is closely related to the aortic arch position. Due to the combined variation of the recurrent laryngeal nerve, the upper mediastinal lymph nodes dissection would be conservative, but with acceptable oncologic results.

One-Stage Hybrid Repair of a Kommerell Diverticulum with Right Aortic Arch and Aberrant Left Subclavian Artery

One-Stage Hybrid Repair of a Kommerell Diverticulum with Right Aortic Arch and Aberrant Left Subclavian Artery

Dexmedetomidine reduces acute kidney injury after endovascular aortic repair of Stanford type B aortic dissection: A randomized, double-blind, placebo-controlled pilot study

Study objectiveTo determine the effect of dexmedetomidine on acute kidney injury (AKI) following endovascular aortic repair (EVAR) for Stanford type B aortic dissection (TBAD). DesignRandomized, double-blind, placebo-controlled, pilot study. SettingUniversity Hospital. Patients102 TBAD patients undergoing EVAR procedures were enrolled. Patients with dissection involving aortic arch or renal artery were excluded. InterventionsPatients were randomly assigned, in a 1:1 ratio, to a dexmedetomidine group (intravenous dexmedetomidine 0.4 μg/kg/h immediately after anesthesia induction and 0.1 μg/kg/h after extubation, which was maintained until 24 h) or a normal saline control group. MeasurementsThe primary outcome was the incidence of AKI within the first two days after surgery, based on the Acute Kidney Injury Network (AKIN) criteria. The secondary outcomes included serum cystatin C and estimated glomerular filtration rate on postoperative days 1, 2, and 7, and in-hospital need for renal replacement therapy (RRT). Long-term outcomes included RRT and all-cause mortality. Main resultsNinety-eight patients completed the study (dexmedetomidine, n = 48; control, n = 50). AKIN stage 1 AKI occurred in 3/48 (6.3%) patients receiving dexmedetomidine, compared with 11/50 (22%) patients receiving normal saline (odds ratio = 0.24, 95% CI: 0.07 to 0.89, P = 0.041). This difference remained significant after adjusting for baseline covariates (adjusted odds ratio = 0.21, 95% CI: 0.05 to 0.84; P = 0.028). Dexmedetomidine led to a lower serum cystatin C on postoperative day 1 (median [IQR] mg/L: 1.31 [1.02–1.72] vs. 1.58 [1.28–1.96]). There were no between-group differences in other secondary or long-term outcomes. During the follow-up (median = 28.4 months), 1 patient in the dexmedetomidine group and 3 patients in the control group required RRT. ConclusionsDexmedetomidine reduced the incidence of AKI in TBAD patients after EVAR procedures. The long-term benefits of dexmedetomidine in this patient population warrant further investigation.Trial registration: ChiCTR-IPR-15006372.

Two fetuses in one family of arterial tortuosity syndrome: prenatal ultrasound diagnosis

BackgroundArterial tortuosity syndrome (ATS) is a rare autosomal recessive connective tissue disorder chiefly characterized by elongated and tortuosity of the large and medium sized arteries and anomalies of the vascular elastic fibers. Here we reported cases of brother about ATS from the same family on the prenatal ultrasound diagnosis. Reports of this case are rare in antenatally and we draw the vessel simulated diagram to display visually.Case presentationPrenatal ultrasound scanning at 29 weeks of gestation of the first fetus showed obvious tortuous and elongated of the aortic arch, ductus arteriosus, left and right pulmonary arteries, carotid and subclavian arteries. Three months after delivery, Contrast-enhanced computed tomography images (CTA) were performed to clearly display vascular abnormalities consistent with prenatal diagnosis of ultrasound. Whole exome sequencing (WES) was performed eight months after birth, two heterozygous variants of SLC2A10 gene was detected in newborn and their father and mother, respectively. Prenatal ultrasound scan at 22 weeks of gestation of the second fetus showed similar cardiovascular imaging. After birth the siblings have facial characteristic features gradually as aging. No surgical intervention was performed in the siblings follow up 19 months.ConclusionsThe key points of prenatal ultrasound diagnosis of ATS are the elongation and tortuosity of the large and medium sized arteries. Genetic counseling is the process of providing individuals and families with information on the nature, inheritance, and implications of genetic disorders to help them make informed medical and personal decisions.

C-factor: a summary measure for systemic arterial calcifications

BackgroundArterial calcification, the hallmark of arteriosclerosis, has a widespread distribution in the human body with only moderate correlation among sites. Hitherto, a single measure capturing the systemic burden of arterial calcification was lacking. In this paper, we propose the C-factor as an overall measure of calcification burden.MethodsTo quantify calcification in the coronary arteries, aortic arch, extra- and intracranial carotid arteries, and vertebrobasilar arteries, 2384 Rotterdam Study participants underwent cardiac and extra-cardiac non-enhanced CT. We performed principal component analyses on the calcification volumes of all twenty-six possible combinations of these vessel beds. Each analysis’ first principal component represents the C-factor. Subsequently, we determined the correlation between the C-factor derived from all vessel beds and the other C-factors with intraclass correlation coefficient (ICC) analyses. Finally, we examined the association of the C-factor and calcification in the separate vessel beds with cardiovascular, non-cardiovascular, and overall mortality using Cox–regression analyses.ResultsThe ICCs ranged from 0.80 to 0.99. Larger calcification volumes and a higher C-factor were all individually associated with higher risk of cardiovascular, non-cardiovascular, and overall mortality. When included simultaneously in a model, the C-factor was still associated with all three mortality types (adjusted hazard ratio per standard deviation increase (HR) > 1.52), whereas associations of the separate vessel beds with mortality attenuated substantially (HR < 1.26).ConclusionsThe C-factor summarizes the systemic component of arterial calcification on an individual level and appears robust among different combinations of vessel beds. Importantly, when mutually adjusted, the C-factor retains its strength of association with mortality while the site-specific associations attenuate.

Free-floating thrombus of the aorta: 3 case reports

BackgroundIdiopathic free-floating thrombus (FFT) of the aorta is a rare occurrence, but it can lead to catastrophic consequences. The initial symptoms are typically cerebral or peripheral embolisms. Surgical thrombectomy and thrombolysis are two primary treatments for FFT. Here, we report three cases of patients with idiopathic FFT in the absence of coagulopathy who were treated successfully by surgery with no recurrent thrombi or relapse of symptoms.Case presentationCase 1 involved a 72-year-old male patient with a pedunculated thrombus in the distal aortic arch. Case 2 involved a 62-year-old female patient with a cylinder thrombus in the aortic arch and left common carotid artery. Case 3 involved a 65-year-old male patient with three pedunculated thrombi in the ascending aorta, aortic arch, and left subclavian artery. None of the patients had clinical signs of coagulopathy. Pedunculated or cylinder thrombi have a greater risk of breaking off, which can produce severe peripheral embolism in contrast with intramural thrombi (73% vs. 12%). Due to the high embolism risk for each patient, conservative medical treatment by heparinization was deemed inappropriate, so each patient underwent emergency surgical thrombus removal. After surgery, each of the three patients was treated with warfarin for secondary prevention of thromboembolism. At 7-month follow-up in outpatient practice, a computed tomography (CT) scan indicated that Patient 1 had no recurrent thrombus, and the patient has been symptom-free for 11 months. At 1-month follow-up in outpatient practice, a CT scan indicated that Patient 2 had no recurrent thrombus, and the patient has been symptom-free for 8 years. At 3-week follow-up in hospital, a CT scan indicated that Patient 3 had no recurrent thrombus, but he failed to follow-up after discharge, so his follow-up status is unknown.ConclusionsFor a large pedunculated or cylinder thrombus located in the thoracic aorta, surgical thrombectomy should be performed. And, in surgical thrombectomy, the location of the cannulas and cross-clamp should be selected carefully according to the location of the thrombus. After surgery, anticoagulant is important to prevent recurrent idiopathic thrombi.

Intravenous injection of adult human bone marrow mesenchymal stromal cells attenuates spinal cord ischemia/reperfusion injury in a murine aortic arch crossclamping model

ObjectiveWe sought to investigate the efficacy of human bone marrow mesenchymal stem/stromal cell (hBM-MSC) in a murine spinal cord ischemia/reperfusion (SCIR) model. MethodsC57BL/6J mice were subjected to SCIR by crossclamping the aortic arch and left subclavian artery for 5.5 minutes. Two hours after reperfusion, hBM-MSCs (hBM-MSC group) or phosphate-buffered saline (control group) were intravenously injected without immunosuppressant. Hindlimb motor function was assessed until day 28 after reperfusion using the Basso Mouse Scale (BMS). The lumbar spinal cord was harvested at hour 24 and day 28, and the histologic number of NeuN-positive motor neurons in 3 cross-sections of each lumbar spinal cord and the gene expression were evaluated. ResultsBMS score was 0 throughout the study period in all control mice. BMS score was significantly greater in the hBM-MSC group than the control group from hour 8 (P < .05) to day 28 (P < .01). The numbers of motor neurons at hour 24 (P < .01) and day 28 (P < .05) were significantly preserved in the hBM-MSC group than the control group. mRNA expression levels of proinflammatory cytokines were significantly lower (P < .05), and those of insulin-like growth factor-1 (P < .01) and proangiogenic factors (P < .05) were significantly greater in the hBM-MSC group than the control group at hour 24. ConclusionshBM-MSC therapy may attenuate SCIR injury by preserving motor neurons, at least in part, through inhibition of proinflammatory cytokines and upregulation of proangiogenic factors in the reperfusion-injured spinal cord.

Adamts18 modulates the development of the aortic arch and common carotid artery

SummaryMembers of a disintegrin and metalloproteinases with thrombospondin motif (ADAMTS) family have been implicated in various vascular diseases. However, their functional roles in early embryonic vascular development are unknown. In this study, we showed that Adamts18 is highly expressed at E11.5-E14.5 in cells surrounding the embryonic aortic arch (AOAR) and the common carotid artery (CCA) during branchial arch artery development in mice. Adamts18 deficiency was found to cause abnormal development of AOAR, CCA, and the third and fourth branchial arch appendages, leading to hypoplastic carotid body, thymus, and variation of middle cerebral artery. Adamts18 was shown to affect the accumulation of extracellular matrix (ECM) components, in particular fibronectin (Fn), around AOAR and CCA. As a result of increased Fn accumulation, the Notch3 signaling pathway was activated to promote the differentiation of cranial neural crest cells (CNCCs) to vascular smooth muscle cells. These data indicate that Adamts18-mediated ECM homeostasis is crucial for the differentiation of CNCCs.

The wandering bullet: An unusual case of paradoxical embolization involving the neck, aortic arch, and iliac artery

Paradoxical intravascular bullet embolism involving the aortic arch (AA) is a rare and highly lethal condition. We describe an unusual case of a civilian gunshot injury to the neck. A bullet entered in the neck, injured the internal jugular vein (IJV), and then continued into the lumen of the common carotid artery (CCA). The bullet traveled under its own momentum and against the flow of blood, along the carotid and brachiocephalic vessels, finally lodging in the wall of the lesser curvature of the AA. The injury tract resulted in an arterial-venous fistula between IJV and CCA and a pseudoaneurysm of the AA. Open surgical repair of the neck and AA was complicated by secondary distal embolization of the bullet, requiring an embolectomy.

A Rare Anomaly of the Aortic Arch: Kommerell Diverticulum Accompanying the Right-sided Aortic Arch and Aberrant Left Subclavian Artery

A Rare Anomaly of the Aortic Arch: Kommerell Diverticulum Accompanying the Right-sided Aortic Arch and Aberrant Left Subclavian Artery

Hybrid repair for Kommerell's diverticulum and right aortic arch with aberrant right vertebral artery.

Kommerell’s diverticulum (KD) is a rare aneurysm of the origin of an aberrant subclavian artery. Hybrid aortic arch repair for KD is being performed more often. We report hybrid arch repair for KD in a 63-year-old man with a right aortic arch and aberrant right vertebral artery, an extremely rare variant. We performed total arch replacement to completely reconstruct the five cervical arteries with elephant trunk to create an adequate landing zone, followed by second-stage endovascular stent-grafting from the ascending aorta to the proximal descending aorta.

Persistent right aortic arch and absent left subclavian artery in a seven‐week‐old English Springer Spaniel

AbstractA seven‐week‐old English Springer Spaniel presented with recurrent vomiting that began with the introduction of solid food. No musculoskeletal issues such as lameness were identified. On radiography, focal dilation of the oesophagus cranial to the heart base was identified. CT angiography confirmed the suspected persistent right aortic arch. A concurrent absent left subclavian artery was identified that has not been reported in detail previously. In addition, a bicarotid trunk and right subclavian artery branched directly from the aortic arch in lieu of a brachiocephalic trunk. A collateral blood supply via an intercostal and vertebral artery furnished the branches of the absent left subclavian artery. The dog developed aspiration pneumonia, deteriorated and was euthanised.

Two-dimensional and doppler echocardiographic evaluation in twenty-one healthy Python regius.

Echocardiographic evaluation is a diagnostic tool for the in vivo diagnosis of heart diseases. Specific and unique anatomical characteristics of the ophidian heart such as the single ventricular cavity, a tubular sinus venosus opening into the right atrium, the presence of three arterial trunks and extreme mobility in the coelomic cavity during the cardiac cycle directly affect echocardiographic examination. Twenty‐one awake, healthy ball pythons (Python regius) were analysed based on guidelines for performing echocardiographic examinations. Imaging in the sagittal plane demonstrated the caudal vena cava, sinus venosus valve (SVV) and right atrium and the various portions of the ventricle, horizontal septum, left aortic arch and pulmonary artery. Transverse imaging depicted the spatial relationship of the left and right aortic arches, the pulmonary artery and the horizontal septum. Basic knowledge of cardiac blood flow in reptiles is necessary to understand the echocardiographic anatomy. The flow of the arterial trunks and SVV was analysed using pulsed‐wave Doppler based on the approach used for humans and companion mammals. The walls and diameters of the cavum arteriosum, cavum venosum and cavum pulmonale were also evaluated. This study should improve the veterinarian's knowledge of ophidian heart basal physiology and contribute to the development of cardiology in reptiles.

Endovascular treatment of extracranial carotid artery aneurysms using self-expandable covered stent grafts: A single center retrospective study.

This study was aimed to evaluate the safety and efficacy of endovascular treatment of extracranial carotid artery aneurysms (ECAAs) using self-expandable covered stent grafts. All patients with ECAA at a single institution were reviewed from February 2014 to February 2020. Eight consecutive patients (three men, mean age 64.5 years) treated with endovascular repair with self-expandable covered stent graft were retrospectively reviewed. Patient characteristics, angiographic results, and follow-up outcomes were retrospectively recorded. Access to ECAA was gained via a femoral approach or a direct puncture of common carotid artery after surgical exposure because of kinking of the aortic arch and common carotid artery. A self-expandable covered stent graft (Viabahn; W. L. Gore, Flagstaff, AZ) was deployed to exclude the aneurysm. Based on imaging features, there were five peudoaneurysms and three true aneurysms. The technical success rate was 100%. Cerebral protection devices were not used in all the patients during the procedures. Immediate absolute obliteration of the ECAA with no endoleak was documented in all the patients. Perioperative complications included one internal carotid-cavernous sinus fistula, one bleeding at the puncture site, and one stroke. The mean follow-up period was 35.5 months (range, 9-72 months). All the patients were alive, with an obligation rate of 100%. No transient ischemia attack, stroke, or reoccurrence of symptoms was identified during the follow-up period. Radiological examinations identified patency of the stent grafts and revealed no endoleaks, stent fracture, stent migration, or aneurysm rupture. Endovascular treatment of ECAAs with self-expandable covered stent grafts appears to be a safe and feasible alternative for traditional open surgery, especially in the challenging anatomy and instable physical conditions. Although cerebrovascular accidents can occur as the result of hemodynamic changes during the perioperative period, the minimal alternative can yield satisfactory midterm follow-up clinical outcomes.

Kommerell diverticulum, vascular ring, and aberrant left subclavian artery: LSCA translocation or aortopexy

Background/PurposeKommerell diverticulum (KD) in children with right aortic arch and aberrant left subclavian artery can be treated surgically via left subclavian artery (LSCA) translocation or aortopexy. These procedures remain controversial. We compared the outcomes of both procedures. MethodsThis retrospective study included 48 patients who underwent LSCA translocation (n = 26) or aortopexy (n = 22). Lateral thoracotomy and sternotomy were performed in 38 and 10 patients with severe intracardiac malformations, respectively. ResultsThe median age of the aortopexy group was 10 months, median follow-up was 14 months, and mean length of hospitalization was 11.41 ± 6.8 days. The median age of the LSCA translocation group was 12 months, median follow-up was 22 months, and mean length of hospitalization was 14.96 ± 7.74 days. Two patients (LSCA translocation group) required secondary intubations and none suffered from recurrent laryngeal nerve injury or complications related to LSCA translocation. Breathing and swallowing symptoms were relieved in all patients. No additional thoracotomies due to respiratory symptoms were required. ConclusionsThese two procedures relieve the pressure on the trachea and esophagus with similar short-term outcomes. The long-term outcomes are unknown. LSCA translocation may be the best strategy to prevent symptom recurrence and late complications in these patients. Level of EvidenceLevel III

ADAMTS18 Modulates the Development of Aortic Arch and Common Carotid Artery

ADAMTS members have been implicated in vascular diseases by degrading extracellular matrix (ECM), but their functional significance in vascular development remains unknown. We now show that the secreted protease ADAMTS18 is produced by mesenchymal cells and fibroblasts surrounding the embryonic aortic arch (AOAR) and the common carotid artery (CCA). ADAMTS18 deficiency leads to aberrant AOAR and CCA, which further disturbs the development of the 3rd and 4th branchial arch appendages, leading to lack of formation of the carotid body, generation of ectopic and hypoplastic thymus, and variation of intracranial vascular branches of CCA. ADAMTS18 was shown to modulate the ECM abundance around AOAR and CCA, in particular fibronectin (Fn). Adamts18 deficiency lead to Fn accumulation, thus activating the Notch3 signaling pathway to promote the differentiation of cranial neural crest cells (CNCCs) to vascular smooth muscle cells. These data disclose that ADAMTS18-mediated ECM homeostasis is crucial for the differentiation of CNCCs.