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Objectives: Our goal was to describe postoperative complications and outcomes in a large contemporary cohort of children with an isolated double aortic arch (DAA) or a right aortic arch (RAA) with left arterial ligament and to assess the impact of foetal diagnosis on outcomes., Methods: We performed a retrospective analysis of all patients who underwent surgery for DAA or RAA with left arterial ligament between 2005 and 2019., Results: A total of 132 children were operated on for a DAA (n = 77) or a RAA (n = 55). Prenatal diagnosis was made in 100/132 (75.8%). Median age at surgery for DAA was 5.0 (1.7-13.3) months and for RAA was 13.9 (6.4-20.1) months. There was no difference in the age at surgery between the prenatal and postnatal cases (8.6 [4.0-15.6] vs 5.4 months [1.8-17.7]; P = 0.37). No surgical deaths occurred. Vocal cord palsy was the most common complication, occurring in 12/132 (9%): of these, 11 resolved spontaneously and 1 required a temporary tracheostomy. Logistic regression demonstrated that older age at operation was the only predictor for a postoperative complication (P = 0.02). Overall, 21/67 (31%) of prenatally detected, symptomatic cases reported residual symptoms/signs 1 year after surgery compared to 18/28 (64%) of postnatally detected cases. Postnatal diagnosis was associated with persistent postoperative symptoms/signs [P = 0.006, odds ratio = 3.9 (95% confidence interval 1.5-9.4)]., Conclusions: Surgery to relieve a vascular ring resolves trache-oesophageal compressive symptoms in most cases, but parents/patients should be aware that symptoms/signs may persist in the first postoperative year despite effective release of the vascular ring. Earlier surgery and prenatal diagnosis may improve outcomes., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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.)

Background: Coronary involvement is rare but can be critical in patients with aortitis. Although cardiac ischemia can be resolved by coronary artery bypass grafting (CABG), patients complicated with cardiac ischemia, calcified aorta, and valve insufficiency pose difficult problems for surgeons., Case Presentation: A 71-year-old woman was referred to our institution because of unstable angina. She had been previously diagnosed with aortitis and left subclavian artery occlusion. Contrast-enhanced computed tomography revealed severe left coronary main trunk stenosis, right coronary artery occlusion, and porcelain aorta. Ultrasonic echocardiogram showed severe aortic regurgitation. We performed emergent coronary artery bypass grafting, aortic valve replacement and ascending aorta replacement under hypothermic circulatory arrest., Conclusions: The technique of circumferential calcified intimal removal and reinforcement with felt strips was effective for secure anastomosis. Unilateral cerebral perfusion from the right subclavian artery enabled good visualization and sufficient time to perform distal anastomosis.

A right-sided descending aorta with a left aortic arch is a rare congenital anomaly in which the aortic arch crosses the midline from the left side of the trachea coursing to the descending aorta in the right thoracic cavity. The surgical repair of an interrupted aortic arch with a right-sided descending aorta carries great risks of bronchial and oesophageal obstruction. Herein, we describe a case of successful surgical repair of an interrupted aortic arch with a right-sided descending aorta using the swing-back and trap-door techniques., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Aortic arch anomalies are rare congenital cardiovascular malformations. We present a case of a 9-year-old asymptomatic boy with an unusual, unknown arch anomaly.

Objective: To establish the impact that timing of diagnosis and place of birth have on neonatal outcomes in those with readily treatable critical congenital heart disease., Study Design: This was a population-based study with a complete national cohort of live-born infants with transposition of the great arteries and aortic arch obstruction in New Zealand between 2006 and 2014. Timing of diagnosis, place of birth, survival to surgery, in-hospital events, and neonatal mortality were reviewed. Live births with a gestation of ≥35 weeks and without associated major extracardiac anomalies were included for analysis., Results: A total of 166 live-born infants with transposition of the great arteries and 87 with aortic arch obstruction were included. Antenatal detection increased from 32% in the first 3 years to 47% in the last 3 years (P = .05). During the same period, neonatal mortality decreased from 9% to 1% (P = .02). No deaths occurred after surgical intervention. An antenatal diagnosis was associated with decreased mortality (1/97 [1%] vs 11/156 [7%]; P = .03) and birth outside the surgical center was associated with increased risk of mortality (11/147 [7%] vs 1/106 [1%]; P = .02). Those with an antenatal diagnosis required fewer hours of mechanical ventilation (P = .02) and had shorter durations of hospital stay (P = .05) compared with those diagnosed >48 hours after birth., Conclusions: The mortality risk for transposition of the great arteries and critical aortic arch obstruction is greatest before cardiac surgery. Improved antenatal detection allowing delivery at a surgical center is associated with reduced mortality., (Copyright © 2018 Elsevier Inc. All rights reserved.)

A 72-year-old man presented with epistaxis on two occasions requiring admission. Prior to performing a bilateral sphenopalatine artery ligation diathermy, anaesthetic concern was raised regarding what the patient described as a 'congenital aortic aneurysm', with an overlying scar secondary to explorative surgery as a child. The abnormality was a cervical aortic arch (CAA). CAA is a rare vascular anomaly, which most commonly manifests as a pulsatile neck mass. In this case, we discuss the differential diagnosis for a pulsatile neck mass and considerations to be made in the workup. We also highlight the importance of cardiovascular risk factor management in patients with CAA., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: In children and adolescents, a Ross/Konno operation is commonly done to both enlarge the aortic root and provide a competent aortic valve with relief of left ventricular outflow tract obstruction (LVOTO). Optimum management is not so straightforward in adults., Methods: Between 1995 and 2014, 16 patients of mean age 39.4 years (18-57 years) with hypoplastic aortic annulus (AA) measuring 20mm and less, and mean aortic valve/LVOT gradient of 61mmHg (30-70mmHg) presented for surgery., Results: Eight patients with mean LVOT/AA diameter 19.6mm (18-20mm) underwent an "inclusion-cylinder" type Ross procedure (RP). Eight patients with more severe LVOT/AA obstruction, with mean diameter of 17.4mm (16-19mm) underwent mechanical aortic valve replacement (AVR) with standard Konno-type aortoventriculoplasty. There was zero early and late mortality; with mean follow-up of 11.6 years (3-21 years) in the Ross group and 6 years (2-10 years) in the Konno-AVR group. One patient in the Konno-AVR group had reoperation after 2 years for RVOT obstruction. The postoperative echocardiograms of these patients at last follow-up show residual mean gradient across LVOT/AA of 4.4mmHg (2-6mmHg) after RP, and 11.9mmHg (8-17mmHg) after Konno-AVR., Conclusions: In adults, the "inclusion-cylinder" Ross-procedure is a good alternative for mild to moderate aortic root hypoplasia. However, for cases with severe LVOT obstruction, a Ross-Konno is not possible with the same method of autologous support used in a non-Konno RP, and this could be expected to have an impact on late durability and the need for further intervention, in a group that has already undergone multiple procedures in childhood. Both methods of RP and Konno-AVR lead to excellent early and late results., (Copyright © 2017. Published by Elsevier B.V.)

A right aortic arch (RAA) with a left arterial duct (LAD) together encircle the trachea and have the potential to cause tracheobronchial compression and published guidelines recommend bronchoscopy in symptomatic patients. The aim of the study was to describe the incidence of tracheal compression in a cohort of prenatally diagnosed RAA and LAD. Retrospective review of clinical course and imaging of prenatal cases of RAA and LAD assessed with flexible bronchoscopy over an 11-year period. 34 cases of prenatally diagnosed RAA with LAD underwent bronchoscopy at median age of 9 months (range 0.4-123) of whom 11 had respiratory symptoms and 23 were asymptomatic. In the neonatal period, three cases demonstrated respiratory symptoms. An aberrant left subclavian artery (ALSA) was identified in 29 cases. Pulsatile tracheal compression was identified in 32/34 (94%) cases and two cases showed normal tracheal appearances. Significant tracheal compression (> 70% occlusion) was present in 25/34 (74%) cases of which 16 were asymptomatic. Significant carinal compression (> 70% occlusion) was identified in 14/34 (42%) cases, an ALSA was observed in 13/14. Surgical relief of a vascular ring has been performed in 27 (79%) cases at a median age of 15 months (range 0.6-128 months). At surgery, a fibrous remnant of an atretic left aortic arch was identified in 11/27 (41%) cases. Significant tracheal compression may be present in infants even without symptoms. If early relief of airway compression is to be achieved to promote normal development of tracheal cartilage, early bronchoscopy should be considered.

Interrupted aortic arch is a rare congenital anomaly in newborns and infants and is commonly associated with other cardiovascular anomalies. Here, we report an unusual case of type A interrupted cervical aortic arch associated with long segment coarctation of the descending thoracic aorta. Patent ductus arteriosus reconstituted the descending thoracic aorta. Proximal segments of the left common carotid and left subclavian arteries were atretic. Echocardiography-gated multidetector CT angiography not only identified the type of aortic arch interruption in the neonate but also delineated the exact anatomical details.

A 50-year-old man presented to the emergency department with interscapular pain, diaphoresis and restlessness. Initial examination raised the possibility of aortic dissection; however, the CT scan did not concur with the diagnosis. An ECG showed ST segment elevation in leads V1-V6 and echocardiography showed severe left ventricular systolic dysfunction. Coronary angiography through the right femoral artery was attempted but the diagnostic catheter could not be advanced to the ascending aorta. Radiocontrast injection showed complete obstruction of the descending aorta. Coronary angiography through right radial approach showed mild left anterior descending disease. The aortogram showed complete interruption of the ascending aorta with extensive collateral network. Left ventricle gram was consistent with stress-induced cardiomyopathy. We noticed intermittent confusion and agitation. MRI of the brain showed areas of deep white matter ischaemia as well as microhaemorrhages, suggesting posterior reversible leucoencephalopathy syndrome. He unfortunately went into cardiac arrest and could not be revived., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Objectives: Our goal was to report the selection schemes, technical variations and long-term outcome of extra-anatomical bypass to correct complex, recurrent aortic coarctation and hypoplastic aortic arch., Methods: Between 1989 and 2012, 53 patients (mean age 13.2 ± 4.3, median 11.6, range 9-23 years) with complex aortic coarctation (n = 33; long-segment hypoplastic aortic arch in 15), recurrent coarctation (n = 20; anastomosic pseudoaneurysm in 10), underwent correction using extra-anatomical bypass, either with (n = 18: femoral bypass = 13, left heart bypass = 5) or without (n = 35) extracorporeal circulation via a left lateral thoracotomy (n= 48) and combined median sternotomy and median laparotomy (n = 5). The decision to use extracorporeal circulation was based on the anatomical location of the coarctation, the length of the hypoplasia and a history of previous repair. Preoperatively, mean systolic blood pressure was 130 ± 30 mmHg at rest and 180 ± 40 mmHg during exercise, with a mean pressure gradient of 80 ± 11.6 (range 40-120) mmHg., Results: Various extra-anatomical bypass strategies included left subclavian artery to descending aorta (n = 38), ascending aorta to left subclavian artery (n = 3), ascending aorta to descending aorta (n = 4), aortic arch to descending aorta (n = 3) and ascending aorta to abdominal aorta (n = 5). Graft size (median 18, range 10-26, mm) was chosen according to the diameter of the vessel proximal and distal to the planned graft. No operative deaths, paraplegia or abdominal malperfusion occurred. The mean reduction in systolic blood pressure was 60 ± 25 mmHg without pressure gradients. During a mean follow-up of 18.3 ± 3.7 years, there were no reoperations, graft complications or pseudoaneurysm formation on anastomotic sites. Seven (11.6%) patients are on antihypertensive medications. No patient presented with claudication nor did anyone experience orthostatic problems from the steal phenomenon., Conclusions: Extra-anatomical bypass is safe, an effective technique, and achieves satisfactory long-term results., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

We report the successful surgical repair of a cervical aortic arch and diverticulum with a brain circulation anomaly through a clamshell incision. Because of the reliability of selective antegrade cerebral perfusion and superior exposure, we chose an approach through a clamshell incision. We describe the utility of this approach for treating a cervical aortic arch with a diverticulum., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The two most common surgical strategies for the treatment of neonates born with single-ventricle anomalies associated with aortic arch obstruction are the Norwood operation and pulmonary artery banding plus coarctation repair (PAB+COA). We reviewed characteristics and outcomes of neonates who underwent those two surgical strategies at our institution., Methods: Between 2002 and 2012, 94 neonates with a single ventricle and aortic arch obstruction (excluding hypoplastic left heart syndrome) underwent Norwood (n = 65) or PAB+COA (n = 29). Outcomes were parametrically modeled, and risk factors associated with early and late death were analyzed., Results: Competing-risks analysis showed that, at 2 years after the operation, 24% of patients had died or received transplantation and 75% had undergone a Glenn shunt. At 5 years after the Glenn shunt, 10% of patients had died or received transplantation, 62% had undergone Fontan, and 28% were alive awaiting Fontan. Overall 8-year survival was 70%. Outcomes after Norwood included extracorporeal membrane oxygenation use in 9 (14%), unplanned reoperation in 13 (20%), hospital death in 10 (15%), and interstage death in 8 (12%), with 8-year survival of 66%. Outcomes after PAB+COA included extracorporeal membrane oxygenation use in 1 (3%), unplanned reoperation in 9 (30%), hospital death in 1 (3%), and interstage death in 3 (10%), with 8-year survival of 76%. There was an association trend between underlying anatomy and survival (hazard ratio [HR], 2.1; 95% confidence interval [CI], 0.9 to 4.7; p = 0.087). On multivariable analysis, factors associated with death were extracorporeal membrane oxygenation use (HR, 5.5; 95% CI, 1.9 to 15.9; p = 0.002), genetic syndromes/extracardiac anomalies (HR, 3.5; 95% CI, 1.5 to 8.2; p = 0.003), and weight of 2.5 kg or less (HR, 3.0; 95% CI, 1.3 to 7.2; p = 0.012)., Conclusions: Anatomic and patient characteristics influence palliation outcomes in neonates born with single-ventricle anomalies associated with aortic arch obstruction. Although the Norwood operation is applicable in most of these patients, the PAB+COA strategy is a valid alternative in well-selected patients., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Aortic arch reconstruction is the key to successfully repairing an interrupted aortic arch (IAA) with tubular hypoplasia of the aortic arch (THAA), especially in older children. We report a novel reconstruction technique involving aortapulmonary fusion that was used to treat THAA in a 9 year-old patient with IAA. In this procedure, the underside of the aortic arch and the upside of the main pulmonary artery were fused to reconstruct the aortic arch. The short-term outcome of the procedure has been promising. This procedure may represent an alternative for repairing extensive THAA in older children., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Three-dimensional (3D) printed prototypes of malformed hearts have been used for education, communication, presurgical planning and simulation. We present a case of a 5-month old infant with complex obstruction at the neoaortic to transverse arch and descending aortic junction following the neonatal modified Norwood-1 procedure for hypoplastic left heart syndrome. Digital 3D models were created from a routine 64-slice CT dataset; then life-size solid and magnified hollow models were printed with a 3D printer. The solid model provided further insights into details of the anatomy, whereas the surgical approach and steps of the operation were simulated on the hollow model. Intraoperative assessment confirmed the anatomical accuracy of the 3D models. The operation was performed in accordance with preoperative simulation: sliding autologous flaps achieved relief of the obstruction without additional patching. Knowledge gained from the models fundamentally contributed to successful outcome and improved patient safety. This case study presents an effective use of 3D models in exploring complex spatial relationship at the aortic arch and in simulation-based planning of the operative procedure., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Vascular rings can be challenging to diagnose because they can contain atretic portions not detectable with current imaging modalities. In these cases, where the compressed airway and esophagus are not encircled by patent, opacified vessels, there are useful secondary signs that should be considered and should raise suspicion for the presence of a vascular ring. These signs include a double aortic arch, the four-vessel sign, the distorted subclavian artery sign, a diverticulum of Kommerell, a ductal diverticulum contralateral to the aortic arch, and a descending aorta contralateral to the arch or circumflex aorta. If none of these findings is present, a ring can be excluded with confidence.

Background: Right aortic arch with aberrant left subclavian artery is the most frequent cause of vascular ring. Ligamentum arteriosus division opens the ring but leaves the Kommerell diverticulum in place, with a risk of residual compression, aneurysmal dilation, dissection, or even rupture. A procedure consisting of translocation of the aberrant left subclavian artery to the left carotid artery with removal of the Kommerell diverticulum and division of the ligamentum is currently advocated., Methods: Between September /2009 and August 2011, 12 consecutive patients underwent the foregoing procedure. Clinical findings, surgical procedure, complications, histopathologic findings, and follow-up data were retrospectively analyzed., Results: Mean age at the time of operation was 7.7 years (median, 4.3 years, range, 0.9 to 18.9 years), and mean weight was 26 kg (median, 18 kg; range, 8.4 to 59 kg). All patients had symptoms. Computed tomography scan or magnetic resonance imaging, or both, had confirmed the diagnosis in all patients. Postoperative events, all transient, included chylothorax (1 patient), phrenic palsy (1 patient), pneumonia (1 patient), and the need for prolonged chest drainage (3 patients). Mean follow-up reached 19 months (median, 13 months; range, 12 to 41 months). Mild residual respiratory symptoms were noted in 3 patients. Echo-Doppler analysis showed a patent left subclavian-to-carotid artery anastomosis. Histopathologic analysis of the resected diverticulum (n = 6) showed cystic medial necrosis in four diverticula (focal in one)., Conclusions: Translocation of the aberrant left subclavian artery with diverticulum resection and ligamentum division is an efficient procedure for symptom relief. Profound wall abnormalities such as medial necrosis in at least 50% of the analyzed diverticula encourage us to maintain this strategy, to reduce the risk of aneurysm formation and dissection., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The aim of this article is to describe the clinical presentation and imaging findings of the right-sided aortic arch, an uncommon anatomical variant. We present a case of associated steal syndrome, to our knowledge the first such case in the literature. Diagnostic imaging of a 49-year-old woman with symptoms of cerebrovascular insufficiency revealed the presence of a right aortic arch, with an aberrant left subclavian artery (lusorian artery). This arrangement placed pressure on the right common carotid artery at the level of its departure with the steal phenomenon dependent on the respiratory phase., (Copyright © 2015 Elsevier Inc. All rights reserved.)

A double aortic arch is a congenital anomaly associated with the formation of a vascular ring, which can cause varying degrees of airway compression. This can be subclinical or clinical, manifesting as acute stridor, severe respiratory compromise or symptoms of chronic airway compression. An accurate diagnosis of double aortic arch can be made prenatally by fetal echocardiography. In this report, we describe two infants with normal karyotypes in whom a prenatal diagnosis of double aortic arch was made at our fetal cardiology center, between July 2012 and August 2013. The diagnosis was confirmed postnatally by two-dimensional echocardiography and cardiac magnetic resonance imaging (MRI). The presence of a right aortic arch should prompt more careful evaluation to exclude a double aortic arch. Postnatal assessment should comprise echocardiography and cross-sectional imaging in the form of cardiac MRI/computed tomography. Bronchoscopy may be considered to exclude subclinical airway compression and reduce potential long-term respiratory sequelae., (Copyright © 2014 ISUOG. Published by John Wiley & Sons Ltd.)

Background: The entity of crossed pulmonary arteries was first described by Jue, Lockman, and Edwards in 1966, in a patient with trisomy 18. Since then, several series have been described, both in terms of the isolated anatomic variant, or its association with other intracardiac or extracardiac anomalies. We describe a rare association that has previously not been reported. Methods and results Institutional Review Board approval for a retrospective chart review was obtained. Over the period 2011 through 2013, we have encountered six patients in whom the crossed origins of the pulmonary arteries from the pulmonary trunk were associated with hypoplasia of the transverse aortic arch, an association that, to the best of our knowledge, has previously not been reported. In all of the patients, the isthmic component of the aortic arch was inserted in an end-to-side manner into the ductal arch, with additional discrete coarctation in half of the patients., Conclusion: To the best of our knowledge, no cases of crossed pulmonary arteries have been described in association with hypoplasia of the transverse aortic arch. We draw comparisons between the cases with exclusively tubular hypoplasia, and those with the added problem of the more typical isthmic variant of aortic coarctation. In all cases, the ability to reconstruct cross-sectional images added significantly to the diagnosis and understanding of these complex lesions. These findings have specific surgical implications, which are discussed.

A 57-year old female was referred to our hospital after symptoms of hoarseness and dysphagia for a few years. She was diagnosed with a cervical vessel anomaly during childhood and had undergone surgery for a right-sided thoracic descending aortic aneurysm through a right thoracotomy at the age of 23 years. Now, enhanced computed tomography revealed newly developed multiple aneurysms in the aorta between the left carotid artery and the proximal descending aorta in the left hemithorax. A successful aortic surgical repair was accomplished via a left posterolateral thoracotomy. To our knowledge, this is the first case of a cervical aortic arch with formation of multiple aneurysms that required multiple surgeries via bilateral thoracotomies. This case suggests the inherent fragility and possibility of later aneurysmal formation in malformed vessels, thereby emphasizing the importance of periodical imaging tests in this rare congenital anomaly., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: There is a paucity of data on the current outcomes of surgical intervention for recurrent aortic arch obstruction (RAAO) after initial aortic arch repair in children. The goal of this study is to report the long-term results in these patients., Methods: All patients undergoing surgical intervention for RAAO at Texas Children's Hospital from 1995 to 2012 were included. The cohort was divided into four groups based on initial procedure: (1) simple coarctation repair, (2) Norwood procedure, (3) complex congenital heart disease, and (4) interrupted aortic arch., Results: A total of 48 patients age 9 months (range, 22 days to 36 years) underwent 49 procedures for RAAO. All patients had an anatomic repair consisting of either patch aortoplasty (n=27, 55%), aortic arch advancement (n=8, 16%), sliding arch aortoplasty (n=6, 12%), placement of an interposition graft (n=2, 17%), reconstruction with donor allograft (n=4, 8%), extended end-to-end anastomosis (n=1, 2%), or redo Norwood-type reconstruction (n=1, 2%). Most procedures (n=46, 94%) were performed through a median sternotomy using cardiopulmonary bypass. At a median follow-up of 6.1 years (range, 9 days to 17 years), only 2 patients required surgical or catheter-based intervention for RAAO. Hypertension was present in 10% of patients at last follow-up. There were no neurologic or renal complications. There was 1 perioperative death after an aortic arch advancement in group 1. Four other patients have died during follow-up, none of the deaths related to RAAO., Conclusions: Anatomic repair of RAAO is a safe procedure associated with low morbidity and mortality, and low long-term reintervention rates., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Vascular rings can present with non-specific respiratory and/or oesophageal symptoms. Early diagnosis requires a high index of suspicion. This case report describes an uncommon acute presentation of a vascular ring. We report a thriving 14-month-old child with a long history of recurrent wheeze and 'noisy breathing'. He presented acutely with food bolus impaction in the oesophagus which led to a respiratory arrest. Oesophagoscopy and bronchoscopy suggested vascular ring anomaly. A contrast-enhanced CT scan demonstrated a right-sided aortic arch with left ligamentum arteriosum encircling the oesophagus and airway. The ligament was ligated and divided. At follow-up 6 months later, the infant had mild persistent stridor but was otherwise well., (2014 BMJ Publishing Group Ltd.)

Objective: Patients with suspected acute aortic syndromes (AAS) often undergo computed tomography (CT) with negative results. We sought clinical and diagnostic criteria to identify low-risk patients, an initial step in developing a clinical decision rule., Methods: We retrospectively identified all adults presenting to our emergency department (ED) from January 1, 2006, to August 1, 2010, who underwent CT angiography for suspected AAS without prior trauma or AAS. A total of 1465 patients met inclusion criteria; a retrospective case-controlled review (ratio 1:4) was conducted. Cases were diagnosed with aortic dissection, intramural hematoma, penetrating atherosclerotic ulcer, or ruptured aneurysm., Results: Of the patients who underwent CT, 2.7% (40/1465) had an AAS; 2 additional cases were diagnosed after admission (ED miss rate, 5% [2/42]). Patients with AAS were significantly older than controls (66 vs 59 years; P = .008). Risk factors included abnormal chest radiograph (sensitivity, 79% [26/33]; specificity, 82% [113/137]) and acute chest pain (sensitivity, 83% [29/35]; specificity, 71% [111/157]). None of the 19 patients with resolved pain upon ED presentation had AAS. These data support a 2-step rule: first screen for ongoing pain; if present, screen for acute chest pain or an abnormal chest radiograph. This approach achieves a 54% (84/155) reduction in CT usage with a sensitivity for AAS of 96% (95% confidence interval, 89%-100%), negative predictive value of 99.8% (99.4%-100%), and a false-negative rate of 1.7% (1/84)., Conclusions: Our results demonstrate a need to safely identify patients at low risk for AAS who can forgo CT. We developed a preliminary 2-step clinical decision rule, which requires validation., (© 2013.)

Background: Descending thoracic acute aortic syndromes (DTAASs) are life-threatening pathologies in which thoracic endovascular aortic repair (TEVAR) is an attractive therapeutic option. There are few data in the literature on the survival and morbidity rates of TEVAR in such indications. We provide the results of TEVAR from our single-center cohort of DTAASs: ruptured aneurysms (RAs), acute symptomatic dissections (ADs), and traumatic ruptures (TRs)., Methods: Between 2004 and 2011, data from all patients treated by TEVAR for DTAAS in our university center were collected prospectively. Primary end points were rates of 30-day mortality and morbidity. Secondary end points were mid-term outcomes and reinterventions., Results: Forty-eight patients underwent TEVAR for DTAASs: 19 RAs (39.6%), 12 ADs (25.0%), and 17 TRs (35.4%). The cumulative 30-day mortality and morbidity rate was 33%. There were 10 deaths (6 RAs, 1 AD, and 3 TRs), 2 cases of paraplegia (0 postoperative and 2 preoperative cases (2 TRs), and no major reinterventions and 4 minor reinterventions (embolization for endoleak exclusion). Long-term outcomes were known in 33 patients (5 patients were lost to follow-up), with a mean duration follow-up of 27 months. The cumulative mortality and morbidity rate was 9%: no deaths were reported, and 2 major reinterventions (open aneurysm repair) and 1 minor reintervention (endoleak exclusion) were required., Conclusions: Our results confirm that TEVAR, as an emergency therapeutic option, is suited to DTAASs. The 21% perioperative mortality rate for such dramatic lesions remains high but is lower than medical or open repair therapeutic options previously reported. No additional deaths occurred during the 2-year follow-up period, and a low incidence of endograft-related reinterventions was observed. Nevertheless, scheduled follow-up visits with computed tomodensitometry scans on a regular basis are mandatory., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Four different aortic diseases are summarized under the term "acute aortic syndrome". Aortic dissection, contained aortic rupture, intramural aortic hematoma, and penetrating aortic ulcer. Therapeutic decision making depends on both the localization within the aorta and the presence of a complicated course. If the ascending aorta is involved surgical therapy has to be considered. In the presence of a complicated course of a disease of the descending aorta interventional or surgical therapy may be indicated. An interdisciplinary approach between Internal Medicine, Surgery, interventional Radiology, and anesthesiology is helpful., (© Georg Thieme Verlag KG Stuttgart · New York.)

Objective: An evaluation is presented on the clinical, pathological and sociodemographic characteristics of a sample of sudden death victims secondary to an Acute Aortic Syndrome (AAS) in the north of Portugal, submitted to medico-legal autopsy, during the period between January 2008 and December 2012., Methods: Autopsy reports were analyzed in which, through necropsy and histological examination, the confirmed cause of death was an Acute Aortic Syndrome., Results: From the studied cases, 70.3% of the victims were male. The median age was 65,19 ± 14,35 years (minimum of 27 years and maximum of 88 years). Death occurred mainly at home (40,5%) and during the normal activities of daily living (43,1%). There was an history of prodromes in 64,9% of the cases, mainly syncope and pain or respiratory symptoms. In 62,2% of the cases no pain history was reported. In the internal examination three presentation forms were identified: cardiac tamponade (51,4%); hemotorax (16,2%) and intra-abdominal bleeding (32,4%). The occurence of an Acute Aortic Syndrome was related to an aneurysmatic formation in 81% of the cases. Atherosclerotic plaques were identified in 51,4% of the cases and left ventricular hypertrophy was identified in 54,1%. None of the victims had a family history of aortic pathology and 54,1% of them presented a history of arterial hypertension., Conclusions: Necropsy data analysis of sudden death victims allows a better understanding of the AAS presentation, thus complementing the existent clinical studies. This work reveals how forensic medicine can be a privileged medium for articulation with clinical practice.

Neo-aortic arch obstruction (NAAO) is a common complication following the Norwood/Sano procedure (NP) for hypoplastic left heart syndrome (HLHS) and is associated with increased morbidity and mortality. However, there is currently no objective method for predicting which patients will develop NAAO. This study was designed to test the hypothesis that hemodynamic changes from development of NAAO after NP in patients with HLHS will lead to changes in myocardial dynamics that could be detected before clinical symptoms develop with strain analysis using velocity vector imaging. Patients with HLHS who had at least one cardiac catheterization after NP were identified retrospectively. Strain analysis was performed on all echocardiograms preceding the first catheterization and any subsequent catheterization performed for intervention on NAAO. Twelve patients developed NAAO and 30 patients never developed NAAO. Right ventricular strain was worse in the group that developed NAAO (-6.2 vs. -8.6 %, p = 0.040) at a median of 59 days prior to diagnosis of NAAO. Those patients that developed NAAO following NP were significantly younger at the time of first catheterization than those that did not develop NAAO (92 ± 50 vs. 140 ± 36 days, p = 0.001). This study demonstrates that right ventricular GLS is abnormal in HLHS patients following NP and worsening right ventricular strain may be predictive of the future development of NAAO.

Background: The standard operation for vascular ring with right aortic arch and aberrant left subclavian artery is ligamentum arteriosum division. A new surgical approach with primary translocation of the aberrant left subclavian artery to the left carotid artery, removal of the Kommerell diverticulum, and division of the ligamentum through left thoracotomy was recently applied. This study assessed the early outcomes of this approach., Methods: This is a retrospective review of all patients having an operation for right aortic arch with aberrant left subclavian artery and the Kommerell diverticulum between January 2001 and April 2011 at a single institution., Results: Eight patients had a division of the ligamentum and 10 had a primary translocation of an aberrant left subclavian artery with diverticulum removal and ligamentum division. The median operative age and weight for each surgical technique group were, respectively, 2.2 and 1.8 years (p = 0.56) and 10.7 and 12.6 kg (p = 0.30). All patients were symptomatic preoperatively. No deaths occurred. One patient from each group had chylous effusion that was medically treated. The median hospital stay for each group was 4 and 5 days (p = 0.45). During the median follow-up of 47 months, 3 patients in the division of ligamentum group required albuterol aerosol or oral antiinflammatory medication for respiratory symptoms. All patients in the primary translocation group remained asymptomatic and required no medication (p = 0.069)., Conclusions: Primary translocation of aberrant left subclavian artery with removal of the diverticulum and division of the ligamentum had excellent early outcomes and can potentially eliminate residual symptoms and late complications., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Congenital malformations of the thoracic aorta can be discovered on chest radiographs when associated with symptoms or found incidentally. We review the imaging anatomy and associations of many of the aortic arch malformations that can be encountered in adults and highlight key points with regard to their treatment and prognoses. An understanding of the normal and abnormal embryologic development of the aortic arch, with knowledge of their imaging features, may be important for improving diagnostic accuracy and patient care.

Vascular rings and pulmonary slings are congenital anomalies of the aortic arch/great vessels and pulmonary arteries, respectively, that commonly present early during infancy and childhood with respiratory and/or feeding difficulties. The diagnosis of these conditions frequently utilizes a multi-modality radiological approach, commonly utilizing some combination of radiography, esophagography, CT angiography and MR angiography. The purpose of this pictorial review is to illustrate the radiological findings of common and uncommon vascular rings and pulmonary slings in children using a state-of-the-art multi-modality imaging approach.

In this article the authors examine 4 diseases specific to thoracic surgery in children: patent ductus arteriosus, vascular rings, congenital tracheal stenosis, and pectus deformities. The pathophysiology, clinical presentation, and results of contemporary surgical management are discussed for each diagnosis. Surgical techniques are described and illustrated in detail., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Objectives: To analyse early and midterm results of thoracic aortic endografting (TEVAR) in the aortic arch., Methods: Between January 1997 and February 2009 178 patients received TEVAR in the aortic arch at our institution. This population was subdivided into four groups according to the proximal landing zone (LZ) classification in the aortic arch by Ishimaru et al. and a retrospective analysis regarding perioperative mortality, morbidity and endoleak formation was performed., Results: The overall 30-day mortality rate was 14% with no statistical significant difference between LZ's 0-3 (p=0.274). Renal insufficiency (hazard ratio (HR) 2.5; p=0.0119), age >75 years (HR 3.1; p=0.0019) and emergency procedures (HR 8.9; p < 0.0001) were independent predictors of death. There was no significant difference regarding type I (p=0.07) or type III (p=0.49) endoleaks between the proximal LZs, but a significant difference regarding the development of type II endoleaks (p=0.01)., Conclusions: The present study showed no influence of the proximal LZ on perioperative mortality and morbidity rate. Furthermore it did not influence relevant (type I/III) endoleak formation., (Copyright (c) 2010 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.)

The follow-up of medically treated acute aortic syndromes relies on CT and MR imaging. Comparison with prior examinations is essential. For aortic dissections, progressive enlargement of the false lumen, visceral hypoperfusion, and extension should be excluded. Mural hematomas and ulcers also undergo close follow-up to detect progression and recanalization. It is important to be familiar with the risk factors of disease progression for medically treated acute aortic syndromes and their management. It is also important to be familiar with the imaging features of disease progression. Acute aortic syndromes managed medically should undergo routine follow-up with CT or MR because these lesions may evolve silently over time and present with complications.