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Objective To analyse the main characteristics, associated conditions and outcome of right aortic arch (RAA) detected in fetal life, and to assess if further investigation is required in cases of isolated RAA. Methods Retrospective observational study of all fetuses diagnosed with a RAA between 2004 and 2012 at a tertiary centre for fetal cardiology. Results A RAA was identified in 98 fetuses: 27 had normal intracardiac anatomy and 71 were associated with other congenital heart disease (CHD); conotruncal anomalies being the most common. An aberrant left subclavian artery was diagnosed in 18.4% of cases, a double aortic arch in 6.1%, and 12.2% had a vascular ring confirmed after birth. Overall, an extracardiac anomaly was present in 31.6% of the patients and a chromosomal anomaly in 15.3%, with half of the latter cases being 22q11.2 microdeletion. Extracardiac and chromosomal anomalies were more commonly associated with RAA with structural CHD (39.4% and 19.7%, respectively), compared to cases of RAA with normal intracardiac anatomy (11.1% and 3.7%, respectively) (p<0.05). First year mortality was 10.3%, with all deaths being in cases with associated structural CHD. Conclusions Detailed fetal extracardiac examination should be undertaken in all cases of RAA. Isolated RAA has a good prognosis, and in the majority of the patients it is an asymptomatic vascular variant with a relatively low risk for chromosomal anomaly. The prognosis of RAA with CHD depends on the complexity of the CHD and/or the associated extracardiac anomalies. In these cases, there is a higher risk for chromosomal anomaly, particularly 22q11.2 microdeletion.

We aimed to assess the frequency of criss-cross pulmonary arteries and associated intracardiac and vascular anomalies in patients who underwent CT angiography due to suspected congenital heart disease or vascular anomaly at our hospital. We retrospectively evaluated the CT angiography images of 355 patients aged 0–18 years between April 2018 and December 2022. The presence of the criss-cross pulmonary artery anomaly was assessed. Additionally, in patients with a criss-cross pulmonary artery anomaly, accompanying branch pulmonary artery anomalies, aortic arch anomalies, and other vascular-cardiac anomalies were also evaluated. A total of 331 patients’ images were evaluated. Criss-cross pulmonary artery anomaly was present in 57 patients (17.2%). Pulmonary artery branch anomaly was present in 16, aortic arch anomaly in 40 patients (70%) with criss-cross pulmonary artery anomaly, while associated intracardiac pathology (by echocardiography) was detected in 43 patients (75.4%). The frequency of criss-cross pulmonary artery was found to be significantly higher in patients with any aortic arch anomaly ( p  = 0.01). This study represents one of the largest series of patients with criss-cross pulmonary artery anomalies. Our results suggests that it may be more common than previously recognized and potentially overlooked. It is crucial to consider the presence of this anomaly in patients with complex aortic arch anomalies or cardiac pathologies, as it may have implications for surgical approaches and potential complications. Increased awareness of this anomaly among cardiologists and radiologists is necessary for accurate diagnosis and appropriate management.

Objectives:To evaluate the use of MRI and FDG-PET for the diagnosis and measurement of disease activity of inflammatory aortic arch syndrome in patients with complicated giant cell arteritis.Methods:MRI and FDG-PET were performed for 25 patients with giant cell arteritis who presented with a complicated disease course despite immunosuppressive therapy. Disease activity of the thoracic aorta and the supra-aortic arteries as assessed by both modalities was compared with serological (C-reactive protein (CRP), erythrocyte sedimentation rate (ESR)) and clinical findings (Birmingham vasculitis activity score (BVAS.2)). Additionally, the usefulness of MRI for assessment of vessel wall thickening, aneurysms and stenoses was evaluated.Results:In 17/25 patients, MRI disclosed structural vessel lesions suspicious for vasculitis. Active disease was detected by MRI, thoracic PET, and whole body PET in 22, 14 and 20 patients, respectively. While serological and clinical findings correlated significantly with each other, there was no concordance with MRI and only low, non-significant correlation of PET with CRP (rs =  −0.158, 0.136), ESR (rs =  −0.232, 0.320) and BVAS.2 (rs =  −0.064, 0.221) for disease activity.Conclusions:MRI and PET are unreliable for assessing large-vessel inflammation in patients with giant cell arteritis and pre-existing immunosuppressive therapy. MRI is valuable for its ability to detect morphological vessel lesions, such as aneurysms and stenoses.

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.

The right aortic arch is a congenital vascular anomaly, which may form a vascular ring. However, prenatal identification of the branching pattern of brachiocephalic vessels is often limited. In this paper, we clearly demonstrated the branching pattern of brachiocephalic vessels in a case of right aortic arch with an aberrant left subclavian artery using HDlive Flow imaging.

The purpose of the study was to explore the prognosis, as well as antenatal ultrasonic features of isolated left subclavian artery (ILSCA) and isolated left brachiocephalic trunk (ILBCT) malformations, in order to improve prognosis and provide guidance for prenatal diagnosis. The origin and routing of cephalic and cervical vessels were observed in patients diagnosed with right aortic arch or right arterial duct arch in our hospital from March 2015 to March 2017, and the spectrum features related to ILSCA and ILBCT were analyzed. Fetuses diagnosed as ILSCA, or, and ILBCT were followed up for 3 months after birth. At the same time, a literature review was carried out for ILBCT and ILSCA in Pubmed. In our study, two cases with ILSCA and ILBCT were both diagnosed prenatally. They are not accompanied by other congenital malformations or chromosome abnormalities. No abnormality was found during postnatal follow-up except that left radial pulsation was weakened and blood pressure of the left upper limb decreased in baby with ILSCA. In baby with ILBCT, in addition to these abnormal changes, the left common carotid artery pulse disappearance too. In pubmed, three of 12 ILSCA or ILBCT did not have other congenital malformation or chromosome abnormalities. They were not diagnosed until the age of 3, 10, and 47 because of school exams or atypical symptoms, such as headaches, chest pain. Symptom of ILBCT or ILSCA without other abnormality is silent, and therefore they cannot be diagnosed timely after birth prenatal diagnosis is necessary for they can be treated in time.

Left aortic arch with right descending aorta is a rare congenital anomaly. We describe the clinical presentation of this unusual anomaly associated with cardiorespiratory compromise from severe aortic obstruction and left main bronchus compression. The anatomical peculiarities, embryological basis, and surgical solutions are presented.

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. 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. 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). 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.

In this report, a unique case of a symptomatic vascular ring formed by right aortic arch, aberrant left subclavian artery, and left ligamentum arteriosus in which there is atresia of the proximal left subclavian artery is described. Imaging modalities were non-diagnostic and the patient was sent to surgery based on strong clinical suspicion. Her anatomy was delineated in the operating room and the ring was successfully repaired.

Fifth arch anomalies are rare and complex and frequently misdiagnosed or mistaken for other entities. We report a double arch vascular ring that is thought to consist of right fourth arch and left fifth arch components, a previously undescribed persistent fifth arch variant. The currently recognized spectrum and classification of fifth arch vascular anomalies are expanded along with illustrative images to justify the proposed changes. Reviewing and expanding the classification of fifth arch anomalies to include a double arch ring variant will promote recognition, correct diagnosis and appropriate management of these anomalies.