Fibromuscular dysplasia of subclavian artery: A case report and mini-review.

A case of a 40-year-old male patient with a right subclavian artery aneurysm of fibromuscular dysplasia origin is reported. The patient presented with thoracic outlet-like symptoms and underwent aneurysm resection. Microscopic examination revealed intimal and medial fibroplasia. Additional cases of fibromuscular dysplasia at this rare location are reviewed, indicating a male and right-sided predominance. The most frequent clinicopathological manifestation was an aneurysm, with the histopathological pattern characterized by medial fibroplasia. Treatment modalities included the use of either graft prosthesis or end-to-end anastomosis.

Modified transmanubrial approach for complicated type 1 cervical rib resection requiring subclavian artery reconstruction.

The transmanubrial musculoskeletal sparing approach (TMA) is commonly used for resecting apical lung tumours with vascular involvement. Non-neoplastic conditions which might require surgical exploration of the thoracic outlet include the 'cervical rib', a clinical condition consisting of an additional rib forming above the first rib and growing from the base of the neck just above the clavicle. Type 1 cervical rib-when a complete cervical rib articulates with the first rib or manubrium of the sternum-is the most challenging scenario where the subclavian artery can be damaged by continuous compression due to the narrow space between clavicle, first rib and supernumerary cervical rib, requiring prosthetic reconstruction of the involved tract. Here, we describe a modified TMA in which the incision in the neck is conducted posteriorly to the sternocleidomastoid muscle, thus allowing safe dissection of the superior and middle trunk of the brachial plexus.

Brachial and subclavian arteries aneurysms due to tuberous sclerosis complex mechanisms - case report and literature review.

INTRODUCTION: Tuberous sclerosis complex (TSC) is a rare autosomal dominant condition characterized by cutaneous, cerebral, and other multiorgan involvement. Aneurysms due to TSC pathogenic mechanism are rarely present, mainly aortic, renal, or intracranial and very few associated with peripheral circulation. A TSC patient, aged 31 years, who developed brachial and subclavian arteries aneurysms is presented. The question of a random association of the aneurysms with TSC versus aneurysms within pathogenic released mammalian target of rapamycin (mTOR) pathway effect was raised. CASE PRESENTATION: Patient's file, available from the age of six months, was analyzed for demonstration of the TSC diagnosis. Patient was examined, and cerebral magnetic resonance imaging (MRI) was repeated. Surgery and angiographic reports and images were reviewed. Pathology of the aneurysmal wall available from surgery was reexamined and special stainings and immunohistochemistry markers were applied. Genetic characterization of the patient was performed. Definite TSC was diagnosed based on major criteria [ungual fibromas, shagreen patch, cortical tubers, subependymal nodules (SENs), subependymal giant cell astrocytoma (SEGA)], minor criteria (confetti skin lesions, dental enamel pits, gingival fibromas), genetic result showing heterozygous variant in exon 8 of TSC1 gene (c.733C>T-p.Arg245*). Pathology analysis revealed markedly thickened aneurysmal wall due to smooth muscle cells (SMCs) proliferation in media and neoformation vessels with similar characteristics in the aneurysmal wall. DISCUSSIONS AND CONCLUSIONS: This is a rare case with aneurysms related to TSC, with an exceptional peripheral localization. Pathology exam is the key investigation in demonstrating the TSC-related pathogenic mechanism. A literature review showed 73 TSC cases presenting aneurysms published until now.

Machine learning for detection of stenoses and aneurysms: application in a physiologically realistic virtual patient database.

This study presents an application of machine learning (ML) methods for detecting the presence of stenoses and aneurysms in the human arterial system. Four major forms of arterial disease-carotid artery stenosis (CAS), subclavian artery stenosis (SAS), peripheral arterial disease (PAD), and abdominal aortic aneurysms (AAA)-are considered. The ML methods are trained and tested on a physiologically realistic virtual patient database (VPD) containing 28,868 healthy subjects, adapted from the authors previous work and augmented to include disease. It is found that the tree-based methods of Random Forest and Gradient Boosting outperform other approaches. The performance of ML methods is quantified through the [Formula: see text] score and computation of sensitivities and specificities. When using six haemodynamic measurements (pressure in the common carotid, brachial, and radial arteries; and flow-rate in the common carotid, brachial, and femoral arteries), it is found that maximum [Formula: see text] scores larger than 0.9 are achieved for CAS and PAD, larger than 0.85 for SAS, and larger than 0.98 for both low- and high-severity AAAs. Corresponding sensitivities and specificities are larger than 90% for CAS and PAD, larger than 85% for SAS, and larger than 98% for both low- and high-severity AAAs. When reducing the number of measurements, performance is degraded by less than 5% when three measurements are used, and less than 10% when only two measurements are used for classification. For AAA, it is shown that [Formula: see text] scores larger than 0.85 and corresponding sensitivities and specificities larger than 85% are achievable when using only a single measurement. The results are encouraging to pursue AAA monitoring and screening through wearable devices which can reliably measure pressure or flow-rates.

Extent and progression of atherosclerosis in carotid and subclavian arteries: the Carotid Artery Subclavian Artery study.

AIMS: To define the prevalence, progression, and the relationship between carotid and subclavian artery atherosclerosis and to identify factors associated with disease progression in a population of asymptomatic patients. METHODS: Among all consecutive patients without a history of cardiovascular disease admitted to our hospital for duplex ultrasound examinations of the supra-aortic arteries, from January to December 2012, we retrospectively identified 530 patients with two evaluations at least 3 years apart. Each artery was graded according to stenosis degree, as absent or less than 20%, 20-49%, 50-69%, 70-99% and total occlusion. Disease progression was defined for any class increase at any time interval. Patients were grouped according to the presence of a more than 20% stenosis of the supra-aortic district at baseline, as controls, without atherosclerosis: n = 111, 21%; isolated carotid artery disease: n = 390, 74%; concomitant subclavian artery-carotid artery disease: n = 29, 5%. There were no cases with isolated subclavian artery atherosclerosis. RESULTS: The mean time-lapse between the two evaluations was 3.1 ±â€Š0.3 years; we documented disease progression in 32 patients (6%), all limited to the carotid artery (P = 0.009 vs. controls, with no differences between isolated carotid artery disease and concomitant carotid and subclavian artery disease). Hypertension was significantly (P < 0.001) associated with disease progression, regardless of the single or double district involvement. CONCLUSION: The subclavian artery is far less prone to atherosclerosis than the carotid artery, and features lesser disease progression. Understanding factors for the different susceptibility to atherosclerosis in these two close arterial districts provides insight into local factors prompting vascular disease.

Midterm Outcomes of Zone 0 Antegrade Endograft Implantation During Type I Hybrid Aortic Arch Repair.

OBJECTIVE: Type I hybrid arch repair has become popular as a procedure that is less invasive than total arch replacement. The major advantage of this technique is that antegrade endograft implantation can be performed during the procedure, thereby avoiding the complications of introducing the endograft from the groin. The aim of this study was to assess the midterm outcomes of type I hybrid aortic arch repair with antegrade endograft implantation. METHODS: Thirty consecutive patients who underwent type I hybrid repair with antegrade endograft implantation from 2009 to 2015 were reviewed retrospectively. Patient demographics, and peri-operative and late results were collected from a prospective database and analysed. RESULTS: Four patients (13%) were female and the median age was 78 years. Median aneurysm size was 64 mm. Six patients (20%) developed stroke, and the 30 day mortality rate was 3%. Two patients suffered aortic dissection at the site of debranching anastomosis. The median follow up was 5.2 years. All aneurysms remained stable or had decreased in size at three years, and 82% were stable at five years. Overall survival was 79% at three years and 71% at five years. The rates of freedom from aorta related death were 86% at three and five years, respectively. During the follow up period, three additional left subclavian artery embolisations and one endograft relining due to type IIIb endoleak were required. CONCLUSION: Midterm outcomes of type I hybrid aortic arch repair with antegrade endograft implantation for aortic arch aneurysms are reported. Although the incidence of peri-operative stroke was high, late sac behaviour was acceptable.

Systolic murmur in disguise: subclavian artery stenosis as an overlooked cause of missed case of hypertension.

In this report we present a case of missed hypertension due to subclavian artery stenosis. A 77 year-old female patient, initially thought as being normotensive, was referred to us due to newly discovered systolic heart murmur suspicious for aortic stenosis. We noted inter-arm blood pressure difference of 30 mmHg, with higher, hypertensive values on right arm. Further workup and medical imaging excluded aortic stenosis and revealed an asymptomatic, hemodynamically significant, stenosis of left subclavian artery. Due to absence of symptoms, the patient has been managed with conservative therapy for subclavian stenosis and hypertension, and she is currently in good conditions and followed up for any signs of disease progression. This case clearly shows importance of measuring blood pressure on both arms when initially diagnosing hypertension as this is often overlooked and is key to properly diagnose hypertension and possible subclavian stenosis.

Focal Liver 18F-FDG Uptake in a Patient Who Underwent Thrombolysis for Brachiocephalic and Subclavian Thrombosis: A Potential Pitfall.

ABSTRACT: A 19-year-old woman presented with a primary mediastinal B-cell lymphoma invading the superior vena cava with associated thrombosis of the left brachiocephalic and subclavian vein. She underwent thrombolysis followed by chemotherapy. The midtreatment 18F-FDG PET/CT demonstrated important regression of the primary mediastinal B-cell lymphoma, but showed intense focal hepatic uptake in segment IV, without a corresponding lesion on ultrasonography, non-contrast-enhanced low-dose CT, and MRI. This focal uptake disappeared on a subsequent 18F-FDG PET/CT study when the radiotracer was injected in the foot, suggesting an anomalous venous return pathway that persisted despite thrombolysis.

First MDCT evidence of ruptured aberrant left subclavian artery aneurysm in right aortic arch, Kommerell's diverticulum and extrapleural hematoma treated by emergency thoracic endovascular aortic repair.

Spontaneous ruptured aneurysm involving an aberrant subclavian artery with a right-sided aortic arch and Kommerell's diverticulumis a rare life-threatening condition that can be treated successfully if promptly identified. Multidetector Computed Tomography angiography is the first line imaging modality of thoracic vascular anomalies diagnosis. We report the case of a 74-year-old man suffering from this emergency ondition with mediastinal hematoma mostly extending to the left-side extrapleural cavity. The patient underwent successful emergency thoracic endovascular aortic repair and an Amplatzer vascular plug was placed into the first segment of the ALSA. Post-procedural imaging showed complete exclusion of the aneurysm. Emergency endovascular repair can be effective in such cases.

Separate arch origin of the left external carotid artery with common trunk giving rise to the left internal carotid artery and left subclavian artery.

We present a case of a 3-year-old boy with tetralogy of Fallot having the direct origin of the left external carotid artery from the aorta with a common trunk giving rise to the left internal carotid artery and left subclavian artery, in a right-sided aortic arch. We also highlight the potential implications in management.

LACHT syndrome (Mardini-Nyhan association) with tracheal stenosis in a Thai newborn.

LACHT syndrome, or Mardini-Nyhan association, is an ultra-rare disorder, diagnosed solely by the clinical characteristics of lung agenesis, complex cardiac defects, and thumb anomalies. Only 12 patients have been reported worldwide, and here, we report a new clinical diagnosis of LACHT syndrome. Our patient was a male full-term newborn with left lung agenesis, congenital heart defects including ventricular septal defect, right-sided aortic arch, with aberrant left subclavian artery and Kommerell diverticulum, as well as left preaxial polydactyly and hemivertebra. Our patient appears to be the second LACHT syndrome case to also suffer from tracheal stenosis, which has only been reported once before in conjunction with this syndrome. In light of this, tracheal stenosis may be a phenotype for LACHT syndrome.

Infrequent origin of a peripheral embolism: Arterial thoracic outlet syndrome in a young woman.

Arterial thoracic outlet syndrome (TOS) is caused by the compression of the subclavian or axillary artery between the ribs and the scalene muscles. Patients may present with various clinical conditions. Herein, we report a case of arterial TOS who presented with peripheral embolism. Arterial TOS is an uncommon cause of peripheral embolism. The diagnostic approach and treatment methods used are discussed. Clinicians should keep this diagnosis in mind and manage these cases according to the clinical appearance.

Vascular ring anomalies in cats: 20 cases (2000-2018).

OBJECTIVE: To report the clinical characteristics, types of vascular ring anomalies (VRA), operative findings, complications, and survival after surgical treatment of cats with VRA. STUDY DESIGN: Retrospective, multi-institutional case series. ANIMALS: Client- or shelter-owned cats presenting to academic, referral veterinary institutions. METHODS: Medical records of cats with VRA that underwent surgical treatment were reviewed. Signalment, relevant medical history, clinical signs, diagnostic imaging, surgical findings, complications, and survival were recorded. RESULTS: Twenty cats with VRA were included. Vascular ring anomalies were most commonly (75% [15/20]) diagnosed in cats less than 1 year old, with no breed or sex predilection. Regurgitation was the most common clinical sign, present in 18 of 20 (90%) cats. A persistent right aortic arch was diagnosed in 17 of 20 (85%) cats, with concurrent aberrant left subclavian artery in four of the cats. Surgical treatment was associated with survival to discharge in 18 of 20 (90%) cats. Persistent clinical signs were reported in nine of 13 (69%) cats, and radiographic evidence of megaesophagus persisted in four of 13 (31%) cats, with a median follow-up of 275 days after discharge. CONCLUSION: Persistent right aortic arch was the most commonly diagnosed VRA in cats in this series, although multiple anomalies were observed. Surgical treatment of VRA in cats was associated with a high survival to discharge, although persistence of clinical signs and megaesophagus was noted in 69% and 31% of the cats, respectively. CLINICAL SIGNIFICANCE: Surgical treatment of VRA in cats is associated with a high survival rate; however, persistence of clinical signs is an expected outcome.