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Journal of the American College of... Mar 2023An aberrant subclavian artery (ASA) (or lusoria) is the most common congenital anomaly of the aortic arch (0.5%-2.2%; female-to-male ratio 2:1 to 3:1). ASA can become...
BACKGROUND
An aberrant subclavian artery (ASA) (or lusoria) is the most common congenital anomaly of the aortic arch (0.5%-2.2%; female-to-male ratio 2:1 to 3:1). ASA can become aneurysmal and result in dissection, involving Kommerell's diverticulum when present and the aorta. Data of its significance in genetic arteriopathies are not available.
OBJECTIVES
The purpose of this study was to assess the prevalence and complications of ASA in gene-positive and -negative nonatherosclerotic arteriopathies.
MATERIALS
The series includes 1,418 consecutive patients with gene-positive (n = 854) and gene-negative arteriopathies (n = 564) diagnosed as part of institutional work-up for nonatherosclerotic syndromic and nonsyndromic arteriopathies. Comprehensive evaluation includes genetic counseling, next-generation sequencing multigene testing, cardiovascular and multidisciplinary assessment, and whole-body computed tomography angiography.
RESULTS
ASA was found in 34 of 1,418 cases (2.4%), with a similar prevalence in gene-positive (n = 21 of 854, 2.5%) and gene-negative (n = 13 of 564, 2.3%) arteriopathies. Of the former 21 patients, 14 had Marfan syndrome, 5 had Loeys-Dietz syndrome, 1 had type-IV Ehlers-Danlos syndrome, and 1 had periventricular heterotopia type 1. ASA did not segregate with genetic defects. Dissection occurred in 5 of 21 patients with genetic arteriopathies (23.8%; 2 Marfan syndrome and 3 Loeys-Dietz syndrome), all with associated Kommerell's diverticulum. No dissections occurred in gene-negative patients. At baseline, none of the 5 patients with ASA dissection fulfilled criteria for elective repair according to guidelines.
CONCLUSIONS
The risk of complications of ASA is higher in patients with genetic arteriopathies and is difficult to predict. In these diseases, imaging of the supra-aortic trunks should enter baseline investigations. Determination of precise indications for repair can prevent unexpected acute events such as those described.
Topics: Humans; Male; Female; Marfan Syndrome; Loeys-Dietz Syndrome; Prevalence; Vascular Diseases; Subclavian Artery; Heart Defects, Congenital; Aorta, Thoracic; Diverticulum
PubMed: 36889877
DOI: 10.1016/j.jacc.2023.01.005 -
Journal of Vascular Surgery Apr 2022An aberrant right subclavian artery (ARSA) is the most common congenital anomaly of the aortic arch. A paucity of reported studies is available regarding the treatment...
OBJECTIVE
An aberrant right subclavian artery (ARSA) is the most common congenital anomaly of the aortic arch. A paucity of reported studies is available regarding the treatment of these patients. The purpose of the present study was to evaluate the contemporary management strategies and natural history of ARSA in these patients.
METHODS
A single-center retrospective review of patients with a diagnosis of ARSA from 2009 to 2019 was performed. Computed tomography scans were analyzed, and the aortic and ARSA diameters were measured at 10 different segments. The demographic data, comorbidities, and operative interventions were collected. The patients were categorized into those who had undergone intervention and those who had undergone expectant management. Linear mixed effect models were used to estimate the annual ARSA diameter changes.
RESULTS
A total of 30 patients with ARSA were identified, 17 (57%) of whom were women. The average age for the cohort was 54.5 ± 14.6 years. Of the 30 patients, 20 (67%) had undergone operative repair at presentation and 10 (33%) were initially observed. The most common presenting symptom was dysphagia (30%). Of the 10 patients who had been initially treated expectantly, 4 had subsequently required intervention. Of the 24 operative interventions, 13 (54%) were hybrid procedures involving right carotid-subclavian bypass or transposition and thoracic endovascular aortic repair. The mean diameter of ARSA at its origin was 20.4 ± 5.7 mm, and the mean cross-sectional aortic diameter at the level of the ARSA was 31.8 ± 8.5 mm for the entire cohort. For the patients who had initially been observed and had subsequently required intervention, the largest change in the ARSA cross-sectional diameter was observed 1 cm distally to the vessel ostium at a rate of 3.05 mm annually (95% confidence interval, 1.54-4.56; P < .001). No statistically significant changes in the annual growth rate of the aortic segments were observed in the entire cohort or for those patients who had undergone intervention (P > .05).
CONCLUSIONS
The decision to intervene on an ARSA should be individualized by the presence of symptoms (eg, dysphagia lusoria) or complications (eg, dissection, concomitant aortic aneurysmal disease, enlarging Kommerell diverticulum). Asymptomatic patients with nonaneurysmal ARSA might not require any intervention and can be safely observed. Measurement of the cross-sectional ARSA diameter 1 cm distally to the ostium of the vessel might aid in the surveillance of vessel diameter changes. Additional studies are required to determine the specific size criteria as an indication for operative repair of asymptomatic Kommerell diverticulum.
Topics: Adult; Aged; Aorta, Thoracic; Blood Vessel Prosthesis Implantation; Cardiovascular Abnormalities; Deglutition Disorders; Diverticulum; Endovascular Procedures; Female; Humans; Male; Middle Aged; Subclavian Artery; Treatment Outcome
PubMed: 34838611
DOI: 10.1016/j.jvs.2021.11.051 -
Journal of Vascular Surgery May 2023Aberrant subclavian artery (ASA) and Kommerell's diverticulum (KD) are rare vascular anomalies that may be associated with lifestyle-limiting and life-threatening...
OBJECTIVE
Aberrant subclavian artery (ASA) and Kommerell's diverticulum (KD) are rare vascular anomalies that may be associated with lifestyle-limiting and life-threatening complications. The aim of this study is to report contemporary outcomes after invasive treatment of ASA/KD using a large international dataset.
METHODS
Patients who underwent treatment for ASA/KD (2000-2020) were identified through the Vascular Low Frequency Disease Consortium, a multi-institutional collaboration to investigate uncommon vascular disorders. We report the early and mid-term clinical outcomes including stroke and mortality, technical success, and other operative outcomes including reintervention rates, patency, and endoleak.
RESULTS
Overall, 285 patients were identified during the study period. The mean patient age was 57 years; 47% were female and 68% presented with symptoms. A right-sided arch was present in 23%. The mean KD diameter was 47.4 mm (range, 13.0-108.0 mm). The most common indication for treatment was symptoms (59%), followed by aneurysm size (38%). The most common symptom reported was dysphagia (44%). A ruptured KD was treated in 4.2% of cases, with a mean diameter of 43.9 mm (range, 18.0-100.0 mm). An open procedure was performed in 101 cases (36%); the most common approach was ASA ligation with subclavian transposition. An endovascular or hybrid approach was performed in 184 patients (64%); the most common approach was thoracic endograft and carotid-subclavian bypass. A staged operative strategy was employed more often than single setting repair (55% vs 45%). Compared with endovascular or hybrid approach, those in the open procedure group were more likely to be younger (49 years vs 61 years; P < .0001), female (64% vs 36%; P < .0001), and symptomatic (85% vs 59%; P < .0001). Complete or partial symptomatic relief at 1 year after intervention was 82.6%. There was no association between modality of treatment and symptom relief (open 87.2% vs endovascular or hybrid approach 78.9%; P = .13). After the intervention, 11 subclavian occlusions (4.5%) occurred; 3 were successfully thrombectomized resulting in a primary and secondary patency of 95% and 96%, respectively, at a median follow-up of 39 months. Among the 33 reinterventions (12%), the majority were performed for endoleak (36%), and more reinterventions occurred in the endovascular or hybrid approach than open procedure group (15% vs 6%; P = .02). The overall survival rate was 87.3% at a median follow-up of 41 months. The 30-day stroke and death rates were 4.2% and 4.9%, respectively. Urgent or emergent presentation was independently associated with increased risk of 30-day mortality (odds ratio [OR], 19.8; 95% confidence interval [CI], 3.3-116.6), overall mortality (OR, 3.6; 95% CI, 1.2-11.2) and intraoperative complications (OR, 8.3; 95% CI, 2.8-25.1). Females had a higher risk of reintervention (OR, 2.6; 95% CI, 1.0-6.5). At an aneurysm size of 44.4 mm, receiver operator characteristic curve analysis suggested that 60% of patients would have symptoms.
CONCLUSIONS
Treatment of ASA/KD can be performed safely with low rates of mortality, stroke and reintervention and high rates of symptomatic relief, regardless of the repair strategy. Symptomatic and urgent operations were associated with worse outcomes in general, and female gender was associated with a higher likelihood of reintervention. Given the worse overall outcomes when symptomatic and the inherent risk of rupture, consideration of repair at 40 mm is reasonable in most patients. ASA/KD can be repaired in asymptomatic patients with excellent outcomes and young healthy patients may be considered better candidates for open approaches versus endovascular or hybrid modalities, given the lower likelihood of reintervention and lower early mortality rate.
Topics: Humans; Female; Middle Aged; Male; Endoleak; Aneurysm; Subclavian Artery; Endovascular Procedures; Stroke; Diverticulum; Aorta, Thoracic; Treatment Outcome; Blood Vessel Prosthesis Implantation
PubMed: 36657501
DOI: 10.1016/j.jvs.2023.01.014 -
Journal of Vascular Surgery Jan 2022
Topics: Aneurysm, False; Arabidopsis Proteins; Computed Tomography Angiography; Debridement; Echocardiography; Female; Ferredoxin-NADP Reductase; Humans; Middle Aged; Subclavian Artery
PubMed: 34949384
DOI: 10.1016/j.jvs.2020.12.101 -
Vascular Health and Risk Management 2021Traumatic subclavian artery injuries are associated with high morbidity and mortality. Thoracic cage and clavicle provide a well protection of the underlying subclavian...
BACKGROUND
Traumatic subclavian artery injuries are associated with high morbidity and mortality. Thoracic cage and clavicle provide a well protection of the underlying subclavian vessels and nerves and also cause a very limited operation space during open surgery. The endovascular modality is less invasive and alternative to conventional open surgical reconstruction.
PURPOSE
The purpose of this study was to analyze the different therapeutic effects on limb salvage.
METHODS
A retrospective review of patients who presented with blunt or penetrating injuries to the subclavian arteries between March 2012 and March 2021.
RESULTS
Endovascular and open repairs were both effective for traumatic subclavian artery injury. There was no statistical difference in the limb salvage, mortality, procedure-related complication, reintervention rate and in-hospital medical complications. Intraoperative blood loss, red blood cell transfusion requirement and length of hospital stay were significantly lower in the endovascular intervention group.
CONCLUSION
Endovascular treatment represents an attractive alternative to the traditional surgical approach for the treatment of traumatic injuries in the subclavian.
Topics: Adult; Aneurysm, False; Blood Vessel Prosthesis Implantation; Endovascular Procedures; Humans; Male; Middle Aged; Retrospective Studies; Stents; Subclavian Artery; Treatment Outcome; Vascular System Injuries; Wounds, Nonpenetrating
PubMed: 34429608
DOI: 10.2147/VHRM.S322127 -
Surgical and Radiologic Anatomy : SRA Sep 2021A rare branching pattern of the aortic arch in a female cadaver is reported. An aberrant right subclavian artery originated from the distal part of the aortic arch and...
A rare branching pattern of the aortic arch in a female cadaver is reported. An aberrant right subclavian artery originated from the distal part of the aortic arch and following a retroesophageal course was recognized. Next to it, from the left to the right, the left subclavian artery and a short bicarotid trunk originating the left and the right common carotid artery were recognized. An unusual origin of the vertebral arteries was also identified. The left vertebral artery originated directly from the aortic arch, whereas the right vertebral artery originated directly from the right common carotid artery. Retroesophageal right subclavian artery associated with a bicarotid trunk and ectopic origin of vertebral arteries represents an exceptional and noteworthy case.
Topics: Cadaver; Cardiovascular Abnormalities; Carotid Arteries; Female; Humans; Subclavian Artery; Vertebral Artery
PubMed: 33856505
DOI: 10.1007/s00276-021-02746-1 -
Ultrasound in Obstetrics & Gynecology :... Sep 2015The primary objective was to estimate the prevalence of aberrant right subclavian artery (ARSA) in fetuses with Down syndrome. Secondary objectives were to assess the... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVES
The primary objective was to estimate the prevalence of aberrant right subclavian artery (ARSA) in fetuses with Down syndrome. Secondary objectives were to assess the prevalence of ARSA in euploid fetuses, the feasibility of ultrasound evaluation of the right subclavian artery (RSA) in the first and second trimesters of pregnancy, the performance of ARSA in screening for trisomy 21 and its association with other abnormalities.
METHODS
Web-based databases (PubMed, EMBASE and MEDLINE) were searched up to July 2014. The STROBE, PRISMA and QUIPS instruments were used to assess all included studies and for reporting of methodology, results and conclusions. Original studies that reported prenatal ultrasound evaluation of ARSA, assessment of its prevalence in Down-syndrome and euploid fetuses, feasibility of ultrasound evaluation of the RSA in the first and second trimesters of pregnancy and correlation of ARSA with other abnormalities were included, excluding duplications and case reports. Collected data were summarized to estimate prevalence and feasibility. A meta-analysis was performed pooling the study-specific positive and negative likelihood ratios (LR+ and LR-), detection rates and false-positive rates for trisomy 21.
RESULTS
Prevalence of ARSA in Down-syndrome fetuses was 23.6% (95% CI, 19.4-27.9%), whereas in euploid fetuses it was 1.02% (95% CI, 0.86-1.10%). Ultrasound evaluation of the RSA course and origin in the first and second trimesters of pregnancy was feasible in 85% and 98% of cases (first and second trimester, respectively) and it was directly related to sonographic experience and fetal crown-rump length and inversely related to maternal body mass index. In more than 20% of fetuses with ARSA there was an association with other abnormalities but ARSA seemed to be an independent marker of trisomy 21. The meta-analysis showed that ARSA is a significant risk factor for Down syndrome (pooled LR+ = 26.93, 95% CI, 19.36-37.47, P for effect < 0.001, P for Q = 0.3, I(2) = 17.3%), whereas normal RSA is a significant protective marker (pooled LR- = 0.71, 95% CI, 0.51-0.99, P for effect = 0.043, P for Q = 0.9, I(2) = 0%).
CONCLUSIONS
ARSA appears to be a clinically useful prenatal ultrasound marker of Down syndrome. Additional testing when ARSA is diagnosed should involve evaluation of all risk factors by applying a mathematical model. There is insufficient evidence to recommend fetal karyotyping in cases with isolated ARSA. If the background risk is higher or additional markers are present, full fetal karyotyping is advisable, including analysis for 22q11 microdeletion.
Topics: Aneurysm; Cardiovascular Abnormalities; Deglutition Disorders; Down Syndrome; Female; Humans; Models, Statistical; Pregnancy; Pregnancy Trimester, First; Pregnancy Trimester, Second; Prevalence; Subclavian Artery; Ultrasonography, Prenatal
PubMed: 25586729
DOI: 10.1002/uog.14774 -
Neurologia Medico-chirurgica Jun 2023Various surgical treatments are available for occlusive subclavian and common carotid artery diseases. Nevertheless, to date, when cerebral endovascular treatment is...
Various surgical treatments are available for occlusive subclavian and common carotid artery diseases. Nevertheless, to date, when cerebral endovascular treatment is utilized, revascularization via direct surgery may be required. This study reported five symptomatic cases of revascularization for CCA and SCA occlusive and stenotic lesions that were expected to be challenging to treat with endovascular treatment. We performed subclavian artery-common carotid artery or internal carotid artery bypass using artificial blood vessels or saphenous vein grafts in five patients with subclavian steal syndrome, symptomatic common carotid artery occlusion, and severe proximal common carotid artery stenosis. In this study, good bypass patency was achieved in all five cases. Although there were no intraoperative complications, one patient had a postoperative lymphatic leak. Moreover, there was no recurrence of stroke during postoperative follow-up for an average of 2 years. Conclusively, subclavian artery-common carotid artery bypass can be an effective surgical treatment for common carotid artery occlusion, proximal common carotid artery stenosis, and subclavian artery occlusion.
Topics: Humans; Subclavian Artery; Carotid Stenosis; Constriction, Pathologic; Carotid Artery, Common; Carotid Artery, Internal; Carotid Artery Diseases; Thrombosis; Subclavian Steal Syndrome
PubMed: 37005246
DOI: 10.2176/jns-nmc.2022-0307 -
European Journal of Vascular and... May 2022To synthesise and present all available evidence regarding the surgical treatment of patients with aberrant subclavian artery (aSA) pathologies during the last 30 years.... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To synthesise and present all available evidence regarding the surgical treatment of patients with aberrant subclavian artery (aSA) pathologies during the last 30 years. The study also aimed to create a checklist of suggested reporting items to help increase reporting homogeneity.
METHODS
A systematic search of medical databases was performed to identify all studies published between 1990 and 2020 reporting on the outcomes of patients undergoing surgery for an aSA pathology. Patients were divided into two groups; Group A included patients undergoing open or hybrid surgery through sternotomy/thoracotomy and Group B included patients undergoing endovascular or hybrid treatment without chest access. The % crude rates (CR) of all variables of interest were calculated. A proportion meta-analysis was performed reporting pooled rates with 95% confidence intervals (CIs).
RESULTS
Three hundred and fourteen studies were identified reporting on the surgical outcomes of 732 patients (60.1% males; 440/732). The quality of evidence was generally low, with 286 studies including fewer than four patients and 28 studies with five or more cases. Aberrant right subclavian artery (aRSA) in a left sided aortic arch was present in 71.4% of the patients and a Kommerell's diverticulum was present in 50.1% of the cases. A total of 68% of the patients were symptomatic on presentation, with the majority complaining of dysphagia (49.6%). Group A included 453 and Group B 279 patients. Studies reporting on five or more patients were included in the main meta-analysis. The pooled early mortality rate was 1.62% (95% CI 0.05% - 4.53%) in Group A and 1.96% (95% CI 0 - 6.34%) in Group B. Pooled rates of symptom relief were 99.52% (95% CI 92.05% - 100.00%) in Group A and 95.79% (95% CI 83.96% - 100.00%) in Group B.
CONCLUSION
The surgical techniques used to treat aSA and aortic pathologies involving an aSA had remarkably low mortality rates and high clinical success, regardless of the technique used.
Topics: Aorta, Thoracic; Cardiovascular Abnormalities; Diverticulum; Female; Humans; Male; Subclavian Artery
PubMed: 35459610
DOI: 10.1016/j.ejvs.2022.02.027 -
Interactive Cardiovascular and Thoracic... Jan 2021To assess the safety and efficacy of the translocation of the aberrant left subclavian artery (LSCA) and resection of the Kommerell diverticulum during the concomitant...
OBJECTIVES
To assess the safety and efficacy of the translocation of the aberrant left subclavian artery (LSCA) and resection of the Kommerell diverticulum during the concomitant repair of intracardiac anomalies for paediatric patients who had a right-sided aortic arch.
METHODS
A retrospective review of paediatric patients who were diagnosed right-sided aortic arch, aberrant LSCA, Kommerell diverticulum and intracardiac anomalies between 2015 and 2019 was conducted. Patients who underwent translocation of the aberrant LSCA, diverticulum resection and concomitant intracardiac repair were included.
RESULTS
Eight patients underwent translocation of aberrant LSCA, diverticulum resection, ligamentum division and concomitant repair of the associated intracardiac anomalies. All patients were male. The median age was 1.3 years (range 0.4-5.5 years) and the median weight was 10.0 kg (range 6.1-21.0 kg). The most commonly combined intracardiac anomaly was a ventricular septal defect. Seven patients (87.5%) had preoperative respiratory or gastrointestinal symptoms. There was no early mortality and no postoperative complications. During the median follow-up of 23 months (range 4-43 months), no patient had residual respiratory or gastrointestinal symptoms. A postoperative computed tomography scan was performed in 3 patients, all of which showed patent LSCA-left carotid artery anastomosis.
CONCLUSIONS
Translocation of the aberrant LSCA and resection of the Kommerell diverticulum can be safely performed during the concomitant repair of intracardiac anomalies for paediatric patients. This approach could eliminate residual respiratory and gastrointestinal symptoms, and prevent reintervention in the future.
Topics: Cardiovascular Abnormalities; Child, Preschool; Diverticulum; Heart Defects, Congenital; Humans; Infant; Male; Postoperative Care; Retrospective Studies; Subclavian Artery; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 33221838
DOI: 10.1093/icvts/ivaa226