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Transradial access (TRA) has become increasingly utilized in neurointerventions because it reduces access site complications. However, radial artery anomalies can be difficult to navigate, often necessitating conversion to femoral access. We describe the case of a female patient in her early 70s who underwent preoperative embolization of a carotid body tumor via right TRA. Her radial angiogram demonstrated the presence of a radial artery loop which was successfully navigated with a triaxial system but would not spontaneously reduce, even after the guide catheter was advanced into the subclavian artery. However, manual manipulation of the catheters in the antecubital fossa under direct fluoroscopic visualization reduced the loop, allowing the procedure to continue transradially. Although most radial loops can be traversed and reduced using standard techniques, this case demonstrates that manual reduction can be successful when other measures fail. We recommend attempting this method prior to converting the access site.

Various surgical options have been described for the treatment of aberrant subclavian arteries and an associated Kommerell diverticulum.Between 1999 and 2019, 43 patients underwent a repair, comprising 26 (61%) endovascular and 17 (39%) open approaches. The endovascular approach consisted of initial subclavian revascularization followed by thoracic endovascular aortic repair. The open approach included total arch replacement (12%) and reverse hemiarch repair with left thoracotomy (53%) or right thoracotomy (35%). The perioperative and long-term outcomes were retrospectively reviewed.No mortality occurred in the endovascular group, whereas there was 1 (6%) in the open approach group. Patients in the endovascular group demonstrated a shorter hospital stay (3.5 days vs 10.0 days; P = .001) and less frequent prolonged mechanical ventilation (0% vs 24%; P = .019), with a lower occurrence of pneumonia (0% vs 24%; P = .019). Among patients who had the endovascular approach, shrinkage of Kommerell diverticulum or aberrant vessel origin was seen in 96%. Furthermore, relief of dysphagia was confirmed in 92% (12/13), including patients without Kommerell diverticulum (n = 3) after endovascular repair. The cumulative incidence of treatment failure or aortic reintervention at 7 years was 21% and 14 % in the endovascular and open approach groups, respectively (P = .62). Two (8%) patients in the endovascular group required an open reintervention. One reintervention was performed for persistent dysphagia in the setting of an untreated complete vascular ring, and the other was for persistent false lumen flow associated with aortic dissection.The treatment approach should be individualized on the basis of the aortic disease and comorbidities. The endovascular approach is a viable and effective alternative in the presence of suitable landing zones.

Kommerell diverticulum (KD) is a dilated proximal aberrant right or left subclavian artery associated with either right or left aortic arches (RAA-ARSA or LAA-ALSA). Although case series suggest that KD may be a liability for vascular complications, the risk, pattern of dilation throughout the life span, and differences between arch sides are not known.This study was a single-center retrospective review of patients of all ages with KD on cross-sectional imaging. Maximal short-axis diameter of KD (KDmax), absolute and indexed to descending aortic diameter (DAo), was correlated with age. Comparisons were made between arch sides. Patients with vascular complications were described.A total of 104 patients with KD were included: 68 (65%) with RAA-ALSA, 36 (35%) with LAA-ARSA, 43 (41%) asymptomatic. Although KDmax was correlated with age (RAA-ALSA r = 0.84; [P.0001]; LAA-ARSA r = 0.51 [P = .001]), KDmax indexed to DAo was not (RAA-ALSA r = 0.14 [P = .27]; LAA-ARSA r = -0.22 [P = .21]). Patients with RAA-ALSA had larger KDmax indexed to DAo (1.02 ± 0.20 mm/mm vs 0.89 ± 0.18 mm/mm; P = .002) and more symptoms (75% vs 28%; P.0001), and they were younger (median, 9.5 years vs 61.7 years; P.0001). Six patients (58 to 80 years of age) had vascular complications, and all 6 had LAA-ARSA and risk factors for acquired aneurysms.In older patients, KDmax indexed to DAo was not larger, thus arguing against isolated KD dilation with age. Diverticula from RAA-ALSA and LAA-ARSA demonstrated different phenotypes, a finding suggesting different disease processes and likely different risk. The incidence of vascular complications was lower than in previous reports, and these complications occurred exclusively in patients with LAA-ARSA and aneurysm risk factors. This finding suggests that conservative management of asymptomatic KD is often reasonable, especially in patients with RAA-ALSA.

We present a 54-year-old Caucasian woman, who presented with acute symptomatic type B aortic dissection with deteriorating renal function. She was a known smoker with a 2-year history of dysphagia. CT angiography documented the artery of lusoria arising from the mid-thoracic aorta, aneurysmal dilation of her descending aorta, and kinetic and static flaps around her visceral ostia. The patient was managed by staged hybrid single lumen reconstruction and bilateral subclavian to carotid transpositions. During follow-up, there was no aortic rupture or retrograde type A dissection. There were no renal, visceral, cardiac, pulmonary or spinal complications. The patient went off her antihypertensive medication with a normal estimated glomerular filtration rate and accelerated aortic modulation.

Isolation of the left subclavian artery or its anomalous origin from the pulmonary artery has been documented in several cases, especially in association with a right-sided aortic arch. However, similar anomaly involving the right subclavian artery has been less frequently reported. Isolated right subclavian artery in association with interrupted aortic arch (IAA) is extremely rare, and only three cases have been reported so far. Here, we have presented yet another case of isolated right subclavian artery associated with ventricular septal defect, type B IAA and bilateral patent ductus arteriosus.

BACKGROUND: Duplex ultrasound (DU) is used in the diagnosis of neurogenic thoracic outlet syndrome (NTOS) to measure compression of the subclavian artery (SCA) which is thought to strengthen the NTOS diagnosis. However, the value of DU in NTOS remains unclear.METHODS: A retrospective review of a prospectively acquired database from the TOS center of the Catharina Hospital Eindhoven was performed of patients referred between January 2017 and December 2019. Only "proven NTOS" patients, defined as a successful response to thoracic outlet decompression (TOD) surgery based on patient-reported outcomes (NRS pain scale, CBSQ and DASH score) were included to exclude wrongfully diagnosed NTOS patient. The presence of vascular symptoms (defined as discoloration, edema or temperature changes of the hand or fingers), results of provocative maneuvers, and outcome of DU was used for analysis. To assess the link between vascular symptoms and compression on DU, a chi-squared test was performed. Further, we looked for a correlation between vascular symptoms, compression on DU and clinical outcome using a repeated measures analysis of variance (ANOVA).RESULTS: Vascular symptoms were seen in 49 of 133 patients (36.8%). In total, 51 of 133 patients (38.3%) had at least 50% variation in SCA peak systolic velocity (PSV) during DU at the level of SCA stenosis. SCA occlusion was seen in 11 patients (8.3%) during provocative maneuvers. The presence of clinical "arterial symptoms" was not significantly correlated with vascular laboratory findings, neither for alterations in PSV during DU (P = 0.245) nor for positional SCA occlusion (P = 0.540). No statistically significant correlations between the degree of SCA stenosis and postoperative outcomes, as measured with the DASH, CBSQ, or NRS scale for pain were found (P = 0.787).CONCLUSIONS: The role of DU in the work-up of NTOS in patients with vascular complaints is questionable. Changes in flow velocities are seen in NTOS patients and do not correlate with "vascular symptoms" or clinical outcome.

Background: We describe a simple and novel technique for cerebral protection during left subclavian artery (SA) stenting by inflation of a balloon-guiding catheter inside the aortic arch at the SA origin. Methods: A total of 12 patients with left SA stenosis underwent SA stenting with inflation of a balloon-guiding catheter inside the aortic arch at the left SA origin between January 2020 and December 2020. The SA stenting procedures under proximal protection were retrospectively reviewed to assess ischemic complications, hyperintense spots on diffusion-weighted imaging (DWI), and flow direction of left vertebral artery (VA) during balloon-guiding catheter inflation using ultrasonography (US). Results: The success rate of the SA stenting procedure was 100% with no symptomatic ischemic complications. None of the 12 patients showed small hyperintense spots in DWI. In all patients, the left VA on US during balloon inflation showed retrograde blood flow. Conclusions: The left VA on US had retrograde blood flow during inflation of the balloon-guiding catheter inside the aortic arch at the SA origin. Therefore, the proximal balloon protection inside the aortic arch at the SA origin is a simple and maybe a safe technique for cerebral protection during SA stenting.

Subclavian artery aneurysms are rare peripheral artery aneurysms, and open surgical repair is the reference standard treatment. We have reported the case a patient with a right subclavian artery aneurysm who was not indicated for open surgical repair because of comorbidities. Thus, endovascular treatment using the Gore Excluder Iliac Branch Endoprosthesis (WL Gore and Associates, Flagstaff, Ariz) was performed, leading to complete aneurysmal exclusion without perioperative complications. Although anatomic limitations exist, this technique could be alternative treatment option for right subclavian artery aneurysms.

The Blalock–Taussig–Thomas Shunt (BTTS) procedure was first introduced by Alfred Blalock, Hellen B. Taussig, and Vivien Thomas in 1944. The procedure is performed by making a connection between the subclavian artery to the pulmonary artery to augment pulmonary blood flow. BTTS is used to palliate neonates born with restrictive pulmonary blood flow disorders (cyanotic congenital heart disease). The modified BTTS (MBTTS) procedure is still seen as high risk and has quite high morbidity and mortality rates, especially in the neonatal group. Recent studies suggest a mortality of up to 15% in the single ventricle group and 3% in the biventricular group. Based on a literature search, several risk factors such as patient weight, shunt size/body weight ratio, underlying cardiac malformations, use of mechanical ventilator support, sternotomy approach and use of CPB machines have been shown to increase mortality. Current perioperative risk assessment methods and recommendations are also covered. In addition, our review article specifically discusses perioperative strategies including strategies for surgeons using the MBTTS procedure (surgical approach, determining shunt size and anastomosis techniques) and postoperative strategies (anticoagulation management and strategy to deal with undershunting/overshunting problems). Resumen: El procedimiento de derivación de Blalock-Taussig-Thomas fue introducido por primera vez por Alfred Blalock, Hellen B. Taussig y Vivien Thomas en 1944. El procedimiento se realiza al hacer una conexión entre la arteria subclavia y la arteria pulmonar para aumentar el flujo sanguíneo pulmonar. El procedimiento de derivación de Blalock-Taussig-Thomas se utiliza para paliar a los recién nacidos que nacen con trastornos restrictivos del flujo sanguíneo pulmonar (cardiopatía congénita cianótica). El procedimiento MBTTS todavía se considera un procedimiento de alto riesgo y tiene tasas de morbilidad y mortalidad bastante altas, especialmente en el grupo neonatal. Estudios recientes sugieren que hay una mortalidad de hasta el 15% en el grupo de un solo ventrículo y del 3% en el grupo de anomalías cardíacas biventrículos. Según la búsqueda bibliográfica, se ha demostrado que varios factores de riesgo, como el peso del paciente, la relación tamaño de la derivación/peso corporal, malformaciones cardíacas subyacentes, uso de asistencia respiratoria mecánica, abordaje de esternotomía y uso de máquinas de CEC, aumentan la mortalidad. También se tratan los métodos y recomendaciones actuales de evaluación de riesgos perioperatorios. Además, nuestro artículo de revisión analiza específicamente las estrategias perioperatorias, incluidas las estrategias para los cirujanos en el procedimiento MBTTS (abordaje quirúrgico, determinación del tamaño de la derivación y técnicas de anastomosis) y las estrategias postoperatorias (manejo de anticoagulación y estrategia para lidiar con problemas de sobrecapacidad/sobrecadencia).

Background Resection of lung cancer infiltrating the aortic arch or the subclavian artery can be accomplished in selected patients with the use of cardiopulmonary bypass (CPB). Direct cross-clamping of the aortic arch and the left subclavian artery without CPB for radical resection of the tumor can be an alternative. This study presents one group’s experience with this technique. Methods Between October 2016 and May 2019, 9 patients (5 male, 4 female) underwent radical resection of lung cancer infiltrating the aortic arch (n = 5) or the left subclavian artery (n = 4) by direct cross-clamping technique at Sapienza University of Rome, Italy. Seven left upper lobectomies, 1 left pneumonectomy, and 1 left upper sleeve lobectomy were performed. Reconstruction of the aortic arch was performed by direct suturing or polyethylene terephthalate (Dacron) patch, whereas the subclavian artery was reconstructed with a Dacron conduit. Three patients received neoadjuvant chemotherapy. Results Patients’ mean age was 64.7 ± 13.3 years (range, 36 to 78 years). Aortic arch resection was partial in all cases (adventitial in 1 and full thickness in 4); left subclavian artery resection was adventitial in 2 patients and circumferential in 2. All the resections were complete. Prosthetic reconstruction was performed in 4 cases. Mean operative time was 130 ± 25.6 minutes; mean vascular clamping time was 28.2 ± 3.2 minutes. No mortality occurred. The major complication rate was 11.1 %. At a mean follow-up of 17 ± 9 months (range, 5 to 29 months), the recurrence rate was 33.3%. Median survival was 20 months. Conclusions Direct cross-clamping as an alternative to CPB for resection of lung cancer infiltrating the aortic arch or the subclavian artery is a feasible, safe, and reliable procedure in selected patients.

INTRODUCTION Inadvertent implantation from the subclavian/axillary arteries is uncommon and has been rarely reported. Complications are frequent, mainly driven by thromboembolic events. Lead extraction, depending on the dwell time may be challenging and associated with thromboembolic events Case Report: We report the case of an 87-year-old man who had involuntarily pacemaker lead implanted into the left ventricle through the left subclavian artery. An extraction procedure was performed with surgical dissection to the artery and simple traction of the probe without complication. CONCLUSION Percutaneous lead extraction using surgical dissection is a therapeutic option for less than one year old left ventricle inadvertent lead implantation.

Purpose: The purpose of this study was to evaluate the association between the distance from the primary intimal tear (PIT) to the left subclavian artery (LSA) (PIT–LSA distance) and the risk of aortic enlargement after thoracic endovascular aortic repair (TEVAR). Methods: This is a retrospective cohort study. A total of 228 patients were reviewed from the database of the Registry Of type B aortic dissection with the Utility of STent graft (ROBUST) study performed from January 1, 2011, to December 31, 2016. Of them, 196 patients were eligible for analysis. The PIT–LSA distance was defined as the length from the distal edge of the LSA orifice to the proximal edge of the PIT along the centerline of the true lumen. According to the border between zone 3 and zone 4 of the Ishimaru classification, patients were divided into group A (n = 117, PIT–LSA distance ≤ 2 cm) and group B (n = 79, PIT–LSA distance > 2 cm). Thoracic aortic enlargement (TAE) was defined as a thoracic aortic volume increase of ≥20%. Multivariate Cox regression was used to estimate the association between the PIT–LSA distance and risk of TAE after TEVAR. Results: The mean age was 52.3 ± 11.6 years, and 88.8% of patients were male. There were no significant differences between groups in demographic and baseline characteristics. The PIT–LSA distance was 1.1 cm (range, −1.6 to 2.0 cm) in group A, and 2.9 cm (range, 2.1–12.6 cm) in group B. TAE occurred in 27 patients in group A, and 6 in group B. The mean follow-up was 12.4 months (range, 0.10–83.1 months) in group A, and 12.63 months (range, 0.10–82.77 months) in group B. The cumulative 12- and 24-month rates of freedom from TAE were 79.0% and 71.3% in group A, versus 92.5% and 92.5% in group B, respectively. Multivariate Cox regression analysis revealed that the PIT–LSA distance was an independent predictor of TAE after TEVAR (adjusted hazard ratio, 0.66; 95% confidence interval, 0.48–0.90; p = 0.009). Conclusion: Patients with a more proximal PIT location have a higher incidence of thoracic aortic enlargement after TEVAR. The location of the PIT in relation to the LSA can be used to identify patients who need closed surveillance after TEVAR or early preemptive intervention.

A 75-year-old man, who underwent inadvertent misplacement of pacemaker leads into the left coronary artery and left ventricle through the subclavian artery, was referred to our hospital. We safely performed percutaneous lead extraction in collaboration with surgeons and with the patient under general anesthesia. (Level of Difficulty: Advanced.), Central Illustration

BACKGROUND Iatrogenic aortic dissection (IAD) is a rare but fatal complication of interventional treatment for the proximal supra-aortic large vessels. Several cases of IAD after endovascular treatment of subclavian artery have been reported. Nevertheless, the pathogenesis of IAD is still unclear. Here we report a patient with IAD following a balloon expandable stent implanted into the left subclavian artery (LSA). CASE SUMMARY An 84-year-old man with a history of hypertension was admitted to the Neurology Department of our hospital complaining of dizziness and gait disturbance for more than 1 mo. Computed tomography angiography of the head and neck showed severe stenosis at the proximal LSA and the origin of the left vertebral artery. Magnetic resonance diffusion-weighted imaging of the brain revealed subacute infarctions in cerebellum, occipital lobe and medulla oblongata. He suffered a Stanford type B aortic dissection after the proximal LSA angioplasty with a balloon expandable stent. Thoracic endovascular aortic repair was performed immediately with the chimney technique and he was discharged 20 d later. After exploring the pathogenesis with multimodal imaging analysis, an easily neglected focal intramural hematoma (IMH) in the aorta near the junction of the LSA was found to be the main cause of the IAD. The risk of IAD should be sufficiently evaluated according to the characteristics of aortic arch lesions before the proximal LSA angioplasty. CONCLUSION Focal aortic IMH is a potential risk factor for IAD during a seemingly simple stenting of the proximal LSA.

Background Degenerative aortic arch aneurysms are known to develop through a pathological process of arterial atherosclerosis, which could be accompanied by peripheral artery diseases and resultant development of intrapelvic collateral arteries to the ischemic lower limbs. The aim of this study was to investigate the relationship between peripheral collateral circulation and postoperative paraplegia after total arch repair with a frozen elephant trunk in patients with degenerative aortic arch aneurysms and peripheral artery diseases. Methods Between October 2014 and March 2020, 27 patients (20 men; 69.8 ± 7.7 years old) underwent total arch repair with a frozen elephant trunk. Two of the 27 patients developed paraplegia postoperatively. The patients were divided into two groups, spinal cord ischemia (SCI) group (2 patients) and no-SCI group (25 patients). The aortic shagginess score, arterial calcification (subclavian artery; hypogastric artery) score, and the number of hypogastric artery branches, assessed using preoperative contrast-enhanced computed tomography images, were compared between the two groups. Results The ankle brachial artery pressure index (i.e., lower side value each patient) was lower in the SCI group than that in the no-SCI group (0.64, 0.71, and 1.09±0.07, respectively). There was no difference between the two groups in the arterial calcification scores or the aortic shagginess score. The number of hypogastric artery branches was greater in the SCI group than in the no-SCI group (66, 66, and 30.7±7.5, respectively). Conclusions Enhanced collateral circulation to the ischemic lower limbs in patients with combination of degenerative aortic arch aneurysms and peripheral artery diseases may be involved in paraplegia the upper thoracic spinal cord injury after total arch repair with a frozen elephant trunk.

Purpose: Acute thromboembolic disease of the innominate artery (IA) poses a unique set of therapeutic challenges, owing to its contribution to both the cerebral and upper extremity circulation, and risks of distal embolization via the carotid and subclavian arteries, respectively. Herein, we present a 74-year-old female who presents with acute IA thrombus treated successfully with right axillary and common carotid exposure and aspiration catheter-directed mechanical thrombectomy (CDT). Furthermore, an emerging use of CDT and its application in acute thromboembolism are outlined. Case report: A 74-year-old female with history of right lung transplant for pulmonary fibrosis with severe pulmonary hypertension, and stage IIIA left lung adenocarcinoma status post left lower lobectomy undergoing adjuvant chemotherapy presented with acute IA thrombus and right-sided stroke. She was treated successfully with right axillary and common carotid exposure and aspiration CDT. Computed tomography angiography performed 1 month postoperatively confirmed patent IA with no evidence of residual or recurrent thrombus. Conclusion: There are currently no standard guidelines on the management of acute IA thromboembolism, with mostly individual cases reported in the literature describing this rare entity. Nevertheless, this unique clinical entity mandates expeditious diagnostic and therapeutic approaches in order to avoid permanent neurologic deficits from distal embolization. Our case demonstrates that aspiration CDT may be an effective treatment modality for patients with acute IA thrombus.

OBJECTIVES Vascular rings are rare anomalies of congenital heart disease that cause respiratory and gastrointestinal symptoms. This study assessed the long-term outcomes of patients with vascular ring division. METHODS A multi-institution retrospective review of 371 patients with vascular rings undergoing surgical division at 3 paediatric cardiac institutions between November 2007 and October 2019 was performed. RESULTS The complete vascular rings consisted of a double aortic arch (24.5%), right aortic arch with left ligamentum arteriosum (36.7%) and left aortic arch, with right ligamentum arteriosum (0.5%). The incomplete vascular rings consisted of a pulmonary artery sling (22.9%), left aortic arch with aberrant right subclavian artery (15.1%) and innominate artery compression syndrome (0.3%). Respiratory symptoms included stridor (71.4%), wheezing (49.1%), coughing (31.5%), gastrointestinal symptoms included choking (12.4%), dysphagia (3.2%) and emesis (1.9%). Only one patient died after discharge, yielding a late mortality rate of 0.3% (1/360). The 10-year overall survival rate was 96.8%. Postoperative complications were reported in 51 patients, 15 of whom required reoperation. The 10-year freedom from reoperation rate was 95.9%. Follow-up was completed in 95.4% (354/371) of patients, with a mean follow-up time of 4.3 ± 2.9 years (range from 1 to 13 years). Twenty patients (5.6%) experienced residual symptoms during long-term follow-up. CONCLUSIONS The outcomes of vascular ring division are excellent. A Kommerell diverticulum >1.5 times the aberrant left subclavian artery origin is an operative indication for primary resection. Tracheomalacia is a risk factor for reoperation and residual symptoms, and preoperative fibrobronchoscopy is important for evaluation.

Background. Central venous catheter represents an important tool in the management of critically ill patient. In this report, we described a COVID-19-positive case who had COVID-related complications and iatrogenic left subclavian artery pseudoaneurysm after central venous catheter insertion. Case Presentation. A 58-year-old male patient presented with a high-grade fever, myalgia, and shortness of breath due to COVID-19 infection. He required mechanical ventilation support and hemodialysis. He also developed uneventful deep vein thrombosis and myocardial infarction. As a complication of central line insertion, the patient developed pseudoaneurysm that originated from the subclavian artery with significant bleeding and large hematoma. Balloon-assisted percutaneous thrombin injection was done under ultrasound guidance. The patient was extubated 2 days later with no evidence of flow in the pseudoaneurysm. However, he lost movement in the left arm secondary to the compression of the brachial plexus from the pseudoaneurysm/hematoma, and therefore, 1.5 litres of the hematoma was evacuated in the operating room through a lateral left chest wall incision along the anterior axillary line to relieve the compression over the brachial plexus. The patient declined surgical reconstruction of the brachial plexus, and the flaccid paralysis of the arm did not recover during the follow-up. Conclusion. This is a case of unusual complications of COVID infection and iatrogenic left subclavian artery pseudoaneurysm postcentral vein cannulation. Balloon-assisted percutaneous thrombin injection for treatment of left subclavian artery pseudoaneurysm is feasible; however, delayed diagnosis could be associated with long-term or permanent disability.

Objectives This study investigated whether there was a relationship between steno-occlusion of the vertebral artery (VA) segments and the dominant VA side. Materials and methods Angiography results of 215 patients (146 men; 69 women) were retrospectively analyzed in this study. The patients were divided into three groups: dominant, non-dominant and co-dominant. These groups were compared according to the presence of steno-occlusion in the vertebral artery segments. The results were evaluatedusing X2, Mann-Whitney U and Kruskal-Wallis tests. For correlation analysis, Spearman's Rho test was used. Results The findings showed that 55 of 215 patients (25.6%) had a right dominant VA, and 103 (47.9%) had left dominant VA. There was no significant relationship between dominance and age or gender (p > 0.05). More vertebral artery stenosis (VAS) was found on the dominant side. However, a significant relationship only in the right dominant V1 segment (p = 0.044) was noticed. Hypoplastic VA was detected in 13 patients (6%). Most of the VAs (98.4%, n = 423) arose from the subclavian artery. Conclusions In conclusion, we found more vertebral artery stenosis on the dominant side than the co-dominant side, especially on the origin of the vertebral arteries. However, it was only significant on the right dominant V1 segment, regardless of age and gender of the patients (p > 0.05).

Introduction The aetiology of subclavian artery stenosis can be very different, but typically the most common form is due to atherosclerotic disease. Interventional treatment is generally indicated for upper limb ischemia, vertebrobasilar symptoms and subclavian steal syndrome, coronary steal syndrome. Endovascular stenting is preferred over surgery because of its high success rate, less invasive nature, and minimal complication rate. In this study the characteristics of patients treated endovascularly due to subclavian artery stenosis in our centre and their procedural details will be examined. Methods: We retrospectively analyzed patients with subclavian artery stenosis treated by endovascular techniques in our centre between January 2019 and January 2021. Result Twelve (80%) of 15 patients with stenotic subclavian arteries were successfully treated with endovascular techniques. In 3 patients, the procedure was terminated with failure.The mean age of the patients was 64.66 years old and 3(20%) of the patients were female. All of the patients were receiving antihypertensive treatment with the diagnosis of hypertension, and 4 (26.66%) patients had Diabetes mellitus, 13 (86.66%) patients had hyperlipidemia, 13 (86.66%) patients had coronary artery disease. In 2 patients, the lesion was diagnosed in the right subclavian artery. In 7 patients, the procedure was performed with the telescopic method from a transfemoral approach. In 5 patients, the transradial approach was used. In 3 patients, the procedure was performed directly over the transbrachial approach. Balloon expandable stent implantation was performed in 10 (66.66%) patients at the end of the procedure, and self-expandable stent implantation was performed in 1 patient. Conclusion In conclusion, signs and symptoms can be managed effectively with endovascular techniques in patients with significant subclavian artery disease. In this study, we shared our real-life data on subclavian artery endovascular treatment.

Background: Cervical rib, though it is seen incidentally in the radiographic examination of chest and cervical spine, a particular subset of it can present with thoracic outlet syndrome. This article mainly focuses on the symptom causation of articulating cervical rib, as compared to non-articulating cervical rib which is often asymptomatic. There are many articles describing a cervical rib, but those highlighting this particular relation between the types of cervical rib and symptom causation are not present in present database. The review of serial radiographs upon correlating clinically, lead us to conclude that articulating variant of cervical rib is majorly symptomatic and caused grave arterial complications. The article consists brief discussions on the types of cervical ribs, thoracic outlet syndrome and role of imaging in diagnosing a cervical rib. Main Body: We have illustrated five such cases from our tertiary care hospital and highlighted the relationship between the magnitude of symptoms and variations of cervical rib. In compliance with PRISMA guidelines, we screened 33 articles dealing with cervical rib and 13 with thoracic outlet syndrome. Overall, 46 articles were filtered on studying the abstract. Further, 15 more had to be excluded as they were case reports and case series, and finally 31 articles were included in our study. We studied and discussed these articles and research papers in relation to variant anatomy of cervical rib, its types and symptom causation. In our study, also routine sequential chest radiographs were reviewed during the period of January 2020 to January 2021 and those with cervical rib and/or elongated C7 transverse process were included. Conclusion: Thus, to conclude we can state that articulating cervical ribs pose a greater threat and cause arterial occlusion. Key words: Cervical rib, thoracic outlet syndrome, subclavian artery.

Background Congenital aortic arch anomalies are commonly encountered during neurointerventional procedures. While some anomalies are identified at an early age, many are incidentally discovered later in adulthood during endovascular evaluations or interventions. Proper understanding of the normal arch anatomy and its variants is pivotal to safely navigate normal aortic arch branches and to negotiate the catheter through anomalies during neurointerventional procedures. This is particularly relevant in the increasingly “transradial first” culture of neurointerventional surgery. Moreover, some of these anomalies have a peculiar predilection for complications including aneurysm formation, dissection, and rupture during the procedure. Therefore, an understanding of these anomalies, their underlying embryological basis and associations, and pattern of circulation will help endovascular neurosurgeons and interventional radiologists navigate with confidence and consider relevant pathologic associations that may inform risk of cerebrovascular disease. Methods Here, we present a brief review of the basic embryology of the common anomalies of the aortic arch along with their neurological significances and discuss, through illustrative cases, the association of aortic arch anomalies with cerebral vascular pathology. Conclusions Understanding the aortic arch anomalies and its embryological basis is essential to safely navigate the cerebral vascular system during neurointerventional surgeries.

Objectives To describe clinical and imaging characteristics of an isolated subclavian artery (ISA) in pediatric patients. Background ISA is a rare congenital aortic arch anomaly defined as a loss of connection between the subclavian artery and aorta. The clinical manifestations and complications of ISA in children are unclear. Methods This retrospective study included clinical and imaging data of ISA patients younger than 18 years whose data were recorded in the electronic radiology database during January 2006–August 2019. Results Of 102 enrolled patients, 59 had been diagnosed in the first year of life. The majority of the patients also had congenital heart diseases, of which tetralogy of Fallot was the most common. The vertebral artery and collateral branch of the descending aorta served as blood flow supplies in 94 and 8 patients with ISA, respectively, as confirmed using computed tomography or magnetic resonance imaging. However, the blood supply did not influence the development of ISA. Eleven patients exhibited mild or moderate stenosis of the ISA, although only two exhibited coldness or a blood pressure gradient in the upper extremities. These two symptomatic patients also presented with patent ductus arteriosus, and this association was significant (P = 0.008). Conclusion ISA management is often determined based on symptoms and associated congenital heart diseases. The ISA is prone to stenosis in patients with ipsilateral patent ductus arteriosus. We recommend early surgical ligation or percutaneous closure of the ductus arteriosus in patients with ISA.

Penetrating injuries to the subclavian artery carry a high mortality rate, especially when the patient presents in shock. Rapid and effective haemorrhage control is challenging due to the anatomical location at the thoracic outlet. Historically, vessel ligation has been used to control bleeding, but this is often performed late, when metabolic exhaustion is established, and is associated with upper-limb ischaemia and limb loss. Rapid proximal control through the chest with temporary intravascular shunting is the damage control technique of choice to temporise blood loss and restore perfusion until the patient is physiologically optimised for a delayed definitive vascular repair. We describe a case of vascular damage control in a patient after gunshot wound.

Subclavian artery aneurysms (SAAs) are rare, and their repair can be technically complex. We have reported the redo repair of a large, expanding, right SAA after primary repair consisting of total aortic arch replacement with bilateral subclavian artery ligation and bypass. The redo repair used claviculectomy to facilitate exposure, ligation of the right deep cervical and internal thoracic arteries from within the aneurysm sac, and revision of the previous axillary artery bypass that had thrombosed owing to the mass effect of the expanding SAA. Claviculectomy can facilitate repair of large SAAs that are poorly suited to more routine exposure approaches, with acceptable risk and functional outcomes.

Patients with hostile chests are often precluded from the use of left ventricular assist device therapies for advanced heart failure. We describe a technique for left ventricular assist device implantation using a left thoracotomy approach with outflow graft anastomosis to the left subclavian artery and placement of a distal band to the left subclavian artery. Distal banding of the left subclavian artery prevents over-circulation to the left upper extremity. This technique provides an alternative approach for left ventricular assist device implantation in patients who otherwise would be deemed inoperable because of hostile chest.

Objective Isolated true subclavian artery aneurysm (SAA) without aberrant subclavian artery or coarctation of descending aorta is a rare peripheral aneurysm. Herein, the experience of our medical center in the treatment of this disease is presented. Methods The Division operative log was queried to identify cases of SAA repair between January 2012 and September 2019 that were not associated with coarctation of the aorta or the presence of an aberrant subclavian artery. A total of 22 cases were identified. The characteristics, treatment and clinical outcomes of these cases were assessed. Results The mean age of patients was 53.5 ± 14.3 years and 14 patients were male (63.6%). Half of the cases were attributed to atherosclerotic degeneration. The clinical symptoms of aneurysms were varied, including asymptomatic, pulsatile mass of supraclavicular fossa, local pain, upper limb embolism, Horner's syndrome and hoarseness. Aneurysms were located on the right in 17 cases, on the left in 3 cases and on both sides in 2 cases. Fifteen (68%) patients underwent an intervention, of which 11 (50%) underwent an open surgical repair, and 4 (18%) underwent endovascular repair. The mean diameter of the aneurysms was 39.5 ± 20.7 mm in the open surgery group, and 24.0 ± 4.7 mm in the endovascular group. The follow-up duration ranged from 2 months to 12 years. One patient died of cardiogenic disease in the untreated group. Patients undergoing open operative repair had 100% patency of the reconstruction. In the endovascular group, one patient had stent occlusion 2 years after the operation. Conclusions The most common cause of isolated subclavian aneurysm without aberrant subclavian artery or coarctation of descending aorta is atherosclerosis. The clinical symptoms of aneurysms are varied, and the aneurysms tend to occur on the right side. Based on the anatomical conditions of SAAs, open surgery and endovascular repair can be used for treatment.

We present a case of subclavian artery perforation with mediastinal hematoma following elective percutaneous coronary intervention. A conservative approach was preferred over invasive correction. Although no outcome data exist specifically for subclavian artery injury, registry data in patients with iatrogenic aortic dissection suggest that long-term outcomes are good without vascular repair. (Level of Difficulty: Advanced.), Central Illustration

Introduction: Left internal mammary artery (LIMA) grafts should be used in patients undergoing CABG. No other procedure results in patency equivalent to that of the left anterior descending coronary artery (LAD)–LIMA bypass graft. The CHA2DS2-Vasc-HS scoring system can be used to successfully predict CAD severity in stable CAD patients. We aimed to investigate the relationship between LIMA flow and the CHA2DS2-Vasc-HS score. Methods: A total of 684 patients, who underwent CABG surgery, were included in this study. Previous history of bypass surgery, emergency operations, patients with Leriche syndrome and patients with severe obstructive pulmonary and subclavian artery disease were excluded from our study. Patients with a LIMA flow that was suitable for bypass grafting, as determined during the intraoperative evaluation, were included in the low LIMA flow group, and the CHA2DS2-Vasc-HS score was calculated for all patients. Results: Patients in the low LIMA flow group (Group 1) were older. The CHA2DS2-Vasc-HS score (P < 0.001), presence of mild or moderate COPD (P = 0.022), number of severely diseased vessels (P = 0.036), and BMI (P < 0.001) were independent predictors of poor LIMA flow. The cutoff value of the CHA2DS2-VASc-HS score for the prediction of poor LIMA flow was >5.5, with a sensitivity of 92.9% and specificity of 83.4% (AUC: 0.938, 95% Cl: 0.906 – 0.970, P < 0.001). Conclusion: A preoperative high CHA2DS2-Vasc-HS score can be used to predict low intraoperative LIMA flow. The CHA2DS2-Vasc-HS score is an easy-to-use and reliable estimation method and can be used as an additional preoperative of LIMA flow in patients undergoing CABG due to severe CAD.

Background and aim of study The origin of the vertebral artery (VA) from the left subclavian (LSA) is variable and must be considered when proximal ligation or embolization is performed post thoracic endovascular aortic repair (TEVAR) and extra-anatomical bypass (EAB). A retrospective study was conducted to understand the patency of the LSA and VAs after TEVAR and the relationship of the EAB to the LSA. Methods Fifty-six patients underwent TEVAR where the LSA origin was occluded. A comparison was performed between the length of the proximal LSA from the arch of the aorta to the origin of the VA. Patient outcomes included posterior or anterior circulation cerebrovascular accident, spinal cord ischemia (SCI), and symptoms and signs of left arm ischemia (LAI). Thirty one underwent EAB with 8 undergoing occlusion of the LSA proximal to the origin of the left VA. A further 25 underwent TEVAR with no EAB performed. The mean (standard deviation) of origin of the VA from the origin at the arch was 37.0 (12.9) mm compared to 34.0 (13.7) mm in those where no bypass was performed (p 0.45). Four patients underwent intraluminal plug occlusion and four had external ligation of the proximal LSA in those undergoing EAB. Conclusions Careful evaluation of the LSA is needed when planning TEVAR as occlusion techniques may be dependent on a minimum length of the VA from the aortic arch. The mean length of the VA from the aorta has high heterogeneity which may dictate the optimum occlusion method for LSA.

The optimal approach for patients undergoing transcatheter aortic valve replacement (TAVR), who are contraindicated for a transfemoral (TF) approach, is still controversial. The present study aimed to evaluate the utility of the TAVR via a subclavian artery with a small diameter, by minimal incision and a double Z suture hemostasis technique using 18 Fr DrySeal Flex sheath, namely minimum-incision transsubclavian TAVR (MITS-TAVR), in patients contraindicated for the TF approach.We included consecutive patients who underwent the MITS-TAVR (MITS group; n = 21) and TF-TAVR (TF group; n = 81) using the CoreValve Evolut R/PRO valves and examined the incidence of in-hospital adverse events and post-discharge mortality between the two groups.The mean body surface area was significantly smaller in the MITS group (1.33 ± 0.04 vs. 1.43 ± 0.02 m2; p = 0.045). The minimal lumen diameter of the femoral artery was significantly smaller in the MITS group (5.01 vs. 6.43 mm; p 0.01). The lumen diameter of the left subclavian artery (LSA) in the MITS group was 4.97 ± 0.14 mm. The duration of the TAVR procedure to discharge was not significantly different (9.7 ± 2.0 days vs. 13.2 ± 1.0 days; p = 0.239). We did not experience in-hospital death in both groups, and no significant differences were observed in the incidence of major adverse cardiac and cerebrovascular events between the two groups. The post-discharge survival rate was not significantly different between the groups (at 2-year; MITS group vs. TF group = 91.0% vs. 89.0%; p = 0.725).The MITS-TAVR using 18 Fr Dryseal Flex sheath was safe and effective and might be a promising alternative approach even in patients with a small body and small LSA diameter, who are contraindicated to the TF approach.

The vascular involvement in patients with Behcet's disease is defined as vascular Behcet's disease, which can greatly influence the prognosis. However, there are few reports on endovascular treatment (EVT) for subclavian pseudoaneurysms with long-term prognosis over 10 years. We present the case of a 42-year-old man with left subclavian artery pseudoaneurysm due to vascular Behcet's disease who was treated with EVT along with immunosuppressive therapies. Subsequently, 8 years after, the aneurysm recurred in the stent with stent fracture. Therefore, additional EVT was performed. We present the details of the patient's clinical course over 10 years after the EVT.

In this study, thrombolytic therapy is described and a case of left subclavian artery thrombosis with acute clinical symptoms is presented. The patient was an 82-year-old woman with the symptoms of axillary artery thrombosis. Catheter-directed thrombolysis was performed and thrombolytic medication was injected via a catheter. In addition, a low dose of heparin was infused intravenously. Following treatment, clinical symptoms were normal. The repeated angiography indicated a good blood flow to the left upper distal limb. Minimally invasive methods, such as catheter-based thrombolytic therapy may be a suitable alternative for patients with viable limb acute ischemia. Keywords:Axillary artery,Subclavian artery,Thrombolytic therapy,Thrombosis

Subclavian and proximal axillary arterial injuries are rare and difficult to manage. Eight patients were managed from January 2008 to December 2018 at The Aga Khan University Hospital, Karachi, Pakistan with mean age of 36.13 ± 14.48 years. All patients had penetrating injuries, from gunshot in 5 (62.5%), bomb blast in 2 (25%), and stab wound in 1 (12.5%) case. Six (75%) patients presented in haemodynamically stable condition. The mean time between the injury and patient presentation was 28 ± 8.39 hours. The injuries were approached via supraclavicular incision in 3 (37.5%) patients, infraclavicular incisions in 2 (25%) patients and median sternotomy in 2 (25%) patients. Three (37.5%) patients had false aneurysm, while 5 (62.5%) had transected artery. Primary repair was performed in 2 (25%) cases, while 6 (75%) patients were treated with interposition graft with polytetrafluoroethylene (PTFE). All patients have salvaged limbs with good functional outcomes. Key Words: Subclavian artery, Penetrating injury, Vascular trauma, Vascular repair.

La falla cardíaca avanzada es un importante problema de salud, siendo la única alternativa definitiva de manejo el trasplante cardíaco o los dispositivos de asistencia ventricular. El balón de contrapulsación intraaórtico por acceso femoral como puente a trasplante, que es aún de uso frecuente, tiene la desventaja de limitar la actividad del paciente, exponiéndolo a las complicaciones de la inmovilidad, lo que puede amenazar el éxito del procedimiento o, al menos, complicar la recuperación después del trasplante. El acceso por la arteria subclavia para el implante se ha convertido en una alternativa atractiva pues evita todas estas limitaciones del acceso femoral y hay evidencia que favorece su utilización como primera alternativa en este contexto. Presentamos los primeros casos de implante de balón de contrapulsación por vía subclavia en un centro de atención cardiovascular de alta complejidad en Colombia.

Neck cannulation is the most common cannulation strategy performed to provide veno-arterial extracorporeal membrane oxygenation support in paediatric patients, especially in small children. Upper limb ischaemia is a rare complication of neck cannulation and is likely caused by arterial cannula malposition. We describe a case of right arm ischaemia caused by extrinsic compression of the right subclavian artery by the venous drainage cannula inserted through the right internal jugular vein. Upper limb hypoperfusion was resolved immediately after changing the venous drainage cannula from the right jugular vein to the right femoral vein.

We describe a 17-year-old boy with capillary malformation-arteriovenous malformation syndrome and a massive vascular malformation of the right chest wall, shoulder, and upper arm. Persistent growth of the malformation caused cutaneous ulcerations and recurrent massive bleeding episodes. We proceeded with a modified shoulder disarticulation preceded by ligation of the subclavian artery and innominate vein by median sternotomy. After a staged debulking resection of the residual chest wall arteriovenous malformation with rotational transverse rectus abdominis myocutaneous flap coverage, the patient was discharged home safely. This report demonstrates that a multidisciplinary approach is critical for management of life-threatening complications in capillary malformation-arteriovenous malformation patients.

Background: Posterolateral thoracotomy provides limited access to the thoracic apex that can result in poor visualization of subclavian vessels, their branches, and the brachial plexus. A thoracoscopic approach may overcome these limitations. Purpose: We report a thoracoscopic approach and associated technical challenges in resecting apical thoracic neuroblastoma encasing the subclavian artery. Methods: A single-institution retrospective chart review was performed (2018-2020) for patients undergoing thoracoscopic resection of apical neuroblastoma encasing the subclavian artery. Patient demographics, imaging, and hospital course were reviewed. Operative video recordings were assessed for exposure quality, technical challenges, and percentage of tumor resection. Patients were placed laterally, with three 5-mm ports triangulated to the apex. Dissection started at the tumor edge and followed along the vessel and branches. Results: Four patients (median age 2.7 years) underwent thoracoscopic apical neuroblastoma resection. Median length of stay was 2.5 days. One low-risk patient underwent resection for tumor growth during observation. One intermediate and 2 high-risk patients received neoadjuvant chemotherapy. Two patients continued having persistent vascular encasement, whereas in 1 patient the mass decreased in size and only abutted the subclavian and vertebral arteries. In 1 patient, tumor involved the brachial plexus, which was freed and preserved thoracoscopically. All cases had substantial tumor-feeding vessels branching from the subclavian artery. There was one conversion to open thoracotomy due to dense tumor adherence to the subclavian artery and vein. More than 95% resection was achieved in all cases. All patients had baseline Horner syndrome. No complications were reported. Conclusion: The thoracoscopic approach for resecting apical neuroblastoma provides optimal exposure and safe access in selected patients.

Aberrant extracranial internal carotid artery and nonrecurrent laryngeal nerve (NRLN) are neurovascular anomalies, which clinicians should be aware of to prevent severe complications while performing transoral or open surgery in the head and neck area. These two variants can occur simultaneously because of similar etiology of embryological maldevelopment of arch arteries. In this study, a 64-year-old woman complaining of hoarseness presented a bulge in the left hypopharynx. A contrasted computed tomography scan indicated left thyroid mass with hypopharynx invasion, accompanied by right aberrant extracranial internal carotid artery and subclavian artery. Fine needle aspiration biopsy of the mass revealed papillary thyroid carcinoma (PTC). She underwent total thyroidectomy, partial hypopharyngectomy, neck dissection, and tracheotomy. Intraoperatively, a right NRLN was found above the level of the cricothyroid junction that entered the larynx. The patient did not present severe complications postoperatively. Radioactive iodine therapy was applied after the surgery. At the 3-year follow-up, she remained cancer-free, and the hoarseness has improved. This is the first report of concurrent aberrant extracranial internal carotid artery and NRLN. NRLN of right side can be predicted in the computed tomography scan by the sign of the right subclavian artery origining from the left side of the aortic arch and running an abnormal course between the esophagus and the vertebra. Meanwhile, aberrant extracranial internal carotid artery may occur simultaneously. Recognition of aberrant neurovascular structures enables clinicians to prevent severe surgical complications.

Innominate artery ligation emerged in the 19th century as an early operation for right subclavian aneurysm. Clinical outcomes were often dire, but undeterred surgeons believed that ligation represented an opportunity that outweighed the risks of nonoperative aneurysm management. Valentine Mott of New York performed the procedure in 1818; his patient died 26 days later. Variations on Mott's approach were undertaken 13 more times from 1822 to 1861 by surgeons in the United States and abroad, all of which proved fatal. Andrew Woods Smyth of New Orleans was the first to successfully control a subclavian artery aneurysm with innominate ligation in 1864. The Charity Hospital house surgeon used a series of ligations on the innominate, common carotid, vertebral, and internal mammary arteries to prevent collateral and recurrent blood flow to the aneurysmal sac. These physiologically-oriented operations kept Smyth's patient alive and functional for ten years. New Orleans became an internationally-recognized hub for advancements in aneurysm surgery. One of Smyth's students, Rudolph Matas, went on to revolutionize vascular surgery. Along his path to becoming the Father of modern vascular surgery, Matas documented his own performance of Smyth's operation at Charity 4 times over the course of his career. Although later supplanted by primary vascular anastomosis and grafting, the first successful innominate ligation was a collaborative effort between New York and New Orleans that served as a foundation for the development of modern aneurysm repair.

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 ± 0.3 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

In situ laser fenestration (ISLF) is currently used to reconstruct the aortic major branches during thoracic endovascular aortic repair (TEVAR). To our knowledge, there have been no reports on the application of ISLF for delayed revascularization of the LSA previously sealed in TEVAR. This report describes 5 patients who underwent ISLF for delayed LSA revascularization, with a technical success rate of 80%. No endoleakage occurred, and stents remained patent during more than 6-month follow-up. ISLF is an effective, safe and minimally invasive method for delayed revascularization of the LSA following TEVAR for type B aortic dissection (TBAD) when patients are selected appropriately.

Background Free flaps are widely used in maxillofacial reconstruction; however, this approach was not feasible in the current case. It was not possible because the free flap method requires microvascular anastomosis expertise, which is difficult, time-consuming and costly. Case presentation An 86-year-old woman suffered squamous cell carcinoma on the right side of her face, which resulted in a large soft-tissue defect. Here, we present a case of facial reconstruction from the inferior margin of the jaw to the top of the head. The size of the defect was 18.5 cm × 7.5 cm, which is rare for a patient of this age in the maxillofacial area. We used the supraclavicular artery island flap (SCAIFP) which measured 19.3 cm × 8.3 cm to repair the defect. After the operation, the flap survived without complications. Then, the patient was followed for 10 months and was satisfied with the aesthetic and functional results at the donor and recipient sites following the tumour resection. The tumour did not recur, and facial nerve function was preserved. Conclusion Our results provide a new choice for the reconstruction of large defects of the head and face, and expand the potential applications of the SCAIFP.

Poland's syndrome (PS) is a rare musculoskeletal congenital anomaly with a wide spectrum of presentations. It is typically characterized by hypoplasia or aplasia of pectoral muscles, mammary hypoplasia and variably associated ipsilateral limb anomalies. Limb defects can vary in severity, ranging from syndactyly to phocomelia. Most cases are sporadic but familial cases with intrafamilial variability have been reported. Several theories have been proposed regarding the genesis of PS. Vascular disruption theory, "the subclavian artery supply disruption sequence" (SASDS) remains the most accepted pathogenic mechanism. Clinical presentations can vary in severity from syndactyly to phocomelia in the limbs and in the thorax, rib defects to severe chest wall anomalies with impaired lung function. Most patients have subtle presentation at birth and milder forms in childhood. Functional limitations due to PS are usually minimal. Surgical treatment aims to improve pulmonary functions arising from severe thoracic deformities but is more often done to enhance the cosmesis. The use of adipose-derived mesenchymal stem cells and fat transfer have shown promising results in recent times for correction of chest defects and breast augmentation. Gaining deeper insights into the etiopathogenesis and clinical presentation of PS will improve the clinical recognition and management of this rare condition. In this review article, we aim to outline the details of this syndrome including its etiopathogenesis, evolution, spectrum of clinical manifestations, other systemic associations, diagnostic modalities, and recent advances in treatment.