The wolf in sheep's clothing: vasovagal syncope in acute aortic dissection.

BACKGROUND: The presentation of acute aortic dissection can pose a challenge for emergency physicians, as it may occur without pain. Atypical presentations can lead to significant delays in diagnosis and increased mortality rates. CASE DESCRIPTION: Our case illustrates that isolated painless syncope can be a rare presenting symptom of acute aortic dissection type A. What is unique about our case is the limited extension of the dissection tear and the availability of Holter monitoring during the syncopal episode. CONCLUSION: This constellation provides insight into the pathophysiological mechanism of the syncope in this patient. Mechanisms of syncope related to acute aortic dissection are diverse. We show that vasovagal activation not related to pain can be the underlying mechanism of syncope in acute aortic dissection type A. Although excessive vasovagal tone in the setting of aortic dissection has been hypothesized in the past, it has never been as clearly illustrated as in the present case. This also highlights the challenge in risk stratification of syncope in the emergency department.

Current techniques of repair of aortic arch pathologies and the role of the aortic team.

The treatment of aortic arch pathologies is becoming progressively more complex and multidisciplinary. Despite progresses in open surgical techniques, the high rate of surgical morbidity and mortality, especially in frail and elderly patients, has led to the development of alternative treatment options to conventional open surgery such as hybrid and endovascular procedures. Our purpose is to summarize the advantages and disadvantages of the different approaches and investigate the role of a dedicated aortic team in the choice of the most appropriate treatment for each patient.

A case of right aortic arch type II combined with Kommerell's diverticulum and left innominate vein beneath the aortic arch.

The combination of the right aortic arch and aberrant left subclavian artery (ALSA) with Kommerell's diverticulum (KD) is rare to coexist with the left innominate vein (LINV) beneath the aortic arch. It escalates the surgical risk undoubtedly and increases the difficulty of clinical procedures. We report one case diagnosed by Ultrasound and Computed Tomography Angiography (CTA).

Less invasive replacement of aortic root, ascending aorta and hemiarch via partial upper sternotomy: a propensity-score matched comparison with full sternotomy.

OBJECTIVES: Less invasive surgery has emerged as an option for aortic pathologies. The current study compared our experience on early postoperative results of patients with aortic surgery between partial upper sternotomy (PUS) and full sternotomy (FS). METHODS: We performed a retrospective analysis of the data of patients undergoing aortic root surgery with concomitant ascending aorta and hemiarch replacement. Exclusion criteria were type A aortic dissection and other concomitant major cardiac surgery. After propensity-score matching, we compared the perioperative outcomes of patients undergoing surgery with PUS vs FS. RESULTS: 161 patients operated on between January 2013 and September 2022 met the inclusion criteria (PUS: n = 22, FS: n = 139). Propensity score matching yielded 22 pairs with a balanced distribution of propensity scores and covariates between the compared groups. There was no evidence that PUS affects cardiopulmonary bypass [108(67-119) vs 113(87-148) min, p = 0.154; PUS vs FS] and circulatory arrest duration [9(7-10) vs 9(8-13) min, p = 0.264; PUS vs FS]. There was a reduced cross-clamp duration in the PUS group [88(58-96) vs 92(71-122) min, p = 0.032]. Cumulative sum charts (CUSUM) have shown consistently low cross-clamp and circulatory arrest duration for two experienced surgeons who performed 20 of the procedures in the PUS group (10 each). Perioperative mortality and morbidity were low, with no in-hospital mortality in the PUS group [0 vs 1(4.5%), p > 0.999] and absence of strokes in both groups. CONCLUSIONS: In summary, our initial experience suggests that less invasive aortic root, ascending aorta, and hemiarch replacement via partial upper sternotomy could be performed in our patient cohort as safely as via full sternotomy. Advantages for the patient are reduced surgical trauma, improved cosmetic results, and-presumably-less pain.

Transverse arch stenting and its effect on systemic hypertension.

Background: This study aimed to investigate the safety and efficacy of transverse aortic arch stenting and evaluate the course of hypertension and the act of arch stenting on systemic hypertension. Methods: The transverse aortic arch stenting procedures between January 2007 and May 2023 were retrospectively analyzed. Detailed procedure information, technical aspects, pressure measurements, angiographic data, balloons and stents used, complications, and immediate results were examined. Early and mid-term results were assessed. Results: Eighteen patients (10 males and 8 females; mean age: 14.5±5.3 years; range, 4 to 23 years) were included in the study, all of whom were hypertensive before the procedure. The mean weight was 56.8±19.6 kg. In seven patients, the stent struts had to be dilated due to the stent causing jailing at the entrance of nearby arch vessels. After stenting, there was a significant increase in arch diameter and a decrease in ascending aorta pressure and the pressure gradient across the aorta. There were no early mortality or major complications. Late migration of the stent was observed in one patient. Three patients became normotensive immediately after the intervention, and five became drug-free during the follow-up. The requirement for dual antihypertensive therapy was significantly reduced. Conclusion: Residual transverse arch lesions may contribute to the persistence of systemic hypertension after coarctation treatment. Transverse arch stent implantation can be performed safely with favorable outcomes, facilitating better blood pressure control. However, it should be noted that these patients remain at risk for lifelong hypertension and should be closely monitored in this regard.

Selective cerebral extracorporeal circulation-enhanced total endovascular arch replacement using in situ fenestration.

In this article, we present a newly designed cerebral perfusion technique during the in situ fenestration procedure with three covered stent placement in an endovascular total aortic arch repair of a 68-year-old male patient. This technique enables the endovascular repair of the ascending aorta and aortic arch pathologies with commonly available thoracic aorta stent grafts in a safer and more effective manner.

Sex Related Anatomical Differences in Patients With Aortic Arch Pathology and Their Impact on the Feasibility of Double and Triple Branched Endografts.

OBJECTIVE: To evaluate sex based differences in ascending aorta and arch anatomy in patients with underlying proximal aortic disease and to evaluate their impact on feasibility for total endovascular repair with custom made, arch branched devices. METHODS: This was a retrospective cross-sectional review of all patients undergoing open and or total endovascular arch repair due to distal ascending aorta and or aortic arch pathologies in a single high volume aortic centre between 2012 and 2022. Anatomical ascending aorta and aortic arch parameters were analysed on a centreline of flow on a dedicated 3D workstation. Sex related differences of the ascending aorta, aortic arch, and supra-aortic vessels were evaluated. Subsequently, four endovascular devices were assessed for feasibility: double and triple branched devices both for the Zenith (Cook Medical) and Relay (Terumo Aortic) platforms, first in accordance with the instructions for use and then considering the possibility of adjunctive cervical debranching. The primary endpoints were sex specific differences in aortic anatomy, while secondary endpoints included sex based feasibility of branched endograft devices. RESULTS: During the study period, 395 patients underwent total aortic arch repair, of whom 152 (51 female, 33.5%) had high quality available computed tomography angiographies and were included in the study. Female patients had a shorter proximal landing zone than male patients (22 mm vs. 47 mm; p < .001). Left subclavian artery dissection was more frequent in men (24.8% vs. 3.9%; p < .001). Other anatomical parameters showed a similar distribution between sexes. Female patients presented a lower feasibility for double branched devices (35.3% vs. 58.4%; p = .015) as well as a tendency for lower feasibility rates for triple branched devices (31.4% vs. 47.5%; p = .081). CONCLUSION: Although most ascending aortic and arch parameters showed similar trends in both sexes, the availability of a suitable proximal landing zone was lower in female patients. Consequently, female patients had lower feasibility rates for double arch branched endografts and, to lesser extent, for triple arch branched endografts.

Frozen elephant use in type a dissection: fundamentals, innovations, and pitfalls.

INTRODUCTION: Type A acute aortic dissection (TA-AAD) is a great challenge for aortic surgeons. The establishment of a standardized surgical approach, particularly the determination of whether and when to address the aortic arch and the distal aorta in the same operation as the proximal aorta, is still unclear. AREAS COVERED: Frozen elephant trunk (FET) has emerged as a valuable treatment for TA-AAD over the last decade. Here, we discuss the fundamentals and pitfalls of frozen elephant trunk procedures and present the latest innovations. EXPERT OPINION: FET has the potential to simplify arch reconstruction in patients with complex arch tears and rupture, optimize perfusion in the distal true lumen for those with a compressed true lumen and malperfusion, address distal reentry tears, and promote false lumen thrombosis and late aortic remodeling. Nevertheless, FET is still associated with non-negligible mortality and morbidity rates. Patient selection, surgical expertise, and postoperative care remain crucial determinants in ensuring successful outcomes. Recent innovations in FET surgery involve the development of techniques to minimize or avoid hypothermic circulatory arrest and new FET devices with different arch branch configurations aiming to facilitate subsequent aortic reinterventions. We believe that both these advancements have the potential to improve patient outcomes.

Right-Sided Aortic Arch With Isolated Left Subclavian Artery, Subclavian Steal Syndrome, and Right Bronchial Compression: A Case Report.

A right-sided aortic arch with an isolated left subclavian artery represents a rare anatomical variant, posing diagnostic challenges and clinical complexities. Here, we present a case of a 14-year-old male presenting with respiratory symptoms, unveiling a right-sided aortic arch with an isolated left subclavian artery. Through detailed clinical evaluation, radiographic imaging, and diagnostic modalities including chest radiography, computed tomography angiography, ultrasound, and time-of-flight magnetic resonance angiography, the anatomical features and associated complications were delineated. The discussion encompasses embryological underpinnings, clinical manifestations, and therapeutic considerations, shedding light on the rarity and clinical implications of this anomaly.

Human Genetics of Semilunar Valve and Aortic Arch Anomalies.

Lesions of the semilunar valve and the aortic arch can occur either in isolation or as part of well-described clinical syndromes. The polygenic cause of calcific aortic valve disease will be discussed including the key role of NOTCH1 mutations. In addition, the complex trait of bicuspid aortic valve disease will be outlined, both in sporadic/familial cases and in the context of associated syndromes, such as Alagille, Williams, and Kabuki syndromes. Aortic arch abnormalities particularly coarctation of the aorta and interrupted aortic arch, including their association with syndromes such as Turner and 22q11 deletion, respectively, are also discussed. Finally, the genetic basis of congenital pulmonary valve stenosis is summarized, with particular note to Ras-/mitogen-activated protein kinase (Ras/MAPK) pathway syndromes and other less common associations, such as Holt-Oram syndrome.

Prevalence of carotid artery stenosis and intra-cranial lesions in patients with aortic arch aneurysm and its association with intraoperative regional cerebral oxygen saturation and postoperative neurological outcomes.

Background: Although aortic aneurysm is associated with vascular aging and atherosclerosis, carotid and intracranial vascular disease prevalence in patients with aortic arch aneurysm remains unclear. Similarly, the effect of carotid and intracranial lesions on postoperative outcomes is unknown. This study aimed to investigate the prevalence of carotid artery stenosis and intracranial lesions in patients with aortic arch aneurysm and its association with intraoperative regional cerebral oxygen saturation (rScO2) and postoperative neurological outcomes, including delirium and cerebral infarction. Methods: This retrospective observational study included 133 patients with true aortic arch aneurysm who underwent preoperative magnetic resonance imaging (MRI). We evaluated the prevalence of carotid and intracranial arterial lesions. Symptomatic cerebral infarction and delirium, defined by the confusion assessment method for the intensive care unit, were evaluated for their association with preoperative cerebrovascular lesions. Additionally, changes in regional saturation of the cerebral tissue at different surgical phases were evaluated for patients with and without cerebrovascular lesions. Results: Fifteen (11.3%) patients experienced symptomatic cerebral infarction, and 64 (48.1%) had postoperative delirium. Preoperative MRI showed old infarction, microbleeds, significant carotid artery stenosis, and intracranial lesions in 21.1%, 14.3%, 10.5%, and 7.5% of the patients, respectively. White matter hyperintensities with Fazekas scale 2 were observed in 40.6% of the patients, while Fazekas scale 3 were observed in 18.8% of the patients. Preoperative MRI findings and postoperative neurological outcomes were not significantly different. Seventy-six patients underwent rScO2 monitoring intraoperatively. Changes in rScO2 in patients with and without carotid/cerebrovascular lesions were not significantly different. However, rScO2 was significantly lower in patients who developed cerebral infarction. Conclusions: Significant carotid artery stenosis and intracranial lesions were observed in 10.5% and 7.5% of the patients, respectively. Although preoperative MRI findings and changes in rScO2 or postoperative outcomes showed no significant association, patients with postoperative cerebral infarction showed significantly lower rScO2 intraoperatively.

Clinical outcomes of the type II hybrid procedure for the repair of extensive aortic arch pathology.

Background: Type II hybrid arch repair (HAR) has been used for the repair of extensive aortic arch pathology. The aim of this study was to retrospectively analyze single-stage hybrid treatment involving replacement of the ascending aorta, arch debranching, and zone 0 stent graft deployment. Methods: We retrospectively analyzed clinical data from 41 patients with acute and chronic aortic disease who underwent a type II hybrid arch procedure at Beijing Anzhen Hospital and Beijing Chaoyang Hospital from January 2020 to August 2022. The femoral arteries and right axillary arteries were used as cannulation sites to decrease the risk of malperfusion. During surgery, the nasopharyngeal temperature was lowered to 30 ℃. Demographic, perioperative, and late results data were retrieved and analyzed. Results: The mean age of the patients was 54.9±11.1 years, and 31 patients (75.6%) were men. In all cases, zone 0 stent graft deployment was successful, with no in-hospital mortality. The median follow-up time was 10.5 [interquartile range (IQR), 4.8-17.6] months, and the survival rate was 94.9% during follow-up. Complications included cerebral infarction (3 patients, 7.3%) and renal failure requiring dialysis (3 patients, 7.3%). There were no occurrences of paraplegia, and no stent-related complications occurred during the follow-up period. Conclusions: The single-stage hybrid arch procedure achieved satisfactory early results and represents a less invasive approach for treating complex diffuse aortic disease that affects the arch. This strategy is an important technical advance in the treatment of high-risk patients with extensive aortic arch pathology.

Right-Sided Aortic Arch: A Computed Tomography Angiography Investigation, A Systematic Review with Meta-Analysis.

Background/Objectives: The right-sided aortic arch (RAA) is an uncommon variation of the aortic arch (AA), characterized by the aorta crossing over the right main bronchus. In the RAA, the descending aorta can be found on either the right or left side of the spine. The current study comprises a comprehensive retrospective computed tomography angiography (CTA) investigation into the prevalence of the RAA within the Greek population. Additionally, we will conduct a systematic review and meta-analysis to elucidate both common and rare morphological variants of the RAA. This research is significant as it sheds light on the prevalence and characteristics of the RAA in a specific population, providing valuable insights for clinical practice. Methods: Two hundred CTAs were meticulously investigated for the presence of a RAA. In addition, the PubMed, Google Scholar, and Scopus online databases were thoroughly searched for studies referring to the AA morphology. The R programming language and RStudio were used for the pooled prevalence meta-analysis, while several subgroup analyses were conducted. Results: Original study: A unique case of 200 CTAs (0.5%) was identified with an uncommon morphology. The following branches emanated from the RAA under the sequence: the right subclavian artery (RSA), the right common carotid artery (RCCA), the left common carotid artery (LCCA), and the left vertebral artery (LVA) in common origin with the aberrant left subclavian artery (ALSA). The ALSA originated from a diverticulum (of Kommerell) and followed a retroesophageal course. Systematic Review and Meta-Analysis: Sixty-two studies (72,187 total cases) met the inclusion criteria. The pooled prevalence of the RAA with a mirror-image morphology was estimated at 0.07%, and the RAA with an ALSA was estimated at <0.01%. Conclusions: AA anomalies, specifically the RAA, raise clinical interest due to their coexistence with developmental heart anomalies and possible interventional complications. Congenital heart anomalies, such as the Tetralogy of Fallot and patent foramen ovale, coexisted with RAA mirror-image morphology.

Minimally Invasive Approach for Replacement of the Ascending Aorta towards the Proximal Aortic Arch.

Objectives: In recent years, minimally invasive approaches have been used with increasing frequency, even for more complex aortic procedures. However, evidence on the practicability and safety of expanding minimally invasive techniques from isolated operations of the ascending aorta towards more complex operations such as the hemiarch replacement is still scarce to date. Methods: A total of 86 patients undergoing elective surgical replacement of the ascending aorta with (n = 40) or without (n = 46) concomitant proximal aortic arch replacement between 2009 and 2023 were analyzed in a retrospective single-center analysis. Groups were compared regarding operation times, intra- and postoperative complications and long-term survival. Results: Operation times and ventilation times were significantly longer in the hemiarch replacement group. Despite this, no statistically significant differences between the two groups were observed for the duration of the ICU and hospital stay and postoperative complication rates. At ten-year follow-up, overall survival was 82.6% after isolated ascending aorta replacement and 86.3% after hemiarch replacement (p = 0.441). Conclusions: Expanding the indication for minimally invasive aortic surgery towards the proximal aortic arch resulted in comparable postoperative complication rates, length of hospital stay and overall long-term survival compared to the well-established minimally invasive isolated supracommissural ascending aorta replacement.

Molecular Pathways and Animal Models of Semilunar Valve and Aortic Arch Anomalies.

The great arteries of the vertebrate carry blood from the heart to the systemic circulation and are derived from the pharyngeal arch arteries. In higher vertebrates, the pharyngeal arch arteries are a symmetrical series of blood vessels that rapidly remodel during development to become the asymmetric aortic arch arteries carrying oxygenated blood from the left ventricle via the outflow tract. At the base of the aorta, as well as the pulmonary trunk, are the semilunar valves. These valves each have three leaflets and prevent the backflow of blood into the heart. During development, the process of aortic arch and valve formation may go wrong, resulting in cardiovascular defects, and these may, at least in part, be caused by genetic mutations. In this chapter, we will review models harboring genetic mutations that result in cardiovascular defects affecting the great arteries and the semilunar valves.

Aortic arch de-branching for suspected expanding perigraft haematoma after previous acute type-A dissection repair with AMDS stent: a technique for a potential future problem.

BACKGROUND: Acute Stanford type- A aortic dissections make up a large part of emergency cardiac surgery. They also carry a significant burden of morbidity. New techniques to aid aortic remodelling include the Ascyrus Medical Dissection Stent (AMDS): Its increasing use, looks to present a potential problem in cases where surgery involving the aortic arch may be required. CASE REPORT: We present the case of a 49-year-old male who underwent urgent redo-surgery for total arch replacement and de-branching following recent replacement of the ascending aorta for acute type-A dissection, where an AMDS stent was deployed. The patient underwent total arch replacement with a stented tri-furcate prosthesis and de-branching of arch vessels with the stent landed inside the previous AMDS, to good effect. CONCLUSION: This case highlights a possible approach to aortic arch surgery in patients who have previous had AMDS insertion.

E-vita OPEN NEO in the treatment of acute or chronic aortic pathologies: first interim results of the NEOS study.

OBJECTIVES: The aim of this multicentre study was to demonstrate the safety and clinical performance of E-vita OPEN NEO Stent Graft System (Artivion, Inc.) in the treatment of aneurysm or dissection, both acute and chronic, in the ascending aorta, aortic arch and descending thoracic aorta. METHODS: In this observational study of 12 centres performed in Europe and in Asia patients were enrolled between December 2020 and March 2022. All patients underwent frozen elephant trunk using E-vita OPEN NEO Stent Graft System. Primary end point was the rate of all-cause mortality at 30 days and secondary end points included further clinical and safety data are reported up to 3-6 months postoperatively. RESULTS: A total of 100 patients (66.7% male; mean age, 57.7 years) were enrolled at 12 sites. A total of 99 patients underwent surgery using the E-vita OPEN NEO for acute or subacute type A aortic dissection (n = 37), chronic type A aortic dissection (n = 33) or thoracic aortic aneurysm (n = 29), while 1 patient did not undergo surgery. Device technical success at 24 h was achieved in 97.0%. At discharge, new disabling stroke occurred in 4.4%, while new paraplegia and new paraparesis was reported in 2.2% and 2.2%, respectively. Renal failure requiring permanent (>90 days) dialysis or hemofiltration at discharge was observed in 3.3% of patients. Between discharge and the 3-6 months visit, no patients experienced new disabling stroke, new paraplegia or new paraparesis. The 30-day mortality was 5.1% and the estimated 6-month survival rate was 91.6% (standard deviation: 2.9). CONCLUSIONS: Total arch replacement with the E-vita OPEN NEO can be performed with excellent results in both the acute and chronic setting. This indicates that E-vita OPEN NEO can be used safely, including in the setting of acute type A aortic dissection.

Anatomical Feasibility of the "Canaud Technique" for Physician-Modified Thoracic Endovascular Grafts for the Treatment of Aortic Arch Disease.

PURPOSE: Thoracic endovascular aortic repair (TEVAR), originally conceived for deployment distal to the left subclavian artery (LSA), has been recently extended to more proximal landing zones. Among total endovascular solutions, the "Canaud technique" for Physician-Modified Thoracic Endovascular Grafts (C-PMEGs) has gained acceptance with good early results. The aim of this study is to report the proportion of patients with zone 0, 1, and 2 aortic arch lesions that could theoretically be treated with a C-PMEG. MATERIALS AND METHODS: Computed tomography angiography (CTA) of all consecutive patient candidates to open, hybrid, or endovascular arch repair from January 2009 to July 2023 at our Institution were analyzed. The assessment of feasibility of C-PEMG was conducted following previously extensively described anatomical criteria. RESULTS: During the study period, 209 consecutive patients were candidates for an open, hybrid, or endovascular aortic arch repair. Of them, 164 patients had a preoperative CTA scan eligible for analysis. One hundred twenty-six patients were male (76.8 %), with a mean age of 67 ± 10.8 years. Ninety-four patients (57.3%) were affected by atherosclerotic aneurysms, 48 post-dissecting aneurysms (29.3%) and 22 penetrating aortic ulcers (PAUs) (13.4%). Twenty patients (12.2%) were suitable for double-fenestrated C-PMEG, and 34 patients (20.7%) for single-fenestrated C-PMEG, totaling 32.9% of the entire cohort. The combination of supra-aortic trunks (SAT) bypass or the use of covered stent in IA or left common carotid artery (LCCA) as adjunctive maneuvers, would extend the C-PMEGs feasibility to 69 patients (42.1%). The use of an iliac graft conduit in 3 (1.8%) patients with inadequate ileo-femoral accesses would increase the C-PMEG suitability to 72 patients (43.9%). The main reason for exclusion was excessive ascending aortic diameter in 78 patients (47.6%). CONCLUSIONS: The use of C-PMEG was shown to be theoretically feasible in a percentage of patients ranging from 32.9 to 43.9% affected by aortic arch aneurysms, dissections, and PAU. The main reason for exclusion was the dimension of the ascending aorta. The C-PMEG technique is a viable option in aortic arch lesions endovascular treatment. An off-the-shelf device with similar characteristics could also be used in emergency, avoiding the limitations of physician modifications (such as time for back-table procedure, sterility, off-label use). CLINICAL IMPACT: Thoracic endovascular aortic repair (TEVAR) has been extended to more proximal landing zones for the treatment of aortic arch aneurysm, dissections, penetrating aortic ulcers. Among total endovascular solutions, the "Canaud technique" for Physician-Modified Thoracic Endovascular Grafts (C-PMEGs) has gained acceptance with good early results. The anatomical feasibility in 164 consecutive patients was 69 patients (42.1%), with the combination of supra-aortic trunks (SAT) bypass or the use of covered stent in IA or left common carotid artery (LCCA) as adjunctive maneuvers, showing that the C-PMEG technique is a viable option in aortic arch lesions endovascular treatment.