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Over the last decades, improvement of medical and surgical therapy has increased life expectancy in Marfan patients. Consequently, the number of such patients requiring secondary interventions on the descending thoracic aorta due to new or residual dissections, and distal aneurysm formation has substantially enlarged. Surgical and endovascular proc...
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Objectives:
This study aimed to investigate the morphological characteristics of the dissected thoracic aorta and brachiocephalic arteries within the Chinese population.
Methods:
A retrospective analysis of computed tomography scans of 387 patients with acute Type A aortic dissection was carried out. The dimensions of the thoracic aorta at multi...
Medicine and engineering are in collaboration to assist in the tackling of daunting surgical techniques which are associated with high rates of morbidity and mortality, in exchange for minimally invasive approaches with lower procedural risk. Endovascular procedures in general have already reduced the risk of surgery by limiting the extent of open...
Background: Outcomes of early-onset acute type A aortic dissection (ATAAD) associated with Marfan syndrome (MFS) are known, but not with other etiologies.
Methods and Results: ATAAD patients from 2 centers (n=1,001) were divided into 2 groups: age ≤45 years (n=93) and age >45 years (n=908). Although in-hospital death and 10-year survival were simil...
Citations
... The current recommendations are to practice open descending aortic surgery for complicated AD, and reserve thoracic endovascular aortic repair (TEVAR) in case of aortic rupture based on the higher risk of post-operative complications and of mid-and long-term reoperation for aneurysmal evolution and aortic rupture after TEVAR [2,3]. Such complications are caused either by a progression of the parietal disease or by the appearance of new entry tears [4][5][6]. These current recommendations are based on dated retrospective studies and the progress observed in recent years in endovascular techniques has changed the viewpoint of modern vascular surgery [7][8][9] and currently, many teams offer endovascular treatment as a first-line therapy to these high surgical risk patients [4,10]. ...
... Such complications are caused either by a progression of the parietal disease or by the appearance of new entry tears [4][5][6]. These current recommendations are based on dated retrospective studies and the progress observed in recent years in endovascular techniques has changed the viewpoint of modern vascular surgery [7][8][9] and currently, many teams offer endovascular treatment as a first-line therapy to these high surgical risk patients [4,10]. ...
Objectives: Aortic dissection in patients with Marfan and related syndromes (HTAD) is a serious pathology whose treatment by thoracic endovascular repair (TEVAR) is still under debate. The aim of this study was to assess the results of the TEVAR for aortic dissection in patients with HTAD as compared to a young population without HTAD. Methods: The study received the proper ethical oversight. We performed an observational exposed (confirmed HTAD) vs. non-exposed ( 10% was observed in ten cases (58.8%) in the HTAD group and in five cases (22.7%) in the non-HTAD group (p = 0.022). FL thrombosis was achieved in nine cases (52.9%) in the HTAD group vs. twenty (90.9%) cases in the non-HTAD group (p < 0.01). The risk factors for unfavorable anatomical evolution were male gender and the STABILISE technique. With a linear model, we observed a significantly different aortic volume evolution between the two groups (p < 0.01) with the STABILISE technique; this statistical difference was not found in the TEVAR subgroup. In the HTAD patients, there was a significant difference in the total aortic volume evolution progression between the patients treated with the STABILISE technique and the patients treated with TEVAR (+160.1 ± 52.3% vs. +47 ± 22.5%, p < 0.01 and +189.5 ± 92.5% vs. +58.6 ± 34.8%, p < 0.01 at 1 year and at the end of follow-up, respectively). Conclusions: TEVAR in the HTAD patients seemed to be associated with poorer anatomical outcomes at 1 year. This result was strongly related to the STABILISE technique which should be considered with care in these specific patients.
... [1][2][3] The ability to achieve an early and specific genetic diagnosis, better medical treatment, and refined management of the proximal aorta has improved life expectancy for patients with CTD. 4 As a result, there is an increasing need for treatment of disease developing in the remaining aortic segments. [5][6][7] For non-CTD-related aortic disease, endovascular repair has emerged as the most common option for treatment both in the thoracic and abdominal aorta. 8,9 Patients undergoing endovascular repairs have shorter hospital stays and lower perioperative mortality compared with patients undergoing open repairs. ...
IMPORTANCE Endovascular treatment is not recommended for aortic pathologies in patients with connective tissue diseases (CTDs) other than in redo operations and as bridging procedures in emergencies. However, recent developments in endovascular technology may challenge this dogma.
OBJECTIVE To assess the midterm outcomes of endovascular aortic repair in patients with CTD.
DESIGN, SETTING, AND PARTICIPANTS For this descriptive retrospective study, data on demographics, interventions, and short-term and midterm outcomes were collected from 18 aortic centers in Europe, Asia, North America, and New Zealand. Patients with CTD who had undergone endovascular aortic repair from 2005 to 2020 were included. Data were analyzed from December 2021 to November 2022. EXPOSURE All principal endovascular aortic repairs, including redo surgery and complex repairs of the aortic arch and visceral aorta. MAIN OUTCOMES AND MEASURES Short-term and midterm survival, rates of secondary procedures, and conversion to open repair. RESULTS In total, 171 patients were included: 142 with Marfan syndrome, 17 with Loeys-Dietz syndrome, and 12 with vascular Ehlers-Danlos syndrome (vEDS). Median (IQR) age was 49.9 years (37.9-59.0), and 107 patients (62.6%) were male. One hundred fifty-two (88.9%) were treated for aortic dissections and 19 (11.1%) for degenerative aneurysms. One hundred thirty-six patients (79.5%) had undergone open aortic surgery before the index endovascular repair. In 74 patients (43.3%), arch and/or visceral branches were included in the repair. Primary technical success was achieved in 168 patients (98.2%), and 30-day mortality was 2.9% (5 patients). Survival at 1 and 5 years was 96.2% and 80.6% for Marfan syndrome, 93.8% and 85.2% for Loeys-Dietz syndrome, and 75.0% and 43.8% for vEDS, respectively. After a median (IQR) follow-up of 4.7 years (1.9-9.2), 91 patients (53.2%) had undergone secondary procedures, of which 14 (8.2%) were open conversions.
CONCLUSIONS AND RELEVANCE This study found that endovascular aortic interventions, including redo procedures and complex repairs of the aortic arch and visceral aorta, in patients with CTD had a high rate of early technical success, low perioperative mortality, and a midterm survival rate comparable with reports of open aortic surgery in patients with CTD. The rate of secondary procedures was high, but few patients required conversion to open repair. Improvements in devices and techniques, as well as ongoing follow-up, may result in endovascular treatment for patients with CTD being included in guideline recommendations.
... Thus, a greater proportion of HCTD patients are likely to undergo more than one surgical procedure during their lifetime. 27 ...
Background
Patients with connective tissue diseases are at high lifetime risk of developing thoracic aortic aneurysms (TAAs) due to defects in extracellular matrix composition which compromise the structural integrity of the aortic wall. It is vital to identify and manage aneurysms early to prevent fatal complications such as dissection or rupture.
Method
This review synthesises information obtained from a thorough literature search regarding the pathophysiology of TAAs in those with heritable connective tissue diseases (HCTDs), the investigations for timely diagnosis and current operative strategies.
Results
Major complications of open repair (OR) include pneumonia (32%), haemorrhage (31%) and tracheostomy (18%), with a minor risk of vocal cord paresis (9%). For thoracic endovascular aortic repair (TEVAR), high rates of endoleak were documented (38‐66.6%). Reintervention rates for TEVAR are also high at 38‐44%. Mortality rates were documented as 25% for open repair and vary from 14% to 44% for TEVAR.
Conclusion
OR remains the mainstay of surgical management. While TEVAR use is expanding, it remains the alternative choice due to concerns over endograft durability, limited long‐term outcome data and the lack of high‐quality evidence regarding its use in HCTD patients.
... These benefits are largely lost in the long term, however, as patients treated endovascularly require more reinterventions and have a higher risk of secondary AAA rupture [12,13]. In TAA, while the rates of paraplegia and stroke with EVAR appear lower than those with OSR, other complications such as endoleaks raise concerns, particularly in the case of connective tissue syndromes where EVAR appears insufficient [4,14]. Further, many patients may not be surgical candidates due to co-morbidities. ...
Aortic aneurysm (AA) is a complex and dangerous vascular disease, featuring progressive and irreversible vessel dilatation. AA is typically detected either by screening, or identified incidentally through imaging studies. To date, no effective pharmacological therapies have been identified for clinical AA management, and either endovascular repair or open surgery remains the only option capable of preventing aneurysm rupture. In recent years, multiple research groups have endeavored to both identify noncoding RNAs and to clarify their function in vascular diseases, including aneurysmal pathologies. Notably, the molecular roles of noncoding RNAs in AA development appear to vary significantly between thoracic aortic aneurysms (TAAs) and abdominal aortic aneurysms (AAAs). Some microRNAs (miRNA - a non-coding RNA subspecies) appear to contribute to AA pathophysiology, with some showing major potential for use as biomarkers or as therapeutic targets. Studies of long noncoding RNAs (lncRNAs) are more limited, and their specific contributions to disease development and progression largely remain unexplored. This review aims to summarize and discuss the most current data on lncRNAs and their mediation of AA pathophysiology.
... Several recent studies have focused on the use of endovascular stenting in patients with MFS. [7][8][9][10][11][12][13][14][15] Parisi et al. 16 noted the technical feasibility of endovascular repair in these patients, quoting success rates approaching 100% and associated low early in-hospital morbidity and mortality rates. However, an analysis of mid-to long-term follow-up data revealed a high reintervention rate secondary to primary and secondary endoleaks and a low rate of positive aortic remodeling. ...
Endovascular repair of thoracic aortic diseases can provide satisfactory outcomes in elective and certain emergency cases involving the descending thoracic and aortic arch. However, open repair remains the gold standard method of aortic root pathologies and certain aortic arch pathologies, such as extended dissection. Nevertheless, the use of endovascular stenting in patients with connective tissue disorders has not been fully explored because the aortic tissues are fragile and the likelihood of keeping the stent in place is low because of its progressive dilatation and subsequent requirement for open repair at a later stage when the stent-graft fails. Our brief review focuses on current evidence of the use of stents in patients with connective tissue disorders and whether such practice can be expanded further.
... Thoracic and abdominal EVAR remains controversial in patients with CTD due to their disproportionately high rates of postoperative complications, reintervention, and mortality after EVAR [33,36,37]. Although early morbidity and mortality rates are comparable for patients with or without MFS after endovascular repair of the DTA for acute or chronic dissection, rates of failure due to endoleak, new dissection, open conversion, and late death are higher in patients with MFS [6•, 34]. ...
... Although type II endoleak is the most common type of endoleak in all patients, type I endoleak is most common in MFS. New aortic dissection, such as retrograde type A aortic dissection (RTAD) and stent-graft-induced new entry tear (SINE), is an additional postoperative complication disproportionately affecting patients with MFS [33,34,36,37,72,73]. Both are consequences of aortic wall fragility, increased shear stress by the stent-graft, and angulation of the aorta when a distal stent-graft is over-sized or short [33,34,[73][74][75][76]. ...
Marfan syndrome is a heritable multisystem connective tissue disease and is the most common genetic cause of aortic disease. Guidelines for surgical repair and recommendations for surveillance imaging aim to prevent aneurysm rupture, aortic dissection, and death. Options for proximal aorta repair include the modified Bentall procedure and valve-sparing aortic root repairs. Hemiarch and total arch replacement are options for aortic arch repair, and the elephant trunk procedure is a special two-staged total arch repair facilitating future descending aorta repair. Endovascular repair is not currently considered an acceptable long-term treatment option in Marfan syndrome but may be performed when open repair is not feasible or in the acute setting as a bridge to definitive surgical treatment. After an initial surgery, patients remain at risk for new aortic dissection, propagation of a pre-existing aortic dissection, and new or enlarging aortic aneurysm. Anastomotic pseudoaneurysm is a potential postsurgical complication at multiple sites including proximal and distal aortic anastomoses, coronary anastomoses, and intercostal and visceral artery patches. Patients undergoing endovascular repair are at increased risk of endoleak and aortic dissection. CT and MR angiography are the main imaging modalities for surveillance of the aorta in Marfan syndrome and are capable of demonstrating these complications, as well as expected postsurgical appearances. 4D flow MRI is an emerging technique providing hemodynamic information that may risk-stratify aneurysms better than size alone and help predict future aortic events.
... In principle, endovascular techniques are not intended for the treatment of aortic or vascular conditions in patients with genetic connective tissue disease. Despite the highly restrictive use of endovascular technology in genetically linked conditions, more than 69 cases have been published (1), with the impression that endovascular treatment may have a place as life-saving bridging procedure until a definitive open repair can be performed, while elective endovascular management is not established. ...
... In conclusion, TEVAR was deemed viable as a bridging method due to the low periprocedural mortality rate. A 2015 review conducted by Parisi et al. [34] summarized the largest published series in table form (. Table 2 ...
Background
The most important structural proteins of the vascular wall are collagen and elastin. Genetically linked connective tissue diseases lead to degeneration, aneurysm formation and spontaneous dissection or rupture of arteries. The most well-known are Marfan syndrome, vascular Ehlers-Danlos syndrome (type IV), Loeys-Dietz syndrome and familial aortic aneurysms and dissections.
Objective
This review article addresses the current status of endovascular treatment options for important connective tissue diseases.
Material and methods
Evaluation of currently available randomized studies and registry data.
Results
The treatment of choice for patients that are mostly affected at a young age is primarily conservative or open repair. There is only limited evidence for endovascular aortic repair (EVAR) of abdominal aneurysms or thoracic endovascular aortic repair (TEVAR).
Conclusion
The progression of the disease with dilatation leads to secondary endoleaks and high reintervention rates with uncertain long-term results. For this reason, there is currently consensus that EVAR and TEVAR should be limited to justified exceptional cases and emergency situations in patients with genetically linked aortic diseases.
... In einem aktuellen Review von Parisi et al. [34] aus dem Jahr 2015 werden die größten publizierten Serien tabellarisch zusammengefasst (. Tab. 2 und 3). ...
FragestellungWelchen Stellenwert haben endovaskuläre Behandlungsoptionen in der Therapie aortaler Pathologien auf dem Boden von Bindegewebserkrankungen. Material und MethodenAuswertung aktueller randomisierter Untersuchungen und Registerstudien. ErgebnisDie Therapie der Wahl der meist bereits in jungem Alter betroffenen Patienten ist primär konservativ bzw. offen-chirurgisch. Zur endovaskulären Therapie abdomineller Aneurysmen (EVAR) oder thorakalen Aortenpathologien (TEVAR) gibt es nur wenig Evidenz. SchlussfolgerungenDie Progression der genetisch bedingten Grunderkrankung mit konsekutiver Aortendilatation führt zu sekundären Endoleckagen und vermehrten Reinterventionen mit unsicherem Langzeitergebnis. Deshalb besteht derzeit Konsensus, dass EVAR und TEVAR bei genetisch bedingten Aortenerkrankungen auf begründete Ausnahmefälle und Notfallsituationen begrenzt sein sollten.
