TY - JOUR
T1 - Aortic treatment in connective tissue disease
AU - Tinelli, Giovanni
AU - Ferraresi, Marco
AU - Watkins, Amelia C.
AU - Hertault, Adrien
AU - Soler, Raphael
AU - Azzaoui, Richard
AU - Fabre, Dominique
AU - Sobocinski, Jonathan
AU - Haulon, Stéphan
PY - 2019
Y1 - 2019
N2 - Connective tissue disease (CTD) represents a group of genetic conditions characterized by disruptive matrix remodeling. When this process involves aortic and vascular wall, patients with CTD have a high risk of developing arterial aneurysms, dissections and ruptures. Open surgical repair is still the gold standard therapy for patients with CTD with reasonable morbidity and mortality risk. The surgical treatment of CTD often requires multiple operations. In the endovascular era, fenestrated and branched stent grafts may play a role in reducing the complications of multiple open operations. Although the long-term results of endovascular treatment in the setting of CTD are unknown, it is generally accepted that endovascular treatment is restricted to selected patients with high surgical risk. In an emergency setting, endovascular intervention can serve as a lifesaving bridge to elective open aortic repair. Aortic centers performing a large volume of complex open and endovascular aortic repairs have started to combine these two techniques in a staged fashion. The goal is to reduce the morbidity and mortality associated with extensive aortic repairs in CTD patients. For this reason, recommend endovascular therapy when a “graft-to-graft” approach is possible. In this scenario, the surgeon who performs the open repair must take into consideration future interventions. Surgical repair in any aortic segment should allow creation of proximal and distal landing zones over 4 cm to secure the sealing of a future stent graft. Connective tissue disease should be treated with a multidisciplinary approach, in high volume centers. Endovascular treatment represents a potential option in patients at high risk for open repair. Staged hybrid procedures have emerged as a way to reduce spinal cord ischemia and avoid multiple open surgeries. The aim of this article is to discuss the management of aortic diseases in CTD, focusing on to the role of standard open surgery and emerging endovascular treatment, and to give an overview of the few series published regarding this topic with a small number of patients.
AB - Connective tissue disease (CTD) represents a group of genetic conditions characterized by disruptive matrix remodeling. When this process involves aortic and vascular wall, patients with CTD have a high risk of developing arterial aneurysms, dissections and ruptures. Open surgical repair is still the gold standard therapy for patients with CTD with reasonable morbidity and mortality risk. The surgical treatment of CTD often requires multiple operations. In the endovascular era, fenestrated and branched stent grafts may play a role in reducing the complications of multiple open operations. Although the long-term results of endovascular treatment in the setting of CTD are unknown, it is generally accepted that endovascular treatment is restricted to selected patients with high surgical risk. In an emergency setting, endovascular intervention can serve as a lifesaving bridge to elective open aortic repair. Aortic centers performing a large volume of complex open and endovascular aortic repairs have started to combine these two techniques in a staged fashion. The goal is to reduce the morbidity and mortality associated with extensive aortic repairs in CTD patients. For this reason, recommend endovascular therapy when a “graft-to-graft” approach is possible. In this scenario, the surgeon who performs the open repair must take into consideration future interventions. Surgical repair in any aortic segment should allow creation of proximal and distal landing zones over 4 cm to secure the sealing of a future stent graft. Connective tissue disease should be treated with a multidisciplinary approach, in high volume centers. Endovascular treatment represents a potential option in patients at high risk for open repair. Staged hybrid procedures have emerged as a way to reduce spinal cord ischemia and avoid multiple open surgeries. The aim of this article is to discuss the management of aortic diseases in CTD, focusing on to the role of standard open surgery and emerging endovascular treatment, and to give an overview of the few series published regarding this topic with a small number of patients.
KW - Aortic Aneurysm
KW - Aortic treatment
KW - Connective Tissue Diseases
KW - Connective tissue diseases
KW - Ehlers-Danlos Syndrome
KW - Endovascular Procedures
KW - Humans
KW - Marfan Syndrome
KW - Vascular surgical procedures
KW - Aortic Aneurysm
KW - Aortic treatment
KW - Connective Tissue Diseases
KW - Connective tissue diseases
KW - Ehlers-Danlos Syndrome
KW - Endovascular Procedures
KW - Humans
KW - Marfan Syndrome
KW - Vascular surgical procedures
UR - http://hdl.handle.net/10807/149960
UR - https://www.minervamedica.it/it/riviste/cardiovascular-surgery/articolo.php?cod=r37y2019n04a0518
U2 - 10.23736/S0021-9509.18.10443-5
DO - 10.23736/S0021-9509.18.10443-5
M3 - Article
SN - 0021-9509
VL - 60
SP - 518
EP - 525
JO - Journal of Cardiovascular Surgery
JF - Journal of Cardiovascular Surgery
ER -