Endovascular Thoracoabdominal Replacement after Total Abdominal Aortic Debranching.

Marfan syndrome is an inherited connective tissue disorder affecting mainly eyes and skeletal and cardiovascular systems. Cardiovascular involvement may lead to life-threatening aortic pathologies including aneurysms and/or dissections. In this report, the authors present images of a patient with Marfan syndrome with a history of Bentall-De Bono procedure followed by aortic arch and infrarenal aortoiliac replacements who strongly refused conventional open repair and underwent abdominal debranching followed by thoracoabdominal endovascular stent grafting for the treatment of thoracoabdominal aneurysm. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Marfan syndrome is a worldwide distributed, well-known autosomal dominant inherited connective tissue disorder affecting mainly the eyes and skeletal and cardiovascular systems. Cardiovascular involvement may lead to life-threatening aortic pathologies including aneurysms and/or dissections. 1

A 27-year-old male Marfan syndrome patient with a history of Bentall-De Bono, aortic arch, and infrarenal abdominal aortic replacements was referred with the diagnosis of giant thoracoabdominal dissecting aneurysm. Treatment options, especially the benefits of conventional thoracoabdominal replacement in this young patient with the particular connective tissue disorder, were explained in detail and discussed with the patient; however, he strongly refused the standard therapy. The alternative, that is, total abdominal debranching followed by endoluminal stenting was the other option and he wanted to receive this therapy despite similar risks of paraplegia with open surgery.

He underwent abdominal aortic debranching (from right iliac artery to the right renal artery and right iliac artery to left renal, superior mesenteric, and celiac artery) followed by endovascular thoracoabdominal stent graft implantation between the aortic arch and infrarenal abdominal aortic grafts, for the treatment of the pathology ( Fig. 1 , Video 1 ). There was no significant blood loss and no blood or blood product transfusion was required.

Fig. 1

Aortic debranching (right iliac artery to right renal artery and right iliac artery to celiac, superior mesenteric and left renal artery) followed by thoracoabdominal endovascular stent grafting.

Video 1

Cine of the postoperative control computed tomography angiography. Online content is viewable at: https://www.thieme-connect.com/products/ejournals/html/10.1055/s-0038-1641607 .

Conventional thoracoabdominal replacement carries serious mortality and morbidity risks including visceral organ dysfunction, paraplegia, and consequences of cardiopulmonary bypass. 2 Although our treatment strategy offered a less invasive alternative to surgical thoracoabdominal aortic replacement, the current article presents only the images of our technique and we did not aim to propose visceral debranching followed by endovascular thoracoabdominal stent graft implantation as a new and safe alternative to open surgery. This is certainly a very controversial topic of treating very young patients with known connective tissue disorders in an endovascular fashion. Literature includes case reports and very small series. 3 However, usually these patients are older and have prohibitive risk for an open procedure. In a young man of 27 years, this may be accepted as borderline. There is risk of paraplegia no matter which approach, that is, endovascular or open, is chosen for the entire aortic replacement and most endovascular series still assure the use of all adjuncts to ameliorate this risk. 4 A hybrid approach is not necessarily a lower risk surgery than a thoracoabdominal approach as demonstrated in experiences of others on hybrid techniques. 5 We believe debranching followed by endoluminal stent graft implantation is a possible treatment option for thoracoabdominal aortic aneurysms; however, it should be considered in selected patients with certain comorbidity factors as well as in whom open techniques are strongly rejected.