Japanese Journal of Cardiovascular Surgery  2016;45(6):290-294

doi:10.4326/jjcvs.45.290

Hybrid-Procedure for the Treatment of Thoraco-abdominal Dissecting Aneurysm of the Aorta in a Patient with Marfan Syndrome

Naoya Sakoda ; Keiji Yunoki ; Shigeru Hattori ; Gaku Uchino ; Takuya Kawabata ; Munehiro Saiki ; Yasuhumi Fujita ; Kunikazu Hisamochi ; Hideo Yoshida

Keywords

Marfan syndrome; extended thoraco-abdominal aneurysm; debranch TEVAR; chimney; dissecting aneurysm of the aorta

Country

Japan

Language

Japanese

Abstract

Endovascular treatment for chronic aortic dissection in patients with Marfan syndrome is still controversial. A 60-year-old man developed an extended chronic type B dissection involving the aortic arch and thoraco-abdominal aorta with a large entry at the distal aortic arch and patent false lumen. He had undergone David procedure for type A aortic dissection at age 42, and aortic valve replacement for recurrent aortic valve insufficiency at 58, which was complicated with mediastinitis. He also suffered drug-induced interstitial pneumonitis. Considering his complicated surgical history and impaired pulmonary function, conventional graft replacement of thoraco-abdominal aorta was thought to be quite a high risk. Thus, we chose debranch TEVAR with a staged approach. First, debranching and Zone 0 TEVAR with the chimney technique were performed. Then, 4 months later, abdominal debranching and TEVAR was performed. The patient tolerated both procedures well and was discharged home. Two years after last procedure, he is in good condition and computed tomography shows that complete entry closure and false lumen had thrombosed. This strategy may be worthy to be considered even for a patient with Marfan syndrome, in case the patient's condition is unsuitable for conventional surgery.