Staged total arch replacement, followed by fenestrated-branched endovascular aortic repair, for patients with mega aortic syndrome

Hidetake Kawajiri, Emanuel R. Tenorio, Mohammad A. Khasawneh, Alberto Pochettino, Bernardo C. Mendes, Giulianna B. Marcondes, Guilherme B.B. Lima, Gustavo S. Oderich

Research output: Contribution to journalArticlepeer-review


Objective: The aim of the present study was to review the clinical outcomes of a staged approach using total arch replacement (TAR) with an elephant trunk or a frozen elephant trunk, followed by fenestrated-branched endovascular aortic repair (F-BEVAR) for patients with mega aortic syndrome. Methods: We reviewed the clinical data and outcomes of 11 consecutive patients (8 men; mean age, 71 ± 7 years) treated by staged TAR and F-BEVAR from January 2014 to December 2018. The F-BEVAR procedures were performed under a prospective, nonrandomized, physician-sponsored investigational device exemption protocol. All patients had had mega aortic syndrome, defined by an ascending aorta, arch, and extent I-II thoracoabdominal aortic aneurysm. The endpoints were 30-day mortality, major adverse events (MAE), patient survival, freedom from reintervention, and freedom from target vessel instability. Results: Of the 11 patients, 6 had developed chronic postdissection aneurysms after previous Stanford A (three A11, two A10, one A9) dissection repair and 5 had had degenerative aneurysms with no suitable landing zone in the aortic arch. The thoracoabdominal aortic aneurysms were classified as extent I in four patients and extent II in seven. One patient had died within 30 days after TAR (9.0%). However, none of the remaining 10 patients who had undergone F-BEVAR had died. First-stage TAR resulted in MAE in three patients (27%), including one spinal cord injury. The mean length of stay was 12 ± 6 days. The mean interval between TAR and F-BEVAR was 245 ± 138 days with no aneurysm rupture during the interval. Second-stage F-BEVAR was associated with MAE in two patients (20%), including spinal cord injury in one patient from spinal hematoma due to placement of a cerebrospinal fluid drain. The mean follow-up period was 14 ± 10 months. At 2 years postoperatively, patient survival, primary patency, secondary patency, and freedom from renal–mesenteric target vessel instability was 80% ± 9%, 94% ± 6%, 100%, and 86% ± 8%, respectively. No aortic-related deaths occurred during the follow-up period. Four patients had required reintervention, all performed using an endovascular approach. Conclusions: A staged approach to treatment of mega aortic syndrome using TAR and F-BEVAR is a feasible alternative for selected high-risk patients. Larger clinical experience and longer follow-up are needed.

Original languageEnglish (US)
Pages (from-to)1488-1497.e1
JournalJournal of vascular surgery
Issue number5
StatePublished - May 2021


  • Elephant trunk
  • Fenestrated-branched endovascular aortic repair
  • Frozen elephant trunk
  • Mega aorta
  • Total arch replacement

ASJC Scopus subject areas

  • Surgery
  • Cardiology and Cardiovascular Medicine


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