Open surgical replacement of the descending thoracic and thoracoabdominal aorta in patients with confirmed Marfan and Loeys-Dietz syndromes: A 20-year single-centre experience

Abstract

OBJECTIVES A relatively small proportion of patients with heritable thoracic aortic disease require open surgical replacement of the distal thoracic aorta. We reviewed the outcome in patients with Marfan and Loeys-Dietz syndromes treated in an aortic centre in the United Kingdom. METHODS We performed a single-centre retrospective study of consecutive patients treated between October 1999 and December 2019. The primary end point was 30-day mortality. Secondary end points were Kaplan–Meier estimates of medium-term survival and freedom from distal reintervention. Data are presented as median (interquartile range). RESULTS A total of 58 patients [33 men; 51 with Marfan syndrome; median age 41 years (35–48); median aneurysm diameter 60 mm (55–74)] underwent open descending (n = 21) or thoracoabdominal aortic replacement (n = 37). All repairs were performed using cardiopulmonary bypass with hypothermic circulatory arrest in 31 patients. The 30-day mortality was 5.2% (n = 3, including 2 patients ≥ 60 years with significant comorbidity). Major non-fatal complications included early reoperation (n = 7), tracheostomy (n = 9), temporary renal replacement therapy (n = 3), permanent spinal cord deficit (n = 2) and permanent stroke (n = 1). Median follow-up was 81 months (48–127). Estimated (±standard error) 5-year survival was 85% ±5%. Seven patients had distal aortic reintervention with no deaths or spinal cord deficit: estimated 5-year freedom from distal reintervention was 94% ±3%. There was no difference in survival or freedom from distal reintervention comparing: elective vs. non-elective; type of heritable thoracic aortic disease; DeBakey type; or extent of surgical repair. CONCLUSIONS Descending thoracic and thoracoabdominal aortic replacement in patients with heritable thoracic aortic disease can be performed with low perioperative morbidity and mortality, satisfactory long-term survival and low requirement for distal reintervention.

Outcomes After Endovascular Aortic Intervention in Patients With Connective Tissue Disease

“…[30][31][32][33] In accordance with previous reports of endovascular treatment of thoracic, thoracoabdominal, and abdominal aortic disease in patients with CTD, the primary technical success was excellent in the EVICTUS cohort. [34][35][36][37][38][39] The perioperative mortality and morbidity were acceptable for patients with Marfan syndrome or Loeys-Dietz syndrome, especially given that compared with the reports of open surgery, this cohort featured patients with an older median age and more patients emergently treated for complicated dissections, as well as symptomatic or ruptured aneurysms. These results are also consistent with a recent report of thoracic endovascular repairs in patients with CTD of similar age with an analogous proportion of emergent procedures.…”

Comparison of Immediate, Medium, and Long-Term Postoperative Results of Open Surgery and Fenestrated/Branched Stent Grafts for Extended Thoracoabdominal Aortic Aneurysms

“…Historical data suggested similar mortality rates but higher rates of SCI in patients with Marfan syndrome [59]. More recent series have reported more favorable outcomes, with operative mortality, SCI, and stroke rates not exceeding 5% [35,[60][61][62][63][64][65][66][67]. Early reintervention for bleeding is not rare, occurring in up to 10% of patients.…”

Spinal cord injury after open and endovascular repair of descending thoracic aneurysm and thoracoabdominal aortic aneurysm: an updated systematic review and meta-analysis

“…A total of 239 studies were included in the final analysis; 71 studies from the 252 studies that were retrieved for fulltext review, and additional 168 studies were included from our group's previous meta-analysis (5), and one study (4) was excluded from the analysis due to overlapping patient population (Figure S1). The majority (84.1%) of the studies were retrospective (201/239), 15.5% (37/239) were prospective, and one was a randomized trial.…”

“…Postoperative SCI can either be temporary or permanent. The incidence of SCI has been reported to be between 2% and 10% (3)(4)(5) after open or endovascular repair, although estimating the general incidence rate (IR) is challenging as most of the data are from retrospective, single-center observational studies with major limitations of sample size, disparate surgical techniques, complex patient anatomy and pathology, and preexisting comorbidities. In this meta-analysis, we provide a comprehensive systematic review and an update of a meta-analysis previously published by our group on SCI, mortality, and cerebrovascular events after open or endovascular repair of DTA/TAAA.…”