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.

• Authors: Donald Adam, Mauro Iafrancesco, Maciej Juszczak, Martin Claridge, David Quinn, Eshan Senanayake, Paul Clift, Jorge Mascaro
• Journal: European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery (Eur J Cardiothorac Surg) Vol. 62 Issue 3 (08 03 2022) ISSN: 1873-734X [Electronic] Germany
• PMID: 35285871 (Publication Type: Journal Article)
• Copyright: © The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Topics

• Adult
• Aortic Dissection (surgery)
• Aorta (surgery)
• Aortic Aneurysm, Thoracic
• Blood Vessel Prosthesis Implantation (adverse effects)
• Humans
• Loeys-Dietz Syndrome (complications, surgery)
• Male
• Marfan Syndrome (complications, surgery)
• Postoperative Complications
• Retrospective Studies
• Treatment Outcome