Acute aortic dissection determines the fate of initially untreated aortic segments in Marfan syndrome.
- Schoenhoff FS Department of Cardiovascular Surgery, University Hospital Berne, 3010 Berne, Switzerland.
- Jungi S
- Czerny M
- Roost E
- Reineke D
- Matyas G
- Steinmann B
- Schmidli J
- Kadner A
- Carrel T
- 2013-03-16
Published in:
- Circulation. - 2013
Acute Disease
Adolescent
Adult
Aged
Aneurysm, Dissecting
Aorta
Aortic Aneurysm
Aortic Valve
Blood Vessel Prosthesis Implantation
Cardiomyopathy, Dilated
Cause of Death
Child
Disease Progression
Disease-Free Survival
Elective Surgical Procedures
Female
Follow-Up Studies
Humans
Kaplan-Meier Estimate
Male
Marfan Syndrome
Middle Aged
Postoperative Complications
Reoperation
Retrospective Studies
Risk Factors
Treatment Outcome
Young Adult
English
BACKGROUND
The aim of the current study was to investigate incidence and causes of surgical interventions in primarily nontreated aortic segments after previous aortic repair in patients with Marfan syndrome.
METHODS AND RESULTS
Retrospective analysis of 86 consecutive Marfan syndrome patients fulfilling Ghent criteria that underwent 136 aortic surgeries and were followed at this institution in the past 15 years. Mean follow-up was 8.8±6.8 y. Thirty-day, 6-month, 1-year, and overall mortality was 3.5%, 5.8%, 7.0%, and 12.8%, respectively. Ninety-two percent of patients initially presented with aortic root, ascending aortic or arch lesions, whereas 8% presented with descending aortic or thoraco-abdominal lesions. Primary presentation was acute aortic dissection (AAD) in 36% (77% type A, 23% type B) and aneurismal disease in 64%. Secondary complete arch replacement had to be performed in only 6% of patients without AAD, but in 36% with AAD (P=0.0005). In patients without AAD, 11% required surgery on primarily nontreated aortic segments (5 of 6 patients experienced type B dissection during follow-up), whereas in patients after AAD, 48% underwent surgery of initially nontreated aortic segments (42% of patients with type A and 86% of those with type B dissection; P=0.0002).
CONCLUSIONS
The need for surgery in primarily nontreated aortic segments is precipitated by an initial presentation with AAD. Early elective surgery is associated with low mortality and reintervention rates. Type B dissection in patients with Marfan syndrome is associated with a high need for extensive aortic repair, even if the dissection is being considered uncomplicated by conventional criteria.
The aim of the current study was to investigate incidence and causes of surgical interventions in primarily nontreated aortic segments after previous aortic repair in patients with Marfan syndrome.
METHODS AND RESULTS
Retrospective analysis of 86 consecutive Marfan syndrome patients fulfilling Ghent criteria that underwent 136 aortic surgeries and were followed at this institution in the past 15 years. Mean follow-up was 8.8±6.8 y. Thirty-day, 6-month, 1-year, and overall mortality was 3.5%, 5.8%, 7.0%, and 12.8%, respectively. Ninety-two percent of patients initially presented with aortic root, ascending aortic or arch lesions, whereas 8% presented with descending aortic or thoraco-abdominal lesions. Primary presentation was acute aortic dissection (AAD) in 36% (77% type A, 23% type B) and aneurismal disease in 64%. Secondary complete arch replacement had to be performed in only 6% of patients without AAD, but in 36% with AAD (P=0.0005). In patients without AAD, 11% required surgery on primarily nontreated aortic segments (5 of 6 patients experienced type B dissection during follow-up), whereas in patients after AAD, 48% underwent surgery of initially nontreated aortic segments (42% of patients with type A and 86% of those with type B dissection; P=0.0002).
CONCLUSIONS
The need for surgery in primarily nontreated aortic segments is precipitated by an initial presentation with AAD. Early elective surgery is associated with low mortality and reintervention rates. Type B dissection in patients with Marfan syndrome is associated with a high need for extensive aortic repair, even if the dissection is being considered uncomplicated by conventional criteria.
- Language
-
- English
- Open access status
- bronze
- Identifiers
-
- DOI 10.1161/CIRCULATIONAHA.113.001457
- PMID 23493319
- Persistent URL
- https://sonar.ch/global/documents/131673
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