TY - JOUR
T1 - Aortic root replacement in patients with Marfan's syndrome
T2 - The Southampton experience
AU - Alexiou, Christos
AU - Langley, Stephen M.
AU - Charlesworth, Phillip
AU - Haw, Marcus P.
AU - Livesey, Steven A.
AU - Monro, James L.
PY - 2001
Y1 - 2001
N2 - Background. The purpose of this study was to evaluate the early and late clinical outcome after aortic root replacement (ARR) in patients with Marfan's syndrome. Methods. A total of 65 consecutive patients with Marfan's syndrome (mean age 41.7 ± 10.7 years, range 15 to 76 years) undergoing ARR between 1972 and 1998 in Southampton were studied. Of the patients, 45 had a chronic aneurysm of the ascending aorta and 20 had a type A dissection (16 acute and 4 chronic). The operations Were elective in 38 and nonelective in 27 cases (emergency in 22 and urgent in 5). Mean size Of the ascending aorta was 6.3 ± 1.4 cm (3.8 to 12 cm). A Bentall procedure was performed in 62 and a homograft root replacement in 3 patients. Mean follow-up was 8 ± 4.1 years (0 to 22.9 years). Results. Operative mortality was 6.1% (4 deaths) (for the elective vs nonelective procedures it was 2.6% Vs 11%, p = 0.2). The 10-year freedom from thromboembolism, hemorrhage, and endocarditis was 88%, 89.8%, and 98.4% (0.9%, 0.9%, and 0.2% per patient-year) and from late aortic events it was 86.3% (1.3% per patient-year). Aortic root replacement for dissection was an independent predictor of occurrence of late aortic events (p = 0.01). Five patients had a reoperation with one early death. The 10-year freedom from reoperation was 89.2% (1.1% per patient year) (for elective and nonelective procedures, 90.8% vs 84.6%, p = 0.6). The 10-year survival, including operative mortality, was 72.7% (for elective and nonelective procedures, 78% vs 66.5%, p = 0.6). Late aortic events was an independent adverse predictor of survival (p = 0.02). Conclusions. In patients with Marfan's syndrome, elective ARR, usually for chronic aneurysm, is associated with a low mortality, low rate of aortic complications, and good late survival. Nonelective ARR, mostly for dissection, has a greater operative risk and a significantly higher incidence of late catastrophic aortic events. Early prophylactic surgery in these patients is therefore recommended, Long-term clinical and radiologic follow-up to prevent or to treat late aortic events is highly desirable.
AB - Background. The purpose of this study was to evaluate the early and late clinical outcome after aortic root replacement (ARR) in patients with Marfan's syndrome. Methods. A total of 65 consecutive patients with Marfan's syndrome (mean age 41.7 ± 10.7 years, range 15 to 76 years) undergoing ARR between 1972 and 1998 in Southampton were studied. Of the patients, 45 had a chronic aneurysm of the ascending aorta and 20 had a type A dissection (16 acute and 4 chronic). The operations Were elective in 38 and nonelective in 27 cases (emergency in 22 and urgent in 5). Mean size Of the ascending aorta was 6.3 ± 1.4 cm (3.8 to 12 cm). A Bentall procedure was performed in 62 and a homograft root replacement in 3 patients. Mean follow-up was 8 ± 4.1 years (0 to 22.9 years). Results. Operative mortality was 6.1% (4 deaths) (for the elective vs nonelective procedures it was 2.6% Vs 11%, p = 0.2). The 10-year freedom from thromboembolism, hemorrhage, and endocarditis was 88%, 89.8%, and 98.4% (0.9%, 0.9%, and 0.2% per patient-year) and from late aortic events it was 86.3% (1.3% per patient-year). Aortic root replacement for dissection was an independent predictor of occurrence of late aortic events (p = 0.01). Five patients had a reoperation with one early death. The 10-year freedom from reoperation was 89.2% (1.1% per patient year) (for elective and nonelective procedures, 90.8% vs 84.6%, p = 0.6). The 10-year survival, including operative mortality, was 72.7% (for elective and nonelective procedures, 78% vs 66.5%, p = 0.6). Late aortic events was an independent adverse predictor of survival (p = 0.02). Conclusions. In patients with Marfan's syndrome, elective ARR, usually for chronic aneurysm, is associated with a low mortality, low rate of aortic complications, and good late survival. Nonelective ARR, mostly for dissection, has a greater operative risk and a significantly higher incidence of late catastrophic aortic events. Early prophylactic surgery in these patients is therefore recommended, Long-term clinical and radiologic follow-up to prevent or to treat late aortic events is highly desirable.
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U2 - 10.1016/S0003-4975(01)02993-9
DO - 10.1016/S0003-4975(01)02993-9
M3 - Article
C2 - 11722033
AN - SCOPUS:0035171062
SN - 0003-4975
VL - 72
SP - 1502
EP - 1507
JO - Annals of Thoracic Surgery
JF - Annals of Thoracic Surgery
IS - 5
ER -