Surgical Management
of the Aortic Root in Patients with Marfan Syndrome
Kenton J. Zehr, Alireza Matloobi, Heidi M. Connolly, Thomas A. Orszulak,
Francisco J. Puga, Hartzell V. Schaff
Divisions of Cardiovascular Surgery and
Cardiology, Mayo Clinic, Rochester, MN, USA |
Background and aim of the study: Surgical aortic
root reconstruction techniques are standard therapy to avoid catastrophic
vascular events in patients with Marfan syndrome with a dilated and/or
dissected aortic root. The study aim was to evaluate the long-term results
of aortic root reconstruction.
Methods: Eighty-three patients (54 males, 29 females;
mean age 37 ± 17 years) fulfilling strict Ghent criteria
for Marfan syndrome underwent aortic root surgery between 1971
and 2001. Of these patients, 65 (78%) underwent a composite valve
conduit repair and 18 (22%) a valve-sparing aortic root reconstruction.
Six patients (7%) suffered from an acute type A dissection, and
16 (19%) a chronic type A dissection.
Results: In-hospital and 30-day mortality was 3.6% (n
= 3). Morbidity included stroke (1.2%; n = 1), perioperative myocardial
infarction (1.2%; n = 1) and reoperation for bleeding (10%; n =
8). Of 21 late deaths, the cause was cardiac in nine cases. Actuarial
survival at 5, 10, 15 and 20 years was 84% (95% CI 76-93%), 73%
(CI 61-86%), 59% (CI 45-77%) and 43% (CI |
26-72%), respectively. Multivariate predictors for late
death were postoperative dysrhythmias and need for inotropes (p £0.01).
Freedom from reoperation at 5, 10, 15 and 20 years was 86% (CI 78-95%),
69% (CI 56-85%), 53% (CI 38-74%) and 48% (CI 23-71%), respectively. Multivariate
predictors for reoperation were preoperative mitral valve prolapse and
an initial valve-sparing aortic procedure (p
£0.05). In the composite valve conduit patients, freedom from thromboembolism
was 88% (CI 76-100%), and from endocarditis was 99% (CI 93-100%) at 15
years.
Conclusion: Composite valve conduit replacement of the
aortic root in patients with Marfan syndrome offers a durable result,
with low mortality and long-term complication rates. Reoperation
was most commonly required for cardiac and vascular disease unrelated
to the initial operation and in patients undergoing a valve-sparing
aortic root procedure.
The Journal of Heart Valve Disease 2005;14:121-129 |