Surgical management of the aortic root in patients with Marfan syndrome.
OBJECTIVE
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 < or =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 < or =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.


CONCLUSIONS
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.
Zehr
Kenton J
KJ
Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905, USA. zehr.kenton@mayo.edu
Matloobi
Alireza
A
Connolly
Heidi M
HM
Orszulak
Thomas A
TA
Puga
Francisco J
FJ
Schaff
Hartzell V
HV
D000328:Adult; D000784:Aortic Dissection / Q000601:surgery*; D001014:Aortic Aneurysm / Q000601:surgery*; D001021:Aortic Valve / Q000601:surgery*; D001145:Arrhythmias, Cardiac / Q000150:complications; D002316:Cardiotonic Agents / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006350:Heart Valve Prosthesis; D017052:Hospital Mortality; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000401:mortality / Q000601:surgery*; D008945:Mitral Valve Prolapse / Q000150:complications; D015999:Multivariate Analysis; D011183:Postoperative Complications; D012086:Reoperation / Q000706:statistics & numerical data; D012189:Retrospective Studies; D016019:Survival Analysis; D014184:Transplantation, Homologous
D002316:Cardiotonic Agents