title: The Beneficial Effect of Renin-Angiotensin-Aldosterone System Blockade in Marfan Syndrome Patients after Aortic Root Replacement.
abstract: In this study, we evaluated the long term beneficial effect of Renin-Angiotensin-Aldosterone System (RAAS) blockade therapy in treatment of Marfan aortopathy.
  
  
  
  We reviewed Marfan syndrome (MFS) patients who underwent aortic root replacement (ARR) between January 1996 and January 2011. All patients were prescribed β-blockers indefinitely. We compared major aortic events including mortality, aortic dissection, and reoperation in patients without RAAS blockade (group 1, n=27) to those with (group 2, n=63). The aortic growth rate was calculated by dividing the diameter change on CT scans taken immediately post-operatively and the latest scan available.
  
  
  
  There were no differences in clinical parameters except for age which was higher in patients with RAAS blockade. In group 1, 2 (7%) deaths, 5 (19%) aortic dissections, and 7 (26%) reoperations occurred. In group 2, 3 (5%) deaths, 2 (3%) aortic dissections, and 3 (5%) reoperations occurred. A Kaplan-Meier plot demonstrated improved survival free from major aortic events in group 2. On multivariate Cox, RAAS blockade was an independent negative predictor of major aortic events (hazard ratio 0.38, 95% confidence interval 0.30-0.43, p=0.002). Mean diameter change in descending thoracic and supra-renal abdominal aorta was significantly higher in patients without RAAS blockade (p<0.05).
  
  
  
  In MFS patients who underwent ARR, the addition of RAAS blockade to β-blocker was associated with reduction of aortic dilatation and clinical events.
authors.affiliation: Cardiology Division, Department of Internal Medicine, Severance Cardiovascular Hospital, Yonsei University Health System, Seoul, Korea.
authors.forename: Seung Jun
authors.initials: SJ
authors.lastname: Lee
authors.affiliation: Cardiology Division, Department of Internal Medicine, Severance Cardiovascular Hospital, Yonsei University Health System, Seoul, Korea.
authors.forename: Jaewon
authors.initials: J
authors.lastname: Oh
authors.affiliation: Cardiology Division, Department of Internal Medicine, Severance Cardiovascular Hospital, Yonsei University Health System, Seoul, Korea.
authors.forename: Young Guk
authors.initials: YG
authors.lastname: Ko
authors.affiliation: Department of Cardiovascular Surgery, Severance Cardiovascular Hospital, Yonsei University Health System, Seoul, Korea.
authors.forename: Sak
authors.initials: S
authors.lastname: Lee
authors.affiliation: Department of Cardiovascular Surgery, Severance Cardiovascular Hospital, Yonsei University Health System, Seoul, Korea.
authors.forename: Byung Chul
authors.initials: BC
authors.lastname: Chang
authors.affiliation: Department of Radiology, Research Institute of Radiological Science, Yonsei University Health System, Seoul, Korea.
authors.forename: Do Yun
authors.initials: DY
authors.lastname: Lee
authors.affiliation: Department of Anesthesiology and Pain Medicine, Severance Cardiovascular Hospital, Yonsei University Health System, Seoul, Korea.
authors.forename: Young Ran
authors.initials: YR
authors.lastname: Kwak
authors.affiliation: Cardiology Division, Department of Internal Medicine, Severance Cardiovascular Hospital, Yonsei University Health System, Seoul, Korea. cdhlyj@yuhs.ac.
authors.forename: Donghoon
authors.initials: D
authors.lastname: Choi
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000494:pharmacology; D000368:Aged; D000784:Aortic Dissection / Q000150:complications / Q000401:mortality / Q000601:surgery; D057911:Angiotensin Receptor Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D001011:Aorta / Q000473:pathology / Q000601:surgery*; D001014:Aortic Aneurysm / Q000150:complications / Q000401:mortality / Q000601:surgery; D001021:Aortic Valve; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000401:mortality / Q000601:surgery*; D008875:Middle Aged; D012084:Renin-Angiotensin System / Q000187:drug effects*
chemical_list: D000319:Adrenergic beta-Antagonists; D057911:Angiotensin Receptor Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors
keywords: ACE inhibitor; Marfan syndrome; RAAS blockade; angiotensin receptor blocker