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The Beneficial Effect of Renin-Angiotensin- Aldosterone System Blockade in Marfan Syndrome Patients after Aortic Root Replacement

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Purpose: In this study, we evaluated the long term beneficial effect of Renin-Angiotensin-Aldosterone System (RAAS) blockadetherapy in treatment of Marfan aortopathy. Materials and Methods: We reviewed Marfan syndrome (MFS) patients who underwent aortic root replacement (ARR) betweenJanuary 1996 and January 2011. All patients were prescribed β-blockers indefinitely. We compared major aortic events includingmortality, aortic dissection, and reoperation in patients without RAAS blockade (group 1, n=27) to those with (group 2, n=63). Theaortic growth rate was calculated by dividing the diameter change on CT scans taken immediately post-operatively and the latestscan available. Results: There were no differences in clinical parameters except for age which was higher in patients with RAAS blockade. Ingroup 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 eventsin 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 aortawas significantly higher in patients without RAAS blockade (p<0.05). Conclusion: In MFS patients who underwent ARR, the addition of RAAS blockade to β-blocker was associated with reduction ofaortic dilatation and clinical events.

Abstract

Purpose: In this study, we evaluated the long term beneficial effect of Renin-Angiotensin-Aldosterone System (RAAS) blockadetherapy in treatment of Marfan aortopathy. Materials and Methods: We reviewed Marfan syndrome (MFS) patients who underwent aortic root replacement (ARR) betweenJanuary 1996 and January 2011. All patients were prescribed β-blockers indefinitely. We compared major aortic events includingmortality, aortic dissection, and reoperation in patients without RAAS blockade (group 1, n=27) to those with (group 2, n=63). Theaortic growth rate was calculated by dividing the diameter change on CT scans taken immediately post-operatively and the latestscan available. Results: There were no differences in clinical parameters except for age which was higher in patients with RAAS blockade. Ingroup 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 eventsin 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 aortawas significantly higher in patients without RAAS blockade (p<0.05). Conclusion: In MFS patients who underwent ARR, the addition of RAAS blockade to β-blocker was associated with reduction ofaortic dilatation and clinical events.

참고문헌 (34)

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