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

Abstract

PurposeIn this study, we evaluated the long term beneficial effect of Renin-Angiotensin-Aldosterone System (RAAS) blockade therapy in treatment of Marfan aortopathy.Materials and MethodsWe 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.ResultsThere 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).ConclusionIn MFS patients who underwent ARR, the addition of RAAS blockade to β-blocker was associated with reduction of aortic dilatation and clinical events.

Interdisciplinary German clinical practice guidelines on the management of type B aortic dissection

“…Calcium antagonists have demonstrated improved survival rates in patients with type B dissections [ 88 ]. Both angiotensin‑1 receptor antagonists (losartan) and general blockades of the renin-angiotensin-aldosterone system (RAAS) have been shown to slow aortic dilation [ 235 , 236 ] and the rate of events after surgery [ 237 ] in patients with Marfan syndrome. Accordingly, a beta-blocker should be selected as basic treatment in combination with a calcium antagonist; additionally, a further combination with an angiotensin‑1 receptor antagonist is beneficial for drug-based blood pressure control in the context of rehabilitation for type B dissections.…”

Possible Effect of Alcohol Consumption on Aortic Dilatation by Inducing Renin–Angiotensin–Aldosterone System

“…The mineralocorticoid receptor antagonists significantly attenuated salt-induced AA. Human studies 11,12 also demonstrated the association between RAAS and AA. In a metaanalysis, 11 ACE I/D polymorphism was a risk factor for AA.…”

“…In a meta-analysis, 11 ACE I/D polymorphism was a risk factor for AA. Furthermore, Lee et al 12 reported that the addition of RAAS blockade to β-blocker treatment decreased aortic dilatation and clinical events in patients with Marfan syndrome.…”