Identification of Molecular Defects in Korean Patients with Marfan Syndrome

Shin, Mi Seung; Park, Hyun-Young; Lim, Yeunhwan; Shin, Gil Ja; Jang, Yangsoo; Jang, Byung Chul; Chung, Namsik

doi:10.4070/kcj.2003.33.11.1018

Cited by 1 publication

(1 citation statement)

References 24 publications

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“…Fibrillin-1 regulates TGF-β signaling, in addition to being a major structural component of extracellular matrix microfibrils. 1 2 3 4 Fibrillin-1 is important in multiple organ systems, including the musculoskeletal, ocular, pulmonary, cardiovascular, and central nervous systems. Defects in the cardiovascular system cause death in MFS patients.…”

Section: Introductionmentioning

confidence: 99%

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

Lee

et al. 2016

Yonsei Med J

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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.

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Section: Introductionmentioning

confidence: 99%

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

Lee

et al. 2016

Yonsei Med J

View full text Add to dashboard Cite

show abstract

Identification of Molecular Defects in Korean Patients with Marfan Syndrome

Cited by 1 publication

References 24 publications

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

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

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