Literature DB >> 16948755

Cardiovascular reoperations in Marfan syndrome.

Vedat Erentug1, Adil Polat, Nilgun Ulusoy Bozbuga, Ebru Polat, Hasan Basri Erdogan, Kaan Kirali, Mustafa Guler, Esat Akinci, Cevat Yakut.   

Abstract

BACKGROUND AND AIM OF THE STUDY: The purpose of this study is to analyze the outcome results of reoperations in Marfan syndrome patients.
METHODS: Between 1985 and December 2004, 49 patients with Marfan syndrome were operated for aortic aneurysms. Of these 49 patients, 9 (18,4%) required > or =1 reoperations after a mean duration of 32.2 +/- 26.6 months. The mean duration of follow-up was 52.0 +/- 46.8 months, a total of 39 patient/years. Survival free of reoperation was calculated by Cox regression analysis.
RESULTS: Surgical indication for operation was a chronic aneurysmal dilatation of the ascending or abdominal aorta in seven patients (77.8%) and aortic dissection in two (22.2%) at the initial operation. In the reoperations, repair of thoracoabdominal aortic aneurysm with separated graft interposition in six patients (66.7%), replacement of ascending aorta in one (11.1%), replacement of ascending and hemiarchus aorta in one (11.1%), and mitral valve replacement in three patients (33.3%) were performed. The hospital mortality was 11.1% with one patient. Among the survivors, one expired in the follow-up five months after the second operation (12.5%). With the Cox regression analysis, survival without reoperation for 13, 24, and 123 months are 95.56 +/- 3.04%, 90.66 +/- 4.40%, and 60.32 +/- 12.63%, respectively. Mean survival for reoperated patients is 99 +/- 14 months (95% confidence interval 72-127 months).
CONCLUSIONS: Reoperations can be done with low morbidity and mortality. Patients should be kept under close follow-up using imaging techniques infinitely.

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Year:  2006        PMID: 16948755     DOI: 10.1111/j.1540-8191.2006.00276.x

Source DB:  PubMed          Journal:  J Card Surg        ISSN: 0886-0440            Impact factor:   1.620


  3 in total

1.  External aortic root support for Marfan syndrome.

Authors:  Adil Polat; Ebru Bal Polat; Vedat Erentug
Journal:  J R Soc Med       Date:  2010-12       Impact factor: 5.344

2.  A novel mutation in fibrillin-1 gene identified in a Chinese family with marfan syndrome.

Authors:  Dan-Li Liu; Juan-Hui Cao; Jie Yang; Fen He; Yun Wang; Ning Fan; Xu-Yang Liu
Journal:  Int J Clin Exp Med       Date:  2015-05-15

3.  A new novel mutation in FBN1 causes autosomal dominant Marfan syndrome in a Chinese family.

Authors:  Jiamei Dong; Juan Bu; Wei Du; Yuan Li; Yanlei Jia; Jianchang Li; Xiaoli Meng; Minghui Yuan; Xiaojuan Peng; Aimin Zhou; Lejin Wang
Journal:  Mol Vis       Date:  2012-01-13       Impact factor: 2.367

  3 in total

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