Total Aortic and Branches Repair with Hybrid Therapy in the setting of Marfan Syndrome: A Case Report

Author(s):  
Sheng Yan ◽  
Zhi-Yuan Wu ◽  
Li Ma ◽  
Zuo-Guan Chen ◽  
Yong-Peng Diao ◽  
...  
2010 ◽  
Vol 1 (3) ◽  
pp. e180-e183 ◽  
Author(s):  
Hiroshi Nishino ◽  
Kenji Suda ◽  
Akitaka Kuramaoto ◽  
Yoshihiro Honda ◽  
Kiyoko Takemiya ◽  
...  

2015 ◽  
Vol 4 (4) ◽  
pp. 269
Author(s):  
Hemanth Kumar Kalla ◽  
Swarna Kumari ◽  
CH Rama rao ◽  
MKR Parthasarathy ◽  
S Surya prakash Reddy ◽  
...  

Marfan syndrome(MFS) is a connective tissue disorder that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Its incidence is among the highest of any heritable disorder.Most patients who have Marfan syndrome are usually diagnosed incidentally when they present for a routine physical examination for various reasons. The purpose of this paper is to provide a review of the literature, as well as describe a 22-year-old male with MFS and right hydroureteronephrosis diagnosed incidentally when he attended our hospital for complaints of fever and right loin pain. This case report emphasizes importance of “Revised Ghent criteria” for the diagnosis of MFS and highlights various clinical signs of MFS<strong>.</strong>


Author(s):  
Timothy Smith ◽  
Jose Sleiman ◽  
Nikita Zadneulitca ◽  
Cedric Sheffield ◽  
Viviana Navas ◽  
...  

Abstract Background: Marfan syndrome (MFS) is a connective tissue disorder that can lead to aortic disease, arrhythmias and heart failure. Many centers are reluctant to offer orthotopic heart transplantation (OHT) for patients with MFS with concurrent aortic disease due to complexity of the surgery and perceived inferior results when compared to patients without MFS. Methods: We present a case of a patient with MFS with previous Bentall procedure who underwent successful OHT, accompanied by a literature review on OHT performed for patients with MFS. Results and Conclusions: Patients with MFS who underwent OHT had no difference in mortality compared to patients without MFS. Even though OHT is technically more challenging when combined with concurrent intervention for aortic disease, it should be considered as a life-saving operation for patients with MFS.


2020 ◽  
Vol 9 (3) ◽  
pp. 136-142
Author(s):  
Mediha Gönenç Ortaköylü ◽  
Tuğçe Özen ◽  
Belma Akbaba Bağcı ◽  
Esma Seda Akalın Karaca
Keyword(s):  

2014 ◽  
Vol 4 (2) ◽  
pp. 111-114
Author(s):  
Sharmin Mahbuba ◽  
Fauzia Mohsin ◽  
Rubaiya Islam ◽  
Tahmina Begum

Marfan syndrome is an inherited connective tissue disorder that is transmitted as an autosomal dominant trait. These cases can be diagnosed by molecular cytogenetic techniques. A modified Ghent criteria using systemic scoring system can also identify these cases in absence of molecular cytogenetic techniques.We report a case of a 6 year 5 month old boy who presented with the complaints of excessive sweating sinceinfancy and protrusion of both eye balls which was non progressive since early childhood. On examination, some skeletal features of Marfan syndrome was found and echocardiogram showed huge dilatation of root of aorta which helped in diagnosis by scoring system.Birdem Med J 2014; 4(2): 111-114


2019 ◽  
Vol 33 (14) ◽  
pp. 2493-2504 ◽  
Author(s):  
Amanda Veiga-Fernández ◽  
Laura Joigneau Prieto ◽  
Teresa Álvarez ◽  
Yolanda Ruiz ◽  
Ricardo Pérez ◽  
...  

2015 ◽  
Vol 55 (1) ◽  
pp. 101-105 ◽  
Author(s):  
Kazuki SAKAKURA ◽  
Hiroyoshi AKUTSU ◽  
Tetsuya YAMAMOTO ◽  
Yosuke MASUDA ◽  
EIICHI ISHIKAWA ◽  
...  

2015 ◽  
Vol 4 (4) ◽  
pp. 269
Author(s):  
Hemanth Kumar Kalla ◽  
Swarna Kumari ◽  
CH Rama rao ◽  
MKR Parthasarathy ◽  
S Surya prakash Reddy ◽  
...  

Marfan syndrome(MFS) is a connective tissue disorder that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Its incidence is among the highest of any heritable disorder.Most patients who have Marfan syndrome are usually diagnosed incidentally when they present for a routine physical examination for various reasons. The purpose of this paper is to provide a review of the literature, as well as describe a 22-year-old male with MFS and right hydroureteronephrosis diagnosed incidentally when he attended our hospital for complaints of fever and right loin pain. This case report emphasizes importance of “Revised Ghent criteria” for the diagnosis of MFS and highlights various clinical signs of MFS<strong>.</strong>


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