Login | Users Online: 33798  
Home Print this page Email this page Small font sizeDefault font sizeIncrease font size   
Home | About us | Editorial board | Search | Ahead of print | Current Issue | Archives | Submit article | Instructions | Subscribe | Advertise | Contact us
 


 
Table of Contents
A PICTURE IS WORTH A THOUSAND WORDS
Year : 2012  |  Volume : 13  |  Issue : 1  |  Page : 22-23  

Marfan's syndrome


Department of Cardiology, Sanjay Gandhi PGIMS, Lucknow, India

Date of Web Publication26-May-2012

Correspondence Address:
Aditya Kapoor
Department of Cardiology, Sanjay Gandhi PGIMS, Lucknow 226014
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1995-705X.96666

Rights and Permissions

How to cite this article:
Bhatia T, Kapoor A, Kumar S. Marfan's syndrome. Heart Views 2012;13:22-3

How to cite this URL:
Bhatia T, Kapoor A, Kumar S. Marfan's syndrome. Heart Views [serial online] 2012 [cited 2023 Oct 2];13:22-3. Available from: https://www.heartviews.org/text.asp?2012/13/1/22/96666

A 25-year-old young male presented with progressive shortness of breath and palpitations for 4 years. Clinical examination revealed a collapsing water-hammer pulse (100/min), BP 140/50/0 mm Hg and raised jugular venous pressure (5 cm above the sternal angle); on auscultation severe aortic regurgitation (AR) and mild mitral valve regurgitation (MR) was present. In addition, the patient had typical skeletal features of Marfan's syndrome with positive "wrist" and "thumb" sign [Figure 1]a and b. A transthoracic 2D echocardiography demonstrated aneurysmally dilated aortic root and ascending aorta, non-stenotic bicuspid aortic valve and severe AR. [Figure 2], [Videos 1] and [Video 2]. The proximal aorta was so enormously dilated that the left atrium was severely compressed and barely visible posterior to the aorta in the parasternal view. The aortic annulus, aortic arch, and descending aorta were all normal in size. Mitral valve prolapse with mild mitral valve regurgitation was also present. A 64-slice computed tomography (CT) examination confirmed the echocardiography findings of the generalized dilatation of ascending aorta [Figure 3]a and b.
Figure 1 a,b: Thumb (1a) and wrist sign (1b) in the patient with Marfans' syndrome highlighting the hyper-extensile joints

Click here to view
Figure 2: 2 D echocardiogram demonstrating aneurysmally dilated aortic root

Click here to view
Figure 3a: A 64-slice CT image demonstrating the generalized ascending aortic dilatation
Figure 3b: A 64-slice CT image demonstrating the generalized ascending aortic dilatation


Click here to view









    Figures

  [Figure 1], [Figure 2], [Figure 3]


This article has been cited by
1 “Elephant-Trunk Shaped” Urethral Deformity in a Young Man with Marfan Syndrome
Wei-Tang Kao,Kuan-Chou Chen,Chia-Chang Wu,Chia-Hung Liu,Yi-Te Chiang
Journal of Experimental & Clinical Medicine. 2014; 6(1): 33
[Pubmed] | [DOI]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Article Figures

 Article Access Statistics
    Viewed4226    
    Printed202    
    Emailed0    
    PDF Downloaded161    
    Comments [Add]    
    Cited by others 1    

Recommend this journal