ARTICLE DE REVUE 

Syndrome de Marfan
Christine H. Attenhofer Josta, Marianne Rohrbachb, Gabor Matyasc, Florian Schoenhoffd, Matthias Baumgartnerb,
Angela Oxeniuse, Thierry Carreld, Michael Hueblerf, Kan Ming, Gabriella De Pasqualeh, Klara Landaui,
Oliver Kretschmare, Dragan Odavick, Paul Vogta, Francesco Faletral, Matthias Greutmannm
a HerzGefässZentrum Zürich, Klinik Im Park, Zürich; b Stoffwechselabteilung, Forschungszentrum für das Kind, Universitäts-Kinderspital Zürich;
c Zentrum für Kardiovaskure Genetik und Gendiagnostik, Schlieren-Zürich; d Herz- und Gefässchirurgie, Inselspital Bern;
e Kardiologie, Universitäts-Kinderspital Zürich, f Klinik für Herz- und Gefässchirurgie, UniversitätsSpital Zürich; g Swiss Scoliosis, Klinik Im Park, Zürich;
h Kardiologie, Stadtspital Triemli, Zürich; i Augenklinik, UniversitätsSpital Zürich; k Klinik für Herzchirurgie, Stadtspital Triemli, Zürich;
l Cardiocentro Ticino, Lugano; m Abteilung Erwachsene mit Kongenitalen Vitien, Klinik für Kardiologie, UniversitätsSpital Zürich
Introduction

     
    

    


     
  







   
    






    

   




   

    





    

      





   













ARTICLE DE REVUE 
     
     

   

Forum Médical Suisse
   
    



Nosologie actuelle

   
  








Dilatation aortique et dissection aortique
     
  




  




   





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  
  

 
    



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     
     
     
  


   


     

Tableau 1:
Sans anamnèse familiale positive pour le syndrome de Marfan:




Avec anamnèse familiale positive pour le syndrome de Marfan:




Pas de syndrome de Marfan:











ARTICLE DE REVUE 
Score sysmique


     


    



   







     
    
   
  

 
 


(striae dis-
tensae)

    




    
 
    
  
 
   
Tableau 2: 
Atteinte systémiquePoints

 






 








(striae)


Figure 1: 


Figure 2: 




Segment
supérieur
Segment
inférieur

ARTICLE DE REVUE 





  






  
   

     




     


   






      

   
      
       

     
    
 

 
     

    



       



     
    



   

Anamse familiale et analyse génétique

       
   


Figure 3: 
A: 
B:


Main normale Syndrome de MarfanSigne du pouceSigne du poignet
AB
Figure 4:





ARTICLE DE REVUE 




  

      

 

    




   

Echocardiographie, IRM ou tomodensito-
trie: quel examen à quel moment?
 


  





    

    

   




 


   
American College of
Cardiology / American Heart Association / American As-
sociation for Thoracic Surgery



   




 
    
 

Figure 5:



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