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ULTIMO FASCICOLOTHE JOURNAL OF CARDIOVASCULAR SURGERY

Rivista di Chirurgia Cardiaca, Vascolare e Toracica


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The Journal of Cardiovascular Surgery 2002 Ottobre;43(5):665-9

CARDIAC SECTION 

 CASE REPORTS

Surgical treatment of a Budd-Chiari syndrome secondary to hepatic inferior vena cava agenesia

Rinckenbach S. 1, Chakfé N. 1, Beaufigeau M. 1, Hassani O. 1, Bachelier P. 2, Jaeck D. 2, Kretz J.-G. 1

1 Depart­ment of Car­di­o­vas­cular Sur­gery
2 Gen­eral Sur­gery and Trans­plan­ta­tion Stras­burg University Hospital, Strasburg, ­France

We ­report the ­case of a sur­gi­cally ­treated con­gen­ital ­Budd-­Chiari syn­drome ­related to age­nesia of the ret­ro­he­patic infe­rior ­vena ­cava. The ­first symp­toms of the dis­ease ­were ­noticed in child­hood. The ­increasing symp­tom­a­tology led to pro­pose, at the age of 30 ­years, ­first a per­cut­aneous trans­lu­minal angio­plasty ­which ­failed, ­because of the impos­sibility to rec­a­nalize the ­obstructed seg­ment. ­Then a sur­gical pro­ce­dure con­sisting of the implan­ta­tion of ­ePTFE pros­thesis ­between the ­right ­atria and the ret­ro­he­patic infe­rior ­vena ­cava was per­formed. The ­hepatic ­biopsy ­showed a cen­tro­lob­ular ­fibrosis and an old sub­glis­so­nian infarc­tion. The ­patient was ­improved, ­allowing him to ­recover a ­normal ­life. How­ever, ­three ­years ­later, an angio­graphic eval­u­a­tion per­formed ­because of a recur­rence of a ­slight abdom­inal ­pain, ­showed a throm­bosis of the ­bypass. An ­attempt at throm­bol­ysis ­failed. ­Since the ­patient did not ­present ­major clin­ical and bio­log­ical con­se­quences we ­only pro­posed a sur­veil­lance and no endo­vas­cular pro­ce­dure ­because of the ­fear of a pul­mo­nary ­emboli. The pur­pose of ­this ­case ­report is to ­review the lit­er­a­ture and dis­cuss the etio­pa­thology of con­gen­ital ­Budd-­Chiari syn­drome ­with ­regard to the dif­ferent ther­a­peutic ­options.

lingua: Inglese


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