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The Journal of Cardiovascular Surgery 1998 February;39(1):65-74

language: English

Surgical treat­ment of Marfan ­patients ­with aneu­rysms and dis­sec­tion of ­the prox­i­mal aor­ta

Ming­ke D., Dres­ler Ch., Pethig K., Heinemann M., Borst H. G.

From ­the Department of Cardiovascular and Thoracic Surgery Hannover Medical School Hannover, Germany


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Background. The ­authors ret­ro­spec­tively ana­lyzed ear­ly ­and ­late ­results of sur­gi­cal treat­ment ­for 79 Marfan ­patients ­with aneu­rysms ­and dis­sec­tion of ­the prox­i­mal aor­ta.
Methods. From September 1979 to February 1996, 79 ­patients ­with Marfan syn­drome under­went aor­tic ­root replace­ment ­using com­pos­ite ­grafts (n=68, Bentall-tech­nique 63, but­ton-tech­nique 5), ­and ascend­ing aor­tic replace­ment ­with a ­valve-spar­ing pro­ce­dure (n=11). There ­were 12 ­patients (15.2%) ­who simul­ta­ne­ous­ly ­received par­tial or ­total ­arch replace­ment. 55 ­patients (69.6%) ­were ­male, ­and 24 ­female (30.4%). The aver­age ­age ­was 33.8 ­years. Forty-­one ­patient (51.9%) ­had ­non-dis­sect­ing aneu­rysms ­while ­the remain­ing 38 ­patients suf­fered ­from ­either ­acute (24.0%) or chron­ic aor­tic dis­sec­tion (24.0%). The aor­tic ­valve ­was ­involved in 97.5% of ­all cas­es.
Results. The ­total ear­ly mor­tal­ity (≤30 ­days) ­was 3.8%, 10.5% ­for ­acute aor­tic dis­sec­tion ­and 2.4% ­for ­non-dis­sest­ing aneu­rysms. There ­were no ear­ly post­op­er­a­tive ­deaths in ­patients ­after ­valve-spar­ing oper­a­tion ­and in ­those ­with chron­ic aor­tic dis­sec­tion. The fol­low-up ­rate ­was 98.7%. During a ­mean fol­low-up of 68±25 ­months 10 ­patients (13.3%) ­died ­and car­diac com­pli­ca­tions ­were a com­mon ­cause of ­the ­late ­deaths. There ­was no ­late mor­tal­ity in ­the ­valve-spar­ing oper­a­tions dur­ing a ­mean fol­low-up peri­od of 8±6 ­months, how­ev­er, 1 ­required ­valve replace­ment. 19 (25.3%) of ­the 75 ­patients sur­viv­ing ­late ­have under­gone 25 sec­on­dary oper­a­tions on ­the car­di­o­vas­cu­lar ­system. Reoperations at aor­tic ­sites dis­tant ­from ­the orig­i­nal ­were ­much ­more fre­quent ­after pri­mary ­repair ­for ­acute ­and chron­ic dis­sec­tion ­when com­pared to ­non-dis­sect­ing aneu­rysms (p<0.005). Actuarial sur­vi­val ­rate of ­all ­patients ­with com­pos­ite ­graft replace­ment includ­ing ear­ly ­deaths ­was 91.2% at 1 ­year, 84.4% at 5 ­years ­and 75.2% at 10 ­years.
Conclusions. Composite ­graft inser­tion ­has ­become ­the ­gold stan­dard ­for treat­ing Marfan-­patients ­with ­non-dis­sect­ing ­and dis­sect­ing aneu­rysms of ­the aor­tic ­root. Our ear­ly expe­ri­ence in 11 ­patients ­with ­valve-spar­ing pro­ce­dures indi­cat­ed ­that ­this var­i­ant ­may be ­the bet­ter ­choice in select­ed ­patients.

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