Type A aortic dissection in Marfan syndrome: extent of initial surgery determines long-term outcome.


BACKGROUND:Data on outcomes after Stanford type A aortic dissection in patients with Marfan syndrome are limited. We investigated the primary surgery and long-term results in patients with Marfan syndrome who suffered aortic dissection. METHODS AND RESULTS:Among 1324 consecutive patients with aortic dissection type A, 74 with Marfan syndrome (58% men; median age, 37 years [first and third quartiles, 29 and 48 years]) underwent surgical repair (85% acute dissections; 68% DeBakey I; 55% composite valved graft, 30% supracoronary ascending replacement, 15% valve-sparing aortic root replacement; 12% total arch replacement; 3% in-hospital mortality) at 2 tertiary centers in the United States and Europe over the past 25 years. The rate of aortic reintervention with resternotomy was 24% (18 of 74) and of descending aorta (thoracic+abdominal) intervention was 30% (22 of 74) at a median follow-up of 8.4 years (first and third quartiles, 2.2 and 12.7 years). Freedom from need for aortic root reoperation in patients who underwent primarily a composite valved graft or valve-sparing aortic root replacement procedure was 95±3%, 88±5%, and 79±5% and in patients who underwent supracoronary ascending replacement was 83±9%, 60±13%, 20±16% at 5, 10, and 20 years. Secondary aortic arch surgery was necessary only in patients with initial hemi-arch replacement. CONCLUSIONS:Emergency surgery for type A dissection in patients with Marfan syndrome is associated with low in-hospital mortality. Failure to extend the primary surgery to aortic root or arch repair leads to a highly complex clinical course. Aortic root replacement or repair is highly recommended because supracoronary ascending replacement is associated with a high need (>40%) for root reintervention.






Rylski B,Bavaria JE,Beyersdorf F,Branchetti E,Desai ND,Milewski RK,Szeto WY,Vallabhajosyula P,Siepe M,Kari FA




Has Abstract


2014-04-01 00:00:00














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    pub_type: 临床试验,杂志文章,多中心研究,随机对照试验


    authors: Doughty RN,Whalley GA,Walsh HA,Gamble GD,López-Sendón J,Sharpe N,CAPRICORN Echo Substudy Investigators.

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  • Thromboxane A2, prostacyclin and aspirin: effects on vascular tone and platelet aggregation.

    abstract::Novel compounds that induce or inhibit platelet aggregation and constrict or dilate blood vessels were recently discovered. These compounds are all derivatives of arachidonic acid and include prostaglandin endoperoxides, thromboxane A2, prostaglandin E2, prostaglandin D2 and prostacyclin. Thromboxane A2 (TxA2) could b...


    pub_type: 杂志文章,评审


    authors: Smith JB,Araki H,Lefer AM

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  • Comparison of techniques for measuring baroreflex sensitivity in man.

    abstract::Because discrepancies about baroreflex sensitivity in essential hypertension may have resulted from the use of different measurement techniques, we assessed the extent to which the results of different techniques agree in the same subjects. The eight techniques studied were the change in RR interval per unit change in...


    pub_type: 杂志文章


    authors: Goldstein DS,Horwitz D,Keiser HR

    更新日期:1982-08-01 00:00:00

  • In vivo evidence for nitric oxide-mediated calcium-activated potassium-channel activation during human endotoxemia.

    abstract:BACKGROUND:During septic shock, the vasoconstrictor response to norepinephrine is seriously blunted. Animal experiments suggest that hyperpolarization of smooth muscle cells by opening of potassium (K) channels underlies this phenomenon. In the present study, we examined whether K-channel blockers and/or nitric oxide (...


    pub_type: 临床试验,杂志文章


    authors: Pickkers P,Dorresteijn MJ,Bouw MP,van der Hoeven JG,Smits P

    更新日期:2006-08-01 00:00:00

  • Gender gap in aortic cholesterol accumulation in cholesterol-clamped rabbits: role of the endothelium and mononuclear-endothelial cell interaction.

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    pub_type: 杂志文章


    authors: Holm P,Andersen HL,Arrøe G,Stender S

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  • Locus for atrial fibrillation maps to chromosome 6q14-16.

    abstract:BACKGROUND:Atrial fibrillation (AF), the most common clinical arrhythmia, is a major cause of morbidity and mortality. Although AF is often associated with other cardiovascular conditions, many patients present without an obvious etiology. Inherited forms of AF exist, but the causative gene has been defined only in a s...


    pub_type: 杂志文章


    authors: Ellinor PT,Shin JT,Moore RK,Yoerger DM,MacRae CA

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    pub_type: 杂志文章


    authors: Zhang J,Ding L,Zhao Y,Sun W,Chen B,Lin H,Wang X,Zhang L,Xu B,Dai J

    更新日期:2009-04-07 00:00:00

  • AMP-activated protein kinase inhibits angiotensin II-stimulated vascular smooth muscle cell proliferation.

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    pub_type: 杂志文章


    authors: Nagata D,Takeda R,Sata M,Satonaka H,Suzuki E,Nagano T,Hirata Y

    更新日期:2004-07-27 00:00:00

  • Inhibition of glycosphingolipid synthesis ameliorates atherosclerosis and arterial stiffness in apolipoprotein E-/- mice and rabbits fed a high-fat and -cholesterol diet.

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    pub_type: 杂志文章


    authors: Chatterjee S,Bedja D,Mishra S,Amuzie C,Avolio A,Kass DA,Berkowitz D,Renehan M

    更新日期:2014-06-10 00:00:00

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    pub_type: 杂志文章


    authors: Fosbøl EL,Zhao Y,Shahian DM,Grover FL,Edwards FH,Peterson ED

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    pub_type: 杂志文章


    authors: Schnittger I,Fitzgerald PJ,Gordon EP,Alderman EL,Popp RL

    更新日期:1984-08-01 00:00:00

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    pub_type: 杂志文章,多中心研究,随机对照试验


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    pub_type: 杂志文章


    authors: Moreau M,Brocheriou I,Petit L,Ninio E,Chapman MJ,Rouis M

    更新日期:1999-01-26 00:00:00

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    pub_type: 杂志文章


    authors: Nozawa T,Cheng CP,Noda T,Little WC

    更新日期:1994-02-01 00:00:00

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    pub_type: 杂志文章


    authors: Chen PS,Wolf PD,Claydon FJ,Dixon EG,Vidaillet HJ Jr,Danieley ND,Pilkington TC,Ideker RE

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    authors: Yaoita H,Ogawa K,Maehara K,Maruyama Y

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    authors: Christen T,Nahrendorf M,Wildgruber M,Swirski FK,Aikawa E,Waterman P,Shimizu K,Weissleder R,Libby P

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    pub_type: 杂志文章


    authors: Yasue H,Takizawa A,Nagao M,Nishida S,Horie M,Kubota J,Omote S,Takaoka K,Okumura K

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    pub_type: 杂志文章


    authors: Michelson EL,Morganroth J

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    pub_type: 杂志文章,评审


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    pub_type: 杂志文章


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    pub_type: 杂志文章


    authors: Zhang P,Kirk JA,Ji W,dos Remedios CG,Kass DA,Van Eyk JE,Murphy AM

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    pub_type: 杂志文章


    authors: Dufouil C,Beiser A,McLure LA,Wolf PA,Tzourio C,Howard VJ,Westwood AJ,Himali JJ,Sullivan L,Aparicio HJ,Kelly-Hayes M,Ritchie K,Kase CS,Pikula A,Romero JR,D'Agostino RB,Samieri C,Vasan RS,Chêne G,Howard G,Seshadri S

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    pub_type: 杂志文章


    authors: Yamanami M,Yahata Y,Uechi M,Fujiwara M,Ishibashi-Ueda H,Kanda K,Watanabe T,Tajikawa T,Ohba K,Yaku H,Nakayama Y

    更新日期:2010-09-14 00:00:00

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    pub_type: 杂志文章


    authors: Roberts-Thomson KC,Steven D,Seiler J,Inada K,Koplan BA,Tedrow UB,Epstein LM,Stevenson WG

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    pub_type: 杂志文章


    authors: Cappato R,Schlüter M,Mont L,Kuck KH

    更新日期:1994-08-01 00:00:00

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    pub_type: 临床试验,杂志文章,多中心研究,随机对照试验


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    pub_type: 杂志文章,多中心研究


    authors: Adler Y,Finkelstein Y,Guindo J,Rodriguez de la Serna A,Shoenfeld Y,Bayes-Genis A,Sagie A,Bayes de Luna A,Spodick DH

    更新日期:1998-06-02 00:00:00

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    pub_type: 杂志文章,评审


    authors: Yeung AC,Barry J,Selwyn AP

    更新日期:1990-09-01 00:00:00