Vincentelli André, Susen Sophie, Le Tourneau Thierry, Six Isabelle, Fabre Olivier, Juthier Francis, Bauters Anne, Decoene Christophe, Goudemand Jenny, Prat Alain, Jude Brigitte
Equipe d'Accueil 2693, University of Lille II, Faculté de Médecine, Lille, France.
N Engl J Med. 2003 Jul 24;349(4):343-9. doi: 10.1056/NEJMoa022831.
Aortic-valve stenosis can be complicated by bleeding that is associated with acquired type 2A von Willebrand syndrome. However, the prevalence and cause of the hemostatic abnormality in aortic stenosis are unknown.
We enrolled 50 consecutive patients with aortic stenosis, who completed a standardized screening questionnaire to detect a history of bleeding. Forty-two patients with severe aortic stenosis underwent valve replacement. Platelet function under conditions of high shear stress, von Willebrand factor collagen-binding activity and antigen levels, and the multimeric structure of von Willebrand factor were assessed at base line and one day, seven days, and six months postoperatively.
Skin or mucosal bleeding occurred in 21 percent of the patients with severe aortic stenosis. Platelet-function abnormalities under conditions of high shear stress, decreased von Willebrand factor collagen-binding activity and the loss of the largest multimers, or a combination of these was present in 67 to 92 percent of patients with severe aortic stenosis and correlated significantly with the severity of valve stenosis. Primary hemostatic abnormalities were completely corrected on the first day after surgery but tended to recur at six months, especially when there was a mismatch between patient and prosthesis (with an effective orifice area of less than 0.8 cm2 per square meter of body-surface area).
Type 2A von Willebrand syndrome is common in patients with severe aortic stenosis. Von Willebrand factor abnormalities are directly related to the severity of aortic stenosis and are improved by valve replacement in the absence of mismatch between patient and prosthesis.
主动脉瓣狭窄可能并发与获得性2A型血管性血友病综合征相关的出血。然而，主动脉狭窄中止血异常的患病率和病因尚不清楚。
我们连续纳入了50例主动脉狭窄患者，这些患者完成了标准化筛查问卷以检测出血史。42例重度主动脉狭窄患者接受了瓣膜置换术。在基线以及术后1天、7天和6个月时评估高剪切应力条件下的血小板功能、血管性血友病因子胶原结合活性和抗原水平以及血管性血友病因子的多聚体结构。
重度主动脉狭窄患者中21%发生皮肤或黏膜出血。重度主动脉狭窄患者中67%至92%存在高剪切应力条件下的血小板功能异常、血管性血友病因子胶原结合活性降低以及最大多聚体缺失，或这些情况的组合，且与瓣膜狭窄的严重程度显著相关。原发性止血异常在术后第1天完全纠正，但在6个月时倾向于复发，尤其是当患者与假体不匹配时（有效瓣口面积每平方米体表面积小于0.8 cm²）。
2A型血管性血友病综合征在重度主动脉狭窄患者中很常见。血管性血友病因子异常与主动脉狭窄的严重程度直接相关，并且在患者与假体无不匹配的情况下通过瓣膜置换可得到改善。
