Cacciapuoti Fulvio, Mauro Ciro, Caso Ilaria, Nasti Rodolfo, Tirelli Paolo, Cacciapuoti Federico
Department of Cardiology, "V. Monaldi" Hospital, Naples, Italy.
Department of Cardiology, "A. Cardarelli" Hospital, Naples, Italy.
J Cardiol Cases. 2023 Jan 25;27(4):188-191. doi: 10.1016/j.jccase.2023.01.002. eCollection 2023 Apr.
There has been an exponential increase in incidence of severe aortic stenosis partially due to the lengthening of average lifespan. Among the most disabling symptoms of aortic stenosis are chest pain, fatigue, and dyspnea up to heart failure and pulmonary edema. In some cases, to worsen this symptomatology, there are coagulation disorders linked to an alteration of functional von Willebrand factor, responsible for progressive anemia. In elderly patients with severe aortic stenosis, the simultaneous presence of an angiodysplasia of the colon can favor blood dripping, which may cause iron deficiency anemia. The coexistence of colonic angiodysplasia and acquired von Willebrand disease in patients with aortic stenosis was identified as Heyde's syndrome. In the long term, Heyde's syndrome can contribute to worsen the clinical manifestations of severe aortic stenosis leading to heart failure. Here, we describe the case of a patient suffering of severe calcific aortic stenosis who developed Heyde's syndrome achieving a condition of heart failure with mildly reduced ejection fraction.
Severe aortic stenosis can alter the conformation of the circulating von Willebrand glycoprotein, causing an alteration of the hemostatic balance. When angiodysplasia of the colon coexists with aortic stenosis, a gastrointestinal blood drip can occur inducing an iron deficiency anemia that worsens the symptoms of aortic valvulopathy. This condition often remains undiagnosed. We discuss the pathophysiologic and hemodynamic mechanisms responsible for acquired von Willebrand syndrome in patients with severe aortic stenosis focusing on the clinical elements useful to raise the diagnostic suspicion and analyzing different alternative tools to recognize it promptly.
严重主动脉瓣狭窄的发病率呈指数级增长，部分原因是平均寿命延长。主动脉瓣狭窄最致残的症状包括胸痛、疲劳和呼吸困难，直至心力衰竭和肺水肿。在某些情况下，与功能性血管性血友病因子改变相关的凝血障碍会加重这种症状，导致进行性贫血。在老年严重主动脉瓣狭窄患者中，结肠血管发育异常的同时存在可能导致血液渗出，进而引起缺铁性贫血。主动脉瓣狭窄患者中结肠血管发育异常与获得性血管性血友病并存被称为海德综合征。从长远来看，海德综合征会加重严重主动脉瓣狭窄的临床表现，导致心力衰竭。在此，我们描述一例患有严重钙化性主动脉瓣狭窄的患者，其发展为海德综合征，达到射血分数轻度降低的心力衰竭状态。
严重主动脉瓣狭窄可改变循环中的血管性血友病糖蛋白的构象，导致止血平衡改变。当结肠血管发育异常与主动脉瓣狭窄并存时，可发生胃肠道血液渗出，导致缺铁性贫血，加重主动脉瓣病变的症状。这种情况常常未被诊断出来。我们讨论严重主动脉瓣狭窄患者获得性血管性血友病的病理生理和血流动力学机制，重点关注有助于提高诊断怀疑的临床因素，并分析不同的辅助工具以便及时识别该病。
