Department of Cardiac Surgery, University Hospital Zürich, Zürich, Switzerland.
Department of Cardiology, University Hospital Zürich, Zürich, Switzerland.
Braz J Cardiovasc Surg. 2020 Dec 1;35(6):1007-1009. doi: 10.21470/1678-9741-2020-0061.
We present a case of a 36-year-old male patient with known arthrogryposis multiplex congenita and an associated unicuspid aortic valve. The patient later developed a significant aneurysm of the ascending aorta, however refused surgical intervention and missed follow-up appointments for 5 years. During an urgent, general practitioner-initiated transthoracic echocardiography follow-up, a chronic type A aortic dissection was diagnosed as a result of progressive aortic dilatation. Due to the stationary pressure gradients and non-progressive leaflet fibrosis, a conservative approach for to the unicuspid aortic valve was chosen, combined with replacement of the ascending aorta and partial replacement of the aortic arch.
我们报告了一例 36 岁男性患者，患有先天性多发性关节挛缩症和单一尖瓣主动脉瓣。该患者后来发展为升主动脉显著动脉瘤，但拒绝手术干预，并在 5 年内错过了随访预约。在一次紧急的全科医生发起的经胸超声心动图随访中，由于升主动脉进行性扩张，诊断为慢性 A 型主动脉夹层。由于固定的压力梯度和非进行性瓣叶纤维化，选择了对单一尖瓣主动脉瓣进行保守治疗，同时进行升主动脉置换和部分主动脉弓置换。
