背景:间接性大疱性表皮松解症是一种罕见的皮肤和粘膜疾病,其特征是由于轻微创伤和皮肤外表现而形成水疱。由于皮肤和粘膜脆性,大疱性表皮松解症患者没有心脏手术和预后的报道。
方法:一位55岁的男性,患有先天性交界性大疱性表皮松解症,高血压,血管痉挛型心绞痛.他抱怨劳累时呼吸困难,经胸超声心动图显示主动脉瓣严重反流,中度主动脉瓣狭窄(三尖瓣),和严重的二尖瓣反流.考虑到右胸壁的皮肤状况相对健康,右胸切开术是首选方法,并使用无缝合生物瓣膜进行完全内镜下伴随二尖瓣修复和主动脉瓣置换(Perceval™(Corcym,Group,米兰,意大利))。聚氨酯和硅敷料泡沫用于保护与袋阀面罩接触的部位的皮肤,动脉压导管,静脉导管,和气管插管.垂直床垫缝线用于皮肤缝线。术后进展顺利,病人在手术后九天出院。直到三年随访期才有再次手术的迹象。
结论:对于患有交界性大疱性表皮松解症的患者,通过充分保护皮肤和粘膜,可以安全地进行使用Perceval™假体的完全内镜下伴随主动脉瓣和二尖瓣手术。
BACKGROUND: Junctional epidermolysis bullosa is a rare skin and mucosal disorder characterized by blister formation in response to minor trauma and extracutaneous manifestations. There have been no
reports of cardiac surgery and prognostication in patients with epidermolysis bullosa due to skin and mucosal fragility.
METHODS: A 55-year-old man presented with congenital junctional epidermolysis bullosa, hypertension, and vasospastic angina. He complained of dyspnea on exertion, and transthoracic echocardiography revealed severe aortic valve regurgitation, moderate aortic valve stenosis (tricuspid valve), and severe mitral valve regurgitation. Considering that the skin condition in the right chest wall was relatively healthy, the right thoracotomy approach was preferred and totally endoscopic concomitant mitral valve repair and aortic valve replacement were performed using a sutureless bioprosthetic valve (Perceval™ (Corcym, Group, Milan, Italy)). Polyurethane and silicon dressing foams were used to protect the skin at the site of contact with the bag valve mask, arterial pressure catheter, intravenous catheter, and the tracheal intubation tube. Vertical mattress sutures were used for the skin sutures. The postoperative course was uneventful, and the patient was discharged nine days after the operation. There was no indication for reoperation until three years follow-up period.
CONCLUSIONS: The totally endoscopic concomitant aortic and mitral valve surgery using Perceval™ prosthesis can be performed safely in patients with junctional epidermolysis bullosa by adequate protection of the skin and mucosa.