Nguyen Hien Sinh, Nguyen Hung Dang, Tong Phuc Duy, Pham Thanh Tan, Ngo Thanh Van
Center of Cardiovascular Surgery, Hanoi Heart Hospital, Hanoi, Viet Nam.
Department of Pediatric Cardiac Surgery, Hanoi Heart Hospital, Hanoi, Viet Nam.
Int J Surg Case Rep. 2021 May;82:105930. doi: 10.1016/j.ijscr.2021.105930. Epub 2021 Apr 29.
Aortic regurgitation and aortic root dilatation are common features in adults with both repaired and unrepaired tetralogy of Fallot (TOF). Valve-sparing aortic root replacement (VSARR) is an effective repair for aortic regurgitation due to progressive aortic root dilatation with TOF after repair. However, the effectiveness of this technique for unrepaired patients has rarely been reported.
We reported a case of a 30-year-old man with cyanosis and exertional dyspnea. Echocardiography and computed tomography showed unrepaired TOF with significant aortic regurgitation and massively dilated aortic root. Aortic root replacement was mandatory. He underwent successful concomitant VSARR with TOF repair. At 6-month follow-up, he remains stable with trivial aortic regurgitation on echocardiogram.
In unrepaired TOF, the absence of sub-annular muscular rim and the unbalance of aortic sinuses make VSARR utmost challenging. If can be done successfully, VSARR preserves the native aortic valve and avoids lifelong anticoagulation therapy.
Concomitant VSARR with TOF repair can be safely and effectively applied for unrepaired patients presenting with progressive aortic root dilatation and significant aortic regurgitation.
主动脉瓣反流和主动脉根部扩张是法洛四联症(TOF)患者(无论是否接受过修复手术)的常见特征。保留瓣膜的主动脉根部置换术(VSARR)是治疗因修复后法洛四联症患者主动脉根部逐渐扩张导致的主动脉瓣反流的有效方法。然而,该技术在未修复患者中的有效性鲜有报道。
我们报告了一例30岁男性患者,有紫绀和劳力性呼吸困难。超声心动图和计算机断层扫描显示为未修复的法洛四联症,伴有严重的主动脉瓣反流和主动脉根部大量扩张。必须进行主动脉根部置换术。他成功地接受了同期VSARR和法洛四联症修复手术。在6个月的随访中,他情况稳定,超声心动图显示有轻微主动脉瓣反流。
在未修复的法洛四联症中,瓣环下肌肉边缘的缺失和主动脉窦的失衡使得VSARR极具挑战性。如果能成功实施,VSARR可保留天然主动脉瓣并避免终身抗凝治疗。
同期VSARR和法洛四联症修复术可安全有效地应用于出现主动脉根部逐渐扩张和严重主动脉瓣反流的未修复患者。