Ghanta Ravi K, Green Susan Y, Price Matt D, Arredondo Courtney C, Wainwright D'Arcy, Preventza Ourania, de la Cruz Kim I, Aftab Muhammad, LeMaire Scott A, Coselli Joseph S
Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas; CHI St. Luke's Health-Baylor St. Luke's Medical Center, Houston, Texas; Division of Thoracic and Cardiovascular Surgery, University of Virginia, Charlottesville, Virginia.
Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas.
Ann Thorac Surg. 2016 Apr;101(4):1402-9; discussion 1409. doi: 10.1016/j.athoracsur.2015.10.018. Epub 2016 Jan 13.
Pathologic conditions of the aorta remain a major source of morbidity and mortality for patients with Marfan syndrome (MFS). Extensive thoracoabdominal aortic aneurysm (TAAA) repair can prevent aortic catastrophe but carries substantial risk of perioperative adverse events. We evaluated midterm survival and quality of life (QoL) after contemporary Crawford extent II TAAA repair in patients with MFS.
From 2004 to 2010, 49 consecutive patients with MFS (mean age, 43.4 ± 12.0 years) underwent extent II TAAA repair (41 elective and 8 urgent/emergent procedures) with intercostal reimplantation. Thirty-six patients (73%) had aorta-related symptoms, and 45 (92%) had distal aortic dissection. Operative adjuncts included cerebrospinal fluid drainage (n = 47 [96%]), left heart bypass (n = 46 [94%]), and cold renal perfusion (n = 47 [96%]). Kaplan-Meier survival analysis was performed. QoL was assessed in 24 patients with a 12-item survey (12-Item Short Form Health Survey version 2 [SF-12v2]) a median of 5.3 (interquartile range [IQR], 4.0-7.9) years postoperatively. QoL data were normalized and compared with data from the general population.
There were no operative deaths, strokes, paraparesis, or paraplegia. Two patients (4%) had permanent renal failure necessitating hemodialysis. The most frequent complication was vocal cord paralysis (n = 21 [43%]). Six-year Kaplan-Meier survival was 84% ± 6%. The 24 patients with QoL data had slightly worse physical component scores (46.0 ± 10.6) and slightly better mental component scores (51.4 ± 10.4) than the general population (50 ± 10 for both scores).
Operative treatment of extensive TAAA in patients with MFS enables excellent midterm survival and QoL. Cerebrospinal fluid drainage, left heart bypass, and cold renal perfusion probably aid in achieving excellent outcomes.
主动脉病理状况仍是马方综合征（MFS）患者发病和死亡的主要原因。广泛的胸腹主动脉瘤（TAAA）修复可预防主动脉灾难，但围手术期不良事件风险很大。我们评估了当代克劳福德II型TAAA修复术后MFS患者的中期生存率和生活质量（QoL）。
2004年至2010年，49例连续的MFS患者（平均年龄43.4±12.0岁）接受了II型TAAA修复（41例择期手术和8例急诊/紧急手术）并进行肋间血管再植入。36例患者（73%）有主动脉相关症状，45例（92%）有远端主动脉夹层。手术辅助措施包括脑脊液引流（n = 47 [96%]）、左心转流（n = 46 [94%]）和冷肾灌注（n = 47 [96%]）。进行了Kaplan-Meier生存分析。在术后中位时间5.3年（四分位间距[IQR]，4.0 - 7.9年）时，对24例患者用12项调查（12项简明健康调查第2版[SF - 12v2]）评估生活质量。生活质量数据进行了标准化处理，并与一般人群的数据进行比较。
无手术死亡、中风、轻截瘫或截瘫发生。2例患者（4%）出现永久性肾衰竭，需要进行血液透析。最常见的并发症是声带麻痹（n = 21 [43%]）。Kaplan-Meier六年生存率为84%±6%。有生活质量数据的24例患者的身体成分得分（46.0±10.6）略低于一般人群（两项得分均为50±10），心理成分得分（51.4±10.4）略高于一般人群。
MFS患者广泛TAAA的手术治疗可实现良好的中期生存率和生活质量。脑脊液引流、左心转流和冷肾灌注可能有助于取得良好的治疗效果。
