PMID- 26794897 OWN - NLM STAT- MEDLINE DCOM- 20160801 LR - 20160322 IS - 1552-6259 (Electronic) IS - 0003-4975 (Linking) VI - 101 IP - 4 DP - 2016 Apr TI - Midterm Survival and Quality of Life After Extent II Thoracoabdominal Aortic Repair in Marfan Syndrome. PG - 1402-9; discussion 1409 LID - S0003-4975(15)01678-1 [pii] LID - 10.1016/j.athoracsur.2015.10.018 [doi] AB - BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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. CI - Copyright (c) 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved. FAU - Ghanta, Ravi K AU - Ghanta RK AD - 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. FAU - Green, Susan Y AU - Green SY AD - 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. FAU - Price, Matt D AU - Price MD AD - 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. FAU - Arredondo, Courtney C AU - Arredondo CC AD - 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. FAU - Wainwright, D'Arcy AU - Wainwright D AD - 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. FAU - Preventza, Ourania AU - Preventza O AD - Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Cardiovascular Research Institute, 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. FAU - de la Cruz, Kim I AU - de la Cruz KI AD - Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Cardiovascular Research Institute, 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. FAU - Aftab, Muhammad AU - Aftab M AD - 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. FAU - LeMaire, Scott A AU - LeMaire SA AD - Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Cardiovascular Research Institute, 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. Electronic address: slemaire@bcm.edu. FAU - Coselli, Joseph S AU - Coselli JS AD - Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Cardiovascular Research Institute, 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. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20160113 PL - Netherlands TA - Ann Thorac Surg JT - The Annals of thoracic surgery JID - 15030100R SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aortic Aneurysm, Thoracic/etiology/*mortality/*surgery MH - *Blood Vessel Prosthesis Implantation MH - Female MH - Humans MH - Male MH - Marfan Syndrome/*complications/mortality/surgery MH - Middle Aged MH - *Quality of Life MH - Retrospective Studies MH - Survival Analysis MH - Survival Rate MH - Treatment Outcome MH - Young Adult EDAT- 2016/01/23 06:00 MHDA- 2016/08/02 06:00 CRDT- 2016/01/23 06:00 PHST- 2015/01/28 00:00 [received] PHST- 2015/08/27 00:00 [revised] PHST- 2015/10/09 00:00 [accepted] PHST- 2016/01/23 06:00 [entrez] PHST- 2016/01/23 06:00 [pubmed] PHST- 2016/08/02 06:00 [medline] AID - S0003-4975(15)01678-1 [pii] AID - 10.1016/j.athoracsur.2015.10.018 [doi] PST - ppublish SO - Ann Thorac Surg. 2016 Apr;101(4):1402-9; discussion 1409. doi: 10.1016/j.athoracsur.2015.10.018. Epub 2016 Jan 13.