TY - JOUR
T1 - Editor's Choice - Open Thoracic and Thoraco-abdominal Aortic Repair in Patients with Connective Tissue Disease
AU - Keschenau, Paula R.
AU - Kotelis, Drosos
AU - Bisschop, Jeroen
AU - Barbati, Mohammad E.
AU - Grommes, Jochen
AU - Mees, Barend
AU - Gombert, Alexander
AU - Peppelenbosch, Arnoud G.
AU - Schurink, Geert Willem H.
AU - Kalder, Johannes
AU - Jacobs, Michael J.
PY - 2017/11
Y1 - 2017/11
N2 - Objective/Background: The aim is to present current results of open complex aortic repair in patients with connective tissue disease (CTD).Methods: This was a retrospective cross-border, single centre study. From February 2000 to April 2016 72 aortic operations were performed on 65 patients with CTD (41 male, median age 41 years [range 19-70 years]). Fifty-six patients (86%) underwent at least one previous aortic repair (71 open, four endovascular), including 33 patients (51%) operated before at the site of the procedure reported here. The open procedures, counting eight emergency operations (11%), included aortic arch revision (n = 1; 1%), descending thoracic aortic repair (n = 11; 15%), TAAA type I repair (n = 12; 17%), type II repair (n = 29; 40%), type III repair (n = 12; 17%), and type IV repair (n = 5; 7%). Simultaneous repair of the ascending aorta and/or the aortic arch was performed in two (3%) and eight cases (11%), respectively. Seven patients (10%) underwent staged procedures. Median follow-up was 42 months (0.5-180 months).Results: The in hospital mortality was 14% (n = 9) as a result of haemorrhage (n = 3/9), neurological (n = 3/9), cardiac (n = 2/9), and pulmonary (n = 1/9) complications. Paraplegia and paraparesis occurred in one (2%) and three patients (5%), respectively. Seven patients (11%) required temporary dialysis; none needed permanent dialysis. Major complications were revision surgery for bleeding or haematoma (n = 20/65), sepsis (n = 10/65), myocardial infarction/severe cardiac arrhythmia (n = 2/65), stroke (n = 2/65), as well as multiorgan failure, abdominal compartment syndrome, mesenteric and peripheral ischaemia (all n = 1/65). Multivariate analysis identified an operating time > 7 hours (p = .006) as an independent predictor of increased mortality. Freedom from re-intervention was 85%, 1 year survival was 80%, and overall survival was 75%.Conclusion: Open TAA(A) repair is a durable therapy for patients with CTD. Often being performed as revision surgery, it can be associated with relevant risks and should therefore be reserved for specialised centres. Staged procedures and thus reducing operating time, if applicable, should be preferred. (C) 2017 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.
AB - Objective/Background: The aim is to present current results of open complex aortic repair in patients with connective tissue disease (CTD).Methods: This was a retrospective cross-border, single centre study. From February 2000 to April 2016 72 aortic operations were performed on 65 patients with CTD (41 male, median age 41 years [range 19-70 years]). Fifty-six patients (86%) underwent at least one previous aortic repair (71 open, four endovascular), including 33 patients (51%) operated before at the site of the procedure reported here. The open procedures, counting eight emergency operations (11%), included aortic arch revision (n = 1; 1%), descending thoracic aortic repair (n = 11; 15%), TAAA type I repair (n = 12; 17%), type II repair (n = 29; 40%), type III repair (n = 12; 17%), and type IV repair (n = 5; 7%). Simultaneous repair of the ascending aorta and/or the aortic arch was performed in two (3%) and eight cases (11%), respectively. Seven patients (10%) underwent staged procedures. Median follow-up was 42 months (0.5-180 months).Results: The in hospital mortality was 14% (n = 9) as a result of haemorrhage (n = 3/9), neurological (n = 3/9), cardiac (n = 2/9), and pulmonary (n = 1/9) complications. Paraplegia and paraparesis occurred in one (2%) and three patients (5%), respectively. Seven patients (11%) required temporary dialysis; none needed permanent dialysis. Major complications were revision surgery for bleeding or haematoma (n = 20/65), sepsis (n = 10/65), myocardial infarction/severe cardiac arrhythmia (n = 2/65), stroke (n = 2/65), as well as multiorgan failure, abdominal compartment syndrome, mesenteric and peripheral ischaemia (all n = 1/65). Multivariate analysis identified an operating time > 7 hours (p = .006) as an independent predictor of increased mortality. Freedom from re-intervention was 85%, 1 year survival was 80%, and overall survival was 75%.Conclusion: Open TAA(A) repair is a durable therapy for patients with CTD. Often being performed as revision surgery, it can be associated with relevant risks and should therefore be reserved for specialised centres. Staged procedures and thus reducing operating time, if applicable, should be preferred. (C) 2017 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.
KW - Connective tissue diseases
KW - Ehlers-Danlos syndrome
KW - Loeys-Dietz syndrome
KW - Marfan syndrome
KW - Open aortic repair
KW - Thoraco-abdominal aortic aneurysm
KW - SYNDROME TYPE-IV
KW - MARFAN-SYNDROME
KW - ANEURYSM REPAIR
KW - ENDOVASCULAR TREATMENT
KW - B DISSECTION
KW - DISORDERS
KW - STRATEGIES
KW - MANAGEMENT
KW - OUTCOMES
KW - SURGERY
U2 - 10.1016/j.ejvs.2017.07.026
DO - 10.1016/j.ejvs.2017.07.026
M3 - Article
C2 - 28916127
SN - 1078-5884
VL - 54
SP - 588
EP - 596
JO - European Journal of Vascular and Endovascular Surgery
JF - European Journal of Vascular and Endovascular Surgery
IS - 5
ER -