PC170. National Review of Vascular Surgery in Marfan Syndrome and Ehlers-Danlos Syndrome

Abstract

Marfan Syndrome (MFS) and Ehlers-Danlos Syndrome (EDS) represent two connective tissue vascular diseases requiring unique consideration in their vascular surgical (VS) care. A comprehensive national review encompassing all hospitalizations for the MFS and EDS patient population is lacking. The National Inpatient Sample 2010 to 2014 was reviewed for all inpatient VS procedures. National estimates of VS rates were generated from provided weights. Patient demographics, procedure type and outcomes were measured. There were 3103 MFS, 476 EDS, and 3,895,381 other procedures identified. Open aortic aneurysm repair was greater in both MFS (16.8%) and EDS (11.2%) compared with controls (4.4%). While EVAR was similar among the groups, MFS (7.7%) and EDS (5.1%) had more TEVAR performed than controls (0.7%; P < .01). Although percutaneous procedures were fewer in MFS (6.3%) than controls (31.3%) and EDS (26.3%), repair of peripheral arteries was greater in MFS (5.9%) than EDS (4.1%) and controls (1.5%; P < .01; Table). Because aortic repair represented a significant fraction of the procedures in MFS (28%) and EDS (23.5%), the demographics and outcomes of these operations were compared with controls (9.2%). Mean age of aortic procedures was 68.2 years in controls vs MFS 45.8 years and EDS 55.3 years; P < .01. MFS and EDS had fewer comorbidities with controls having significantly higher rates of coronary artery disease (controls 39.9% vs MFS 8.3% and EDS 13.0%), chronic obstructive pulmonary disease (controls 30.0% vs MFS 16.7% and EDS 26.1%), and diabetes (controls 20.6% vs MFS 6.6 and EDS 4.4%). EDS had higher preoperative stroke rate (21.7%) compared with MFS (6.6%) and controls (7.1%; P < .01). MFS and EDS had higher complication rate (65.5% and 52.2%) compared with controls (44.6%; P < .01). Postoperative bleeding was more likely in MFS (42.9%) and EDS (39.1%) than controls (22.2%; P < .01). Increased respiratory failure was noted in MFS (20.2%) vs controls (10.7%) and EDS (8.7%; P < .01). Duration of stay was increased in MFS 12.5 days versus EDS 7.4 days and controls 7.2 days. Median costs of index hospitalization was greatest in MFS ($57,084 vs EDS $22,032 and controls $26,520, P < .01). Patients with MFS and EDS significantly differ from other patients undergoing VS procedures, with a significantly greater proportion of aortic interventions including more EVAR and TEVAR placement. While they are younger with fewer comorbidities, owing to the unique pathogenesis of their underlying connective tissue disorder, there is an overall higher rate of procedural complications.TablePercutaneous proceduresControlsMarfan SyndromeEhlers-Danlos SyndromePOpen aortic repair34,743 (4.4%)106 (16.8%)11 (11.2%)<.0001Endovascular aneurysm repair33,653 (4.2%)22 (3.5%)7 (7.1%).2302Thoracic endovascular aortic repair5,569 (0.7%)49 (7.7%)5 (5.1%)<.0001Aortic endo total39,038 (4.9%)69 (10.9%)12 (12.2%)<.0001Carotid98,454 (12.3%)8 (1.3%)2 (2.0%)<.0001Thoracic aorta/subclavian20,209 (2.5%)400 (63.2%)13 (13.3%)<.0001Lower extremity revascularization97,530 (12.2%)33 (5.2%)7 (7.1%).1611Upper extremity revascularization10,959 (1.4%)5 (0.8%)3 (3.1%)<.0001Percutaneous interventions250,327 (31.4%)40 (6.3%)26 (26.5%)<.0001Dialysis access47,784 (6.0%)5 (0.8%)3 (3.1%)<.0001Major amputation144,287 (18.1%)25 (4.0%)5 (5.1%)<.0001Peripheral arterial repair11,706 (1.5%)37 (5.9%)4 (4.1%)<.0001Inferior vena cava filter113,682 (14.2%)22 (3.5%)14 (14.3%)<.0001 Open table in a new tab