Open Thoracic and Thoracoabdominal Aortic Repair in Patients with Connective Tissue Disease

Abstract

Introduction - Aortic dissections and aneurysms represent a leading cause for increased morbidity and mortality in Connective Tissue Disease (CTD) patients and the surgical treatment of CTD-based thoracic aortic aneurysms (TAA) and thoracoabdominal aortic aneurysms (TAAA) remains a challenge. Although the patients are usually younger and have less comorbidities than degenerative TAAA patients without CTD, many have undergone previous aortic surgery and necessitate subsequent replacement of the residual native aorta or a reintervention at the site of the previous operation. Due to high failure rates in CTD patients, especially in the mid- and long-term, endovascular aortic repair is currently not considered as a standard option for these patients. Open aortic repair has reportedly good mid- and long-term results and can be performed with reasonable morbidity and mortality rates in high volume centers. Thus, it still is the gold standard of therapy for CTD patients. The aim of this study was to present current results of open complex aortic repair in patients with connective tissue disease (CTD). Methods - This is a retrospective cross-border single-center study. From February 2000 to April 2016 72 aortic operations were performed on 65 CTD patients (41 male, median age 41 years, range 19-70 years). 56 patients (86%) underwent at least one previous aortic repair (71 open, 4 endovascular operations) including 33 patients (51%) operated before at the site of the herein reported procedure. The open procedures, counting 8 emergency operations (11%), included aortic arch revision (n=1, 1%), descending thoracic aortic (DTAA) 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 2 (3%) and 8 cases (11%), respectively. Seven patients (10%) underwent staged procedures. Median follow-up (FU) was 42 months (0.5-180 months). Results - The in-hospital mortality was 14% (n=9) due to hemorrhage (3/9), neurological (3/9), cardiac (2/9) and pulmonary (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 hematoma (20/72), sepsis (10/72), myocardial infarction/severe cardiac arrhythmia (3/72), stroke (2/72) as well as multi-organ failure, abdominal compartment syndrome, mesenteric and peripheral ischemia (1/72, respectively). Multivariate analysis identified an operating time >7 hours (p=0.006) as independent predictor of increased mortality. Freedom from reintervention was 85%, 1-year-survival was 80% and overall survival was 75%. Conclusion - This is the largest European (published) series of open TAA(A) repair in CTD patients. It demonstrates that open TAA(A) repair is a durable therapy for CTD patients. However, being often performed as revision surgery, it is associated with relevant mortality and morbidity risks. Staged procedures and thus reducing operating time, if applicable, should be preferred.