BMT is justified in uncomplicated IMHB. However, IMHB becomes complicated in the majority of patients within 20 days. TEVAR in complicated IMHB is feasible but associated with a substantial aortic reintervention rate, reflecting technical challenges and fragile aortic wall conditions.

BackgroundThe most important structural proteins of the vascular wall are collagen and elastin. Genetically linked connective tissue diseases lead to degeneration, aneurysm formation and spontaneous dissection or rupture of arteries. The most well-known are Marfan syndrome, vascular Ehlers-Danlos syndrome (type IV), Loeys-Dietz syndrome and familial aortic aneurysms and dissections.ObjectiveThis review article addresses the current status of endovascular treatment options for important connective tissue diseases.Material and methodsEvaluation of currently available randomized studies and registry data.ResultsThe treatment of choice for patients that are mostly affected at a young age is primarily conservative or open repair. There is only limited evidence for endovascular aortic repair (EVAR) of abdominal aneurysms or thoracic endovascular aortic repair (TEVAR).ConclusionThe progression of the disease with dilatation leads to secondary endoleaks and high reintervention rates with uncertain long-term results. For this reason, there is currently consensus that EVAR and TEVAR should be limited to justified exceptional cases and emergency situations in patients with genetically linked aortic diseases.

Long-term results after TEVAR for aneurysmatic aortic disease demonstrate considerable overall and procedure-related mortality in both elective and urgent indications. Complications and RIs occur still as late events and emphasize the necessity for long-term follow-up.

BackgroundThe aim of this study was to analyze device conformability in TEVAR of acute and chronic (a/c) type B aortic dissections (TBAD) using the Gore Conformable Thoracic Aortic Stent-graft (CTAG).Material/MethodsFrom January 1997 to February 2014, a total of 90 out of 405 patients in our center received TEVAR for TBAD. Since November 2009, 23 patients (16 men; median age: 62 years) were treated with the CTAG. Indications were complicated aTBAD in 15 (65%) and expanding cTBAD in 8 (35%) patients. Primary endpoints were the assessment of device conformability by measuring the distance (D) from the radiopaque gold band marker (GM) at the proximal CTAG end to the inner curvature (IC) of the arch on parasagittal multiplanar reformations of CT angiography, as well as the evaluation of aortic diameter changes following TEVAR. Median follow-up was 13.3 months (range: 2 days to 35 months).ResultsPrimary and secondary success rates were 91.3% (21/23) and 95.6% (22/23), respectively. There was 1 type Ia endoleak, retrograde dissection or primary conversion was not observed. Median GM-IC-D was 0 mm (range: 0 mm to 10 mm). GM-IC-D was associated with zone 2 placement compared to zone 3 (P=0.036). There was no association between GM-IC-D formation and arch type. In aTBAD cases the true lumen significantly increased after TEVAR (P=0.017) and the false lumen underwent shrinkage (P=0.025). In cTBAD patients the false lumen decreased after TEVAR (P=0.036).ConclusionsThe CTAG shows favorable conformability and wall apposition in challenging arch pathologies such as TBAD.

Objective: The aim of this study was to report 10-year real-world single-center experience with the GORE TAG conformable thoracic aortic graft (CTAG), focusing on rupture-free survival, aortic-related reintervention, and device-related complications during midterm and long-term follow-up (FU). Methods: This retrospective study analyzes results of thoracic endovascular aortic repair (TEVAR) performed between January 2009 and December 2018. Out of 419 TEVAR procedures within this period, 194 patients (male 57.2%, 111/194), with a mean age of 65 ± 13 years, were treated with the CTAG device. Indication for TEVAR was a thoracic aortic aneurysm in 24.7% (48/194), type B aortic dissection in 32.5% (63/194), penetrating aortic ulcer 15.5% (30/194), and miscellaneous 27.3% (53/194). Emergently were operated 43.8% (85/194) patients. Median follow-up (FU) including computed tomography imaging was 43.5 months (Q1-Q3: 8.6–67.0) and was completed in 91.2% (177/194) of patients. Results: Overall survival rates were 75.8% (95% confidence interval [CI] = [0.76–0.70]) and 56.6% (95% CI = [0.57–0.50]) at 12 and 60 months, respectively. Cumulative incidence for aortic rupture was 11.9% (95% CI = [0.07–0.17]) at 60 and 90 months, respectively. Cumulative incidence for aortic-related reintervention was 27.5% (95% CI = [0.21–0.34]) at 60 and 90 months. Cumulative incidence for migration was 2.8% (95% CI = [0.004–0.05]) and 3.9% (95% CI = [0.007–0.07]) at 60 and 90 months, respectively. New endograft infections or material fatigue were not observed. Conclusions: The herein reported 10-year real-world single-center experience with the CTAG observed favorable long-term outcome. Thus, the device demonstrates appropriate persistent safety, efficacy, and clinical durability up to long-term FU in the treatment of diverse thoracic aortic pathologies.

OBJECTIVES To evaluate the impact of the coronavirus disease 2019 (COVID-19) pandemic on acute and elective thoracic and abdominal aortic procedures. METHODS Forty departments shared their data on acute and elective thoracic and abdominal aortic procedures between January and May 2020 and January and May 2019 in Europe, Asia and the USA. Admission rates as well as delay from onset of symptoms to referral were compared. RESULTS No differences in the number of acute thoracic and abdominal aortic procedures were observed between 2020 and the reference period in 2019 [incidence rates ratio (IRR): 0.96, confidence interval (CI) 0.89–1.04; P = 0.39]. Also, no difference in the time interval from acute onset of symptoms to referral was recorded (<12 h 32% vs > 12 h 68% in 2020, < 12 h 34% vs > 12 h 66% in 2019 P = 0.29). Conversely, a decline of 35% in elective procedures was seen (IRR: 0.81, CI 0.76–0.87; P < 0.001) with substantial differences between countries and the most pronounced decline in Italy (−40%, P < 0.001). Interestingly, in Switzerland, an increase in the number of elective cases was observed (+35%, P = 0.02). CONCLUSIONS There was no change in the number of acute thoracic and abdominal aortic cases and procedures during the initial wave of the COVID-19 pandemic, whereas the case load of elective operations and procedures decreased significantly. Patients with acute aortic syndromes presented despite COVID-19 and were managed according to current guidelines. Further analysis is required to prove that deferral of elective cases had no impact on premature mortality.

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