In situ bending of thoracic stent grafts: Clinical application of a novel technique to improve conformance to the aortic arch

PurposeA straight thoracic stent graft often complies poorly with the curvature of the aortic arch. We have previously reported an in vitro model of a modified stent graft that can be bent in situ after deployment to improve conformance to the aortic arch. We now report the first clinical experience with this technique in three consecutive patients.MethodsBetween September 2007 and August 2008, three patients were treated for different pathologies of the aortic arch with a modified thoracic stent graft that was fitted with a sliding self-locking knot and a detachable Bowden cable. Transfemoral traction on the Bowden cable enables controlled shortening of the proximal part of the stent graft at the inner curve after deployment. The stent graft is thereby directed to allow for better apposition to the aortic wall.ResultsThe modified thoracic stent grafts were correctly orientated and deployed in all patients. Transfemoral traction on the Bowden cable successfully bent all stent grafts and improved vessel wall apposition without a residual gap on the inner curve. The Bowden cable was successfully released and withdrawn in all patients.ConclusionIn situ bending of thoracic stent grafts with a sliding self-locking knot is feasible and improves proximal apposition of the device at the inner curve of the aortic arch. More data and longer follow-up are required to confirm the applicability of this technique

Perspectives on the revised Ghent criteria for the diagnosis of Marfan syndrome

Three international nosologies have been proposed for the diagnosis of Marfan syndrome (MFS): the Berlin nosology in 1988; the Ghent nosology in 1996 (Ghent-1); and the revised Ghent nosology in 2010 (Ghent-2). We reviewed the literature and discussed the challenges and concepts of diagnosing MFS in adults. Ghent-1 proposed more stringent clinical criteria, which led to the confirmation of MFS in only 32%-53% of patients formerly diagnosed with MFS according to the Berlin nosology. Conversely, both the Ghent-1 and Ghent-2 nosologies diagnosed MFS, and both yielded similar frequencies of MFS in persons with a causative FBN1 mutation (90% for Ghent-1 versus 92% for Ghent-2) and in persons not having a causative FBN1 mutation (15% versus 13%). Quality criteria for diagnostic methods include objectivity, reliability, and validity. However, the nosology-based diagnosis of MFS lacks a diagnostic reference standard and, hence, quality criteria such as sensitivity, specificity, or accuracy cannot be assessed. Medical utility of diagnosis implies congruency with the historical criteria of MFS, as well as with information about the etiology, pathogenesis, diagnostic triggers, prognostic triggers, and potential complications of MFS. In addition, social and psychological utilities of diagnostic criteria include acceptance by patients, patient organizations, clinicians and scientists, practicability, costs, and the reduction of anxiety. Since the utility of a diagnosis or exclusion of MFS is context-dependent, prioritization of utilities is a strategic decision in the process of nosology development. Screening tests for MFS should be used to identify persons with MFS. To confirm the diagnosis of MFS, Ghent-1 and Ghent-2 perform similarly, but Ghent-2 is easier to use. To maximize the utility of the diagnostic criteria of MFS, a fair and transparent process of nosology development is essential

Urgent and emergent repair of complex aortic aneurysms using an off-the-shelf branched device

IntroductionEndovascular repair using off-the-shelf endografts is a viable solution in patients with ruptured or symptomatic complex aortic aneurysms. This analysis aimed to present the peri-operative and follow-up outcomes in urgent and emergent cases managed with the t-Branch multibranched thoracoabdominal endograft.MethodsProspectively collected data from all consecutive urgent and emergent cases managed in two aortic centers between January 1st, 2014, to November 30th, 2022, using the t-Branch device (Cook Medical Inc., Bjaeverskov, Denmark) were analyzed. Patients presenting with ruptured aortic complex aneurysms were characterized as emergent and patients with aneurysms >90 mm of diameter, or symptomatic aneurysms were characterized as urgent. Technical success, 30-day mortality, major adverse events (MAE) and spinal cord ischemia (SCI) rates were assessed.Results225 patients (36.5% females, 72.5 ± 2.8 years) were included; 73.0% were urgent. The mean aneurysm diameter was 109 ± 3.9 mm and 44.4% were type I–III TAAAs. Females (p = .03), para-renal aneurysms (p = .02) and ASA score IV (p < .001) were more common in emergent cases. Technical success was 97.8%. Thirty-day mortality and MAE rates were 17.8% and 30.6%, respectively. SCI rate was 14.7%, (4.8% paraplegia rate) with 22.2% of patients receiving prophylactic cerebrospinal drainage. Thirty-day mortality (13.3% vs. 26.7%, p = .04) and MAE (26.0% vs. 43.0%, p = .02) were more common among emergent cases while technical success (97.6% vs. 98.3%, p = .9), and SCI (13.3% vs. 18.3%, p = .4) were similar. Survival at 12-months was 83.5% (SE 5.9%) for the urgent and 77.1% (SE 8.2%) for the emergent group (log rank, p = 0.96).ConclusionT-Branch represents an effective and safe solution for the management of urgent and emergent cases with complex aortic aneurysms, with high technical success, promising early mortality and SCI rates

Case-matched comparison of cardiovascular outcome in Loeys-Dietz syndrome versus Marfan syndrome

Background: Pathogenic variants in TGFBR1, TGFBR2 and SMAD3 genes cause Loeys-Dietz syndrome, and pathogenic variants in FBN1 cause Marfan syndrome. Despite their similar phenotypes, both syndromes may have different cardiovascular outcomes. Methods: Three expert centers performed a case-matched comparison of cardiovascular outcomes. The Loeys-Dietz group comprised 43 men and 40 women with a mean age of 34 +/- 18 years. Twenty-six individuals had pathogenic variants in TGFBR1, 40 in TGFBR2, and 17 in SMAD3. For case-matched comparison we used 83 age and sex-frequency matched individuals with Marfan syndrome. Results: In Loeys-Dietz compared to Marfan syndrome, a patent ductus arteriosus (p = 0.014) was more prevalent, the craniofacial score was higher (p < 0.001), the systemic score lower (p < 0.001), and mitral valve prolapse less frequent (p = 0.003). Mean survival for Loeys-Dietz and Marfan syndrome was similar (75 +/- 3 versus 73 +/- 2 years; p = 0.811). Cardiovascular outcome was comparable between Loeys-Dietz and Marfan syndrome, including mean freedom from proximal aortic surgery (53 +/- 4 versus 48 +/- 3 years; p = 0.589), distal aortic repair (72 +/- 3 versus 67 +/- 2 years; p = 0.777), mitral valve surgery (75 +/- 4 versus 65 +/- 3 years; p = 0.108), and reintervention (20 +/- 3 versus 14 +/- 2 years; p = 0.112). In Loeys-Dietz syndrome, lower age at initial presentation predicted proximal aortic surgery (HR = 0.748; p < 0.001), where receiver operating characteristic analysis identified <= 33.5 years with increased risk. In addition, increased aortic sinus diameters (HR = 6.502; p = 0.001), and higher systemic score points at least marginally (HR = 1.175; p = 0.065) related to proximal aortic surgery in Loeys-Dietz syndrome. Conclusions: Cardiovascular outcome of Loeys-Dietz syndrome was comparable to Marfan syndrome, but the severity of systemic manifestations was a predictor of proximal aortic surgery

a controlled multicenter study with assessment of echocardiographic reference values, and the frequency of dilatation and aneurysm in Marfan syndrome

Background Echocardiographic upper normal limits of both main pulmonary artery (MPA) diameters (MPA-d) and ratio of MPA to aortic root diameter (MPA-r) are not defined in healthy adults. Accordingly, frequency of MPA dilatation based on echocardiography remains to be assessed in adults with Marfan syndrome (MFS). Methods We enrolled 123 normal adults (72 men, 52 women aged 42 ± 14 years) and 98 patients with MFS (42 men, 56 women aged 39 ± 14 years) in a retrospective cross-sectional observational controlled study in four tertiary care centers. We defined outcome measures including upper normal limits of MPA-d and MPA-r as 95 quantile of normal persons, MPA dilatation as diameters > upper normal limits, MPA aneurysm as diameters >4 cm, and indication for surgery as MPA diameters >6 cm. Results MPA diameters revealed normal distribution without correlation to age, sex, body weight, body height, body mass index and body surface area. The upper normal limit was 2.6 cm (95% confidence interval (CI) =2.44-2.76 cm) for MPA-d, and 1.05 (95% CI = .86–1.24) for MPA-r. MPA dilatation presented in 6 normal persons (4.9%) and in 68 MFS patients (69.4%; P < .001), MPA aneurysm presented only in MFS (15 patients; 15.3%; P < .001), and no patient required surgery. Mean MPA-r were increased in MFS (P 1.05 were equally frequent in 7 normal persons (5%) and in 8 MFS patients (10.5%; P = .161). MPA-r related to aortic root diameters (P = .042), reduced left ventricular ejection fraction (P = .006), and increased pulmonary artery systolic pressures (P = .040). No clinical manifestations of MFS and no FBN1 mutation characteristics related to MPA diameters. Conclusions We established 2.6 cm for MPA-d and 1.05 for MPA-r as upper normal limits. MFS exhibits a high prevalence of MPA dilatation and aneurysm. However, patients may require MPA surgery only in scarce circumstances, most likely because formation of marked MPA aneurysm may require LV dysfunction and increased PASP

Aorto-bronchial and aorto-pulmonary fistulation after thoracic endovascular aortic repair: an analysis from the European Registry of Endovascular Aortic Repair Complications

OBJECTIVES To learn upon incidence, underlying mechanisms and effectiveness of treatment strategies in patients with central airway and pulmonary parenchymal aorto-bronchial fistulation after thoracic endovascular aortic repair (TEVAR). METHODS Analysis of an international multicentre registry (European Registry of Endovascular Aortic Repair Complications) between 2001 and 2012 with a total caseload of 4680 TEVAR procedures (14 centres). RESULTS Twenty-six patients with a median age of 70 years (interquartile range: 60-77) (35% female) were identified. The incidence of either central airway (aorto-bronchial) or pulmonary parenchymal (aorto-pulmonary) fistulation (ABPF) in the entire cohort after TEVAR in the study period was 0.56% (central airway 58%, peripheral parenchymal 42%). Atherosclerotic aneurysm formation was the leading indication for TEVAR in 15 patients (58%). The incidence of primary endoleaks after initial TEVAR was n = 10 (38%), of these 80% were either type I or type III endoleaks. Fourteen patients (54%) developed central left bronchial tree lesions, 11 patients (42%) pulmonary parenchymal lesions and 1 patient (4%) developed a tracheal lesion. The recognized mechanism of ABPF was external compression of the bronchial tree in 13 patients (50%), the majority being due to endoleak formation, further ischaemia due to extensive coverage of bronchial feeding arteries in 3 patients (12%). Inflammation and graft erosion accounted for 4 patients (30%) each. Cumulative survival during the entire study period was 39%. Among deaths, 71% were attributed to ABPF. There was no difference in survival in patients having either central airway or pulmonary parenchymal ABPF (33 vs 45%, log-rank P = 0.55). Survival with a radical surgical approach was significantly better when compared with any other treatment strategy in terms of overall survival (63 vs 32% and 63 vs 21% at 1 and 2 years, respectively), as well as in terms of fistula-related survival (63 vs 43% and 63 vs 43% at 1 and 2 years, respectively). CONCLUSIONS ABPF is a rare but highly lethal complication after TEVAR. The leading mechanism behind ABPF seems to be a continuing external compression of either the bronchial tree or left upper lobe parenchyma. In this setting, persisting or newly developing endoleak formation seems to play a crucial role. Prognosis does not differ in patients with central airway or pulmonary parenchymal fistulation. Radical bronchial or pulmonary parenchymal repair in combination with stent graft removal and aortic reconstruction seems to be the most durable treatment strateg

Results of Iliac Branch Devices in Octogenarians Within the pELVIS Registry

Purpose:To evaluate if the elderly could benefit from the implantation of iliac branch devices (IBDs) to preserve the patency of the internal iliac artery (IIA) in aneurysms involving the iliac bifurcation.Materials and Methods:From January 2005 to April 2017, 804 patients enrolled in the pELVIS registry underwent endovascular aneurysm repair with 910 IBDs due to aneurysmal involvement of the iliac bifurcation. Among the 804 patients, 157 (19.5%) were octogenarians (mean age 82.9 +/- 2.5 years; 157 men) with 171 target IIAs for preservation. Outcomes at 30 days included technical success, death, conversion to open surgery, and major complications. Outcomes evaluated in follow-up were patency of the IBD and target vessels, type I and type III endoleaks, aneurysm-related reinterventions, aneurysm-related death, and overall patient survival. Kaplan-Meier analyses were employed to evaluate the late outcome measures; the estimates are presented with the 95% confidence interval (CI).Results:Technical success was 99.4% with no intraoperative conversions or deaths (1 bridging stent could not be implanted, and the IIA was sacrificed). Perioperative mortality was 1.9%. The overall perioperative aneurysm-related complication rate was 8.9% (14/157), with an early reintervention rate of 5.1% (8/157). Median postoperative radiological and clinical follow-up were 21.8 months (range 1-127) and 29.3 months (range 1-127), respectively. Estimated rates of freedom from occlusion of the IBD, the IIA, and the external iliac artery at 60 months were 97.7% (95% CI 96.1% to 99.3%), 97.3% (95% CI 95.7% to 98.9%), and 98.6% (95% CI 97% to 99.9%), respectively. Estimated rates of freedom from type I and type III endoleaks and device migration at 60 months were 90.9% (95% CI 87% to 94.3%), 98.7% (95% CI 97.5% to 99.8%), and 98% (95% CI 96.4% to 99.6%), respectively. Freedom from all cause reintervention at 60 months was 87.4% (95% CI 82.6% to 92.2%). The estimated overall survival rate at 60 months was 59% (95% CI 52.4% to 65.6%).Conclusion:IBD implantation in octogenarians provided acceptable perioperative mortality and morbidity rates, with satisfying long-term freedom from IBD-related complications and should be considered a feasible repair option for selected elderly patients affected by aneurysms involving the iliac bifurcation

The main pulmonary artery in adults : a controlled multicenter study with assessment of echocardiographic reference values, and the frequency of dilatation and aneurysm in Marfan syndrome

BACKGROUND: Echocardiographic upper normal limits of both main pulmonary artery (MPA) diameters (MPA-d) and ratio of MPA to aortic root diameter (MPA-r) are not defined in healthy adults. Accordingly, frequency of MPA dilatation based on echocardiography remains to be assessed in adults with Marfan syndrome (MFS). METHODS: We enrolled 123 normal adults (72 men, 52 women aged 42 ± 14 years) and 98 patients with MFS (42 men, 56 women aged 39 ± 14 years) in a retrospective cross-sectional observational controlled study in four tertiary care centers. We defined outcome measures including upper normal limits of MPA-d and MPA-r as 95 quantile of normal persons, MPA dilatation as diameters > upper normal limits, MPA aneurysm as diameters >4 cm, and indication for surgery as MPA diameters >6 cm. RESULTS: MPA diameters revealed normal distribution without correlation to age, sex, body weight, body height, body mass index and body surface area. The upper normal limit was 2.6 cm (95% confidence interval (CI) =2.44-2.76 cm) for MPA-d, and 1.05 (95% CI = .86–1.24) for MPA-r. MPA dilatation presented in 6 normal persons (4.9%) and in 68 MFS patients (69.4%; P < .001), MPA aneurysm presented only in MFS (15 patients; 15.3%; P < .001), and no patient required surgery. Mean MPA-r were increased in MFS (P < .001), but ratios >1.05 were equally frequent in 7 normal persons (5%) and in 8 MFS patients (10.5%; P = .161). MPA-r related to aortic root diameters (P = .042), reduced left ventricular ejection fraction (P = .006), and increased pulmonary artery systolic pressures (P = .040). No clinical manifestations of MFS and no FBN1 mutation characteristics related to MPA diameters. CONCLUSIONS: We established 2.6 cm for MPA-d and 1.05 for MPA-r as upper normal limits. MFS exhibits a high prevalence of MPA dilatation and aneurysm. However, patients may require MPA surgery only in scarce circumstances, most likely because formation of marked MPA aneurysm may require LV dysfunction and increased PASP