Severe Tortuosity of the Distal Descending Thoracic Aorta Affects the Accuracy of Distal Deployment During a Thoracic Endovascular Aortic Repair.

PURPOSE: An accurate distal deployment is essential for successful thoracic endovascular aortic repair (TEVAR) of a paradiaphragmatic aortic aneurysm. This study aimed to investigate the anatomical and intraoperative factors that affect the accuracy of distal deployment during TEVAR. METHODS: We conducted a retrospective review of preoperative and postoperative computed tomography scans of 426 patients undergoing TEVAR at our institution between October 2008 and May 2021, of which the stent-graft was attempted to be deployed just above the celiac axis or the superior mesenteric artery in 56 patients. Based on the anatomical factors related to the malposition (deployed >10 mm away from the target vessel) and the greater curve to the straight-line ratio (G/S ratio), the patients were categorized as severe tortuosity (n=21) and mild tortuosity (n=35) groups to compare the operative and clinical outcomes. RESULT: Stent-graft malpositioning occurred in 21 cases. Among all anatomical variables, only the G/S ratio was significantly larger in the malpositioned cases (p=0.049). A cutoff G/S ratio value of 1.15 was determined using the receiver operating curve analysis. In the severe tortuosity group, the distal end of the stent-graft was significantly farther (median: 10.0 [interquartile range (IQR): 2.5-19.5] mm vs 3.0 [0-8.0] mm; p=0.015) from the target vessel, and the tilt angle of the stent-graft's distal edge was larger (median: 21.4 [IQR: 15.8-24.5] vs 9.5 [5.5-12.5] degree; p<0.01) than that in the mild tortuosity group. Both groups were comparable for the incidence of a primary type Ib endoleak (p=0.454), a secondary type Ib endoleak (p=1.0), and the rate of distal reintervention (p=0.276). CONCLUSION: Severe tortuosity in the distal descending thoracic aorta is associated with a malpositioned and tilted distal end of the stent-graft. CLINICAL IMPACT: Thoracic endovascular aortic repair (TEVAR) for paradiaphragmatic thoracic aortic aneurysms requires accurate distal landing. In this paper, a retrospective CT analysis revealed that the greater curve to the straight-line ratio (G/S ratio) was associated to affects the malposition of the stent graft, defined as being deployed more than 10 mm away from the target vessel. Further, a comparative analysis based on the G/S ratio demonstrated that severe aortic tortuosity was associated with a more distal and tilted deployment of the stent graft.

Geometric Pattern of Proximal Landing Zones for Thoracic Endovascular Aortic Repair in the Bovine Arch Variant.

OBJECTIVE: The aim was to investigate whether the "bovine" aortic arch (i.e. arch variant with a common origin of the innominate and left carotid artery (CILCA)) is associated with a consistent geometric configuration of proximal landing zones for thoracic endovascular aortic repair (TEVAR). METHODS: Anonymised thoracic computed tomography (CT) scans of healthy aortas were reviewed to retrieve 100 cases of CILCA. Suitable cases were stratified according to type 1 and 2 CILCA, and also based on type of arch (I, II, and III). Further processing allowed calculation of angulation and tortuosity of the proximal landing zones. Centre lumen line lengths of each proximal landing zone were measured in a view perpendicular to the centre line. All geometric features were compared with those measured in healthy patients with a standard arch configuration (n = 60). Two senior authors independently evaluated the CT scans, and intra- and interobserver repeatability were assessed. RESULTS: The 100 selected patients (63% male) were 71.4 ± 7.7 years old. Type 1 CILCA (62/100) was more prevalent than type 2 CILCA (38/100), and the two groups were comparable in age (p = .11). Zone 3 presented a severe angulation (i.e. > 60°), which was greater than in Zone 2 (p < .001), and a consistently greater tortuosity than Zone 2 (p = .003). This pattern did not differ between type 1 and type 2 CILCA. A greater tortuosity was also observed in Zone 0, which was related to increased elongation of the ascending aorta (i.e. Zone 0), than the standard configuration. The CILCA had an overall greater elongation, and Zone 2 also was specifically longer. When stratifying by type of arch, reversely from Type III to Type I, the CILCA presented a gradual flattening of its transverse tract, which entailed a consistent progressive elongation (p = .03) and kinking of the ascending aorta, with a significant increase of Zone 0 angulation to even a severe degree (p = .001). Also, from Type III to Type I, Zone 2 presented a progressively shorter length (p = .004), which was associated with increased tortuosity (p < .05). Mean intra- and interobserver differences for angulation measurements were 1.4° ± 6.8° (p = .17) and 2.0° ± 10.1° (p = .19), respectively. CONCLUSION: CILCA presents a consistent and peculiar geometric pattern compared with standard arch configuration, which provides relevant information for TEVAR planning, and may have prognostic implications.

Geometry and flow in ascending aortic aneurysms are influenced by left ventricular outflow tract orientation: Detecting increased wall shear stress on the outer curve of proximal aortic aneurysms.

BACKGROUND: The geometrical characterization of ascending thoracic aortic aneurysms in clinical practice is limited to diameter measurements. Despite growing interest in hemodynamic assessment, its relationship with ascending thoracic aortic aneurysm pathogenesis is poorly understood. This study examines the relationship between geometry of the ventriculo-aortic junction and blood flow patterns in ascending thoracic aortic aneurysm disease. METHODS: Thirty-three patients with ascending thoracic aortic aneurysms (exclusions: bicuspid aortic valves, connective tissue disease) underwent 4-dimensional flow magnetic resonance imaging. After image segmentation, geometrical parameters were measured, including aortic curvature, tortuosity, length, and diameter. A unique angular measurement made by the trajectory of the left ventricular outflow tract axis and the proximal aorta was also conducted. Velocity profiles were quantitatively and qualitatively analyzed. In addition, 11 patients (33%) underwent wall shear stress mapping of the ascending thoracic aortic aneurysm region using computational fluid dynamics simulation. RESULTS: Greater left ventricular outflow tract aortic angles were associated with larger aortic diameters at the levels of the sinus (coefficient = 0.387, P = .014) and ascending aorta (coefficient = 0.284, P = .031). Patients with left ventricular outflow tract aortic angles greater than 60° had marked asymmetric flow acceleration on the outer curvature in the proximal aorta, ascertained from 4-dimensional flow analysis. For patients undergoing computational fluid dynamics assessment, regression analysis found that higher left ventricular outflow tract aortic angles were associated with significantly higher wall shear stress values in the outer curve of the aorta (coefficient 0.07, 95% confidence interval 0.04-0.11, P = .002): Angles greater than 50° yielded time-averaged wall shear stress values greater than 2.5 Pa, exhibiting a linear relationship. CONCLUSIONS: Our findings strengthen the hypothesis of flow-mediated ascending thoracic aortic aneurysm disease progression and that left ventricular outflow tract aortic angle may be a predictor of disease severity.

Aortic tortuosity in Turner syndrome is associated with larger ascending aorta.

Turner syndrome (TS) is associated with aortic coarctation, dissection and dilation/aneurysm. Predictors of dissection are not well delineated, making decisions regarding prophylactic root replacement challenging. In other disorders, arterial tortuosity is an imaging biomarker associated with increased risk for aortic dissection and adverse cardiovascular events. We aimed to determine if, in TS, arterial tortuosity was associated with aortic dilation or aortic events. We performed a retrospective cohort analysis of unselected women and children with TS who underwent cardiovascular magnetic resonance angiography (MRA) for a prior prospective study. We calculated tortuosity indices including vertebral artery tortuosity index, aortic arch tortuosity index, thoracic aortic tortuosity index (ATI-D), and aortic tortuosity index to the celiac artery (ATI-C). We compared tortuosity in TS patients against age and gender matched controls. We evaluated univariable and multivariable associations between the tortuosity indices and aortic root and ascending aorta size as defined by z-scores, which give a sense of how far a measurement deviates from the mean. We also studied associations between tortuosity and need for aortic root replacement or aortic dissection. Of 184 subjects, with median age 34 years, mean general aortic root z-score was 0.1 ± 1.2 and mean general ascending aortic z-score was 0.4 ± 1.5. Three patients had aortic dissection, and one had prophylactic root replacement, which all occurred prior to first MRA. Vertebral tortuosity index, ATI-D, and ATI-C all increased with age (p < 0.0001) for all. ATI-C was associated with larger general ascending z-score. In multivariable analysis, ATI-C remained independently associated with larger ascending aortic z-scores. The relationship between aortic indices and surgery/dissection could not be evaluated since all were collected post-surgery/dissection. Thoracic aortic tortuosity as measured by ATI-C is independently associated with larger ascending aortic dimensions. In this population with only three aortic dissections occurring prior to imaging assessment, we could not assess for associations between aortic tortuosity and dissection. Studies including more patients with aortic dissection are needed to draw further conclusions.

Aortic Geometric Alteration Associated With Acute Type B Aortic Dissection: Angulation, Tortuosity, and Arch Type.

Background: Acute type B aortic dissection is a highly serious aortic pathology. Aortic geometric parameters may be useful variables related to the occurrence of acute type B aortic dissection (aTBAD). The aim of the study is to delineate the alteration in aortic geometric parameters and analyze the specific geometric factors associated with aTBAD. Methods: The propensity score matching method was applied to control confounding factors. The aortic diameter, length, angulation, tortuosity, and type of aortic arch of the aTBAD and control group were retrospectively analyzed via three-dimensional computed tomography imaging created by the 3mensio software (version 10.0, Maastricht, The Netherlands). The geometric variables of true lumen and false lumen in the descending aorta were measured to estimate the severity of aortic dissection. Multivariable logistic regression models were used to investigate the significant and specific factors associated with aTBAD occurrence. The area under the receiver operating characteristic curve (AUC) was used to estimate the performance of the model. Results: After propensity score matching, 168 matched pairs of patients were selected. The ascending aorta and aortic arch diameters were dilated, and the ascending aorta and total aorta lengths were elongated in aTBAD group significantly (P < 0.001). The ascending aorta and aortic arch angulations in the aTBAD group were sharper than those of the controls (P = 0.01, P < 0.001, respectively). The aortic arch and total aorta tortuosities were significantly higher in the aTBAD group (P = 0.001, P < 0.001, respectively). There were more type III arch patients in the aTBAD group than the controls (67.9 vs. 22.6%). The true lumen angulation was sharper than that in the false lumen (P < 0.01). The true lumen tortuosity was significantly lower than that in the false lumen (P < 0.001). The multivariable models identified that aortic arch angulation, tortuosity, and type III arch were independent and specific geometric factors associated with aTBAD occurrence. The AUC of the multivariable models 1, 2, 3 were 0.945, 0.953, and 0.96, respectively. Conclusions: The sharper angulation and higher tortuosity of aortic arch and type III arch were the geometric factors associated with aTBAD in addition to the ascending aorta elongation and aortic arch dilation. The angulation and tortuosity of the true and false lumens may carry significant clinical implications for the treatment and prognosis of aTBAD.

Function of Ltbp-4L and fibulin-4 in survival and elastogenesis in mice.

LTBP-4L and LTBP-4S are two isoforms of the extracellular matrix protein latent-transforming growth factor beta-binding protein 4 (LTBP-4). The mutational inactivation of both isoforms causes autosomal recessive cutis laxa type 1C (ARCL1C) in humans and an ARCL1C-like phenotype in Ltbp4-/- mice, both characterized by high postnatal mortality and severely affected elastogenesis. However, genetic data in mice suggest isoform-specific functions for Ltbp-4 because Ltbp4S-/- mice, solely expressing Ltbp-4L, survive to adulthood. This clearly suggests a requirement of Ltbp-4L for postnatal survival. A major difference between Ltbp4S-/- and Ltbp4-/- mice is the matrix incorporation of fibulin-4 (a key factor for elastogenesis; encoded by the Efemp2 gene), which is normal in Ltbp4S-/- mice, whereas it is defective in Ltbp4-/- mice, suggesting that the presence of Ltbp-4L might be required for this process. To investigate the existence of a functional interaction between Ltbp-4L and fibulin-4, we studied the consequences of fibulin-4 deficiency in mice only expressing Ltbp-4L. Resulting Ltbp4S-/-;Fibulin-4R/R mice showed a dramatically reduced lifespan compared to Ltbp4S-/- or Fibulin-4R/R mice, which survive to adulthood. This dramatic reduction in survival of Ltbp4S-/-;Fibulin-4R/R mice correlates with severely impaired elastogenesis resulting in defective alveolar septation and distal airspace enlargement in lung, and increased aortic wall thickness with severely fragmented elastic lamellae. Additionally, Ltbp4S-/-;Fibulin-4R/R mice suffer from aortic aneurysm formation combined with aortic tortuosity, in contrast to Ltbp4S-/- or Fibulin-4R/R mice. Together, in accordance with our previous biochemical findings of a physical interaction between Ltbp-4L and fibulin-4, these novel in vivo data clearly establish a functional link between Ltbp-4L and fibulin-4 as a crucial molecular requirement for survival and elastogenesis in mice.

Innervation patterns may limit response to endovascular renal denervation.

BACKGROUND: Renal denervation is a new interventional approach to treat hypertension with variable results. OBJECTIVES: The purpose of this study was to correlate response to endovascular radiofrequency ablation of renal arteries with nerve and ganglia distributions. We examined how renal neural network anatomy affected treatment efficacy. METHODS: A multielectrode radiofrequency catheter (15 W/60 s) treated 8 renal arteries (group 1). Arteries and kidneys were harvested 7 days post-treatment. Renal norepinephrine (NEPI) levels were correlated with ablation zone geometries and neural injury. Nerve and ganglion distributions and sizes were quantified at discrete distances from the aorta and were compared with 16 control arteries (group 2). RESULTS: Nerve and ganglia distributions varied with distance from the aorta (p < 0.001). A total of 75% of nerves fell within a circumferential area of 9.3, 6.3, and 3.4 mm of the lumen and 0.3, 3.0, and 6.0 mm from the aorta. Efficacy (NEPI 37 ng/g) was observed in only 1 of 8 treated arteries where ablation involved all 4 quadrants, reached a depth of 9.1 mm, and affected 50% of nerves. In 7 treated arteries, NEPI levels remained at baseline values (620 to 991 ng/g), ≤20% of the nerves were affected, and the ablation areas were smaller (16.2 ± 10.9 mm(2)) and present in only 1 to 2 quadrants at maximal depths of 3.8 ± 2.7 mm. CONCLUSIONS: Renal denervation procedures that do not account for asymmetries in renal periarterial nerve and ganglia distribution may miss targets and fall below the critical threshold for effect. This phenomenon is most acute in the ostium but holds throughout the renal artery, which requires further definition.