Coarctation of the aorta (CoA) is a congenital heart defect that is associated with a bicuspid aortic valve (BAV), ascending aorta dilatation, intracerebral aneurysms, and premature atherosclerotic... Show moreCoarctation of the aorta (CoA) is a congenital heart defect that is associated with a bicuspid aortic valve (BAV), ascending aorta dilatation, intracerebral aneurysms, and premature atherosclerotic disease. The first presentation during late adulthood is rare and is frequently driven by late sequelae. Hypertrophic collateral arteries can develop aneurysms which are at risk for spontaneous rupture, however, treatment recommendations for these aneurysms are scarce. Here, we describe the clinical course and percutaneous treatment strategy of a patient with a late diagnosis of a pin-point CoA, a BAV with moderate regurgitation, and an exceptionally large aneurysm of a collateral artery. A 59-year-old woman was diagnosed with Streptococcus bovis endocarditis of a BAV with moderate aortic valve regurgitation and small vegetation (<5 mm) on the non-coronary cusp. Work-up revealed hypertension and adenocarcinoma in situ of the ascending colon, considered the bacteremia porte d'entree, for which a curative hemicolectomy was performed. Echocardiography showed a narrowing of the aorta distal from the origin of the left subclavian artery with the antegrade diastolic flow with a pathognomonic "sawtooth" pattern and an estimated pressure gradient of >70 mmHg. Computed tomography angiography (CTA) showed a network of well-developed collateral arteries and a levoatriocardinal vein. One of the collateral arteries arising from the left subclavian artery revealed an exceptionally large aneurysmatic dilation (29 x 24 x 24 mm). The invasive assessment confirmed a hemodynamically significant CoA. Treatment involved balloon dilatation and placement of a covered stent at the site of the pin-point CoA and a percutaneous coronary intervention (PCI) of the stenosis in the left anterior descending artery. No residual gradient over the CoA was observed. Antihypertensive drugs could be discontinued, and CTA performed 4 months later showed regression and thrombosis of the numerous collaterals and, importantly, thrombosis of the large aneurysm. This case illustrates the late diagnosis of CoA with associated congenital heart defects and late sequelae including hypertension, BAV endocarditis, coronary artery disease, and aneurysm formation of the extensive collateral network. The patient underwent pharmacological and percutaneous treatment, ultimately resulting in the alleviation of the CoA, normalization of the blood pressure, reduction of collateral flow, and thrombosis of the large aneurysm of the collateral artery. Show less
Juffermans, J.F.; Assen, H.C. van; Kiefte, B.J.C. te; Ramaekers, M.J.F.G.; Palen, R.L.F. van der; Boogaard, P. van den; ... ; Westenberg, J.J.M. 2022
(1) Background: Aorta hemodynamics have been associated with aortic remodeling, but the reproducibility of its assessment has been evaluated marginally in patients with thoracic aortic aneurysm ... Show more(1) Background: Aorta hemodynamics have been associated with aortic remodeling, but the reproducibility of its assessment has been evaluated marginally in patients with thoracic aortic aneurysm (TAA). The current study evaluated intra- and interobserver reproducibility of 4D flow MRI-derived hemodynamic parameters (normalized flow displacement, flow jet angle, wall shear stress (WSS) magnitude, axial WSS, circumferential WSS, WSS angle, vorticity, helicity, and local normalized helicity (LNH)) in TAA patients; (2) Methods: The thoracic aorta of 20 patients was semi-automatically segmented on 4D flow MRI data in 5 systolic phases by 3 different observers. Each time-dependent segmentation was manually improved and partitioned into six anatomical segments. The hemodynamic parameters were quantified per phase and segment. The coefficient of variation (COV) and intraclass correlation coefficient (ICC) were calculated; (3) Results: A total of 2400 lumen segments were analyzed. The mean aneurysm diameter was 50.8 +/- 2.7 mm. The intra- and interobserver analysis demonstrated a good reproducibility (COV = 16-30% and ICC = 0.84-0.94) for normalized flow displacement and jet angle, a very good-to-excellent reproducibility (COV = 3-26% and ICC = 0.87-1.00) for all WSS components, helicity and LNH, and an excellent reproducibility (COV = 3-10% and ICC = 0.96-1.00) for vorticity; (4) Conclusion: 4D flow MRI-derived hemodynamic parameters are reproducible within the thoracic aorta in TAA patients. Show less
Andel, M.M. van; Waard, V. de; Timmermans, J.; Scholte, A.J.H.A.; Berg, M.P. van den; Zwinderman, A.H.; ... ; Groenink, M. 2021
Objectives Patients with Marfan syndrome (MFS) are prone to develop aortic aneurysms due to fragmentation of elastic fibres, resulting in reduced distensibility of the aorta. Reduced distensibility... Show moreObjectives Patients with Marfan syndrome (MFS) are prone to develop aortic aneurysms due to fragmentation of elastic fibres, resulting in reduced distensibility of the aorta. Reduced distensibility was previously shown to predict progressive descending aorta dilatation. Here, we investigated longitudinal changes in distensibility, as a potential predictor of aortic events.Methods This retrospective study included all patients with MFS with at least four cardiac magnetic resonance examinations performed between 1996 and 2012. Aortic distensibility was assessed, in the ascending (level 1), proximal descending (level 2) and distal descending (level 3) aorta. Changes in distensibility were studied using linear mixed-effects regression models.Results In total, 35 patients with MFS (age at inclusion 28 (IQR 23-32) years, 54% men) were included. Mean aortic distensibility was already low (between 2.9x10(-3)/mm Hg/year and 6.4x10(-3)/mm Hg/year) at all levels at baseline, and significantly decreased over time at levels 2 and 3 (respectively, p=0.012 and p=0.002). The rate of distensibility loss per year (x10(-3)/mm Hg/year) was 0.01, 0.03 and 0.06x10(-3)/mm Hg at levels 1, 2 and 3, respectively. At inclusion, men exhibited very low distensibility, whereas women showed moderately reduced distensibility, gradually decreasing with age. Aortic dilatation rate at level 2 was associated with reduced aortic distensibility. However, we could not demonstrate a direct correlation between distensibility and clinical events during a follow-up of 22 years.Conclusion Patients with MFS display reduced aortic distensibility already at an early age, inversely relating to aortic dilatation rate. However, in this selected patient group, distensibility seems less suitable as an individual predictor of aortic events. Show less
Objectives Degenerative thoracic aortic aneurysm (TAA) patients are known to be at risk of life-threatening acute aortic events. Guidelines recommend preemptive surgery at diameters of greater than... Show moreObjectives Degenerative thoracic aortic aneurysm (TAA) patients are known to be at risk of life-threatening acute aortic events. Guidelines recommend preemptive surgery at diameters of greater than 55 mm, although many patients with small aneurysms show only mild growth rates and more than half of complications occur in aneurysms below this threshold. Thus, assessment of hemodynamics using 4-dimensional flow magnetic resonance has been of interest to obtain more insights in aneurysm development. Nonetheless, the role of aberrant flow patterns in TAA patients is not yet fully understood. Materials and Methods A total of 25 TAA patients and 22 controls underwent time-resolved 3-dimensional phase contrast magnetic resonance imaging with 3-directional velocity encoding (ie, 4-dimensional flow magnetic resonance imaging). Hemodynamic parameters such as vorticity, helicity, and wall shear stress (WSS) were calculated from velocity data in 3 anatomical segments of the ascending aorta (root, proximal, and distal). Regional WSS distribution was assessed for the full cardiac cycle. Results Flow vorticity and helicity were significantly lower for TAA patients in all segments. The proximal ascending aorta showed a significant increase in peak WSS in the outer curvature in TAA patients, whereas WSS values at the inner curvature were significantly lower as compared with controls. Furthermore, positive WSS gradients from sinotubular junction to midascending aorta were most prominent in the outer curvature, whereas from midascending aorta to brachiocephalic trunk, the outer curvature showed negative WSS gradients in the TAA group. Controls solely showed a positive gradient at the inner curvature for both segments. Conclusions Degenerative TAA patients show a decrease in flow vorticity and helicity, which is likely to cause perturbations in physiological flow patterns. The subsequent differing distribution of WSS might be a contributor to vessel wall remodeling and aneurysm formation. Show less
Objective: To evaluate the impact of changes in elective Abdominal Aortic Aneurysm (AAA) management on life-expectancy of AAA patients.Background: Over the past decades AAA repair underwent... Show moreObjective: To evaluate the impact of changes in elective Abdominal Aortic Aneurysm (AAA) management on life-expectancy of AAA patients.Background: Over the past decades AAA repair underwent substantial changes, that is, the introduction of EVAR and implementation of intensified cardiovascular risk management. The question rises to what extent these changes improved longevity of AAA patients.Methods: National evaluation including all 12.907 (82.7% male) patients who underwent elective AAA repair between 2001 and 2015 in Sweden. The impact of changes in AAA management was established by a time-resolved analysis based on 3 timeframes: open repair dominated period (2001- 2004, n = 2483), transition period (2005-2011, n = 6230), and EVAR-first strategy period (2012-2015, n = 4194). Relative survival was used to quantify AAAassociated mortality, and to adjust for changes in life-expectancy.Results: Relative survival of electively treated AAA patients was stable and persistently compromised [4-year relative survival and 95% confidence interval: 0.87 (0.85- 0.89), 0.87 (0.86- 0.88), 0.89 (0.86- 0.91) for the 3 periods, respectively]. Particularly alarming is the severely compromised survival of female patients (4-year relative survival females 0.78, 0.80, 0.70 vs males 0.89, 0.89, 0.91, respectively). Cardiovascular mortality remained the main cause of death (51.0%, 47.2%, 47.9%) and the proportion cardiovascular disease over non-cardiovascular disease death was stable over time.Conclusions: Changes in elective AAA management reduced short-term mortality, but failed to improve the profound long-term survival disadvantage of AAA patients. The persistent high (cardiovascular) mortality calls for further intensification of cardiovascular risk management, and a critical appraisal of the basis for the excess mortality of AAA patients. Show less
Background and Purpose: Delayed cerebral ischemia (DCI) is a major contributor to the high morbidity in patients with aneurysmal subarachnoid hemorrhage (aSAH). Spreading depolarizations may play a... Show moreBackground and Purpose: Delayed cerebral ischemia (DCI) is a major contributor to the high morbidity in patients with aneurysmal subarachnoid hemorrhage (aSAH). Spreading depolarizations may play a role in DCI pathophysiology. Because patients with migraine are probably more susceptible to spreading depolarizations, we investigated whether patients with aneurysmal subarachnoid hemorrhage with migraine are at increased risk for DCI. Methods: We included patients with aneurysmal subarachnoid hemorrhage from 3 hospitals in the Netherlands. We assessed lifetime migraine history with a short screener. DCI was defined as neurological deterioration lasting >1 hour not attributable to other causes by diagnostic work-up. Adjustments were made for possible confounders in multivariable Cox regression analyses and adjusted hazard ratios (aHR) were calculated. We assessed the interaction effects of age and sex. Results: We included 582 patients (mean age 57 years, 71% women) mostly with mild to moderate aneurysmal subarachnoid hemorrhage of whom 108 (19%) had a history of migraine (57 with aura). Patients with migraine were not at increased risk of developing DCI compared with patients without migraine (22% versus 24%, aHR, 0.89 [95% CI, 0.56-1.43]). Additionally, no increased risk was found in patients with migraine with possible aura (aHR, 0.74 [95% CI, 0.39-1.43]), in women (aHR, 0.88 [95% CI, 0.53-1.45],P-interaction=0.859), or in young patients aged <50 years (aHR, 1.59 [95% CI, 0.72-3.49]), although numbers in these subgroups were limited. We found an interaction between migraine and age with an increased risk of DCI among young patients with migraine (P-interaction=0.075). Conclusions: Patients with migraine are in general not at increased risk of DCI. Future studies should focus in particular on young SAH patients, in whom there might be an association between migraine history and development of DCI. Show less
