Background Partial thrombosis of the false lumen has been reported as a significant predictor of mortality during follow-up in patients with acute type B aortic dissection. The purpose of this study ...was to investigate the correlation of false lumen thrombosis and aortic expansion during follow-up in patients with acute type B aortic dissection. Methods All medically treated patients with acute type B aortic dissection observed in 4 cardiovascular referral centers between 1998 and 2011, with admission and follow-up computed tomography or magnetic resonance imaging scans, were included. Aortic diameters of the dissected aortas were measured at 4 levels on the baseline and follow-up scans, and annual growth rates were calculated. Univariate and multivariate regression analyses were used to investigate the effect of false lumen thrombosis on aortic growth rate. Results A total of 84 patients were included, of whom 40 (47.6%) had a partially thrombosed false lumen, 7 (8.3%) had a completely thrombosed false lumen, and 37 (44.0%) had a patent false lumen. A total of 273 of the 336 (81.3%) evaluated aortic levels were dissected segments. Overall, the mean aortic diameter increased significantly at all evaluated levels ( P < .001). Univariate analysis showed that annual aortic growth rates were significantly higher in those segments having a false lumen with partial thrombosis (mean, 4.25 ± 10.2) when compared with the patent group (mean, 2.10 ± 5.56; P = .035). In multivariate analysis, partial lumen thrombosis was an independent predictor of higher aortic growth (adjusted mean difference, 2.05 mm/year; 95% confidence interval, 0.10-4.01; P = .040). Conclusions In patients with acute type B aortic dissection, aortic segments with a partially thrombosed false lumen have a significantly higher annual aortic growth rate when compared with those presenting with patent or complete thrombosis of the false lumen. Therefore, patients with partial thrombosis require more intensive follow-up and may benefit from prophylactic intervention.
Background Conservative management of acute type B aortic dissection (ABAD) is often associated with aortic dilatation during follow-up increasing the risk of aortic rupture. The goal of this study ...was to investigate whether morphologic characteristics of the dissection can predict aortic growth. Methods All conservatively managed ABAD patients from four referral centers were included (2000 to 2010). Aortic diameters were measured at five levels at baseline and at the last follow-up computed tomography angiography, and annual aortic growth rates were calculated for all segments. Linear regression was used to study the influence of aortic morphologic characteristics for aortic dilatation. Results Included were 62 patients (41 men) with a mean age of 60.3 ± 10.7 years. Among the 310 analyzed aortic segments, 248 (80.0%) were dissected, of which 211 (85.1%) showed aortic growth. Overall, the mean diameter increased from 36.1 ± 9.4 to 40.2 ± 11.1 mm ( P < .01), which corresponds with a mean aortic growth rate of 3.1 ± 6.3 mm/y. Multivariate linear regression analysis showed that male sex (95% confidence interval CI, 0.60-4.04; P = .005) and a saccular false lumen (95% CI, 2.07-7.81: P = .001) were associated with a significantly increased aortic growth rate. Increasing age (95% CI, −0.23 to −0.04; P = .005), increased number of entry tears (95% CI, −2.40 to −0.43; P = .005), false lumen located on the aortic outer curvature (95% CI, −4.30 to −0.38; P = .019), and a circular configuration of the true lumen (95% CI, −5.35 to −0.32; P = .027) were associated with a decreased aortic growth rate. Conclusions Multiple morphologic characteristics appear to predict aortic dilatation in ABAD patients treated medically. Early assessment of these morphologic signs may be useful in the selection of ABAD patients who might benefit from closer radiologic surveillance or prophylactic intervention.
Background Patients with uncomplicated acute type B aortic dissection (ABAD) generally can be treated with conservative medical management. However, these patients may develop aortic enlargement ...during follow-up, with the risk for rupture, which necessitates intervention. Several predictors have been studied in recent years to identify ABAD patients at high risk for aortic enlargement who may benefit from early surgical or endovascular intervention. This study systematically reviewed and summarized the current available literature on prognostic variables related to aortic enlargement during follow-up in uncomplicated ABAD patients. Methods Studies were included if they reported predictors of aortic growth in uncomplicated ABAD patients. Studies about type A aortic dissection, aortic aneurysm, intramural hematoma, or ABAD that required acute intervention were excluded. Results A total of 18 full-text articles were selected. The following predictors of aortic growth in ABAD patients were identified: age <60 years, white race, Marfan syndrome, high fibrinogen-fibrin degradation product level (≥20 μg/mL) at admission, aortic diameter ≥40 mm on initial imaging, proximal descending thoracic aorta false lumen (FL) diameter ≥22 mm, elliptic formation of the true lumen, patent FL, partially thrombosed FL, saccular formation of the FL, presence of one entry tear, large entry tear (≥10 mm) located in the proximal part of the dissection, FL located at the inner aortic curvature, fusiform dilated proximal descending aorta, and areas with ulcer-like projections. Tight heart rate control (<60 beats/min), use of calcium-channel blockers, thrombosed FL, two or more entry tears, FL located at the outer aortic curvature, and circular configuration of the true lumen were associated with negative or limited aortic growth. Conclusions Several predictors might be used to identify those ABAD patients at high risk for aortic growth. Although conservative management remains indicated in uncomplicated ABAD, these patients might benefit from closer follow-up or early endovascular intervention.
Objective True splenic artery aneurysms (SAAs) are a rare but potentially fatal pathology. For many years, open repair (OPEN) and conservative management (CONS) were the treatments of choice, but ...throughout the last decade endovascular repair (EV) has become increasingly used. The purpose of the present study was to perform a systematic review and meta-analysis evaluating the outcomes of the three major treatment modalities (OPEN, EV, and CONS) for the management of SAAs. Methods A systematic review of all studies describing the outcomes of SAAs treated with OPEN, EV, or CONS was performed using seven large medical databases. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed to ensure a high-quality review. All articles were subject to critical appraisal for relevance, validity, and availability of data regarding characteristics and outcomes. All data were systematically pooled, and meta-analyses were performed on several outcomes, including early and late mortality, complications, and number of reinterventions. Results Original data of 1321 patients with true SAAs were identified in 47 articles. OPEN contained 511 patients (38.7%) in 31 articles, followed by 425 patients (32.2%) in CONS in 16 articles and 385 patients (29.1%) in EV in 33 articles. The CONS group had fewer symptomatic patients (9.5% vs 28.7% in OPEN and 28.8% in EV; P < .001) and fewer ruptured aneurysms (0.2% vs 18.4% in OPEN and 8.8% in EV; P < .001), but no significant differences were found in existing comorbidities. CONS patients were usually older and had smaller-sized aneurysms than patients in the OPEN and EV groups. The only identified difference in baseline characteristics between OPEN and EV was the number of ruptured aneurysms (18.4% vs 8.8%; P < .001). OPEN had a higher 30-day mortality than EV (5.1% vs 0.6%; P < .001), whereas minor complications occurred in a larger number of the EV patients. EV required more reinterventions per year (3.2%) compared with OPEN (0.5%) and CONS (1.2%; P < .001). The late mortality rate was higher in patients treated with CONS (4.9% vs 2.1% in OPEN and 1.4% in EV; P = .04). Conclusions EV of SAA has better short-term results compared with OPEN, including significantly lower perioperative mortality. OPEN is associated with fewer late complications and fewer reinterventions during follow-up. Patients treated with CONS showed a higher late mortality rate. Ruptured SAAs are predictors of a significantly higher perioperative mortality compared with nonruptured SAAs in the OPEN and EV groups.
Objective This study was conducted to provide insight into the safety, applicability, and outcomes of thoracic endovascular aortic repair (TEVAR) with the chimney graft technique. Methods Original ...data regarding the chimney technique in TEVAR in the emergent and elective setting were collected from MEDLINE, Embase, and Scopus databases. All variables were systematically extracted and included in a database. Patient and procedural characteristics, details, and outcomes were analyzed. Results In total, 94 patients with 101 chimney-stented aortic arch branches were analyzed, consisting of the brachiocephalic artery in 20, the left common carotid artery in 48, and the left subclavian artery in 33. Balloon-expandable stents were used in 36% and self-expandable stents in 64% for the aortic side branch. The interventions were elective in 72% and emergent in 28%. Technical success was achieved in 98% in elective and emergent settings combined. Endoleaks were described in 18%; with type Ia being most frequently reported in 6.4% overall and in 6.5% in the elective setting. Stroke was reported in 5.3% of the patients, of which 40% were fatal. The overall perioperative mortality was 3.2%. Median follow-up time was 11 months, and chimney stents remained patent in all patients. Conclusions TEVAR with the chimney technique is a viable treatment option and may expand treatment strategies for patients with challenging thoracic aortic pathology and anatomy in the emergent and elective setting. Patency of the thoracic chimney stents appears to be good during short-term follow-up. Other complications, such as endoleak and stroke, deserve attention by future research to further improve treatment strategies and the prognosis of these patients.
Background Aortic growth rate in acute type B aortic dissection (ABAD) is a significant predictor for aortic complications and death. To improve the overall outcome, radiologic predictors might ...stratify patients who benefit from successful medical management vs those who require intervention. This study investigated whether the number of identifiable entry tears in ABAD patients is associated with aortic growth. Methods ABAD patients with uncomplicated clinical conditions and therefore treated with medical therapy were evaluated. Those with a computed tomography angiography (CTA) obtained at clinical presentation and a subsequent CTA obtained at least 90 days after medical treatment were included (2005 to 2010). The CTAs were investigated for the number of entry tears between the true and false lumen. Diameters of the dissected aortas were measured at five levels on the baseline and on the last available follow-up CTA, and annual aortic growth rates were calculated. The number of entry tears in these patients and the location in the aorta were compared with the aortic growth rate. Results Included were 60 patients who presented with 243 dissected segments. Mean growth rates during follow-up (median, 23.2; range, 3 to 132 months) were significantly higher in patients with 1 entry tear (5.6 ± 8.9 mm) than in those with 2 (2.1 ± 1.7 mm; p = 0.001) and 3 entry tears (mean 2.2 ± 4.1; p = 0.010). The distance of the primary entry tear from the left subclavian artery did not have an effect on the aortic growth rate (median, 38; interquartile range, 24 to 137 mm; p = 0.434). Conclusions The number of entry tears in ABAD patients detected on the first CTA after clinical presentation is a significant predictor for aortic growth. Patients with 1 entry tear at presentation show a higher growth rate than other patients and might benefit from more strict surveillance or early prophylactic intervention.
Objective An association of intraluminal thrombus (ILT) with abdominal aortic aneurysm (AAA) growth has been suggested. Previous in vitro experiments have demonstrated that aneurysm-associated ...thrombus may secrete proteolytic enzymes and may develop local hypoxia that might lead to the formation of tissue-damaging reactive oxygen species. In this study, we assessed the hypothesis that ventral ILT thickness is associated with markers of proteolysis and with lipid oxidation in the underlying AAA vessel wall. Methods Ventral AAA tissue was collected from asymptomatic patients at the site of maximal diameter during open aneurysm repair. Segments were divided, one part for biochemical measurements and one for histologic analyses. We measured total cathepsin B, cathepsin S levels, and matrix metalloproteinase (MMP)-2 and MMP-9 activity. Myeloperoxidase and thiobarbituric acid reactive substances were determined as measures of lipid oxidation. Histologic segments were analyzed semiquantitatively for the presence of collagen, elastin, vascular smooth muscle cells (VSMCs), and inflammatory cells. Preoperative computed tomography angiography scans of 83 consecutive patients were analyzed. A three-dimensional reconstruction was obtained, and a center lumen line of the aorta was constructed. Ventral ILT thickness was measured in the anteroposterior direction at the level of maximal aneurysm diameter on the orthogonal slices. Results Ventral ILT thickness was positively correlated with aortic diameter ( r = 0.25; P = .02) and with MMP-2 levels ( r = 0.27; P = .02). No biochemical correlations were observed with MMP-9 activity or cathepsin B and S expression. No correlation between ventral ILT thickness and myeloperoxidase or thiobarbituric acid reactive substances was observed. Ventral ILT thickness was negatively correlated with VSMCs (no staining, 18.5 interquartile range, 12.0-25.5 mm; minor, 17.6 10.7-22.1 mm; moderate, 14.5 4.6-21.7 mm; and heavy, 8.0 0.0-12.3 mm, respectively; P = .01) and the amount of elastin (no staining, 18.6 12.2-30.0 mm; minor, 16.5 9.0-22.1 mm; moderate, 11.7 2.5-15.3 mm; and heavy 7.7 0.0-7.7 mm, respectively; P = .01) in the medial aortic layer. Conclusions ILT thickness appeared to be associated with VSMCs apoptosis and elastin degradation and was positively associated with MMP-2 concentrations in the underlying wall. This suggests that ILT thickness affects AAA wall stability and might contribute to AAA growth and rupture. ILT thickness was not correlated with markers of lipid oxidation.
Introduction Aortic intramural hematoma type B (IMHB) is a variant of acute aortic syndrome, which presents with symptoms similar to classic type B aortic dissection (ABAD). However, the natural ...history of IMHB is not well understood. The purpose of this study was to better characterize IMHB, comparing its clinical characteristics, treatment, and in-hospital and long-term outcomes to those with classic ABAD. Methods A total of 107 IMHB and 790 ABAD patients enrolled in the International Registry of Acute Aortic Dissection (IRAD) between January 1996 and June 2012 were analyzed. Accordingly, differences in presentation, diagnostics, therapeutic management, and outcomes were assessed. Results As compared with the ABAD, IMHB presented predominantly in males (62% vs 33%; P < .001) at older age (69 ± 12 vs 63 ± 14; P < .001). IMHB patients more often had chest pain (80% vs 69%; P = .020) and periaortic hematoma (22% vs 13%; P = .020) and were more often treated medically (88% vs 62%; P < .001), with surgical/endovascular interventions being reserved for more complicated patients. Overall in-hospital mortality was 10% (IMHB, 7% vs ABAD, 11%; P = NS). Six out of seven IMHB deaths occurred during medical treatment, two due to aortic rupture. During follow-up in IMHB, patient mortality was 7%, and no adverse events, including progression to an aortic dissection or aortic rupture, were observed. Imaging showed significantly more aortic enlargement at the level of the descending aorta in ABAD patients (39% vs 61%; P = .034). Conclusions Most IMHB patients can be treated medically, and aortic enlargement is less common during follow-up, which may suggest that IMHB may have a slightly more benign course compared with classic ABAD in the acute setting.
Introduction Ruptured descending thoracic aortic aneurysm (rDTAA) is associated with high mortality rates. Data supporting endovascular thoracic aortic aneurysm repair (TEVAR) to reduce mortality ...compared with open repair are limited to small series. We investigated published reports for contemporary outcomes of open and endovascular repair of rDTAA. Methods We systematically reviewed all studies describing the outcomes of rDTAA treated with open repair or TEVAR since 1995 using MEDLINE, Cochrane Library CENTRAL, and Excerpta Medica Database (EMBASE) databases. Case reports or studies published before 1995 were excluded. All articles were critically appraised for relevance, validity, and availability of data regarding treatment outcomes. All data were systematically pooled, and meta-analyses were performed to investigate 30-day mortality, myocardial infarction, stroke, and paraplegia rates after both types of repair. Results Original data of 224 patients (70% male) with rDTAA were identified: 143 (64%) were treated with TEVAR and 81 (36%) with open repair. Mean age was 70 ± 5.6 years. The 30-day mortality was 19% for patients treated with TEVAR for rDTAA compared 33% for patients treated with open repair, which was significant (odds ratio OR, 2.15, P = .016). The 30-day occurrence rates of myocardial infarction (11.1% vs 3.5%; OR, 3.70, P < .05), stroke (10.2% vs 4.1%; OR, 2.67; P = .117), and paraplegia (5.5% vs 3.1%; OR, 1.83; P = .405) were increased after open repair vs TEVAR, but this failed to reach statistical significance for stroke and paraplegia. Five additional patients in the TEVAR group died of aneurysm-related causes after 30 days, during a median follow-up of 17 ± 10 months. Follow-up data after open repair were insufficient. The estimated aneurysm-related survival at 3 years after TEVAR was 70.6%. Conclusion Endovascular repair of rDTAA is associated with a significantly lower 30-day mortality rate compared with open surgical repair. TEVAR was associated with a considerable number of aneurysm-related deaths during follow-up.
The majority of the genetic causes of autosomal recessive (ar) cone dystrophy (CD) and cone-rod dystrophy (CRD) are currently unknown. We used a high-resolution homozygosity mapping approach in a ...cohort of patients with CD or CRD to identify new genes for ar cone disorders.
Case series.
A cohort of 159 patients with ar CD and 91 patients with CRD.
The genomes of 83 patients with ar CD and 73 patients with CRD were analyzed for homozygous regions using single nucleotide polymorphism (SNP) microarrays. One patient showed homozygosity of SNPs across chromosome 6, and segregation analysis was performed using microsatellite markers. Direct sequencing of all retinal disease genes on chromosome 6 revealed a novel pathogenic TULP1 mutation in this patient. A cohort of 159 individuals with CD and 91 individuals with CRD was screened for this particular mutation using the restriction enzyme HhaI. The medical history of patients carrying the TULP1 mutation was reviewed and additional ophthalmic examinations were performed, including electroretinography (ERG), perimetry, optical coherence tomography (OCT), fundus autofluorescence (FAF), and fundus photography.
TULP1 mutations, age at diagnosis, visual acuity, fundus appearance, color vision defects, visual field, ERG, FAF, and OCT findings.
In 1 patient, homozygosity mapping and subsequent segregation analysis revealed maternal uniparental disomy (UPD) of chromosome 6. A novel homozygous missense mutation (p.Arg420Ser) was identified in TULP1, whereas no mutations were detected in other retinal disease genes on chromosome 6. The mutation affects a highly conserved amino acid residue in the Tubby domain and is predicted to be pathogenic. The same homozygous mutation was also identified in an additional, unrelated patient with CRD. Both patients carrying the p.Arg420Ser mutation presented with a bull's eye maculopathy. The first patient had progressive loss of visual acuity with a relatively preserved ERG, whereas the second patient developed loss of visual acuity, peripheral degeneration, and severely reduced ERG responses in a cone-rod pattern.
Maternal UPD of chromosome 6 unmasked a mutation in the TULP1 gene as a novel cause of cone dysfunction. This expands the disease spectrum of TULP1 mutations from Leber congenital amaurosis and early-onset retinitis pigmentosa to cone-dominated disease.
The author(s) have no proprietary or commercial interest in any materials discussed in this article.
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