Frozen elephant trunk aortic reconstruction in a patient with Loeys-Dietz syndrome.
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ABSTRACT:
SUBMITTER: Eldeiry M
PROVIDER: S-EPMC9735389 | biostudies-literature | 2022 Dec
REPOSITORIES: biostudies-literature
Publications
Frozen elephant trunk aortic reconstruction in a patient with Loeys-Dietz syndrome.
Eldeiry Mohamed M Miyamoto Shelley S Chatfield Kathryn K Reece T Brett TB Mitchell Max B MB
JTCVS techniques 20220913
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Project description:BackgroundAim of this study was to report and to identify risk factors for distal aortic failure following aortic arch replacement via the frozen elephant trunk (FET) procedure.MethodsOne hundred eighty-six consecutive patients underwent the FET procedure for acute and chronic aortic dissection. Our cohort was divided into patients with and without distal aortic failure. Distal aortic failure was defined as: (I) distal aortic reintervention, (II) aortic diameter dilatation to ≥ 6 cm or > 5 mm growth within 6 months, (III) development of a distal stent-graft-induced new entry (dSINE) and/or (IV) aortic-related death. Preoperative, intraoperative, postoperative and aortic morphological data were analyzed.ResultsDistal aortic failure occurred in 88 (47.3%) patients. Forty-six (24.7%) required a distal reintervention, aortic diameter dilatation was observed in 9 (4.8%) patients, a dSINE occurred in 22 (11.8%) patients and 11 (6.4%) suffered an aortic-related death. We found no difference in the number of communications between true and false lumen (p = 0.25) but there were significantly more communications between Ishimaru zone 6-8 in the distal aortic failure group (p = 0.01). The volume of the thoracic descending aorta measured preoperatively and postoperatively within 36 months afterward was significantly larger in patients suffering distal aortic failure (p < 0.001; p = 0.011). Acute aortic dissection (SHR 2.111; p = 0.007), preoperative maximum descending aortic diameter (SHR 1.029; p = 0.018) and preoperative maximum aortic diameter at the level of the diaphragm (SHR 1.041; p = 0.012) were identified as risk factors for distal aortic failure.ConclusionThe incidence and risk of distal aortic failure following the FET procedure is high. Especially those patients with more acute and more extensive aortic dissections or larger preoperative descending aortic diameters carry a substantially higher risk of developing distal aortic failure. The prospective of the FET technique as a single-step treatment for aortic dissection seems low and follow-up in dedicated aortic centers is therefore paramount.
| S-EPMC9207307 | biostudies-literature
Project description:The aortic root is the predominant site for development of aneurysm caused by heterozygous loss-of-function mutations in positive effectors of the transforming growth factor-β (TGF-β) pathway. Using a mouse model of Loeys-Dietz syndrome (LDS) that carries a heterozygous kinase-inactivating mutation in TGF-β receptor I, we found that the effects of this mutation depend on the lineage of origin of vascular smooth muscle cells (VSMCs). Secondary heart field-derived (SHF-derived), but not neighboring cardiac neural crest-derived (CNC-derived), VSMCs showed impaired Smad2/3 activation in response to TGF-β, increased expression of angiotensin II (AngII) type 1 receptor (Agtr1a), enhanced responsiveness to AngII, and higher expression of TGF-β ligands. The preserved TGF-β signaling potential in CNC-derived VSMCs associated, in vivo, with increased Smad2/3 phosphorylation. CNC-, but not SHF-specific, deletion of Smad2 preserved aortic wall architecture and reduced aortic dilation in this mouse model of LDS. Taken together, these data suggest that aortic root aneurysm predisposition in this LDS mouse model depends both on defective Smad signaling in SHF-derived VSMCs and excessive Smad signaling in CNC-derived VSMCs. This work highlights the importance of considering the regional microenvironment and specifically lineage-dependent variation in the vulnerability to mutations in the development and testing of pathogenic models for aortic aneurysm.
| S-EPMC6355234 | biostudies-literature
Project description: Not available
| S-EPMC11569935 | biostudies-literature
Project description:BackgroundThe frozen elephant trunk (FET) technique was developed to facilitate the two-stage surgery of extensive pathologies of the thoracic aorta and is now routinely applied in acute and chronic aortic syndromes.MethodsFrom 11/2006 to 07/2017, 68 patients underwent aortic arch repair using the FET technique. Patients received either the Jotec E-vita Open graft (n =?57) or the Vascutek Thoraflex hybrid prosthesis (n =?11). Both, group 1 (acute aortic dissection type A and B; symptomatic penetrating aortic ulcer) and group 2 (aortic aneurysm; chronic aortic dissection) included 34 patients each.ResultsEarly mortality was 13.2% (14.7% in group 1 vs. 11.7% in group 2, p =?0.720). Neurological complications occurred in 12 patients (17.6%) (stroke: 8.8 vs. 11.7%; p =?0.797 and spinal cord injury: 8.8 vs. 5.9%; p =?0.642 in groups 1 vs. 2 respectively). Cardiopulmonary bypass time and cross clamp time were significantly longer in group 1 (252.2?±?73.5 and 148.3?± 34?min vs. 189.2?±?47.8 and 116.3?± 34.5?min; p?<? 0.001). The overall 1-, 3- and 7-year-survival was 80.9, 80.9 and 74.2% with no significant differences between groups 1 and 2. Expansion of true lumen after FET implantation was significant at all levels in both groups for patients with aortic dissection. One-, 3-, and 7-year-freedom from secondary (re-)intervention for patients for aortic dissection was 96.9, 90.2 and 82.7% with no significant differences between groups 1 and 2; p =?0.575.ConclusionThe FET technique can be applied in acute aortic syndromes with similar risks regarding adverse events or mortality when compared to chronic degenerative aortic disease. Postoperative increase in true lumen diameter mirrors decrease of false lumen diameter, goes along with favorable midterm outcome and prolongs freedom from secondary interventions in acute aortic dissection.
| S-EPMC6676558 | biostudies-literature
Project description:Background: This study was aimed to investigate the incidence, risk factors, and outcomes of patients with postoperative hepatic dysfunction (PHD) after frozen elephant trunk (FET) for type A aortic dissection (TAAD). Method: A retrospective study was performed with 492 patients who underwent FET for TAAD between 2015 and 2019. Independent risk factors for PHD were determined by multivariate mixed-effect logistic analysis with surgeon-specific factor as a random effect. Results: The incidence of PHD was 25.4% (n = 125) in our cohort. Patients with PHD presented higher early mortality (10.4 vs. 1.1%, p < 0.001), rates of acute kidney injury (42.4 vs. 12.8%, p < 0.001), and newly required dialysis (23.2 vs. 3.0%, p < 0.001) compared with those without PHD. Moreover, with the median follow-up period of 41.3 months, the survival curve was worse in patients with PHD compared with no PHD group (log-rank p < 0.001), whereas it was similar after excluding patients who died within 30 days (log-rank p = 0.761). Multivariable analyses suggested that PHD was predicted by preoperative aspartate transferase [odds ratio (OR), 1.057; 95% confidence intervals (CI), 1.036-1.079; p < 0.001], celiac trunk malperfusion (OR, 3.121; 95% CI, 1.008-9.662; p = 0.048), and cardiopulmonary bypass time (OR, 1.014; 95% CI, 1.005-1.023; p = 0.003). Retrograde perfusion (OR, 0.474; 95% CI, 0.268-0.837; p = 0.010) was associated with a reduced risk of PHD. Celiac trunk malperfusion was an independent predictor for PHD but not associated with early mortality and midterm survival. Conclusions: PHD was associated with increased early mortality and morbidity, but not with late death in midterm survival. PHD was predicted by preoperative aspartate transferase, celiac trunk malperfusion, and cardiopulmonary bypass (CPB) time, and retrograde perfusion was associated with a reduced risk of PHD.
| S-EPMC8645859 | biostudies-literature
Project description:IntroductionLoeys-Dietz syndrome (LDS) is a genetic syndrome caused by mutations in transforming growth factor beta receptors (TGFBR) 1 and 2. It can manifest with craniofacial, musculoskeletal, cognitive abnormalities, and vascular pathologies including early onset aortic root aneurysms, extensive aortic dissections, and TAAA. Open repair is considered the gold standard treatment but carries morbidity risks, especially in patients with multiple previous aortic procedures. Endovascular treatment is associated with treatment failure when used in the native aorta, because of inherent wall weakness precluding seal. This case report adds to the available literature on hybrid treatment of LDS associated aortic pathologies.ReportThis is the report of staged hybrid TAAA treatment in a 24 year old male patient with multiple previous aortic procedures via sternotomy and thoracotomy. Retrograde infrarenal aortic visceral debranching was performed using 14 mm by 7 mm bifurcated Dacron grafts. These emerged from the limbs of an 18 mm by 9 mm bifurcated Dacron graft in an aortobi-iliac reconstruction. This was followed by staged thoracic endovascular aortic repair (TEVAR) seven days later using three endografts (26 mm-22 mm × 150 mm distal, 30 mm × 200 mm bridging, then 32 mm × 100 mm proximal). The endograft landed in an old thoracic aortic graft proximally and the new infrarenal aortic graft distally. Follow up at 11 months showed patency and no sac expansion.ConclusionHybrid TAAA repair was a valid treatment option in this patient with LDS and multiple previous aortic procedures. It minimised the morbidity of revision surgery and mitigated potential treatment failure by achieving an endovascular seal in surgical grafts. Short term surveillance showed no complications. Limitations to making recommendations include lack of long term follow up.
| S-EPMC8181208 | biostudies-literature
Project description:BackgroundPost-implantation syndrome (PIS) is defined as non-infectious continuous fever and a concomitant rise in inflammatory markers shortly after endovascular aortic repair. PIS occurrence after hybrid procedures, such as the frozen elephant trunk (FET) technique, has not been adequately investigated. The current study aims to define the incidence of PIS after the FET and to identify possible risk factors associated with its occurrence.MethodsThe clinical charts of 59 patients undergoing the FET between February 2015 and April 2020 were reviewed retrospectively. The occurrence of PIS was defined as the presence of fever (>38 °C lasting longer than one day during the hospitalisation) and leucocytosis (white blood cell count >12,000/µL). Patients with concomitant conditions possibly leading to fever and/or leucocytosis were excluded. Beside demographic and procedure-related data, serum/plasma inflammatory markers were evaluated before surgery and daily up to seven days postoperatively. Computed tomography scans (CT) were examined to calculate the volume of pre-existent and new-onset mural thrombus after the FET.ResultsThirty-eight patients met the inclusion criteria. The study cohort was divided into two groups based on the occurrence of PIS (17 cases; 44.7%). Patients with PIS were significantly younger than those without PIS (53.5±8.9 vs. 62.5±9.6 years; P=0.005). Female patients were less likely to develop PIS (5.2% vs. 26.3%, P=0.018). Patients with PIS had a higher volume of new-onset thrombus in the postoperative CT (P<0.001). Patients treated for post-dissection aneurysm had, postoperatively, significantly more thrombus material developed in a false lumen (P=0.02). Among the PIS markers, CRP (C-reactive protein) levels on the third postoperative day were independently associated with the volume of new-onset thrombus (P=0.011). After multivariate analysis, the volume of new-onset thrombus (P=0.028) and age (P=0.036) remained the variable associated with a statistically significant increased incidence of PIS.ConclusionsPIS can occur after the frozen elephant trunk procedure. The volume of new-onset thrombus seems to be associated with an increased incidence of PIS. These findings need to be confirmed in larger patient cohorts.
| S-EPMC8339746 | biostudies-literature
Project description:Treatment of aortic arch aneurysms and dissections require highly complex surgical procedures with devastating complications and mortality rates. Currently, repair of the complete arch until the proximal descending thoracic aorta consists of a two-stage procedure, called elephant trunk (ET) technique, or a single stage a single-stage technique referred to as frozen elephant trunk (FET). There is conflicting evidence about the perioperative results of ET in comparison with FET. We carried out a meta-analysis to investigate possible differences in perioperative and early (up to 30 days) outcomes of ET vs. FET, particularly for mortality, spinal cord injury (SCI), stroke, and renal failure. We also performed a meta-regression to explore the effects of age and sex as possible cofactors. Twenty-one studies containing data from interventions conducted between 1997 and 2019 and published between 2008 and 2021 with 3153 patients (68.5% male) were included. ET was applied to 1,693 patients (53.7%) and FET to 1460 (46.3%). Overall mortality after ET was 250/1693 (14.8%) and after FET 116/1460 (7.9%). Relative risk (RR) and 95% confidence interval (CI) were 1.37 [1.04 to 1.81], p = 0.027. There was no significant effect of age and sex. SCI occurrence after the second stage of ET was 45/1693 (2.7%) and after FET 70/1,460 patients (4.8%) RR 0.53 [0.35 to 0.81], p = 0.004. Age and sex were not associated with the risk of SCI. No significant differences were observed between ET and FET in the incidence of stroke and renal failure. Our results indicate that ET is associated with higher early mortality but lower incidence of SCI compared to FET. When studies published in the last 5 years were analyzed, no significant differences in mortality or SCI were found between ET and FET. This difference is attributed to a decrease in mortality after ET, as the mortality after FET did not change significantly over time.
| S-EPMC9630467 | biostudies-literature
Project description: Not available
| S-EPMC10315428 | biostudies-literature