E-vita OPEN NEO in the treatment of acute or chronic aortic pathologies: first interim results of the NEOS study.

OBJECTIVES: The aim of this multicentre study was to demonstrate the safety and clinical performance of E-vita OPEN NEO Stent Graft System (Artivion, Inc.) in the treatment of aneurysm or dissection, both acute and chronic, in the ascending aorta, aortic arch and descending thoracic aorta. METHODS: In this observational study of 12 centres performed in Europe and in Asia patients were enrolled between December 2020 and March 2022. All patients underwent frozen elephant trunk using E-vita OPEN NEO Stent Graft System. Primary end point was the rate of all-cause mortality at 30 days and secondary end points included further clinical and safety data are reported up to 3-6 months postoperatively. RESULTS: A total of 100 patients (66.7% male; mean age, 57.7 years) were enrolled at 12 sites. A total of 99 patients underwent surgery using the E-vita OPEN NEO for acute or subacute type A aortic dissection (n = 37), chronic type A aortic dissection (n = 33) or thoracic aortic aneurysm (n = 29), while 1 patient did not undergo surgery. Device technical success at 24 h was achieved in 97.0%. At discharge, new disabling stroke occurred in 4.4%, while new paraplegia and new paraparesis was reported in 2.2% and 2.2%, respectively. Renal failure requiring permanent (>90 days) dialysis or hemofiltration at discharge was observed in 3.3% of patients. Between discharge and the 3-6 months visit, no patients experienced new disabling stroke, new paraplegia or new paraparesis. The 30-day mortality was 5.1% and the estimated 6-month survival rate was 91.6% (standard deviation: 2.9). CONCLUSIONS: Total arch replacement with the E-vita OPEN NEO can be performed with excellent results in both the acute and chronic setting. This indicates that E-vita OPEN NEO can be used safely, including in the setting of acute type A aortic dissection.

Challenges in prompt identification and surgical correction of Marfan Syndrome aortic disease in a middle-income country: a case series study.

BACKGROUND: Marfan Syndrome is an autosomal dominant disease caused by pathogenetic variants in the FBN1 gene. The progressive dilatation of the aorta and the potential risk of acute aortic syndromes influence the prognosis of these patients. We aim to describe population characteristics, long-term survival, and re-intervention patterns in patients who underwent aortic surgery with a previously confirmed clinical diagnosis of Marfan Syndrome in a middle-income country. METHODS: A retrospective single-center case series study was conducted. All Marfan Syndrome patients who underwent aortic procedures from 2004 until 2021 were included. Qualitative variables were frequency-presented, while quantitative ones adopted mean ± standard deviation. A subgroup analysis between elective and emergent procedures was conducted. Kaplan-Meier plots depicted cumulative survival and re-intervention-free. Control appointments and government data tracked out-of-hospital mortality. RESULTS: Fifty patients were identified. The mean age was 38.79 ± 14.41 years, with a male-to-female ratio of 2:1. Common comorbidities included aortic valve regurgitation (66%) and hypertension (50%). Aortic aneurysms were observed in 64% without dissection and 36% with dissection. Surgical procedures comprised elective (52%) and emergent cases (48%). The most common surgery performed was the David procedure (64%), and the Bentall procedure (14%). The in-hospital mortality rate was 4%. Complications included stroke (10%), and acute kidney injury (6%). The average follow-up was 8.88 ± 5.78 years. Survival rates at 5, 10, and 15 years were 89%, 73%, and 68%, respectively. Reintervention rates at 1, 2.5, and 5 years were 10%, 14%, and 17%, respectively. The emergent subgroup was younger (37.58 ± 14.49 years), had the largest number of Stanford A aortic dissections, presented hemodynamic instability (41.67%), and had a higher requirement of reinterventions in the first 5 years of follow-up (p = 0.030). CONCLUSION: In our study, surveillance programs played a pivotal role in sustaining high survival rates and identifying re-intervention requirements. However, challenges persist, as 48% of the patients required emergent surgery. Despite not affecting survival rates, a greater requirement for reinterventions was observed, emphasizing the necessity of timely diagnosis. Enhanced educational initiatives for healthcare providers and increased patient involvement in follow-up programs are imperative to address these concerns.

Repair of Dissecting Aortic Aneurysm in Post-LVAD Patient.

Left ventricular assist devices (LVAD) are frequently used in the management of end-stage heart failure, especially given the limited availability of donor hearts. The latest HeartMate 3 LVAD delivers non-physiological continuous flow (CF), although the impact on the aorta is not well established. We highlight a case of aortic aneurysm formation complicated by dissection formation that necessitated high-risk re-operative surgery in a patient post CF-LVAD.

Delayed management of acute type A aortic dissection with concomitant coronary artery bypass graft.

BACKGROUND: Pre-operative coronary angiography and concomitant, planned coronary artery bypass are infrequently performed with type A aortic dissection repair. We present a case in which pre-operative coronary computed tomography angiography was appropriate, and subsequent dissection repair and concomitant coronary artery bypass were successfully performed. CASE PRESENTATION: The patient is a 58-year-old male with heart failure with preserved ejection fraction, renal insufficiency, hypertension, obesity, and smoking history, who presented with a three-to-four-day history of persistent back pain, worsening exertional dyspnea, and orthopnea, as well as a two-to-three month history of dyspnea, lower extremity edema, and intermittent angina. He was diagnosed with an acute type A aortic dissection and anti-impulse control was initiated. However, repair was delayed in order to allow apixaban to metabolize and decrease the risk of bleeding, as the patient was approximately six days post-dissection, without malperfusion, with a well-controlled blood pressure on anti-impulse therapy, and had received five days of anticoagulation. During this time, coronary computed tomography angiography was performed to assess the need for concomitant revascularization and showed coronary artery disease. Ascending aorta hemiarch replacement with aortic valve resuspension, two-vessel coronary artery bypass grafting, and left atrial appendage clipping were performed successfully. CONCLUSIONS: Pre-operative imaging can be considered in a select group of acute type A aortic dissections that present without malperfusion, and with well-controlled blood pressure on anti-impulse/negative inotropic therapy.

Type B Aortic Dissection CTA Collection with True and False Lumen Expert Annotations for the Development of AI-based Algorithms.

Aortic dissections (ADs) are serious conditions of the main artery of the human body, where a tear in the inner layer of the aortic wall leads to the formation of a new blood flow channel, named false lumen. ADs affecting the aorta distally to the left subclavian artery are classified as a Stanford type B aortic dissection (type B AD). This is linked to substantial morbidity and mortality, however, the course of the disease for the individual case is often unpredictable. Computed tomography angiography (CTA) is the gold standard for the diagnosis of type B AD. To advance the tools available for the analysis of CTA scans, we provide a CTA collection of 40 type B AD cases from clinical routine with corresponding expert segmentations of the true and false lumina. Segmented CTA scans might aid clinicians in decision making, especially if it is possible to fully automate the process. Therefore, the data collection is meant to be used to develop, train and test algorithms.

Mortality and hospitalization trends for aortic aneurysms and dissections in Mexico.

BACKGROUND: In Mexico, there is a paucity of evidence on mortality and hospitalization patterns associated with aortic aneurysms and dissections. OBJECTIVE: To analyze national databases and describe the epidemiological characteristics of different aortic pathologies. MATERIAL AND METHODS: Retrospective, cross-sectional, observational study, in which mortality and hospitalization attributed to aortic aneurysms and dissections were analyzed. Statistical analysis was performed on Stata 16. RESULTS: A total of 6,049 deaths were documented in the general population, which included 2,367 hospitalizations and 476 (20.1%) in-hospital deaths. In addition, a statistically significant age difference was found between mean age at death in the general population (69.5 years) and the in-hospital death group (64.1 years, p < 0.001). As for hospitalizations secondary to ruptured abdominal aortic aneurysms, 149 cases were identified, with a mean age of 65.6 years, out of whom 53 (35.5%) were under 65 years of age, with a mean age of 47.8 years. CONCLUSIONS: Epidemiological reports of aortic pathology in Mexico are scarce; therefore, implementation of screening and detection programs for aortic pathologies is necessary in order to address the disparities identified in this analysis.

ANTECEDENTES: Existe evidencia escasa en México respecto a la mortalidad y patrones del ingreso hospitalario asociados a aneurismas y disecciones aórticos. OBJETIVO: Analizar las bases de datos nacionales y describir las características epidemiológicas de diferentes patologías aórticas agudas. MATERIAL Y MÉTODOS: Estudio transversal y observacional de una base de datos retrospectiva, en el que se analizó la mortalidad y hospitalización atribuidas a aneurismas y disecciones aórticos. El análisis estadístico se realizó en Stata 16. RESULTADOS: Se documentaron 6049 muertes en la población general, 2367 hospitalizaciones y 476 muertes intrahospitalarias. Adicionalmente, se encontró una diferencia estadísticamente significativa entre las medias de edad de fallecimiento de la población general (65.5 años) y de los pacientes que murieron en el hospital (64.1 años), p < 0.001. En cuanto a las hospitalizaciones secundarias a aneurisma de aorta abdominal roto, 149 casos fueron evidenciados con una media de edad de 65.6 años; 53 (35.5 %) de estos tenía menos de 65 años, con una media de edad de 47.8 años. CONCLUSIONES: Los reportes epidemiológicos de patología aórtica en México son escasos, por ello la implementación de programas de tamizaje y la detección de patologías aórticas son necesarias para mejorar las disparidades encontradas en este análisis.

Redo partial aortic root remodelling in type A dissection with an aorto-left atrial fistula.

A 72-year-old male with a history of a triple-vessel coronary artery bypass graft years ago presented with a DeBakey type 2 aortic dissection and an aorto-left atrial fistula with patent bypass grafts (left internal mammary artery and saphenous vein grafts). He developed pulmonary oedema and required intubation. The right axillary artery was cannulated. After the ascending aorta and left internal mammary artery were clamped, the aorta was transected, leaving aortic tissue around two saphenous vein grafts as two separate patches. An entry tear was found adjacent to the proximal anastomosis of the saphenous vein graft to the posterior descending artery. A fistula, which was located between a false lumen in the non-coronary sinus and the dome of the left atrium, was primarily closed. Because the adventitia was thinned out in the non-coronary sinus due to aortic dissection, partial aortic root remodelling was performed with resuspension of the commissures. Hemiarch repair was performed under moderate hypothermia and unilateral antegrade cerebral perfusion. After systemic perfusion was resumed, the locations of the saphenous vein graft buttons were determined. The ascending graft was cross-clamped again; the saphenous vein graft to the obtuse marginal branch graft was reimplanted using the Carrel patch technique while a saphenous vein graft to the posterior descending artery required interposition of a 10-mm Dacron graft to accommodate the length.

Study on risk factors and treatment strategies of hypoxemia after acute type a aortic dissection surgery.

Acute type A aortic dissection is a life-threatening cardiovascular disease characterized by rapid onset and high mortality. Emergency surgery is the preferred and reliable treatment option. However, postoperative complications significantly impact patient prognosis. Hypoxemia, a common complication, poses challenges in clinical treatment, negatively affecting patient outcomes and increasing the risk of mortality. Therefore, it is crucial to study and comprehend the risk factors and treatment strategies for hypoxemia following acute type A aortic dissection to facilitate early intervention.

Two surgical strategies (early carotid reperfusion vs. Central aortic repair-first) of acute type a aortic dissection complicated with cerebral malperfusion syndrome: a meta-analysis and systematic review.

OBJECTIVE: Cerebral malperfusion (CM) is a common comorbidity in acute type A aortic dissection (ATAAD), which is associated with high mortality and poor neurological prognosis. This meta-analysis investigated the surgical strategy of ATAAD patients with CM, aiming to compare the difference in therapeutic effectiveness between the central repair-first and the early reperfusion-first according to clinical outcomes. METHODS: The meta-analysis and systematic review was conducted based on studies sourced from the PubMed, Embase, and Cochrane literature database, in which cases of ATAAD with CM underwent surgical repair were included. Data for baseline characteristics, mortality, survival were extracted, and risk ratio (RR) values and the pooled mortality were calculated. RESULTS: A total of 17 retrospective studies were analyzed, including 1010 cases of ATAAD with CM underwent surgical repair. The pooled early mortality in early reperfusion group was lower (8.1%; CI, 0.02 to 0.168) than that in the central repair group (16.2%; CI, 0.115 to 0.216). The pooled long-term mortality was 7.9% in the early reperfusion cohort and 17.4% the central repair-first cohort, without a statistically significant heterogeneity (I [2] = 51.271%; p = 0.056). The mean time of symptom-onset-to-the-operation-room in all the reports was 8.87 ± 12.3 h. CONCLUSION: This meta-analysis suggested that early reperfusion-first may achieved better outcomes compared to central repair-first in ATAAD patients complicated with CM to some extent. Early operation and early restoration of cerebral perfusion may reduce the occurrence of some neurological complications. TRIAL REGISTRATION: The meta-analysis was registered in the International Prospective Register of Systematic Reviews database (No. CRD CRD42023475629) on Nov. 8th, 2023.

Diabetes and aortic dissection: unraveling the role of 3-hydroxybutyrate through mendelian randomization.

BACKGROUND: In observational and experimental studies, diabetes has been reported as a protective factor for aortic dissection. 3-Hydroxybutyrate, a key constituent of ketone bodies, has been found to favor improvements in cardiovascular disease. However, whether the protective effect of diabetes on aortic dissection is mediated by 3-hydroxybutyrate is unclear. We aimed to investigate the causal effects of diabetes on the risk of aortic dissection and the mediating role of 3-hydroxybutyrate in them through two-step Mendelian randomization. MATERIALS AND METHODS: We performed a two-step Mendelian randomization to investigate the causal connections between diabetes, 3-hydroxybutyrate, and aortic dissection and calculate the mediating effect of 3-hydroxybutyrate. Publicly accessible data for Type 1 diabetes, Type 2 diabetes, dissection of aorta and 3-hydroxybutyrate were obtained from genome-wide association studies. The association between Type 1 diabetes and dissection of aorta, the association between Type 2 diabetes and dissection of aorta, and mediation effect of 3-hydroxybutyrate were carried out separately. RESULTS: The IVW method showed that Type 1 diabetes was negatively associated with the risk of aortic dissection (OR 0.912, 95% CI 0.836-0.995), The weighted median, simple mode and weighted mode method showed consistent results. The mediated proportion of 3-hydroxybutyrate on the relationship between Type 1 diabetes and dissection of aorta was 24.80% (95% CI 5.12-44.47%). The IVW method showed that Type 2 diabetes was negatively associated with the risk of aortic dissection (OR 0.763, 95% CI 0.607-0.960), The weighted median, simple mode and weighted mode method showed consistent results. 3-Hydroxybutyrate does not have causal mediation effect on the relationship between Type 2 diabetes and dissection of aorta. CONCLUSION: Mendelian randomization study revealed diabetes as a protective factor for dissection of aorta. The protective effect of type 1 diabetes on aortic dissection was partially mediated by 3-hydroxybutyrate, but type 2 diabetes was not 3-hydroxybutyrate mediated.

Surgical choice for the treatment of partial intestinal ischemic necrosis caused by acute type a aortic dissection combined with malperfusion of superior mesenteric artery.

Acute type A aortic dissection is a severe cardiovascular disease characterized by rapid onset and high mortality. Traditionally, urgent open aortic repair is performed after admission to prevent aortic rupture and death. However, when combined with malperfusion syndrome, the low perfusion of the superior mesenteric artery can further lead to intestinal necrosis, significantly impacting the surgery's prognosis and potentially resulting in adverse consequences, bringing. This presents great significant challenges in treatment. Based on recent domestic and international research literature, this paper reviews the mechanism, current treatment approaches, and selection of surgical methods for poor organ perfusion caused by acute type A aortic dissection. The literature review findings suggest that central aortic repair can be employed for the treatment of acute type A aortic dissection with inadequate perfusion of the superior mesenteric artery. The superior mesenteric artery can be windowed and (/or) stented, followed by delayed aortic repair. Priority should be given to revascularization of the superior mesenteric artery, followed by central aortic repair. During central aortic repair, direct blood perfusion should be performed on the distal true lumen of the superior mesenteric artery, leading to resulting in favorable therapeutic outcomes. The research results indicate that even after surgical aortic repair, intestinal ischemic necrosis may still occur. In such cases, prompt laparotomy and necessary necrotic bowel resection are crucial for saving the patient's life.

Clinical predictive value of the age, creatinine, and ejection fraction score in patients in acute type A aortic dissection after total arch replacement.

The age, creatinine, and ejection fraction (ACEF) score has been accepted as a predictor of poor outcome in elective operations. This study aimed to investigate the predictive value of ACEF score in acute type A aortic dissection (AAAD) patients after total arch replacement. A total of 227 AAAD patients from July 2021 and June 2022 were enrolled and divided into Tertiles 1 (ACEF ≤ 0.73), Tertiles 2 (0.73 < ACEF ≤ 0.95), and Tertiles 3 (ACEF > 0.95). Using inverse probability processing weighting (IPTW) to balance the baseline characteristics and compare the outcomes. Cox logistic regression was used to further evaluate the survival prediction ability of ACEF score. The in-hospital mortality was 9.8%. After IPTW, in the baseline characteristics reached an equilibrium, a higher ACEF score before operation still associated with higher in-hospital mortality. After 1 year follow-up, 184 patients (90.6%) survival. Multivariable analysis revealed that ACEF score (adjusted hazard ratio 1.68; 95% confidence interval 1.34-4.91; p = 0.036) and binary ACEF score (adjusted HR 2.26; 95% CI 1.82-6.20; p < 0.001) was independently associated with 1-year survival. In addition, net reclassification improvement (NRI) and integrated differentiation improvement (IDI) verified that the ACEF score and binary ACEF score is an accurate predictive tool in clinical settings. In conclusions, ACEF score could be considered as a useful tool to risk stratification in patients with AAAD before operation in daily clinical work.

Clinical Outcome of the Type A Acute Aortic Dissection Repair Using the "Tailored Stand-Up Collar" Technique.

PURPOSE: Achieving a secure anastomosis and complete hemostasis is essential for surgically treating type A acute aortic dissection (TAAAD). This study assessed the clinical feasibility of "tailored stand-up collar (TSC)" technique for constructing the distal stump. METHODS: We enrolled 68 patients who underwent ascending aortic repair for TAAAD. Patients were categorized according to the technique for distal stump construction: conventional (C) group using only a felt strip (32 cases); post-aortotomy (P) group, with a Hydrofit-felt strip attached after aortotomy (18 cases), and TSC group, where a Hydrofit-felt strip attached during cooling (18 cases). Pre-operative characteristics, procedural profiles, and post-operative outcomes were evaluated. RESULTS: The pre-operative characteristics were identical among the groups. The durations of cardiopulmonary bypass, hemostasis, and surgery were significantly shorter in the P and TSC groups. The duration of open distal in the TSC group (21 min) was significantly shorter than the other two groups. Post-operative additional procedures were not required for the TSC group and their post-operative hospital stay was significantly shorter (47.1% of patients were discharged within 2 weeks). CONCLUSION: The TSC technique would be practical because of its high reproducibility in terms of ease of use, shorter anastomotic time, and secure hemostasis.

Aortic arch surgery for DeBakey type 1 aortic dissection in patients aged 60 years or younger.

BACKGROUND: Extended aortic repair is considered a key issue for the long-term durability of surgery for DeBakey type 1 aortic dissection. The risk of aortic degeneration may be higher in young patients due to their long life expectancy. The early outcome and durability of aortic surgery in these patients were investigated in the present study. METHODS: The subjects of the present analysis were patients under 60 years old who underwent surgical repair for acute DeBakey type 1 aortic dissection at 18 cardiac surgery centres across Europe between 2005 and 2021. Patients underwent ascending aortic repair or total aortic arch repair using the conventional technique or the frozen elephant trunk technique. The primary outcome was 5-year cumulative incidence of reoperation on the distal aorta. RESULTS: Overall, 915 patients underwent surgical ascending aortic repair and 284 patients underwent surgical total aortic arch repair. The frozen elephant trunk procedure was performed in 128 patients. Among 245 propensity score-matched pairs, total aortic arch repair did not decrease the rate of distal aortic reoperation compared to ascending aortic repair (5-year cumulative incidence, 6.7% versus 6.7%, subdistributional hazard ratio 1.127, 95% c.i. 0.523 to 2.427). Total aortic arch repair increased the incidence of postoperative stroke/global brain ischaemia (25.7% versus 18.4%, P = 0.050) and dialysis (19.6% versus 12.7%, P = 0.003). Five-year mortality was comparable after ascending aortic repair and total aortic arch repair (22.8% versus 27.3%, P = 0.172). CONCLUSIONS: In patients under 60 years old with DeBakey type 1 aortic dissection, total aortic arch replacement compared with ascending aortic repair did not reduce the incidence of distal aortic operations at 5 years. When feasible, ascending aortic repair for DeBakey type 1 aortic dissection is associated with satisfactory early and mid-term outcomes. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT04831073.

Diagnosis and management of acute aortic dissection in the emergency department.

Acute aortic dissection is often misdiagnosed as a result of its atypical presentations. It affects 4000 patients a year in the UK of all ages, not just older patients, with increasing numbers of cases expected in the future because of the ageing population. Dissection of the aortic wall leads to sudden, severe pain, and commonly end-organ symptoms which must be recognised. Acute aortic dissection can be challenging to diagnose in the emergency department because of the multitude of possible presentations and the need for selective testing with Computed Tomography Angiography (CTA). Clinicians often miss acute aortic dissection because it is not considered in the differential diagnosis, and the challenge lies in identifying acute aortic dissection in a sea of complaints of chest, back and abdominal pain. There are several ways to improve diagnosis, including awareness campaigns, better education about patients in which to consider acute aortic dissection, and improved detection strategies including which patients should receive CTA. Clinical decision tools and biomarkers could help, but further research is required and is a research focus in emergency medicine. Once diagnosed, blood pressure control, analgesia and urgent surgery or transfer to enable this to occur with minimal delay is required.

[Retrograde Stanford Type A Aortic Dissection of Marfan Syndrome Long After Thoracic Endovascular Aortic Repair:Report of a Case].

A 52-year-old woman with Marfan syndrome developed Stanford type B aortic dissection and was treated with thoracic endovascular aortic repair. However, 29 months later, she presented with retrograde Stanford type A aortic dissection. We successfully performed aortic arch replacement with the frozen elephant trunk technique and valve-sparing aortic root replacement. The advantages of the frozen elephant trunk technique are that the distal anastomosis can be created without stent-graft resection and the cardiac arrest time is shortened. Therefore, the frozen elephant trunk technique was considered valuable and safe in this potentially lethal situation.

The Association between PDE5 Inhibitors and Aneurysm/Arterial Dissection:A Pharmacovigilance Study Using WHO Safety Database.

Aneurysm and arterial dissection have been reported as adverse drug events, associated with angiogenesis inhibitors and fluoroquinolones. Specifically, several cases of severe arterial disease following cGMP-specific phosphodiesterase type 5 (PDE5) inhibitors usage have recently been reported. It is necessary to ascertain the risks of serious adverse events caused by PDE5 inhibitors. We aimed to evaluate the association of aneurysm and artery dissection with PDE5 inhibitors using VigiBase, which is a World Health Organization database of spontaneously reported adverse events, for explorative hypothesis-generating analysis. We performed disproportionality analysis using a dataset from inception in 1967 to December 2022 and calculated reporting odds ratios (ROR) between PDE5 inhibitors and arterial diseases. We extracted 195,839 reports on PDE5 inhibitors with 254 reports of arterial disease as adverse events from VigiBase. Disproportionality analysis showed disproportional signals for PDE5 inhibitors (ROR, 2.30;95% confidence intervals, 2.04-2.61);disproportional signals were detected in analyses restricting the lesion site to the aorta or cerebral arteries. From stratified analysis, disproportional signals were noted in females, as well as males, generally recognized as a risk factor for artery diseases. This real-world data analysis suggests that PDE5 inhibitors may play a role in the development of lethal arterial disease. J. Med. Invest. 71 : 134-140, February, 2024.

Impact of persistent malperfusion syndrome following central repair of acute type A aortic dissection on early outcomes.

We investigated impact of persistent malperfusion syndrome (MPS) following central repair of acute type A aortic dissection (ATAAD) on outcomes. Thirty patients who underwent central repair for ATAAD with MPS were included. Patients were divided into two groups:23 patients without MPS following central repair (No-MPS group) and 7 with MPS (Persistent-MPS group). The mean age was 66.8±9.6 and 59.4±13.4 years in the No-MPS and Persistent-MPS groups, respectively (P=0.176). Preoperative MPS included the left coronary artery (n=3), brain (n=3), abdomen (n=7), and extremities (n=11) in the No-MPS group. In the Persistent-MPS group, the right coronary (n=1), brain (n=2), abdomen (n=3), and extremities (n=5) were observed. In the No-MPS group, one patient died of extensive cerebral infarction (4.3%). In the Persistent-MPS group, 2 patients died of sepsis and multi-organ failure, respectively (28.6%) (P=0.061). The Persistent-MPS group had more patients requiring hemodialysis than the No-MPS group (P=0.009). Three patients underwent intestinal resection due to persistent MPS (P<0.001). Persistent MPS following central repair for ATAAD significantly contributed to outcomes. J. Med. Invest. 71 : 158-161, February, 2024.