Background Acute aortic dissection type A is a life-threatening disease required emergency surgery during acute phase. Different clinical manifestations, laboratory tests, and imaging features of patients with acute aortic dissection type A are the risk factors of preoperative mortality. This study aims to establish a simple and effective preoperative mortality risk assessment model for patients with acute aortic dissection type A. Methods A total of 673 Chinese patients with acute aortic dissection type A who were admitted to our hospital were retrospectively included. All patients were unable to receive surgically treatment within 3 days from the onset of disease. The patients included were divided into the survivor and deceased groups, and the endpoint event was preoperative death. Multivariable analysis was used to investigate predictors of preoperative mortality and to develop a prediction model. Results Among the 673 patients, 527 patients survived (78.31%) and 146 patients died (21.69%). The developmental dataset had 505 patients, calibration by Hosmer Lemeshow was significant (χ2 = 3.260, df = 8, P = 0.917) and discrimination by area under ROC curve was 0.8448 (95% CI 0.8007–0.8888). The validation dataset had 168 patients, calibration was significant (χ2 = 5.500, df = 8, P = 0.703) and the area under the ROC curve was 0.8086 (95% CI 0.7291–0.8881). The following independent variables increased preoperative mortality: age (OR = 1.008, P = 0.510), abrupt chest pain (OR = 3.534, P < 0.001), lactic in arterial blood gas ≥ 3 mmol/L (OR = 3.636, P < 0.001), inotropic support (OR = 8.615, P < 0.001), electrocardiographic myocardial ischemia (OR = 3.300, P = 0.001), innominate artery involvement (OR = 1.625, P = 0.104), right common carotid artery involvement (OR = 3.487, P = 0.001), superior mesenteric artery involvement (OR = 2.651, P = 0.001), false lumen / true lumen of ascending aorta ≥ 0.75 (OR = 2.221, P = 0.007). Our data suggest that a simple and effective preoperative death risk assessment model has been established. Conclusions Using a simple and effective risk assessment model can help clinicians quickly identify high-risk patients and make appropriate medical decisions.

Objective: This study aimed to establish a risk assessment model to predict postoperative severe acute lung injury (ALI) risk in patients with acute type A aortic dissection (ATAAD). Methods: Consecutive patients with ATAAD admitted to our hospital were included in this retrospective assessment and placed in the postoperative severe ALI and nonsevere ALI groups based on the presence or absence of ALI within 72 h postoperatively (oxygen index [OI] ≤ 100 mmHg). Patients were then randomly divided into training and validation groups in a ratio of 8:2. Univariate and multivariate stepwise forward logistic regression analyses were used to statistically assess data and establish the prediction model. The prediction model's effectiveness was evaluated via 10-fold cross-validation of the validation group to facilitate the construction of a nomogram. Results: After the screening, 479 patients were included in the study: 132 (27.6%) in the postoperative severe ALI group and 347 (72.4%) in the postoperative nonsevere ALI group. Based on multivariate logistics regression analyses, the following variables were included in the model: coronary heart disease, cardiopulmonary bypass (CPB) ≥ 257.5 min, left atrium diameter ≥ 35.5 mm, hemoglobin ≤ 139.5 g/L, preCPB OI ≤ 100 mmHg, intensive care unit OI ≤ 100 mmHg, left ventricular posterior wall thickness ≥ 10.5 mm, and neutrophilic granulocyte percentage ≥ 0.824. The area under the receiver operating characteristic (ROC) curve of the modeling group was 0.805 and differences between observed and predicted values were not deemed statistically significant via the Hosmer-Lemeshow test (χ 2 = 6.037, df = 8, p = .643).For the validation group, the area under the ROC curve was 0.778, and observed and predicted value differences were insignificant when assessed using the Hosmer-Lemeshow test (χ 2 = 3.3782, df = 7; p = .848). The average 10-fold crossvalidation score was 0.756. Conclusions:This study established a prediction model and developed a nomogram to determine the risk of postoperative severe ALI after ATAAD. Variables used in the model were easy to obtain clinically and the effectiveness of the model was good.

Objective: This study aimed to establish a risk assessment model to predict postoperative severe acute lung injury (ALI) risk in patients with acute type A aortic dissection (ATAAD). Methods: Consecutive patients with ATAAD admitted to our hospital were included in this retrospective assessment and placed in the postoperative severe ALI and non-severe ALI groups based on the presence or absence of ALI within 72 h postoperatively (oxygen index (OI) ≤100 mmHg). Patients were then randomly divided into training and validation groups in a ratio of 8:2. Logistic regression analyses were used to statistically assess data and establish the prediction model. The prediction model’s effectiveness was evaluated via tenfold cross-validation of the validation group to facilitate construction of a nomogram. Results: After screening, 479 patients were included in the study: 132 (27.5%) in the postoperative severe ALI group and 347 (72.5%) in the postoperative non-severe ALI group. Based on logistics regression analyses, the following variables were included in the model: coronary heart disease (CHD), cardiopulmonary bypass (CPB) ≥257.5 min, left atrium (LA) diameter ≥35.5 mm, hemoglobin ≤139.5 g/L, preCPB OI ≤100 mmHg, intensive care unit (ICU) OI ≤100 mmHg, left ventricular posterior wall thickness (LVPWT) ≥10.5 mm, and neutrophilic granulocyte percentage (NEUT) ≥0.824. The area under the receiver operating characteristic (ROC) curve of the modeling group was 0.805, and differences between observed and predicted values were not deemed statistically significant via the Hosmer–Lemeshow test (χ2=6.037, df=8, P=0.643). For the validation group, the area under the ROC curve was 0.778, and observed and predicted value differences were insignificant when assessed using the Hosmer–Lemeshow test (χ =3.3782, df=7; P=0.848). The average tenfold cross-validation score was 0.756. Conclusions: This study established a prediction model and developed a nomogram to determine the risk of postoperative severe ALI after ATAAD. Variables used in the model were easy to obtain clinically and the effectiveness of the model was good.

BackgroundAcute type A aortic dissection (ATAAD) is a rare, life-threatening condition affecting the aorta. This study explores the relationship between the level of admission D-dimer, which was assessed during the first 2 h from admission, and in-hospital major adverse events (MAE) with ATAAD.MethodsA total of 470 patients with enhanced computed tomography (CT) confirmed diagnosis of ATAAD who underwent operation treatment in Guangdong Provincial People's hospital between September 2017 and June 2021 were enrolled in the present study. The X-tile program was used to determine the optimal D-dimer thresholds for risk. Restricted cubic spline (RSC) was performed to assess the association between D-dimer and endpoint. The perioperative data were compared between the two groups, univariate and multivariate analyses were used to investigate the risk factors of major adverse events (in-hospital mortality, gastrointestinal bleeding, paraplegia, acute kidney failure, reopen the chest, low cardiac output syndrome, cerebrovascular accident, respiratory insufficiency, MODS, gastrointestinal bleeding, and severe infection).ResultsAmong 470 patients, 151 (32.1%) had MAE. In-hospital mortality was 7.44%. The patients with D-dimer &gt;14,500 ng/ml were more likely to present with acute kidney failure, low cardiac output, cerebrovascular accident, multiple organ dysfunction syndromes (MODS), gastrointestinal bleeding, and severe infection. D-dimer level was an independent risk factor for acute kidney failure (OR 2.09, 95% CI: 1.25–3.51, p = 0.005), MODS (OR 6.40, 95% CI: 1.23–33.39, p = 0.028), gastrointestinal bleeding (OR 17.76, 95% CI: 1.99–158.78, p = 0.010) and mortality (OR 3.17, 95% CI: 1.32–7.63, p = 0.010). Multivariate regression analysis of adverse events also suggested that D-dimer &gt;14,500 ng/ml (OR 1.68, 95% CI: 1.09–2.61, p = 0.020) was the independent risk factor of major adverse events.ConclusionsIncreasing D-dimer levels were independently associated with the in-hospital MAE and thus can be used as a useful prognostic biomarker before the surgery.

Background: Thoracic aortic dissection (TAD) is a very serious vascular condition that requires immediate treatment. Phenotypic conversion of human aortic smooth muscle cells (HASMCs) has been reported to be a causal factor for TAD development. Genetic variations affecting RNA modification may play a functional role in TAD. In this study, we aimed to explore the potential role of the methyltransferase like 3 (METTL3) and notch homolog 1 (NOTCH1) N 6 -methyladenosine (m6A) modification mechanisms in HASMCs.Methods: HASMCs were cultured. METTL3 was knocked down and overexpressed. Then, both METTL3 and NOTCH1 were simultaneously knocked down in HASMCs. HASMC proliferation was determined using Cell Counting Kit-8 (CCK-8). METTL3, NOTCH1, α-smooth muscle actin (α-SMA), smooth muscle protein 22-alpha (SM22α), and calponin expressions were monitored with quantitative real-time polymerase chain reaction (qRT-PCR) and Western blotting. An m6A dot blot assay was used to examine the m6A modification levels. The NOTCH1 3' untranslated region (3'UTR) m6A modification was analyzed using SRAMP and RMBase v. 2.0. A methylated RNA immunoprecipitation (MeRIP) assay was used to evaluate the METTL3 overexpression effect on m6A modification of NOTCH1 messenger RNA (mRNA). A dualluciferase assay was used to investigate the effect of METTL3 binding of the NOTCH1 mRNA m6A modification site. YTH domain family 2 (YTHDF2)-RNA immunoprecipitation (RIP) was used to detect the change in YTHDF2's ability to bind to NOTCH1 mRNA after METTL3 overexpression. Results: Overexpression of METTL3 inhibited α-SMA, SM22α, calponin, and NOTCH1 expressions and promoted HASMC proliferation. Knocking down METTL3 had the opposite effect. The cointerference of the METTL3 and NOTCH1 results suggested that METTL3 regulated NOTCH1, contributing to HASMC phenotypic changes. The MeRIP assay showed that the m6A modification of NOTCH1 mRNA increased after METTL3 overexpression. The dual-luciferase assay indicated that the NOTCH1 mRNA m6A modification site and METTL3 overexpression promoted NOTCH1 mRNA degradation. YTHDF2-RIP further demonstrated that the binding ability of YTHDF2 and NOTCH1 mRNA was enhanced after METTL3 overexpression. Conclusions: METTL3 regulated the phenotypic changes of HASMC by upregulating m6A modification of NOTCH1 and inhibiting NOTCH1. What is the implication, and what should change now? • This study indicated METTL3 and NOTCH1 m6A modification may play an important role in the pathogenesis of TAD. • We can research how to prevent and treat TAD by regulation of METTL3 and NOTCH1 m6A modification.

Acute Type A Aortic Dissection (ATAAD) is a kind of cardiovascular disease which seriously threatens human life and health. Surgical treatment is currently recognized as the standard treatment for ATAAD. It has the characteristics of rapid onset, long operation time, and worse post-operative prognosis than other routine cardiac surgeries. Preoperative biomarkers correlated with the outcome of ATAAD was rarely reported. Future research should be directed toward finding out some useful predictive biomarkers and assessing their potential treatment value, in the hope of improving the postoperative prognosis of ATAAD. Von Willebrand factor (vWF) is considered to be closely related to pathophysiological processes such as blood coagulation and vascular inflammation. Its deficiency or elevation may affect patients' blood coagulation condition and postoperative intravascular inflammation, thus affecting the occurrence of postoperative hemorrhagic complications. The purpose of this study is to investigate the effect of vWF on the early postoperative outcome of patients with ATAAD. Method and analysis: Patients with ATAAD who receive surgical treatment in our center from April 1, 2021 to April 1, 2022 will be prospectively included. According to the preoperative vWF measurement, enrolled patients will be divided into two groups: normal vWF (normal level of vWF: NL-vWF, reference value of 50–160%) group and abnormal vWF (Disrupted level of vWF: DL-vWF) group. The preoperative baseline data (including demographic characteristics, comobidities and malperfusion syndrome state, imaging examination, and laboratory examination), and surgical data will be documented. Primary and secondary endpoints events (described in part 2.4) will be assessed and recorded. We will use propensity score approach to account for baseline differences between DL-vWF group and NL-vWF group, and compare the early postoperative outcomes for a purpose of assessing the effect of vWF on the early postoperative outcomes of patients with ATAAD. Highlights

Background Linkeropathies refers to a series of extremely rare hereditary connective tissue diseases affected by various glycosyltransferases in the biosynthesis of proteoglycans. We report for the first time two heterozygous variants of B3GAT3 in a Chinese infant, in whom Marfan syndrome was suspected at birth. Case presentation A 2-month-old boy from a non-consanguineous Chinese family without a family history presented severe phenotypes of joint dislocation, obvious flexion contractures of the elbow, arachnodactyly with slightly adducted thumbs, cranial dysplasia, foot abnormalities and aortic root dilation; Marfan syndrome was suspected at birth. Our patient was the youngest, at the age of 2 months, to experience aortic root dilation. Two B3GAT3 variants, NM_012200.2, c.752T>C, p.V251A and c.47C>A, p.S16*, with heterozygosity were identified in the patient by whole-exome sequencing; the variants were inherited from his parents. During close follow-up, significant changes in the cranial profile and obvious external hydrocephalus were present at the age of 7 months, which differs from previously reported cases. Conclusion We diagnosed a patient with congenital heart defects at an early age with a B3GAT3-related disorder instead of Marfan syndrome and expanded the spectrum of B3GAT3-related disorders. We also provide a literature review of reported B3GAT3 cases; for at least one of the variants, this is the first report of genotype–phenotype correlations in individuals with cardiovascular defects being related to the acceptor substrate-binding subdomain of B3GAT3.

Background This study investigated the feasibility of a novel preformed artificial chordae tendineaes(ACTs) implantation device (Halochord) for mitral valve repair(MVP) via apical access. Methods Nine pigs were randomly divided into three groups: A, B, and C, and survived for 1, 3, and 6 months after surgery, respectively. The ACTs were anchored in the leaflet throught Halochord, adjusted to the correct length to cause moderate MR guided by echocardiography, and secured at the epicardium. Echocardiography was used to assess hemodynamic data and valve function. Surviving pigs were killed at the end of the follow-up period to confirm the deployment of ACTs. Results The modeling was successful, with no operative mortality. According to the echocardiographic and the cardiac anatomical specimen, all ACT implantation sites were found in the P2 region of MV. No rupture of the ACTs was detected during the observation period. Additionally, the ACTs gradually internalized as time passed, particularly at their extremities. Conclusions We demonstrated that off-pump ACTs implantation from the LV apex is a feasible and reproducible procedure.