Objective To summarize the whole process comprehensive management experience in transfemoral aortic valve replacement(TF-TAVR)using self-expanding valve in 79 patients with pure native aortic valve regurgitation(PNAR).Methods The nursing team adopted a multi-team collaboration approach.Preoperative nursing assessment and full preparation,enhanced psychological support,and sleep management were carefully carried out;during the operation,nurses well cooperated with doctor,implemented predictive care and intervention for possible complications;and after surgery the hemodynamics and respiratory functions were closely monitored to promptly detect and manage the complications,and to implement the infection prevention cluster management process and discharge preparation services.Besides,the comprehensive management measures throughout the entire process,the occurrence of complications and corresponding nursing responses,as well as the six-minute walk test before and after the operation were recorded.The Discharge Readiness Scale was used to evaluate the implementation of patient discharge readiness services so as to check the implementation effect of the comprehensive whole process management measures.Results Through the implementation of comprehensive whole process management measures,the surgery-related ventricular fibrillation and cardiac arrest obtained accurate and timely treatment,no newly-developed complications such as deep vein thrombosis of lower limbs occurred.Through the implementation of infection cluster nursing measures,the incidence of pulmonary infection in patients decreased from 52.00％in the early stage of carrying out the management measures to 9.26％.Through the implementation of discharge preparation services and continuous quality improvement,the left ventricular ejection fraction(LVEF)value in 64 patients receiving successful TAVR increased from(44.06±5.51)％to(54.67±5.20)％,and the difference was statistically significant(t=19.634,P＜0.001).On the day of discharge,the six-minute walk test distance increased from preoperative(131.39±39.36)meters to postoperative(180.77±29.72)meters,and the difference was statistically significant(t=10.898,P＜0.001).The average self-assessment score of discharge readiness of patients was(7.33±1.41)points.All patients were well recovered when discharged from hospital.Conclusion According to the different surgical key points and the difficult problems,full and effective implementation of comprehensive whole process management measures can ensure that the PNAR patients are able to smoothly pass through the perioperative period and obtain a satisfactory recovery after receiving TF-TAVR.

This article reported the diagnosis and management of a case of a child with Kabuki syndrome. Kabuki syndrome is characterized by distinct facial features, skeletal anomalies, abnormal skin texture, and intellectual disabilities, and is primarily caused by heterozygous mutations in the lysine methyltransferase 2D(KMT2D) gene. The onset in this patient was insidious, presenting with short stature and intellectual impairment. Genetic testing identified a pathogenic heterozygous variant in the KMT2D gene. This article focuses on analyzing the necessity and appropriateness of growth hormone therapy in children with Kabuki syndrome, and includes a review of relevant literature to improve clinicians′ understanding of this rare condition.

This article reported the diagnosis and management of a case of a child with Kabuki syndrome. Kabuki syndrome is characterized by distinct facial features, skeletal anomalies, abnormal skin texture, and intellectual disabilities, and is primarily caused by heterozygous mutations in the lysine methyltransferase 2D(KMT2D) gene. The onset in this patient was insidious, presenting with short stature and intellectual impairment. Genetic testing identified a pathogenic heterozygous variant in the KMT2D gene. This article focuses on analyzing the necessity and appropriateness of growth hormone therapy in children with Kabuki syndrome, and includes a review of relevant literature to improve clinicians′ understanding of this rare condition.

Objective:To explore the association of cerebral venous outflow assessed by CT angiography (CTA) with first pass effect (FPE) in patients with acute anterior circulation large vessel occlusion accepted mechanical thrombectomy (MT).Methods:A retrospective analysis was performed; patients with acute anterior circulation large vessel occlusion accepted MT and CTA in Department of Neurology, Affiliated Hospital of Xuzhou Medical University from July 2018 to June 2021 were consecutively enrolled. Cerebral venous outflow in baseline CTA was evaluated using Cortical Vein Opacification Score (COVES). Patients were categorized into either FPE or non-FPE groups based on recanalization of occluded vessels after initial MT. General information, clinical features, radiological data, and surgery-related data between the 2 groups of patients were collected and compared. Significant variables ( P<0.1) from univariate analysis were included into a multivariable Logistic regression model to explore the relation between COVES and FPE. Predictive value of COVES in FPE was assessed using receiver operating characteristic (ROC) curve. Results:Out of the 143 patients enrolled in this study, 52 were into the FPE group and 91 were into the non-FPE group. Compared with the non-FPE group, the FPE group had higher COVES scores, higher proportion of patients with good cerebral venous drainage (COVES≥3), smaller core infarct volume, and shorter time from femoral artery puncture to vessel recanalization, with significant differences ( P<0.05). Multivariable Logistic regression analysis revealed that COVES was still corelated with FPE after adjusting covariates such as baseline NIHSS scores, core infarct volume, and time from femoral artery puncture to vessel recanalization ( OR=0.730, 95% CI: 0.567-0.940, P=0.015). ROC curve demonstrated that the combined model of COVES with aforementioned factors (COVES scores+baseline NIHSS scores+core infarct volume+time from femoral artery puncture to vessel recanalization) had an area under the curve of 0.757 (95% CI: 0.672-0.841, P<0.001), with sensitivity of 61.5% and specificity of 78.0%. Conclusion:Favorable cerebral venous drainage is an independent predictor for successful FPE in patients with acute anterior circulation large vessel occlusion accepted MT.

Objective    To investigate the operation of transcatheter aortic valve replacement (TAVR), the use of TAVR instruments and the current situation of TAVR-related nursing in our country, to reveal the characteristics of TAVR in various hospitals in our country, and to provide reference data for improving perioperative nursing and industry development of TAVR. Methods    A questionnaire survey was conducted among the head nurses of the cardiac catheterization laboratories of 51 hospitals in China that carried out TAVR operations, with a total of 5 items and 23 questions. The current situation of TAVR operation methods, intraoperative instruments and nursing care in China were analyzed. Results    The number of hospitals in China which started conducting TAVR and the beginning year were: 2 in 2010, 1 in 2012, 1 in 2013, 1 in 2015, 11 in 2016, 13 in 2017, 15 in 2018 and 7 in 2019; the number of transfemoral TAVR in 2019: 32 (62.75%) hospitals conducted on less than 20 patients, 7 (13.73%) hospitals 20-<50 patients, 6 (11.76%) hospitals 50-100 patients and 6 (11.76%) hospitals more than 100 patients; TAVR strategies adopted by most hospitals were: general anesthesia (90.20%), the use of vascular sealers (80.39%), backing by cardiac surgeon (74.51%) and using homemade prosthetic valves. Conclusion     At present, the number of TAVR carried out in Chinese hospitals is still far behind that of developed countries in Europe and the United States. Our country has adopted the form of multi-disciplinary cardiac team cooperation and formed a TAVR nursing model with Chinese characteristics.