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Paul J. Devlin Brian W. McCrindle James K. Kirklin Eugene H. Blackstone William M. DeCampli Christopher A. Caldarone Ali Dodge-Khatami Pirooz Eghtesady James M. Meza Peter J. Gruber Kristine J. Guleserian Bahaaladin Alsoufi Linda M. Lambert James E. OBrien Erle H. Austin Jeffrey P. Jacobs Tara Karamlou 《The Journal of thoracic and cardiovascular surgery》2019,157(2):684-695.e8
Objective
Arch obstruction after the Norwood procedure is common and contributes to mortality. We determined the prevalence, associated factors, and practice variability of arch reintervention and assessed whether arch reintervention is associated with mortality.Methods
From 2005 to 2017, 593 neonates in the Congenital Heart Surgeons' Society Critical Left Heart Obstruction cohort underwent a Norwood procedure. Median follow-up was 3.7 years. Multivariable parametric models, including a modulated renewal analysis, were performed.Results
Of the 593 neonates, 146 (25%) underwent 218 reinterventions for arch obstruction after the Norwood procedure: catheter-based (n = 168) or surgical (n = 50) at a median age of 4.3 months (quartile 1-quartile 3, 2.6-5.7). Interdigitation of the distal aortic anastomosis was protective against arch reintervention. Development of ≥ moderate tricuspid valve regurgitation and right ventricular dysfunction at any point was associated with arch reintervention. Nonsignificant variables for arch reintervention included shunt type and preoperative aortic measurements. Surgical arch reintervention was protective against arch reintervention, but transcatheter reintervention was associated with increased reintervention. Arch reintervention was not associated with increased mortality. There was wide institutional variation in incidence of arch reintervention (range, 0-40 reinterventions per 100 years patient follow-up) and in preintervention gradient (range, 0-64 mm Hg).Conclusions
Interdigitation of the distal aortic anastomosis during the Norwood procedure decreased the risk of arch reintervention. Surgical arch reintervention is more definitive than transcatheter. Arch reintervention after the Norwood procedure is not associated with increased mortality. Serial surveillance for arch obstruction, integrated with changes in right ventricular function and tricuspid valve regurgitation, is recommended after the Norwood procedure to improve outcomes. 相似文献45.
Sadanandavalli Retnaswami Chandra Pawan Raj Thomas Gregor Issac 《Indian journal of dermatology》2015,60(3):290-292
Neurodegeneration with brain iron accumulation (NBIA) is the term applied to a heterogeneous group of disorders resulting in iron deposition in the basal ganglia. Well-known phenotypic features are progressive regression with extra pyramidal involvement and a variable course. A 10-year-old child born to consanguineous parents presented with progressive generalized opisthotonic dystonia, retrocollis, oromandibular dyskinesias, apraxia for swallowing, optic atrophy and severe self-mutilation of lips. MR imaging showed brain iron accumulation. Other causes of self-mutilation were excluded. Early infantile onset, ophisthotonic dystonia with oromandibular dyskinesias and characteristic MR images are suggestive of NBIA. There is only one case reported in the literature of self-mutilation in this condition. 相似文献
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《Drug metabolism and pharmacokinetics》2020,35(6):563-570
Minesapride is a novel 5-hydroxytryptamine 4 (5-HT4) receptor partial agonist that is expected to show efficacy in patients with irritable bowel syndrome with predominant constipation and functional constipation. An open-label study was conducted to evaluate pharmacokinetics (PK) and safety of minesapride. Japanese subjects, 12 elderly and 12 young, received a single oral dose of minesapride 40 mg/day in the fasted state. Metabolite profiles were also investigated in this clinical study and in an in vitro study using cryopreserved hepatocytes. Clinical results showed that minesapride was rapidly absorbed (Cmax: 2302.1 ng/mL in the elderly group, 2117.5 ng/mL in the young group), and the plasma concentration then decreased with half-life of approximately 7 h. There were no notable PK differences between elderly and young groups. No serious adverse events (AEs) were observed. The only AE that occurred in 2 or more subjects was diarrhea. Metabolite profiles in plasma and urine were similar between elderly and young groups. No major metabolites exceeded 10% of unchanged minesapride, and results of the in vitro study suggested that there were no human-specific metabolites. From the viewpoints of PK and metabolite profiling, no dose adjustment of minesapride is warranted in elderly population without renal or hepatic impairment. 相似文献
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目的:探讨益气通脉饮治疗气虚血瘀型冠心病心绞痛的临床疗效。方法:选取2017年10月—2019年2月烟台业达医院收治的气虚血瘀型冠心病心绞痛的患者90例作为研究对象,根据随机数字表法将所选患者均分为两组,每组各45例,两组的一般资料经统计学方法,差异无统计学意义(P>0.05),因此具有可比性。对照组给予常规西药进行治疗,观察组在对照组治疗基础上给予益气通脉饮治疗,1个月后比较两组临床的疗效,评估治疗前后中医证候、心绞痛症状分级和血脂水平。临床检测的数据通过统计产品与服务解决方案(Statistical Product and Service Solutions,SPSS) 19.0软件进行分析和处理,计量的数据使用平均数±标准差(x±s)表示,采用双侧t检验,计数的数据采用χ~2检验,以%表示,以P<0.05为差异有统计学意义。结果:治疗后,对照组和观察组都有一定疗效,但观察组的改善中医证候总有效率95.56%(43/45),高于对照组的82.22%(37/45),两组比较具有统计学意义(P<0.05);心绞痛的症状总有效率观察组为93.33%(42/45),对照组为77.78%(35/45),两组比较差异有统计学意义(P<0.05);与治疗前相比较,两组治疗后的三酰甘油(Triglyceride,TG)、血清总胆固醇(Serum Total Cholesterol,TC)、高密度脂蛋白胆固醇(High Density Liptein Cholesterol,HDL-C)水平均降低(P<0.05),且观察组治疗后低于对照组(P<0.05),两组治疗后的HDL-C升高(P<0.05),组间比较,差异无统计学意义(P>0.05)。结论:益气通脉饮治疗气虚血瘀型冠心病心绞痛的疗效显著,值得临床进一步研究与推广应用。 相似文献
49.
冠状病毒(coronavirus,CoVs)感染主要累及肺部,但对心血管系统损伤作用也不容忽视。CoVs感染引起的心脏损伤并非罕见,其发生与病情的严重程度密切相关。本文首先从CoVs引起心血管损伤的证据入手,进一步探讨了CoVs对心肌的直接损伤,以及肾素血管紧张素(RAS)系统激活和细胞因子风暴与炎症反应对心血管损伤的可能作用机制。相关心血管损伤的可能机制包括,(1)病毒直接作用:CoVs在心肌细胞复制,损伤心肌;(2)RAS系统激活:感染CoVs后,心脏血管紧张素转化酶2(angiotensin-converting enzyme 2,ACE2)的表达下调,激活RAS系统,使得血管紧张素Ⅱ(AngiotensinⅡ,AngⅡ)收缩血管功能增强,Ang1-7保护心脏效应减弱;(3)诱发细胞因子风暴:循环细胞因子和全身炎症反应引起心脏损伤;(4)其他:包括低氧血症和儿茶酚胺心脏毒性。本文就相关内容作一综述,为后续的详尽机制和治疗策略研究提供思路。 相似文献
50.
Moises Rodriguez-Gonzalez Antonio Moruno Tirado Reza Hosseinpour Jose Santos de Soto 《Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital》2015,42(4):350-356
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo–8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic. 相似文献