Midterm Outcomes of Angioplasty for Pediatric Renovascular Hypertension

PurposeTo evaluate the midterm outcomes of percutaneous transluminal renal angioplasty (PTRA) for pediatric renovascular hypertension (RVH).Materials and MethodsThe clinical data of patients who underwent PTRA for RVH in the authors’ hospital from 2012 to 2019 were retrospectively analyzed. Postprocedural blood pressure, glomerular filtration rate (GFR) of the affected kidney, restenosis, and complications were closely monitored.ResultsPTRA was performed in a total of 30 children (20 boys and 10 girls), with a mean age of 7.3 years ± 0.7 (range, 40 days to 13.9 years) and a mean weight of 25.0 kg ± 2.3 (range, 3.4–53 kg). The median follow-up period was 26.5 months (range, 1 month to 7.5 years). Technical success was achieved in 26 (86.7%) of the 30 patients. Restenosis developed in 3 patients (10.0%). Only 1 patient underwent stent implantation, and the stent fractured 8 months later, requiring further intervention. There were no other complications. In terms of clinical benefit of blood pressure control after the initial PTRA procedure, 15 patients (50%) were cured and 7 patients (23.3%) showed improvement. There was no significant difference in the etiology, lesion location, and lesion length between patients with clinical benefit and failure (P = .06, P = .202, and P = .06, respectively). GFR of the affected kidney was significantly improved from 19.9 mL/min ± 11.2 to 38.1 mL/min ± 11.9 at the 6-month follow-up after PTRA (P < .001).ConclusionsThe overall results of PTRA for pediatric RVH caused by different etiologies are promising. PTRA not only provided a clinical benefit of blood pressure control in 73.3% of the patients but also significantly improved the function of the affected kidney.     

双水平气道正压对OSAHS患者预后及血管内皮功能的影响

目的 分析双水平气道正压双水平气道正压通气(bi-level positive airway pressure,BiPAP)对阻塞性睡眠呼吸暂停低通气综合征(obstructive sleep apnea hypopnea syndrome,OSAHS)患者血管内皮功能及预后的影响。 方法 将100例OSAHS患者纳入研究，随机分为观察及对照组，各50例。观察组行BiPAP ，对照组行常规治疗，比较两组多导睡眠监测(polysomnography，PSG)检查指标（低通气指数、呼吸暂停指数、呼吸暂停及低通气时间、90%以下血氧饱和度次数、90%以下血氧饱和度时间）、心肺功能［脑钠肽(brainnatriureticpeptide,BNP)、右心室Tei指数、左心室Tei指数、一秒用力呼气容积占用力肺活量的百分比(forced expiratory volume in one second/forced vital capacity，FEV1/FVC)、一秒用力呼气容积占预计值的百分比(FEV1占预计值%)］、血管内皮功能［内皮素1(endothelin-1,ET-1)、一氧化氮(nitric oxide,NO)、凝血酶Ⅲ(antithrombin Ⅲ,AT-Ⅲ)］及生活质量。 结果 观察组低通气指数、呼吸暂停指数、呼吸暂停及低通气时间、90%以下血氧饱和度次数及时间指标均显著低于对照组（P＜0.05）。观察组治疗后BNP以及Tei指数显著低于治疗前及对照组治疗后，FEV1/FVC、FEV1占预计值%显著高于治疗前及对照组治疗后(P＜0.05)。观察组治疗后ET-1、AT-Ⅲ低于治疗前及对照组治疗后，NO高于治疗前及对照组治疗后（P＜0.05）。观察组治疗后生活质量各项目评分高于对照组（P＜0.05）。 结论 BiPAP治疗能够改善OSAHS患者的预后情况及血管内皮功能，效果确切。     

主动脉出口血压对左心室血流特性的影响

采用Mimics对心脏进行三维重构，用3-matic进行模型优化以及模型误差分析，对左心室室壁运动做速度分布假设，基于UDF宏文件对左心室室壁运动编写程序，将血液视为非牛顿流体，采用动网格技术研究不同血压对左心室血液流动的影响。模拟发现当左心室收缩时，压力梯度明显，内部压力减小。当左心室舒张时，内部压力逐渐增高。二尖瓣口处的速率先增大后减小。血压升高，左心室内剪切应力持续增大，极易破坏红细胞结构，产生溶血现象，导致心脏功能紊乱。     

Left ventricular noncompaction associated with a pathogenic mutation in the MYH7 gene: Known mutation,different phenotype

Left ventricular noncompaction (LVNC) is a genetically heterogeneous cardiomyopathy, with familial and sporadic forms, but genetic testing only identifies a pathogenic mutation in a minority of cases. The main complications are heart failure, embolism and dysrhythmias. Herein we report a familial case of LVNC associated with a mutation in the MYH7 gene and review the literature regarding controversies in LVNC. A 50-year-old woman was referred to the cardiology clinic for palpitations. She underwent echocardiography and cardiac magnetic resonance imaging that revealed mild left ventricular systolic dysfunction and LVNC criteria. She had several episodes of non-sustained ventricular tachycardia and received an implantable cardioverter-defibrillator (ICD). Genetic testing revealed the c.1003G>C (p.Ala335Pro) mutation in the MYH7 gene. Familial screening showed clear genotype-phenotype cosegregation, which provided strong evidence for the pathogenic role of this mutation. To the best of our knowledge, this is the first report of LVNC associated with the p.Ala335Pro mutation in the MYH7 gene. This mutation has been described in hypertrophic cardiomyopathy, suggesting that the same pathogenic sarcomere mutation may be associated with different cardiomyopathies. This case also highlights the current difficulties regarding decisions on ICD implantation for primary prevention of sudden cardiac death in LVNC.