改良Nikaidoh手术治疗合并肺动脉瓣狭窄的右室双出口

目的：总结改良Nikaidoh手术治疗右心室双出口（DORV）患者的临床经验,以提高手术疗效。方法：2例先天性心脏病右心室双出口伴肺动脉瓣狭窄行改良Nikaidoh手术,游离主动脉根部及冠状动脉,重建左心室流出道,以带单瓣牛心包片补片重建肺动脉及右心室流出道。结果：术后患者紫绀消失,复查心脏彩超仅有轻度肺动脉瓣关闭不全,未发现左、右心室流出道梗阻,康复出院。结论：采用改良Nikaidoh手术治疗伴肺动脉瓣狭窄的右室双出口,术后可获得良好的血流动力学效果,早期临床结果满意。     

应用同种带瓣管道重建右室流出道的危险因素分析

目的:评价采用同种带瓣管道行右室流出道重建术的临床效果,探讨影响手术效果及临床预后的因素。方法:回顾2002年11月至2010年11月期间应用同种带瓣管道行右室流出道重建患者的临床资料,分析患者手术前后的一般信息、血流动力学表现与临床预后的关系。结果:行右室流出道重建术后49例痊愈出院,5例死亡,存活率90.7%,死亡率9.3%。手术前后比较右室流出道内径较术前明显增加,右室-左室收缩压比值、右室-肺动脉压差较术前明显降低,三尖瓣反流、肺动脉瓣反流较术前加重,肺动脉瓣狭窄较术前减轻。统计分析表明患者死亡的危险因素有术后右室平均压、术后肺动脉-主动脉收缩压比值、术后二尖瓣反流。术后心胸比、术后肺动脉收缩压、术后肺动脉-主动脉收缩压比值、术后三尖瓣反流可能和术后患者ICU时间延长有关。McGoon指数、术后心胸比、术后肺动脉收缩压、术后右室平均压、术后肺动脉-主动脉收缩压比值、合并动脉导管未闭、术后三尖瓣反流可能和术后患者呼吸机时间延长有关。结论:复杂先天性心脏病患者采用同种带瓣管道重建右室流出道可以取得较满意的临床效果,术后流出道梗阻矫正满意,可以防止肺动脉返流导致的心脏损害。     

应用同种带瓣管道重建右室流出道的危险因素分析

目的：评价采用同种带瓣管道行右室流出道重建术的临床效果,探讨影响手术效果及临床预后的因素。方法：回顾2002年11月至2010年11月期间应用同种带瓣管道行右室流出道重建患者的临床资料,分析患者手术前后的一般信息、血流动力学表现与临床预后的关系。结果：行右室流出道重建术后49例痊愈出院,5例死亡,存活率90．7％,死亡率9-3％。手术前后比较右室流出道内径较术前明显增加,右室一左室收缩压比值、右室-肺动脉压差较术前明显降低,三尖瓣反流、肺动脉瓣反流较术前加重,肺动脉瓣狭窄较术前减轻。统计分析表明患者死亡的危险因素有术后右室平均压、术后肺动脉-主动脉收缩压比值、术后二尖瓣反流。术后心胸比、术后肺动脉收缩压、术后肺动脉一主动脉收缩压比值、术后三尖瓣反流可能和术后患者ICU时间延长有关。McGoon指数、术后心胸比、术后肺动脉收缩压、术后右室平均压、术后肺动脉一主动脉收缩压比值、合并动脉导管未闭、术后三尖瓣反流可能和术后患者呼吸机时间延长有关。结论：复杂先天性心脏病患者采用同种带瓣管道重建右室流出道可以取得较满意的临床效果,术后流出道梗阻矫正满意,可以防止肺动脉返流导致的心脏损害。     

特发性右心室流出道室性心律失常射频消融术后复发因素分析

目的:探讨特发性右心室流出道室性心律失常射频消融术后,患者室性心律失常复发的原因,旨在为进一步降低复发率提供线索。方法:1999年12月至2009年12月,在解放军总医院老年心血管内科住院行导管射频消融的特发性右心室流出道室性心律失常患者共145例(男55例,女90例),治疗终点为室性心律失常消失,不能被心室电刺激和静滴盐酸异丙肾上腺素诱发,术后1天复查动态心电图并电话随访观察疗效。结果:在145例患者中,即刻成功136例,成功率为93.8%。随访23.8±6.7月,共有9例患者复发,复发率为6.62%。9例复发患者再次行射频消融术的靶点局部激动(34.0±7.6 ms)明显早于第一次射频消融术(30.4±8.5 ms)(P<0.05);靶点起搏与自发心律失常体表心电图QRS波形的符合数(11.8±0.45)大于第一次射频消融术(11.1±0.78)(P<0.05);复发患者第一次手术在最早激动点处单极标测r波的出现比例大于第二次手术(P<0.05),再次手术均成功。结论:导管射频消融治疗特发性右心室流出道室性心律失常是有效、可行的方法。靶点标测欠精确是术后复发的主要原因。     

右室流出道室性心动过速的发生机制研究进展

右心室流出道室性心律失常是临床上常见的特发性心律失常,占特发性室速的60%~70%,绝大多数右心室流出道室速为腺苷敏感性,其发病机制为儿茶酚胺介导的延迟后除极和触发活动。其发生机制一直是电生理领域研究的热点问题,新近研究表明,L型钙通道的改变与特发性右心室流出道室性心动过速的发生密切相关,提示L型钙通道可能会成为特发性右心室流出道室性心动过速治疗的新靶点。     

矫正型大动脉转位合并心内畸形的外科治疗

目的：为探讨矫正型大动脉转位的病理解剖特点及手术技术。方法：本组6例均为SLL型,手术包括：室间隔缺损修补4例、肺动脉瓣切开1例、静脉室肺动脉外通道1例、房室瓣替换1例。结果：全组手术死亡1例。主要手术并发症为低心排4例、完全性房室传导阻滞1例及残余左房室瓣关闭不全1例。结论：矫正型大动脉转位的病理解剖有一定的特殊性,应按不同的合并畸型选择不同术式,正确处理室间隔缺损、肺动脉流出道狭窄及左房室瓣关闭不全是外科手术的关键。     

改良konno手术治疗肥厚梗阻性心肌病6例及文献复习

目的:总结改良Konno手术治疗6例肥厚型梗阻型心肌病及文献复习。方法:回顾性分析2003年8月至2009年1月我院6例肥厚型梗阻性心肌病,所有病人胸闷、心悸、心前区疼痛、头晕、晕厥。全部接收改良konno手术,2例同时进行二尖瓣成形术,主动脉阻断时间30～63分钟,平均为47.6分钟。体外循环时间45～96分钟,平均为74.5分钟。结果:手术顺利,无并发症出现,病人康复,平均随访2月～5年,左室流出道压差均小于20mmHg(p0.01),症状缓解。结论:改良Konno手术能够彻底解除左室流出道梗阻,并发症较少,仍然是肥厚型梗阻性心肌病的最佳治疗方法之一。     

乳腺癌保乳改良根治术与传统改良根治术临床疗效的比较

目的:对比分析保乳改良根治术与传统改良根治术治疗乳腺癌的临床疗效。方法:将行手术治疗的415例女性乳腺癌患者按手术方式分为保乳改良根治术组76例与传统改良根治术组339例,两组患者术后均接受辅助治疗。比较两组患者术后满意度,术后并发症,复发、转移及死亡率,评价保乳改良根治术的临床疗效。结果:两组患者的发病年龄,术后病理类型,失随访情况,转移、死亡及复发率比较无统计学意义(P0.05)。保乳改良根治术组皮下积液发生率明显低于传统改良根治术组(P0.05),术后满意度明显优于传统改良根治术组(P0.05),其他并发症比较无显著差异(P0.05)。结论:保乳改良根治术能得到和传统改良根治术同样的疗效,且具有损伤小、并发症少、外形美观、术后满意度高等优势。     

高原与平原肺心病右室功能改变的比较研究

目的：检测不同海拔高度肺心病患者右心室肥厚和血清ET水平变化的相关性．方法：肺心病患者高原组和平原组,采用彩色多普勒超声心动图对右心室前壁厚度、右室流出道宽度等指标进行测量比较．血清ET-1含量利用放射免疫技术进行测定。结果：高原组肺动脉瓣a消失、右室前壁增厚和左右肺动脉宽度出现频率显著高于平原组（P〈0．01）;右室流出道增宽、室间隔增宽率高原组高于平原组（P〈0．05）;平均肺动脉压也高于平原组（P〈0．05）;另外,高原组血清ET-1含量也显著高于平原组,并与肺动脉压存在一定的正相关。结论：高原肺心病右心病变较平原组严重,可能与其有更重的低氧血症及ET-1合成增多有关。     

改良后路腰椎体间融合术治疗腰椎管狭窄症的临床观察

目的:研究改良后路腰椎体间融合术(PLIF)治疗腰椎管狭窄症的临床疗效。方法:选取2010年7月到2014年7月我院收治的腰椎管狭窄症患者140例,按照随机数字表法将患者分为研究组和对照组,每组70例。研究组给予改良PLIF治疗,对照组给予传统PLIF治疗,比较两组手术时间、术中出血量、住院时间、术后下地时间,应用视觉模拟评分(VAS)评价术前、术后3个月、术后1年腰痛程度,应用Oswestry生活功能障碍指数(ODI)评分评价患者术前和术后1年的生活功能情况,并比较两组并发症的发生率。结果:两组手术时间、术中出血量比较无统计学意义(P0.05),研究组住院时间、术后下地时间显著短于对照组,差异具有统计学意义(P0.05);研究组术后3个月和术后1年VAS评分均显著低于对照组,差异具有统计学意义(P0.05);研究组术后1年ODI评分显著低于对照组,差异具有统计学意义(P0.05);研究组术后并发症发生率显著低于对照组,差异具有统计学意义(P0.05)。结论:改良PLIF治疗腰椎管狭窄症效果较PLIF好,能有效改善疼痛情况和日常生活,降低并发症的发生率。     

The Hemodynamic Response of the Right Ventricle Following Acute and Chronic Partial Obstruction of the Main Pulmonary Artery in Dogs

In eight adult dogs the main pulmonary artery was constricted to elevate the right ventricular peak systolic pressure to 50% of the peak aortic pressure at rest. The response of the right ventricle was assessed immediately, at 30 minutes and at six months. The right ventricle responded to acute systolic loading by complete compensation. After 30 minutes there was a reduction in the right ventricular outflow tract resistance. The cardiac output, heart rate and aortic pressure were maintained. The right ventricular systolic ejection period, end-diastolic pressure, peak pressure time, mean systolic pressure, right ventricular—main pulmonary artery mean systolic gradient, right ventricular work index, systolic work and outflow tract resistance were all increased.The right ventricle in the dog was shown to have an immediate capacity to compensate for systolic loading and retains this capacity for long periods of time. The ability to increase work is accomplished by adaptations in right ventricular physiology which increase right ventricular mean systolic pressures and prolong the right ventricular ejection period.     

Implantation of the Right and Left Internal Mammary Arteries with Epicardiectomy and Free Omental Graft: A Preliminary Experimental Report

In animal experiments the right internal mammary artery was implanted into the outflow tract of the right ventricle during performance of left mammary artery implantation with epicardiectomy and free omental graft. The effect of double mammary artery implantation operation was tested by triple coronary artery ameroid constriction in 24 animals. In 20 animals studied both implanted arteries remained open and had commenced to bud and branch at the time of examination. The double implant operation is still an experimental procedure.     

兔右室流出道室速对L型钙通道α1c蛋白表达的影响

目的：通过建立右室流出道室速（RVOT-VT）的动物模型,以L型钙通道α1c蛋白作为观察指标,观察RVOT-VT时对L型钙通道α1c蛋白表达的影响,旨在探讨L型钙通道在RVOT-VT中的作用。方法：健康新西兰大耳白兔30只,随机分三组,分别为对照组（10只）、室速组（10只）、室速加维拉帕米干预组（10只）。采用免疫组织化学的方法对三组实验动物的右室流出道心肌组织进行L型钙通道cdc蛋白表达的检测。结果：1、高频刺激主动脉与肺动脉交界处均诱发了起源于右室流出道部位的室速,且室速持续时间均大于4小时。2、室速组L型钙通道α1c蛋白表达量明显下降;干预组L型钙通道α1cc蛋白的表达下降,但与对照组比较无显著差异。结论：1、室速组的心肌L型钙通道α1c蛋白表达发生了重构。2、维拉帕米可以改善心肌L型钙通道α1c蛋白的重构。3、L型钙通道在RVOT-VT发生、持续中起重要作用。     

Ablation of the secondary heart field leads to tetralogy of Fallot and pulmonary atresia

Recent studies in chick and mouse embryos have identified a previously unrecognized secondary heart field (SHF), located in the ventral midline splanchnic mesenchyme, which provides additional myocardial cells to the outflow tract as the heart tube lengthens during cardiac looping. In order to further delineate the contribution of this secondary myocardium to outflow development, we labeled the right SHF of Hamburger-Hamilton (HH) stage 14 chick embryos via microinjection of DiI/rhodamine and followed the fluorescently labeled cells over a 96-h time period. These experiments confirmed the movement of the SHF into the outflow and its spiraling migration distally, with the right side of the SHF contributing to the left side of the outflow. In contrast, when the right SHF was labeled at HH18, the fluorescence was limited to the caudal wall of the lengthening aortic sac. We then injected a combination of DiI and neutral red dye, and ablated the SHF in HH14 or 18 chick embryos. Embryos were allowed to develop until day 9, and harvested for assessment of outflow alignment. Of the embryos ablated at HH14, 76% demonstrated cardiac defects including overriding aorta and pulmonary atresia, while none of the sham-operated controls were affected. In addition, the more severely affected embryos demonstrated coronary artery anomalies. The embryos ablated at HH18 also manifested coronary artery anomalies but maintained normal outflow alignment. Therefore, the myocardium added to the outflow by the SHF at earlier stages is required for the elongation and appropriate alignment of the outflow tract. However, at later stages, the SHF contributes to the smooth muscle component of the outflow vessels above the pulmonary and aortic valves which is important for the development of the coronary artery stems. This work suggests a role for the SHF in a subset of congenital heart defects that have overriding aorta and coronary artery anomalies, such as tetralogy of Fallot and double outlet right ventricle.     

Chromosome 22q11 deletions in patients with selected outflow tract malformations

The incidence of 22q11 deletions and its effect on the phenotype were established in 170 patients with selected outflow tract malformations and transposition of the great arteries (conotruncal defects). Cases were seen both prospectively and retrospectively. All patients had a dysmorphological evaluation by the clinical geneticist and a cytogenetic analysis including FISH analysis for 22q11 deletions. A chromosomal abnormality was present in 29 patients, including a 22q11 deletion in 22/170 patients (13%). The 22q11 deletion was found in 11% of tetralogy of Fallot, in 11% of pulmonary atresia and VSD, in 44% of pulmonary atresia. VSD and collateral arteries, in 20% of truncus arteriosus, in 60% of interrupted aortic arch and in 25% patients with aberrant subclavian artery. They were absent in double outlet right ventricle or in transposition of the great arteries. No parental deletion was found. All patients had clinical characteristics of the velocardiofacial syndrome. This study confirms a high incidence of chromosome 22q11 deletions in patients with selected outflow tract malformations, with great clinical impact for further management and genetic counseling.     

Myocardial deletion of Smad4 using a novel α skeletal muscle actin Cre recombinase transgenic mouse causes misalignment of the cardiac outflow tract

SMAD4 acts as the converging point for TGFβ and BMP signaling in heart development. Here, we investigated the role of SMAD4 in heart development using a novel α skeletal muscle actin Cre recombinase (MuCre) transgenic mouse strain. Lineage tracing using MuCre/ROSA26^LacZ reporter mice indicated strong Cre-recombinase expression in developing and adult heart and skeletal muscles. In heart development, significant MuCre expression was noted at E11.5 in the atrial, ventricular, outflow tract and atrioventricular canal myocardium, but not in the endocardial cushions. MuCre-driven conditional deletion of Smad4 in mice caused double outlet right ventricle (DORV), ventricular septal defect (VSD), impaired trabeculation and thinning of ventricular myocardium, and mid-gestational embryonic lethality. In conclusion, MuCre mice effectively delete genes in both heart and skeletal muscles, thus enabling the discovery that myocardial Smad4 deletion causes misalignment of the outflow tract and DORV.     

Misexpression of noggin leads to septal defects in the outflow tract of the chick heart.

BMP-2 and BMP-4 are known to be involved in the early events which specify the cardiac lineage. Their later patterns of expression in the developing mouse and chick heart, in the myocardium overlying the atrioventricular canal (AV) and outflow tract (OFT) cushions, also suggest that they may play a role in valvoseptal development. In this study, we have used a recombinant retrovirus expressing noggin to inhibit the function of BMP-2/4 in the developing chick heart. This procedure resulted in abnormal development of the OFT and the ventricular septum. A spectrum of abnormalities was seen ranging from common arterial trunk to double outlet right ventricle. In hearts infected with noggin virus, where the neural crest cells have been labelled, the results show that BMP-2/4 function is required for the migration of neural crest cells into the developing OFT to form the aortopulmonary septum. Prior to septation, misexpression of noggin also leads to a decrease in the number of proliferating mesenchymal cells within the proximal cushions of the outflow tract. These results suggest that BMP-2/4 function may mediate several key events during cardiac development.