36例急性StanfordA型主动脉夹层动脉瘤的外科治疗体会

目的：总结急性StanfordA型主动脉夹层动脉瘤的外科治疗体会。方法：选择36例急性StanfordA型主动脉夹层动脉瘤患者,根据病变夹层破口的位置、累及范围、有无合并主动瓣关闭不全选择相应的手术方式,观察患者治疗后的临床疗效并随访治疗6个月后的临床预后。结果：3例患者术中死亡,术中死亡率为8．3％,平均手术时间156．7±56．7min,平均阻断时间98．5±32．7min,平均选择性脑灌注时1．756．1±20．7min,术后平均ICU住院时间6．2±3．8d,术后平均总计住院时间25．4±7．3d;术后6个月后随访．3例患者死亡,其余30例患者恢复满意,生活质量与治疗前相比,均得到显著提高。结论：急性Stanford A型主动脉夹层动脉瘤患者应积极施行早期外科治疗,术前快速准确诊断,根据患者病情制定相应的手术方案,术后采取措施避免术后并发症。     

阴茎阴囊转位合并尿道下裂分期手术修复（附43 例报道）

目的：探讨阴茎阴囊转位合并尿道下裂的手术方法及分期手术修复的临床意义。方法：回顾性分析2005年1 月至2012 年 6 月间兰州军区兰州总医院收治的43 例阴茎阴囊转位伴尿道下裂的病例资料并分析手术方式及术后随访外观情况。结果：43 例 患者经2 期阴囊成形术后疗效满意,其中2 例伴严重尿道下裂患者经分手术后达到预期效果,术后随访6 个月至7 年。所有患者 在阴茎阴囊复位后经同期或分期尿道成形术后最终均达到尿道下裂修复的标准。结论：阴茎阴囊转位合并尿道下裂应及早手术 矫正治疗。分期手术方法使操作简化,阴茎阴囊复位整形效果满意,最终尿道成形术后预后良好,术后并发症少,是一种安全可行 的手术方式,但性器官发育后易复发,术后要随访至青春期以后。     

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。     

马凡综合征合并急性StandfordA型夹层的外科治疗

目的：评价外科治疗马凡综合征合并急性StandfordA型夹层的效果。方法：回顾性分析2007年7月至2010年7月外科治疗12例马凡综合征合并急性StandfordA型夹层病例的临床资料。采用改良Mini—root手术方式代替Bentall手术。结果：主动脉阻断时间69～103min,平均（78．7±28．6）min,体外循环时间79～122min,平均（98．3±23．8）min。术后早期存活11例,死亡1例,死亡率8.3％。术后随访时间2～37个月,平均（18．2±8．6）个月,其中1例术后24月发生急性腹主动脉夹层,予以实施腔内支架隔绝术成功以治愈出院。术后与术前左心室舒张末径分别为45～58mm,平均（50.2±5．6）mnq和53～69mm,平均（61．3±4．6）mm（P〈0．01）。结论：马凡综合征合并急性Standford A型夹层一经确诊则需急诊手术,及时的外科手术是治疗该病的有效方法,而且应用改良Mini—root手术方式疗效满意。     

21例胫骨近端骨肉瘤保肢治疗临床分析

目的：探讨胫骨上端骨肉瘤保肢手术中使用人工关节重建的疗效和并发症。方法：回顾性分析2000年6月-2010年6月对21例胫骨上段骨肉瘤患者行根治性切除人工关节重建手术的临床资料,其中男性13例,女性8例,年龄21—62岁,平均34．1岁。依据Enneking分期：ⅡA期12例、ⅡB期9例。均采用广泛性边缘切除手术治疗。所有患者均接受术前、术后化疗。结果：1例患者发生术后胫前肌群坏死。所有患者均随访6—72个月,平均45．1个月。ⅡA期12例患者中,1例因肺转移死亡（占8．3％）,局部复发1例,行股骨下段截肢,10例无病生存;ⅡB期9例,4例死亡,5例存活;Enneking分期明显影响预后。结论：胫骨上段人工关节重建对胫骨上段骨肉瘤的保肢治疗具有良好的疗效。     

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。     

阴茎阴囊转位合并尿道下裂分期手术修复（附43例报道）

目的：探讨阴茎阴囊转位合并尿道下裂的手术方法及分期手术修复的临床意义。方法：回顾性分析2005年1月至2012年6月间兰州军区兰州总医院收治的43例阴茎阴囊转位伴尿道下裂的病例资料并分析手术方式及术后随访外观情况。结果：43例患者经2期阴囊成形术后疗效满意,其中2例伴严重尿道下裂患者经分手术后达到预期效果,术后随访6个月至7年。所有患者在阴茎阴囊复位后经同期或分期尿道成形术后最终均达到尿道下裂修复的标准。结论：阴茎阴囊转位合并尿道下裂应及早手术矫正治疗。分期手术方法使操作简化,阴茎阴囊复位整形效果满意,最终尿道成形术后预后良好,术后并发症少,是一种安全可行的手术方式,但性器官发育后易复发,术后要随访至青春期以后。     

原发性肝癌合并下腔静脉癌栓的手术治疗

目的：探讨原发性肝癌合并下腔静脉癌栓的手术治疗方法。方法：采用肝切除加下腔静脉取栓治疗2例肝癌合并下腔静脉癌栓患者,取栓方法包括经荷栓肝静脉取栓（1例）和下腔静脉切开取栓（1例）,又分在全肝血流阻断下取栓和在萨氏钳局部血管阻断下取栓。结果：2例肝癌及下腔静脉癌栓均得到成功切除,术中无明显并发症发生;术后无死亡;随访中一例存活11月;另1例已生存8个月。结论：肝癌合并下腔静脉癌栓的手术治疗安全可行,其基本术式为肝切除加下腔静脉切开取栓。     

阿托伐他汀对慢性阻塞性肺疾病合并肺动脉高压患者外周血ROCK2激酶活性及肺动脉压力的影响

目的：探讨口服阿托伐他汀片对慢性阻塞性肺疾病（chronic obstructive pulmonary Disease,COPD）合并肺动脉高压（pul-monary hypertension,PH）患者外周血ROCK2激酶活性及肺动脉压力的影响。方法：选取COPD合并PH患者60例为研究对象,并将其随机分为对照组（给予吸氧、抗感染、化痰、平喘等基础治疗）和阿托伐他汀治疗组（在基础治疗的基础上给予阿托伐他汀片20mg／d治疗）;随访观察周期12周,于试验开始前和结束后检测外周血ROCK2的活性,利用彩色多普勒检测肺动脉压力的变化,肺功能变化（测定FEV1,FVC）。结果：与对照组比较,阿托伐他汀治疗可显著降低患者血浆中ROCK2的水平（P〈0．01）;降低患者的肺动脉压力,改善患者的肺功能（FEV1,FVC）,P均〈0．05。结论：在常规吸氧、抗感染等治疗的基础上,联合应用阿托伐他汀可显著降低ROCK2激酶的活性和肺动脉压力,从而改善肺功能。     

可逆性肺动脉高压的治疗在心脏移植围术期的应用

目的：通过早期判断并治疗心脏移植围术期可逆性肺动脉高压,降低移植手术后右心功能衰竭的发生率。方法：20例接受心脏移植手术的病人,术前放置肺动脉导管,测定肺动脉压、肺循环阻力。对肺动脉高压的病人在肺动脉端泵入硝酸甘油、前列腺素E1以确定可逆性。并在术后早期抗排异治疗的基础上应用增强心肌收缩力、降低肺动脉压力、强化氧疗和呼吸管理等综合措施。结果：20例病人中6例出现急性右心功能衰竭,其中4例经治疗后症状改善、出院,2例死亡。结论：术前早期判断并治疗可逆性肺动脉高压,可以有效预防并减少心脏移植术后右心功能衰竭的发生,提高手术成功率。     

Pregnancy after surgical correction of tetralogy of Fallot.

Forty pregnancies have been documented in 27 patients with surgically corrected tetralogy of Fallot. Infertility was uncommon and there were no premature births and few abortions or small-for-dates babies; this suggests that surgery that corrects cyanosis improves the outcome of pregnancy by correcting the fetal environment. Pregnancy was well tolerated and there were no serious cardiac complications. Thirty of the 31 infants examined were normal, the one abnormal infant having pulmonary atresia. A patient with no major residual defects after surgery for tetralogy of Fallot may be reassured that pregnancy will be well tolerated and that delivery may be managed in the normal manner.     

Per-operative stent placement in the right pulmonary artery; a hybrid technique for the management of pulmonary artery branch stenosis at the time of pulmonary valve replacement in adult Fallot patients

After having undergone surgical correction at an early age, many patients with tetralogy of Fallot develop long-term complications including progressive pulmonary regurgitation and peripheral pulmonary stenosis. A high percentage of these patients need to undergo a second operation in their adolescence or early adulthood. If simultaneous treatment of both pulmonary regurgitation and peripheral pulmonary stenosis is warranted, a complete surgical approach has several disadvantages. We describe four cases of Fallot patients with severe pulmonary regurgitation and peripheral pulmonary stenosis who were treated using a hybrid approach involving surgical implantation of a pulmonary homograft and peroperative stenting of the pulmonary artery.     

小儿法洛四联症根治术后氨基末端脑钠肽前体水平变化及其临床意义分析

目的：探讨小儿法洛四联症根治术后血浆氨基末端脑钠肽前体（N—terminal pro-brain natriuretic peptide,NT-proBNP）水平变化及其临床意义。方法：选择我院2011年10月～2013年4月收治的法洛四联症患儿52例,所有患儿行法洛四联症根治术治疗,并于术前、术后3h、12h、48h、1周、1个月和3个月测定患儿血浆NT-proBNP水平,应用心脏彩超机检查肺动脉跨瓣压差、右室舒张末期容积（rightventricularend．diastolicvolume,RVEDV）、左室射血分数（Leftventricular ejection fraction,LVEF）和右心Tei指数（Teiindex）。结果：①术后3hNT—proBNP水平开始逐渐升高,术后48h达最高,明显高于术前水平（P〈0．01）,术后1个月、3个月明显低于治疗前水平（P〈0．05）。②术后1周,右心功能不全组血浆NT．proBNP水平、肺动脉跨瓣压差、RVEDV明显高于右心功能正常组,LVEF明显低于右心功能正常组（P〈0．05）。③术后3个月,重度返流组、中度返流组患儿术后48h、1周、3个月血浆NT—proBNP明显高于轻度返流组（P〈0．05）;重度返流组患儿术后1周、3个月血浆NT—proBNP明显高于轻度返流组（P〈0．05）。结论：NT．proBNP在小儿法洛四联症根治术后早期的变化水平与右心功能的变化一致,可以作为评估右心功能的客观指标。     

Congenital heart disease cyanotic children

Cyanosis is often the only apparent symptom of congenital heart disease for which a child is brought to a physician. Some of the more common anomalies can be diagnosed from this and other symptoms by a general practitioner. Squatting after exertion is a sign of tetralogy of Fallot; severe disability with relatively mild cyanosis may indicate pure pulmonary stenosis. A brisk, short, rasping systolic murmur is characteristic of these conditions and of tricuspid atresia. Tetralogy of Fallot is further symptomatized by a boot-shaped heart, not greatly enlarged, and right axis deviation on electrocardiograms. Typically the lung fields are clear. The author's treatment of choice is aortic-pulmonary or subclavian-pulmonary anastomosis as indicated, preferably done after the child is three years old if the condition is not so severe as to require earlier operation. Pure pulmonary stenosis, which in some cases cannot be distinguished from tetralogy of Fallot except by cardiac catheterization and angiocardiography, may in more typical cases be diagnosed by convexity rather than concavity in the pulmonary segment and by differences in electrocardiograms. An expanding valvulotome is used to open the stenosed pulmonary valve, which is then dilated.A systolic murmur, a round heart and left axis deviation are usually found in tricuspid atresia. Shunt operations performed for relief of this condition may lead to later heart failure because of the devious rerouting of blood through the heart. The operations here outlined and others are statistically evaluated.     

乌司他丁对体外循环法洛四联症患儿围心脏手术期循环和呼吸功能的影响

目的:探讨乌司他丁对体外循环法洛四联症患儿围心脏手术期循环和呼吸功能的影响。方法:选取我院于收治的60例法洛四联症婴幼儿参与研究,并将其随机分为对照组和试验组两组,每组患儿30例。其中试验组患儿在体外循环前以及患儿进行手术后的三天内每天均给予10000U/kg乌司他丁,而对照组患儿则在相应的时间点给予等量的生理盐水。分析比较两组患儿的体外循环时间、心脏停搏时间、手术时间、在监护室治疗的时间以及患儿术后住院时间和患儿肺部感染发生例数等临床病理情况。结果:所有入选患儿均痊愈出院,在两组患儿的手术操作情况和治疗效果对比中,对照组患儿的手术时间、体外循环时间和心脏停搏时间均显著低于试验组患儿,但重症监护时间及术后住院时间则高于试验组患儿;在两组患儿治疗后的循环功能指标比较中,试验组患儿超滤后CVP和血管活性药物均低于对照组患儿而超滤后MAP则显著高于对照组患儿;在两组患儿治疗后的呼吸功能指标的比较中,试验组患儿的动脉血氧分压明显高于对照组患儿以及试验组患儿的术后机械通气时间和肺部感染例数均低于对照组患儿,两组患儿的数据比较差异除手术时间、体外循环时间和心脏停搏时间外均具有统计学意义(均P0.05)。结论:乌司他丁对体外循环法洛四联症患儿围心脏手术期的循环和呼吸功能具有较好的保护作用,值得在临床上加以广泛推广和运用。     

CONGENITAL HEART DISEASE IN CYANOTIC CHILDREN

Cyanosis is often the only apparent symptom of congenital heart disease for which a child is brought to a physician. Some of the more common anomalies can be diagnosed from this and other symptoms by a general practitioner. Squatting after exertion is a sign of tetralogy of Fallot; severe disability with relatively mild cyanosis may indicate pure pulmonary stenosis. A brisk, short, rasping systolic murmur is characteristic of these conditions and of tricuspid atresia.Tetralogy of Fallot is further symptomatized by a boot-shaped heart, not greatly enlarged, and right axis deviation on electrocardiograms. Typically the lung fields are clear. The author''s treatment of choice is aortic-pulmonary or subclavian-pulmonary anastomosis as indicated, preferably done after the child is three years old if the condition is not so severe as to require earlier operation.Pure pulmonary stenosis, which in some cases cannot be distinguished from tetralogy of Fallot except by cardiac catheterization and angiocardiography, may in more typical cases be diagnosed by convexity rather than concavity in the pulmonary segment and by differences in electrocardiograms. An expanding valvulotome is used to open the stenosed pulmonary valve, which is then dilated.A systolic murmur, a round heart and left axis deviation are usually found in tricuspid atresia. Shunt operations performed for relief of this condition may lead to later heart failure because of the devious rerouting of blood through the heart.The operations here outlined and others are statistically evaluated.     

Acquired left coronary artery fistula to right ventricular outflow tract

A 59-year-old asymptomatic male was referred to our hospital for evaluation 44 years after surgical correction of a Fallot’s tetralogy. Transthoracic echocardiography showed a good surgical result with only a mild subvalvular pulmonary stenosis and mild pulmonary regurgitation. However, in the parasternal short axis a diastolic colour Doppler flow was seen in the right ventricular outflow tract with a maximum velocity over 4 m/s (figure 1). Pulmonary regurgitation seemed very unlikely because of the high velocity in the absence of elevated pulmonary artery pressure. Because a fistula was suspected, a multislice computer tomography scan was performed.     

L'Anastomose cavo-pulmonaire dans les malformations tricuspidiennes: Evolution de 10 cas opérés

Certain congenital malformations affecting the rightsided cavities of the heart and accompanied by a diminished pulmonary blood flow can be improved by an end-to-side anastomosis between the right pulmonary artery and the superior vena cava. Nine out of the 10 patients who underwent the operation survived. The results have been very impressive in six patients with tricuspid atresia who were critically ill. The anoxemic episodes ceased completely and cyanosis has disappeared. Three cases of Ebstein''s anomaly have also been improved. This clinical study has given us the opportunity to review the physiological consequences of the anastomosis, its complications and its surgical indications. We believe that this type of shunt is especially indicated in cases of tricuspid atresia, in which only a palliative form of surgery can be performed.     

多切面法联合彩色多普勒超声在胎儿先天性心脏病诊断中的应用

目的:探讨多切面法联合彩色多普勒超声在胎儿先天性心脏病(congenital heart diseases,CHD)诊断中的应用价值。方法:采用多切面法联合彩色多普勒超声对2015年5月~2016年7月300例胎儿进行CHD筛查,并与随访的产后超声或尸解结果作对照。结果:300例胎儿经产前超声联合多切面法检出CHD胎儿20例,检出率为6.7%,经产后超声或尸解确诊14例:三尖瓣下移畸形1例,室间隔完整型完全性大动脉转位1例,完全性房室间隔缺损1例,室间隔完整型肺动脉瓣闭锁1例,双流入型单心室1例,共同动脉干Ⅰ型2例,单纯室间隔缺损2例,法洛氏四联症2例,主动脉弓缩窄1例,肺动脉瓣轻度狭窄1例,二尖瓣闭锁并共同动脉干1例;误诊为单纯室间隔缺损1例,误诊为法洛氏四联症1例,病例流失4例。产前超声联合多切面法对有、无高危因素的检出率分别为3.79%、13.48%,比较有统计学意义(P0.05)。产前超声联合多切面法诊断CHD的灵敏度为100%、特异度为99.66%、阳性预测值为80.00%、阴性预测值为100%。结论:多切面法联合彩色多普勒超声在胎儿CHD诊断中具有较高的应用价值。