功能性单心室的外科治疗

目的 为了使更多的先天性心脏病患者得到纠治，回顾性总结手术治疗功能性单心室的临床经验。方法 手术纠治127例功能性单心室患者，年龄7个月－12岁，体重7．5－39kg，其中丰唐手术（Fontan operation)72例，半Fontan术3例，双向上腔静脉肺动脉吻合术51例，肺动脉环缩术1例。结果 早期Fontan术19例，死亡11例；改良Fontan术53例，死亡10例；半Fontan术死亡1例；双向上腔静脉肺动脉吻合术死亡4例；总手术死亡率20．5％。结论 功能性单心室必须早期得到纠治，控制肺动脉血流，预防严重缺氧。双向上腔静脉肺动脉吻合术能减少功能性单心室的容量负荷，保持足够的心排血量。改良Fontan术是功能性单心室的最佳手术方案。     

应用系列改良Fontan手术治疗复杂先天性心脏病

目的探讨系列改良Fontan手术的特点和治疗复杂先天性心脏病的效果，进一步提高对其临床应用的认识。方法回顾性分析1992年9月～2006年6月期间77例复杂先天性心脏病患者行改良Fontan手术治疗的临床资料，其中行右心房肺动脉吻合术21例，心房内板障或管道全腔静脉-肺动脉连接术28例，心外人工管道全腔静脉-肺动脉连接术24例，自体右房壁管道全腔静脉肺动脉连接术2例，自体带蒂心包心外管道全腔静脉-肺动脉连接术1例，主肺动脉与下腔静脉吻合全腔静脉-肺动脉连接术1例。结果术后早期死亡5例，其中死于心力衰竭3例，突发心律失常1例，脑出血1例。再次手术1例，术后早期生存率93．5％（72／77），手术成功率92．0％。左心室舒张期末内径（LVEDD）较术前减小（52．5±7．8mm vs．62．5±11．0mm，P=0．013），左心室射血分数（LVEF）较术前增加（68．5％±4．0％vs ．62．0％±4．5％，P=0．032）。随访63例，随访率87．5％（63／72），随访时间1～15年。随访期间死亡4例，远期再次手术1例，远期生存率88．3％，手术成功率86．0％。结论在复杂先天性心脏病的治疗中，改良Fontan手术有良好的疗效；同时根据具体解剖结构可选择不同的手术方式。     

小儿复杂先天性心脏病侧通道和外管道Fontan术比较

目的 总结侧通道和外管道Fontan术纠治小儿危重复杂型先天性心脏病(先心病)的经验.方法 2000年1月至2006年12月128例复杂型心内畸形病儿行改良Fontan术治疗.男76例,女52例.年龄1.9～16.5岁,平均(6.2±3.8)岁;体重8～62 kg,平均(19.0±5.5)kg.病种主要有单心室(SV)59例;右室双出口(DORY)Z5例等.一期Fontan术87例;二期Fontan术41例.两次手术间隔0.8～7.3年,平均(3.9±2.8)年.首次手术为单侧Glenn术(12例)、双侧双向Glenn术(8例)等.行心内侧通道(LT)的Fontan组(LT组)89例,行外管道(ECC)的Fontan组(ECC组)39例.术前均行二维多普勒超声,11例加做核磁共振,30例行心导管和心血管造影.结果 死亡17例(13.3%).LT组病儿年龄明显低于ECC组.32例发生低心输出量综合征,其中11例.肾功能受损导致无尿而行腹膜透析术(2～5 d后尿量恢复).LT组术后心律紊乱的发生率明显高于ECC组.门诊随访3～24个月,均未出现严重并发症,无死亡病例.结论 LT组术后心律紊乱的发生率明显高于ECC组,ECC的Fontan术具有可避免主动脉阻断和心肌缺血、缩短CPB时间和保持更为流线型的下腔静脉回流血的优点,开窗术的远期优越性未能明确.     

单心室的外科治疗

目的 回顾总结单心室的手术治疗经验。方法 1973年1月－1999年12月共纠治单心室47例,其中包括右心室型17例,左心室型14例,另16例诊断不明确。手术方式为肺动脉环缩术1例,丰唐手术（Fontan operation)23例,双向上腔静脉动脉吻合术23例。结果 全组死亡7例,死亡率15％、行双向上腔静脉肺动脉吻合术患者中无死亡。结论 单心室患者早期必须采取手术治疗,防止肺部充血和严重缺氧。双向上腔静脉肺动脉吻合术能改善单心室的压力和容量负荷,降低手术死亡率,减少Fontan手术的危险因素。     

心房内侧隧道Fontan术纠治小儿复杂先天性心脏病47例

目的 总结应用心房内侧隧道Fontan术治疗47例小儿复杂先天性心脏病（先心病）的经验。方法 47例病儿年龄1岁5个月－14岁;体重9－42kg。病种包括三尖闭锁10例,单心室17例,右室双出口14例,完全性大动脉转位2例和纠正性大动脉转位4例。结果 术后早期死亡9例,手术死亡率19.1％。1995年前手术死亡率26.3％（5／19例）,1995年后（含1995年）14.3%(含1995年）14.3%(4/28例）。晚期死亡1例。结论 改良Fontan术适应证已扩大至许多解剖上不能纠治、功能上仅有一个单心室腔的复杂性紫绀型先心病,为提高手术疗效须严格掌握手术适应证;板障上开窗可有效降腔静脉和右房压力,减少胸腔引流量,缩短住院,是高体循环心排出量;少数不能耐受Fontan术后循环生理改变 的难治性低心输出量病儿,应早期果断再手术改建为双向腔肺分流术（半Fontan术）     

改良Fontan手术治疗复杂先天性心脏病

目的 总结改良Fontan手术治疗复杂先天性心脏病的临床经验.方法 1996年11月～2005年5月,采用改良Fontan手术纠治124例复杂先天性心脏病(病种包括三尖瓣闭锁、单心室、右心室双出口、大动脉错位、肺动脉闭锁、矫正型大动脉转位、右心室发育不良等)患者,手术年龄7.6±5.5岁.常温非体外循环下手术19例,体外循环下手术105例.右心房-肺动脉连接17例,右心房-右心室连接19例,全腔静脉-肺动脉连接术(TCPC)88例.23例行分期手术.结果 术后早期(术后30d)死亡17例(13.7%),其中行右心房-肺动脉连接者死亡率为23.5%(4/17),行右心房-右心室连接者死亡率为15.8%(3/19),行TCPC者死亡率为11.4%(10/88),同期预留或术后开窗手术死亡率为14.6%(6/41),分期手术患者死亡率为8.7%(2/23).死亡原因低心排血量、多器官功能衰竭和心室颤动等.术后早期并发症发生率为16.9%(21/124),主要为胸腔积液、心律失常、心包积液和低心排血量综合征等.术后随访89例,随访时间6～65个月.远期再住院率6.5%,再手术率0.9%.3例出现再发性胸腔积液,3例出现心包积液,1例出现下腔静脉梗阻,均经相应的治疗后治愈.其余患者心功能恢复好.结论 改良Fontan手术是治疗复杂先天性心脏病中功能性单心室的最佳手术方案;房间隔开窗可明显提高术后早期疗效,减少渗出.     

心外管道全腔静脉肺动脉连接术

目的 报告心管道全腔静脉肺动脉连接术（TECPC）应用经验。方法 横断上腔静脉与右肺动脉端侧吻合,切断下腔静脉前壁,保留原位吻合口,将下腔静脉通过人工血管与主肺动脉吻合,共治疗9例复杂性先天性心脏病,其中1例单心室改良Fontan术后4年频发室上性心动过速而改行TECPC。结果 全组手术后均生存。3例术后出现胸腔积液、乳糜胸并发症、均治愈。血流动力学指标满意,术后随访心功能Ⅰ-Ⅱ级,无心律紊乱发生。结论 TECPC手术操作简单,并发症少,适应证广泛,优于改良Fontan手术和传统的全腔静脉肺动脉连接术。     

改良Fontan手术的危险因素分析

目的 探讨改良Fontan手术后早期死亡的危险因素和手术适应证。方法 统计分析154例改良Fontan手术病人的17个围手术期指标与手术结果。结果 术后早期死亡37例，死亡率24.0%。单因素分析结果表明，术前McGoon比值≤1.8，术前房室瓣存在反流，手术方式，术后右房压≥20mmHg,心律失常，严重低心输出量综合征是手术早期死亡的高危因素，多因素Logistic逐步回归分析结果显示，手术方式（右房与右室连接，右房与肺动脉连接），术后右房压和术后严重低心输出血量综合征与术后早期死亡有关。结论 术前严格选择心室功能和肺动脉发育好，无明显房室瓣反流的病例，采用全腔静脉与肺动脉连接术式，术后加强监护是预防和降低术后早期死亡的有效措施。     

Fontan类手术治疗心房异构、内脏异位合并心脏畸形的效果

目的 评价Fontan类手术治疗心房异构、内脏异位合并心脏畸形的效果.方法 2002年4月至2010年12月,25例心房异构、内脏异位合并心脏畸形共行手术治疗28次.男10例,女15例,年龄2～18岁,平均(9.1±5.5)岁.右心房异构18例,左心房异构7例.左心室双入口合并肺动脉狭窄15例,左心室双入口和双出口合并肺动脉狭窄5例,右心室双出口合并肺动脉闭锁2例,合并肺动脉狭窄1例,二尖瓣和三尖瓣闭锁合并肺动脉狭窄各1例.Fontan手术16例次,其中15例次行心外管道Fontan手术,1例次行心房内侧隧道Fontan手术;双向腔肺分流术8例次,3例次为双上腔静脉肺动脉分流;全腔静脉肺动脉分流4例次(Kawashima手术).结果 术后早期死亡2例,均为术后严重低心排血量综合征.术后室上性心动过速5例,结性心律2例.术后经皮血氧饱和度0.78 ～ 1.00,平均0.86±0.07.随访6个月以上者15例,最长7年,经皮血氧饱和度0.68 ～0.97,平均0.82±0.08;心室射血分数＞0.55者13例,＜0.50者2例;结性心律4例.结论 Fontan类手术是治疗心房异构、内脏异位合并心内复杂畸形的主要手术方式,术后早、中期效果较好.选择Fontan手术的术式,特别是心外管道,妥善修复心内合并畸形是提高效果的关键.     

完全性大动脉转位的外科治疗

对３３例完全性大动脉转位施行了手术治疗，其中全腔静脉肺动脉连接３例、改良Ｆｏｎｔａｎ手术２例、Ｍｕｓｔａｒｄ及大动脉调转术各１例，Ｒａｓｔｅｌｉ手术２６例，包括１５例心内隧道和心外管道，１１例心内、外双管道。这２６例心外管道中，除４例采用同种主动脉管道外，其余为经处理的猪肺动脉人工管道。全组手术死亡１０例，晚期死亡１例。结论：完全性大动脉转位病理解剖变异性大，应按不同类型的病理解剖特征选择手术方法     

Modified fontan operation

From November 1988 to October 1991 30 patients underwent a total extracardiac right heart bypass for complex cardiac anomalies by means of bidirectional cavopulmonary anastomosis and interposition of a conduit from the inferior vena cava to the pulmonary artery. Mean age at surgery was 6.4 years and mean weight 19.2 kg. There was 1 hospital death (3%) due to a borderline indication for a Fontan operation. 2 patients had further surgery: In 1 the repair was taken down due to the stenosis of the left pulmonary artery and the patient was left with a bidirectional cavopulmonary anastomosis only, the second patient required a revision of the cavopulmonary anastomosis due to a stenosis of the superior vena cavaright pulmonary artery junction. There were no late deaths and the survivors are in good clinical condition a mean of 15.1 months after the operation. We propose this technique as an alternative surgical option in candidates for a Fontan operation in whom atrial septation is hazardous including those with 1) hypoplasia or atresia of the left atrio-ventricular valve, 2) common atrioventricular valve, 3) anomalies of systemic and/or pulmonary venous return, or 4) auricular juxtaposition.     

Physiological rationale for a bidirectional cavopulmonary shunt. A versatile complement to the Fontan principle

The original Fontan procedure included a classic superior vena cava-to-right pulmonary artery (Glenn) shunt. Subsequent experience demonstrated that this anastomosis was not essential and was an unnecessary commitment of the larger right pulmonary circulation to the smaller blood volume of the superior vena caval return. With application of the Fontan principle to more complex cardiac malformations, there has been a reconsideration of possible benefits of a cavopulmonary shunt in selected patients. A modified shunt from the divided end of the superior vena cava to the side of the undivided right pulmonary artery utilized in 21 patients is described. This shunt is designed to allow bidirectional pulmonary arterial distribution of both superior vena caval inflow and right atrial outflow after completion of the Fontan procedure. Twelve patients had the bidirectional shunt performed prior to a Fontan operation; five of these had a subsequent atriopulmonary connection and seven await operation. Eight patients had construction of this shunt at the time of their Fontan procedure. One patient had a bidirectional shunt constructed following atriopulmonary anastomosis to help relieve right atrial outflow obstruction. Two patients with univentricular heart undergoing simultaneous Fontan procedure and a bidirectional shunt died while in the hospital. The remaining 19 patients have been followed up for 2 months to 9 years with one late sudden death at 9 years. There have been no bidirectional cavopulmonary shunt failures, stenoses, kinks, or recognized pulmonary arteriovenous malformations. Postoperatively, eight patients had assessment of pulmonary distribution of shunt blood flow by angiography. Seven of these patients were also evaluated by radionuclide angiography. Superior vena caval blood flow via the bidirectional cavopulmonary shunt tended to be greater to the right lung, but bilateral pulmonary flow was documented in all but one patient. After Fontan operation, six of seven patients tested also demonstrated bilateral distribution of atriopulmonary flow. We concluded from our experience that this modified shunt provides excellent relief of cyanosis, allows bidirectional pulmonary distribution of both superior vena caval return and also the right atrial blood flow after atriopulmonary connection, and may be done before, with, or after a Fontan procedure and is compatible with all currently recommended modifications. Perioperative hemodynamic adjustments to the Fontan procedure may be improved by reducing atrial volume, and this may also be of potential benefit in the long-term adaptation to Fontan physiology by minimizing atrial distention.     

Bidirectional Cavopulmonary Shunt in Patients With Anomalies of Systemic and Pulmonary Venous Drainage

Background. Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic or pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure.

Methods. Between March 1990 and December 1995, 36 patients with anomalous systemic or pulmonary venous drainage underwent bidirectional cavopulmonary shunt. A combination of anomalous systemic and pulmonary venous drainage was present in 12 patients, whereas 19 patients had anomalous drainage only from the systemic circulation and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 18 patients. The median age at operation was 11 months, and bidirectional cavopulmonary shunt was the first surgical procedure performed in 10 of these patients. Techniques of repair are described.

Results. There were two early deaths and one bidirectional cavopulmonary shunt was taken down, for mortality and failure rates not significantly different than those for all patients undergoing bidirectional cavopulmonary shunt during this time period (n = 117). At a mean follow-up of 19.9 months, there have been three late deaths and 11 patients have undergone Fontan completion. Actuarial survival was 87% at 1 year and 81% at 3 years. Among all patients undergoing bidirectional cavopulmonary shunt during this time period, neither heterotaxy syndrome nor anomalies of systemic or pulmonary venous return were significantly associated with decreased survival or poor outcome.

Conclusions. Bidirectional cavopulmonary shunt can be performed in patients with anomalous systemic or pulmonary venous drainage, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients undergoing bidirectional cavopulmonary shunt. In this report, we describe our experience with this group of patients, primarily focusing on outcomes and technical issues that pertain to the use of bidirectional cavopulmonary shunt as a preparatory procedure for the extracardiac conduit Fontan operation.     

双向上腔静脉肺动脉吻合术

双向上腔静脉肺动脉吻合术(bidirectional superior cavopulmonary anastomosis,BCPA)是应用于单心室纠治中的姑息术式,主要形式有双向Glenn分流术和半Fontan手术.适应证包括作为全腔静脉-肺动脉连接术的姑息形式,部分双心室修补或1 1/2心室修补及复杂先天性心脏病手术的辅助步骤.目前对年龄的选择、术后肺动脉的生长发育、额外肺血流是否保留、侧枝血管的形成及转换全腔静脉-肺动脉连接术的时机等问题仍值得进一步研究.     

经皮肾镜碎石取石术后发热原因及治疗分析

目的：探讨引起经皮肾镜取石术（PCNL）后发热的因素及防治措施。方法回顾性分析2011年1月至2012年12月本院收治的158例行PCNL患者的临床资料，分析术后体温＞38.5℃患者的年龄、结石大小、手术时间、术前是否尿路感染、术后是否引流不畅等因素与发热的关系以及相应的治疗措施。结果158例患者中31例（19.6%）出现术后体温升高，其中14例患者术前存在感染，术后引流不畅导致发热者11例，结石直径＞3.0 cm组患者术后发热率为47.61%，≤3.0 cm组为9.48%（χ^2=28.436，P =0.000）；手术时间＞90 min组患者术后发热率为25.80%，≤90 min组为10.76%（χ^2=5.486，P=0.019）；术前存在感染组患者发热率为40.00%，不存在感染组为13.82%（χ^2=11.840，P=0.001）；术后引流不畅组发热率为84.61%，引流通畅组为13.79%（χ^2=37.944，P=0.000）；组间发热率比较差异均具有统计学意义（P ＜0.05）。而不同年龄、同侧上尿路手术史、合并糖尿病组间发热率比较差异无统计学意义。发热患者使用抗菌药物，保持引流通畅等治疗后痊愈。结论发热是PCNL术后常见的并发症，术前尿路感染、结石较大、手术时间长和术后引流不畅是发热的主要原因。保持引流通畅及使用有效抗菌药物是最主要的处理方法。     

甘肃河西地区大肠癌与胆囊结石相关性的初步探讨

目的探讨大肠癌发病与胆囊结石之间的相关性。方法对照分析213例大肠癌患者（大肠癌组）和同期432例胃癌患者（对照组）合并胆囊结石的比率,比较大肠癌组内胆囊结石患者与非胆囊结石患者在肿瘤发生部位、性别、年龄、各影响因素分布等方面的差异。结果 （1）大肠癌组合并胆囊结石41例（19.25%）,对照组合并胆囊结石37例（8.56%）,两组合并胆囊结石的比率比较,χ^2=15.318,P=0.000,差异有高度统计学意义,大肠癌组合并胆囊结石的比率明显升高。（2）大肠癌组内,合并胆囊结石的大肠癌患者与不合并胆囊结石的患者在肿瘤解剖部位间的差异无统计学意义（χ^2=5.386,P=0.068）;但在性别间的差异有高度统计学意义（χ^2=11.056,P=0.001）,女性大肠癌合并胆囊结石的比率明显升高;大肠癌合并胆囊结石的比率在〈50岁、50-60岁、〉60岁3个年龄段间的差异有高度统计学意义（χ^2=10.557,P=0.005）,〉60岁的大肠癌患者合并胆囊结石的比率明显升高;（3）多元素回归分析显示,合并胆囊结石的超重或高血压、糖尿病、肝功能异常、嗜酒、非甾体类抗炎药常规服用等因素与大肠癌发生无相关性,而伴有血脂异常（高血脂）相关（OR=2.742,95%CI：1.114-6.751,P=0.046）。结论胆囊结石可能是大肠癌发生的高危因素之一;女性胆囊结石、〉60岁的胆囊结石以及伴有血脂异常的胆囊结石并发大肠癌的风险明显升高。     

Immediate results of bidirectional cavopulmonary anastomosis and Fontan operations in adults

The purpose of this study is to analyze the immediate results of bidirectional cavopulmonary anastomosis (BCPA) and Fontan operations performed in adults, and to reveal the risk factors. During the years 1983-2010, 681 consecutive patients underwent BCPA or a Fontan operation. Fifty-three of 681 patients were more than 18?years of age. Twenty-nine adults underwent BCPA and 24 underwent a Fontan operation. Immediate results of surgical treatment were followed during the hospital period. The average number of exceeded 'operability' criteria by Choussat et al. [Choussat A, Fontan F, Besse P, Vallot F, Chauve A, Bricaud H. Selection criteria for Fontan procedure. In: Anderson RH, Shinebourne EA, editors. Pediatric Cardiology. Edinburgh: Churchhill Livingstone, 1977:559-566] was significantly higher in patients from the BCPA group compared to the Fontan group (1.3±0.8 vs. 0.9±0.7, P=0.034). Hospital mortality after BCPA in adults was 6.9% (2/29) and did not differ from children (7.1%, 19/268), P=0.634. Hospital mortality after Fontan operation in adults was 8.3% (2/24) and did not differ from children (11.9%, 43/360), P=0.419. The frequency of non-lethal hospital complications was higher in patients after a Fontan operation. Patients from the Fontan group more frequently developed arrhythmias and prolonged pleural effusions. Preoperative regurgitation at atrioventricular valves was an independent risk factor for hospital mortality and morbidity after a Fontan operation. BCPA and Fontan operations performed in adults are accompanied by good immediate results and considerably improves patients' condition.