埃布斯坦综合征的外科治疗

目的 为了改善埃布斯坦综合征（Ｅｂｓｔｅｉｎ ａｎｏｍａｌｙ）的手术疗效,总结３６例埃布斯坦综合征患者的手术治疗经验。方法 ３６例手术中,三尖瓣置换术６例,Ｄａｎｉｅｌｓｏｎ手术２８例,Ｃａｒｐｅｎｔｉｅｒ手术２全;其中５例同时行右心房室旁路切断术。结果 术后早期右侧心力衰竭死亡４例,其中心瓣膜置换术１例,成形术２例,１例成形术并右心房室旁路切断术死于右心室壁血肿。２５例患者随访３个月～１１年,１     

埃布斯坦综合征的外科治疗

目的 为进一步提高埃布斯坦综合征(Ebstein syndrome)的外科治疗效果，总结其临床经验。方法 37例Ebstein综合征患者行三尖瓣成形术31例，三尖瓣置换术4例，全腔静脉-肺动脉连接术2例；同期行房间隔缺损修补术19例，预激综合征旁路切断术4例。结果 术后因严重心律失常死亡1例，其余患者近期效果满意，心功能均达I～Ⅱ级，仅5例患者有轻度三尖瓣反流。结论 Ebstein综合征一旦诊断明确，应尽早手术矫治，防止房化右心室进一步扩大、变薄，以改善右心功能，提高手术效果。     

Ebstein综合征的外科治疗

目的总结Ebstein综合征的外科治疗经验。方法回顾性分析2006年2月至2010年6月济宁医学院附属医院23例Ebstein综合征患者经外科手术治疗的临床资料,其中男5例,女18例;年龄3～40(15.0±7.6)个月。行单纯三尖瓣环成形术1例,Danielson式折叠术5例,Carpentier式折叠术16例(其中5例患者三尖瓣和功能右心室发育差,加行双向Glenn手术),心脏复跳后三尖瓣关闭不全,有大量反流,再次阻断主动脉行三尖瓣置换术1例。结果无手术死亡,术后出现频发性室性早搏4例,室性心动过速2例,Ⅲ°房室传导阻滞1例,均于术后1周内恢复。术后1～2周复查心脏超声心动图提示:三尖瓣有微量至轻度反流19例,中度反流3例。随访19例,随访时间0.5～4.0(3.3±1.6)年,随访期间因右心功能衰竭死亡1例,再次手术行三尖瓣置换术1例;心功能分级(NYHA)Ⅰ级12例,Ⅱ级4例,Ⅲ级2例。失访4例。结论三尖瓣修复术是治疗Ebstein综合征有效的手术方式,可有效地缓解患者的临床症状,改善血流动力学,促进右心室功能的恢复,提高患者的生活质量。     

矫正型大动脉转位形态三尖瓣置换术及中远期结果

目的探讨矫正型大动脉转位（cTGA）患者行形态三尖瓣置换术的手术适应证及其术后中远期结果。方法1997年9月至2007年9月,阜外心血管病医院收治cTGA患者18例,男15例,女3例;年龄16~51岁（33.3±12.8岁）,体重47~90 kg（60.9±14.7 kg）。单纯形态三尖瓣关闭不全10例,合并室间隔缺损3例,合并室间隔缺损及肺动脉瓣狭窄2例,室间隔缺损修补术后形态三尖瓣关闭不全2例,形态三尖瓣置换术后机械瓣功能不良1例。术前形态右心室射血分数为56.2%±11.6%,心功能分级（NYHA）Ⅱ级12例,Ⅲ级6例。18例cTGA患者均施行形态三尖瓣置换术。术后随访患者的心功能、形态右心室射血分数等指标。结果手术死亡1例,死于术后低心排血量综合征。术后发生瓣周漏2例,分别于术后7 d和30 d行瓣周漏修补术后治愈。随访16例,随访时间57.0±40.7个月;随访时形态右心室射血分数与术前比较差异无统计学意义（52.8%±9.2%vs.56.2%±11.6%;t=2.062,P〉0.05）,心功能分级（NYHA）Ⅰ~Ⅱ级12例,Ⅲ级4例,与术前比较心功能分级Ⅲ级者所占百分比差异无统计学意义（χ2=1.532,P〉0.05）。结论cTGA形态三尖瓣置换术能防止形态三尖瓣关闭不全对形态右心室功能的进一步损害;其中远期结果满意;随访期间,形态右心室功能基本正常。     

11／2心室修补手术

目的 应用11／2心室修补手术治疗小儿复杂先天性心脏病,探讨11／2心室修补手术指征。方法 自1996年3月－2000年5月,对11例患者进行了11／2心室修补术。其中包括三尖瓣闭锁（1a型）、三尖瓣狭窄、室间隔完整型肺动脉闭锁、肺动脉狭窄和室间隔缺损等合并右心发育不良6例;埃布斯坦综合征（Ebstein anomaly C 型）、肺动脉闭锁伴Ebstein综合征等右心室功能不良2例以及合并左侧上腔静脉异位引流至左心房3例。所有患者在心内修补手术后进行双向腔静脉肺动脉吻合术,其中房间隔开窗3例。结果 手术死亡1例,存活10例,术后血流动力学稳定,随访结果满意。结论 对复杂先天性心脏病某些选择性病例,11／2心室修补手术是一种安全、可靠、有效的手术方法。     

先天性心脏病术中心脏瓣膜的保护和矫治

目的 总结先天性心脏病术后因瓣膜功能不全再手术的经验。方法 回顾分析先天性心脏病术后再行瓣膜手术13例患者的临床资料,其中室间隔缺损修补术后8例,部分心内膜垫缺损修补术后3例,法洛四联症和房间隔缺损修补术后各1例。第1次手术时即存在二尖瓣轻～中度关闭不全6例,主动脉瓣关闭不全1例;新出现瓣膜功能异常6例,其中2例因补片漏致三尖瓣关闭不全,2例因前叶腱索断裂致三尖瓣关闭不全,1例因残留右心室流出道狭窄继发三尖瓣关闭不全,1例因伤及主动脉瓣并发二尖瓣和三尖瓣关闭不全。13例中,行二尖瓣置换6例,三尖瓣置换2例,主动脉瓣置换1例,行主动脉瓣置换并二尖瓣、三尖瓣成形1例,三尖瓣成形3例。同时修补残余漏,疏通右心室流出道。结果 术后发生低心排综合征3例。2例术后早期分别死于脑气栓和呼吸循环衰竭。11例术后痊愈出院,随访1～8年,心功能良好。结论 先天性心脏病矫治术中应注意心脏瓣膜的保护,合并的瓣膜功能异常应积极修补,及时地再手术可取得良好效果。     

改良Carpentier法治疗埃布斯坦综合征25例

目的探讨改良Carpentier方法矫治埃布斯坦综合征(Ebsteinsyndrome)的疗效。方法采用自体心包片加宽前瓣,以加大面积;后瓣叶边缘对合缝在隔瓣叶或前瓣;自制心包片包裹涤纶环加固瓣环;可调式DeVega三尖瓣成形。结果采用改良Carpentier方法矫治的25例Ebstein综合征患者除1例死于低心排血量综合征外,余24例治愈出院,手术死亡率4%(1/25)。术后随访3～94个月,心功能级3例,级18例,级3例,未发生新的心律失常。结论改良Carpentier方法矫治Ebstein综合征,方法新颖实用,疗效满意。     

二尖瓣置换术后三尖瓣关闭不全的外科治疗

目的总结二尖瓣置换术后三尖瓣关闭不全患者的外科治疗经验。方法14例心脏瓣膜疾病患者行二尖瓣置换术后发生中度或重度三尖瓣关闭不全,其中相对性关闭不全12例,器质性关闭不全2例。行三尖瓣成形术4例,均使用人工瓣环成形;行三尖瓣置换术10例,其中置换生物瓣6例,双叶机械瓣4例。在心脏不停跳下手术4例,常规心脏停搏手术10例。结果全组无手术死亡,术后发生低心排血量综合征3例,用升压药维持循环,患者均顺利出院。术后复查超声心动图,右心房、室均明显缩小,右心室前后径(22.5±5.6mm)较术前(31.9±5.9mm)明显减小(P<0.001)。随访14例,随访时间1.8±1.3年,心功能级2例、级9例,级2例,1例于术后4年因心力衰竭死亡。结论二尖瓣置换术后远期三尖瓣关闭不全的患者经外科矫治后疗效满意,合理掌握手术指征、手术时机和良好的围术期治疗是手术成功的关键。     

艾勃斯坦心脏畸形的手术配合体会

艾勃斯坦畸形是一种少见的先天性心脏畸形。其病理解剖,主要是三尖瓣下移和三尖瓣畸形。三尖瓣下移至右心室腔内,致使一部分心室腔形成房化心室。常有三尖瓣关闭不全,并有房间隔缺损或卵圆孔未闭。多数伴有完全或不完全右束枝传导阻滞,10％的病人伴有预激综合征。     

心室减荷术在Ebstein畸形矫治中的应用

目的　应用心内修复并双向腔肺动脉分流术矫治Ebstein畸形18例。方法　18例中男10例,女8例;年龄9月龄～5 4岁,平均(18.4 7±13.85 )岁;体重8.5～80. 0kg ,平均(36.0 9±19.78)kg。按汪曾炜分型均为II型;按Carpentier分型B型15例,C型3例。手术均在浅低温体外循环心脏跳动下进行,先采用Danielson的方法行房化心室折叠和三尖瓣成形,以及房间隔缺损和卵圆孔未闭修补,后行双向腔肺动脉分流术,即心室减荷术。结果　全组无死亡,无严重心功能不全和顽固低心排出量综合征等发生,无因三尖瓣病变再次手术者。13例随访1～5 3个月,心功能I级12例,II级1例。三尖瓣关闭不全I度4例,II度2例,余7例无关闭不全,上腔—右肺动脉吻合口通畅。结论　Ebstein畸形中三尖瓣显著下移合并三尖瓣中、重度关闭不全和右室流出道生理性梗阻时,修复心内畸形后加行双向腔肺动脉分流术可减轻右心负荷,保护左、右心室和三尖瓣的功能     

慢性肾脏病合并代谢综合征患者的中医证候分析

目的:对慢性肾脏病(CKD)合并代谢综合征(MS)患者的中医证候进行回顾性研究,了解CKD合并MS的证候特征,为CKD的个体化诊治提供指导。方法:收集2007年～2009年住院或门诊的CKD患者,其中CKD合并MS组121例,CKD非合并MS组123例,设计中医证候临床观察表,对两组病人临床脉证进行调查研究。结果:(1)CKD合并MS组男性患病人数明显高于女性,年龄大于CKD非合并MS组。两组病程及慢性肾脏病的分期差异无统计学意义。(2)代谢综合征各组分比较:CKD合并MS组腰围(W)、BMI、高血压发生率、FPG、TG、LDL-C明显高于CKD非合并MS组(P<0.01)。HDL-C明显低于CKD非合并MS组(P<0.05)。(3)两组病人肾损害比较:Scr、BUN、24h尿蛋白定量、血尿及肾衰竭发生率两组相比差异无统计学意义(P<0.05)。CKD合并MS组UA明显高于CKD非合并MS组(P<0.001)。(4)两组病人中医证候的比较:虚证中合并MS组以脾肾气虚多见,非合并MS组以气阴两虚多见;实证中合并MS组以血瘀、痰湿、热盛多见,非合并MS组以湿热、血瘀为多见;合并MS组实证中两证以上相兼的现象较非合并MS组常见;合并MS组血瘀证和热盛证兼夹、血瘀和痰湿证兼夹、血瘀、痰湿和热盛证兼夹多见,非合并MS组以血瘀和湿热证兼夹、湿热和热盛证兼夹多见。两组总的证候均以虚实夹杂为主要特征。结论:CKD合并MS的中医证候特点以虚实夹杂,多证相兼为主要特征。虚证以脾肾气虚为主,实证以血瘀、痰湿、热盛为主。     

Acute Mirizzi Syndrome

Background:

Mirizzi syndrome is a rare complication of cholecystolithiasis characterized by jaundice due to compression of the common hepatic duct. The diagnosis may not be immediately apparent, and management is controversial with open surgery still recommended by some authors.

Method:

A case is detailed herein of a 67-year-old man who presented with abdominal pain, fever, and jaundice. A dilated bile duct was found on ultrasound, but the gallbladder could not be seen. The diagnosis of Mirizzi syndrome was made at ERCP, and a stent was placed through the papilla. Laparoscopic retrograde (fundus first) cholecystectomy was carried out utilizing a laparoscopic liver retractor.

Results:

In this particular case, it was not possible at ERCP to get a guidewire and stent past the obstruction. A stent was left through the papilla, below the obstruction and this allowed primary duct closure during surgery.

Conclusion:

Acute Mirizzi syndrome should be suspected when a patient presents with acute cholecystitis and jaundice with dilated intrahepatic ducts on ultrasound. ERCP is useful to confirm the diagnosis and allows stenting to alleviate the jaundice and facilitate the subsequent operation. Laparoscopic ultrasound is useful to locate the impacted stone and to partially replicate the touch of the surgeon''s hand, which is not available in laparoscopic surgery.     

Metabolic Syndrome and Hypogonadism

Metabolic syndrome (MetS) is a complex entity and continues to be a source of medical controversy. The most important pathogenic factors are insulin resistance and visceral obesity. The clinical heterogeneity of the syndrome results from its significant influence on protein, glucose, and fat metabolism, cellular growth and differentiation, endothelial function, atherosclerotic disease, and inflammation. MetS is linked to a number of urologic diseases. This article reviews its relationship with hypogonadism.     

Bardet—Biedl Syndrome and Cystinuria

An unusual association of Bardet-Biedl syndrome with cystinuria was described in one patient. A 21-year-old mate was admitted to hospital because of renal failure, severe deterioration of visual acuity, Polydactyly, brachydactyly, and mental retardation. Laboratory investigations revealed a serum creatinine of 292 µmol/L (3.3 mgldL) and a GFR of 25 mLlmin per 1.73 m². Quantitative ion exchange chromatography demonstrated an increased urinary excretion rate of cystine, lysine, arginine, and ornithine. The ophthalmologic examination showed a severe atypical retinal dystrophy. Visual acuity was severely deteriorated and the patient could only count the examining physician's fingers. The patient had been previously evaluated at the age of 7 years for polyuria, polydipsia, and growth failure. His workup at that time demonstrated nephrogenic diabetes insipidus, normal GFR, and a urinary amino acid pattern consistent with the cystinuric phenotype. There was mental retardation notwithstanding the normal ophthalmologic examination. Intravenous pielography showed calyceal clubbing, calyceal cysts, and lobulated renal outlines of the fetal type. The patient was evaluated again at the age of 13 years for deterioration of visual acuity and the ophthalmologic examination showed an atypical retinal dystrophy, with sparse pigmentation, central and peripheral atrophy, attenuated vessels, and marked optic disk pallor. To our knowledge the association of Bardet-Biedl syndrome with cystinuria has never been reported. It is unlikely that cystinuria may have contributed to the kidney damage. The possibility that mental retardation has been induced or aggravated by cystinuria cannot be excluded.