房室管畸形的手术治疗

为提高房室管畸形手术疗效，总结１９８２年１月至１９９４年１２月纠治房室管畸形１３０例，其中部分性房室管畸形（ＰＡＶＣ）８８例，完全性房室管畸形（ＣＡＶＣ）４２例。ＰＡＶＣ手术方法采用间断缝合二尖瓣裂缺，心包补片关闭原发孔缺损；本组ＰＡＶＣ手术无死亡，１例术后３年发生二尖瓣重度反流，再次手术时死亡。ＣＡＶＣ采用单片心包补片方法；本组ＣＡＶＣ手术死亡１例，死亡率２．４％，死因为重度肺动脉高压；远期死亡２例，均为二尖瓣反流，肺部感染合并心力衰竭。结论：ＰＡＶＣ如无明显二尖瓣反流，手术可在３～４岁时做，而ＣＡＶＣ为了防止肺血管阻塞性病变，应该在６月龄内手术，最迟不要超过１岁     

部分性房室管畸形合并双口二尖瓣诊断及外科处理

手术治疗４３例部分性房室管畸形，其中并存双口二尖瓣４例，术前确诊２例，作者结合文献对其病理解剖，诊断及外科处理注意问题进行讨论，强调术中切勿切断连接于前后瓣之间组织桥，以免造成严重二尖瓣反流；同时提倡部分修补瓣裂，以避免修补后造成二尖瓣口狭窄。     

66例部分性房室管畸形的外科治疗

目的总结部分性房室管畸形手术治疗的经验,以提高治疗效果。方法回顾性分析我院1984年1月－2007年12月经外科治疗部分性房室管畸形患者66例的临床资料,对二尖瓣大瓣裂的处理：单纯裂缺缝合52例,缝合加交界折叠缝缩8例,加小瓣成形1例,加缝置St．Jude软质人工成形环3例,人工机械瓣置换术2例;对原发孔型房间隔缺损的修补：采用涤纶补片修补12例,自体心包54例;采用Kirklin法将冠状静脉窦口隔入左心房5例,MeGoon法将冠状静脉窦口隔入右心房61例;同期处理其他合并畸形。结果术后早期死亡2例（3．03％）,1例死于心律失常,另1例死于呼吸功能衰竭。术后发生Ⅲ°房室传导阻滞2例,均安装永久性心脏起搏器。术后随访52例（81．3％）,随访时间5个月～22年,平均15年,心功能均有明显改善,尤其是术前心功能Ⅲ～Ⅳ级的19例患者,术后改善为Ⅰ～Ⅱ级。再次手术4例,其中1例经再次行二尖瓣置换术治愈;术后死亡3例,1例死于急性。肾功能衰竭,2例死于低心排血量综合征。结论早期手术治疗可以保全房室瓣结构、功能,避免发生肺动脉高压和降低死亡率。手术治疗的关键是消除二尖瓣关闭不全、修补原发孔型房间隔缺损和避免损伤传导组织,术后近、中期疗效良好;有残留中度以上二尖瓣反流者,远期效果不满意。     

房室间隔缺损矫治术后左侧房室瓣反流的外科治疗

目的为提高房室间隔缺损修补术后左侧房室瓣反流的外科治疗效果,探讨其瓣膜的病理改变、手术方法的选择,总结围手术期处理经验。方法回顾分析上海交通大学医学院附属新华医院1995年1月至2009年12月收治29例房室间隔缺损矫治术后左侧房室瓣反流患者的临床资料,其中男16例,女13例;年龄4~62岁,平均年龄26.5岁;术前心功能分级（NYHA）Ⅱ级10例,Ⅲ级17例,Ⅳ级2例;曾行部分性房室间隔缺损矫治术18例,完全性房室间隔缺损矫治术11例;再次手术行心瓣膜修复术17例,心瓣膜置换术12例。结果手术后早期因多器官功能衰竭死亡1例;1例患者行心瓣膜置换术后发生永久性Ⅲ°房室传导阻滞而安装永久心脏起搏器,1例4岁患者行心瓣膜置换术后不能撤离体外循环而行左心辅助56 h后痊愈出院。术后随访25例,失访3例,均为行瓣膜修复患者;术后随访6个月~14年,平均8.2年;随访14例心瓣膜修复患者,左侧房室瓣前向血流均无明显加快,10例左侧房室瓣轻微或轻度反流,1例中度反流,3例分别于再次手术后10 d、3年和6年再发左侧房室瓣重度反流,最终行心瓣膜置换术;25例患者术后心功能明显改善,Ⅰ级17例、Ⅱ级6例、Ⅲ级2例;胸部X线片示：心影较术前明显缩小,心胸比率0.53~0.67（0.60±0.11）;未发现远期死亡。结论对房室间隔缺损矫治术后再发生的左侧房室瓣反流,进行及时的手术治疗,选择恰当的手术方式,能取得满意的近期和远期疗效。     

完全性房室通道的外科治疗

目的总结94例完全性房室通道(com p lete atrioven tricu lar cana l defect,CAVCD)的外科治疗经验。方法一期手术矫治CAVCD 94例,房、室间隔缺损修补用双片法(涤纶片+心包片)65例,单片法29例,术中行房室瓣成形,并同期修补合并畸形。结果全组死亡10例(10.6%),其中<6个月者4例。4例术后出现二尖瓣中至大量反流,心肺功能衰竭死亡,3例因肺动脉高压危象死亡,3例分别死于低心排血量、脑并发症和气胸。随访84例,随访时间3~6个月,超声心动图复查二尖瓣轻度反流18例,轻至中度反流12例。结论严重二尖瓣关闭不全和肺动脉高压危象是CAVCD矫治术后主要的死亡原因,早期手术矫治和确切的房室瓣成形可获得较好的手术效果,术中常规使用食管超声心动图对提高手术疗效具有重要的作用。     

老年人肘管综合征九例分析

目的探讨老年性肘管综合征的病因、诊断、鉴别诊断和手术疗效。方法对１９８９～１９９５年手术的９例６０岁以上肘管综合征的病因、症状、手术发现和远期随访结果进行分析研究。结果Ｘ线片显示：９例有Ｃ４，５，Ｃ５，６椎间隙狭窄、骨质增生，但部位均不在Ｃ８Ｔ１。３例曾因幼时外伤而致肘关节不能完全伸直或过伸；２例肘部尺神经滑脱；４例肘外翻畸形。９例就诊时间均在晚期，作尺神经前置术后平均随访２．５年，完全恢复７例，明显改善２例；手部肌力改善４例；爪形手畸形消失２例，无改善２例；但手内肌萎缩均无明显恢复。结论对老年性肘管综合征患者要强调和颈椎病的鉴别诊断，注意肘关节有无畸形，一旦确诊应及早手术，以提高治疗效果。     

完全性房室隔缺损的外科治疗

目的回顾性总结手术纠治４５例完全性房室隔缺损（ｃｏｍｐｌｅｔｅａｔｒｉｏｖｅｎｔｒｉｃｕｌａｒｄｅｆｅｃｔ，ＣＡＶＤ），以期把握好手术时机，提高手术成功率和生存质量。方法根据Ｒａｓｔｅｌｉ分型：Ａ型２６例，Ｂ型２例，Ｃ型２例，过渡型１５例。手术采用单片心包补片修补方法。结果手术死亡３例，死亡率６．７％。死亡原因为伴发心内严重畸形法洛四联症２例，重度肺动脉高压１例；远期死亡２例，均为二尖瓣反流并发肺部感染和心力衰竭。结论ＣＡＶＤ早期出现肺动脉高压，手术应在６个月～１岁内为好，术后必须定期随访，早期了解房室瓣反流情况     

改良术式矫正Ebstein畸形

目的　报告改良术式改善重症Ebstein畸形隔、后瓣下移明显、前瓣发育差、功能右室小、手术矫正困难者的手术疗效。方法　 1997年 6月至 1999年 8月用改良术式对 8例Ebstein畸形施行了手术矫正 (改良组 )。并与采用Hardy、Carpentier等术式矫正及三尖瓣置换共 5 5例病人 (对照组 )的疗效进行了跟踪随访比较。结果　改良组无死亡 ,与对照组比较 ,术后心功能的恢复及三尖瓣反流的改善效果更好。结论　改良术式扩大了右心室形态及三尖瓣重建的手术适应证范围 ,使部分重症病人避免了瓣膜置换术的并发症。对隔、后瓣下移明显且前瓣发育不良的病人消除了术后三尖瓣反流并在保持左、右心室几何形态和功能方面效果显著     

改良Carpentier法矫治三尖瓣下移畸形

目的探讨改良Carpentier法矫治三尖瓣下移畸形(Ebstein畸形)的手术方法,总结其临床经验。方法回顾性分析2006年6月至2010年8月安徽医科大学第一附属医院13例Ebstein畸形患者手术治疗的临床资料,其中男6例,女7例;年龄(26.8±13.5)岁。所有患者均采用改良Carpentier法矫治,手术方法包括:切除部分房化右心室,折叠环缩三尖瓣瓣环至适当的大小,用自体心包片扩大后瓣叶/隔瓣叶,移位缝合切下的部分前瓣叶(向内旋转后)、后瓣及隔瓣叶缝至正常的瓣环水平,移位相应的乳头肌、腱索,扩大修复瓣叶,加固瓣环,并矫正其他合并的心血管畸形。结果所有患者术后均恢复顺利,无死亡。超声心动图提示:三尖瓣功能良好,三尖瓣瓣叶均在正常位置;三尖瓣有轻度至中度反流3例,无反流或有轻微反流10例。心功能分级(NYHA)I～Ⅱ级。所有患者均得到随访,随访时间3～15个月,平均8个月。术后3个月和1年分别复查超声心动图提示:三尖瓣启闭及右心室功能良好,三尖瓣无明显反流12例,1例出院时三尖瓣有中度反流患者转为轻度反流。所有患者恢复正常的生活或工作。结论改良Carpentier法矫治Ebstein畸形有较好的近期疗效,该手术方法切除无功能的房化右心室后重新缝合塑形,有利于右心室形态重建和功能恢复,通过自体心包片扩大瓣叶、充分的瓣叶移位及后乳头肌、腱索移位重建可达到良好的瓣膜成形效果。     

风湿性三尖瓣病变的瓣膜成形术

作者报道４９例风湿性三尖瓣病变的成形术，占同期三尖瓣病变的１３．５％。以狭窄为主２６例，以关闭不全为主２３例；后瓣与隔瓣交界融合９例，前瓣与隔瓣交界融合７例，前后瓣交界融合５例，混合存在５例。合并瓣下结构病变１７例，联合其它心脏瓣膜手术：双瓣置换３３例，二尖瓣置换１５例，主动脉瓣置换１例。手术采用闭合后瓣的二叶化手术或节段性ＤｅＶｅｇａ成形术；交界融合切开加瓣环成形术；瓣下结构粘连分离加腱索乳头肌劈开加瓣环成形术。术后早期死亡３例，其余４６例术后随访２个月～７年（平均４．２年）。术后半年超声复查右房均有不同程度的缩小，三尖瓣轻到中度反流８例，无反流３８例（８２．６１％），心功能转为Ｉ级３４例、ＩＩ级１０例、ＩＩＩ级２例，无晚期死亡。对手术选择的理论依据进行了讨论     

Mitral valve replacement in the first year of life

From 1973 through 1987 25 patients underwent mitral valve replacement in the first year of life for mitral stenosis and mitral regurgitation. The patients with mitral stenosis included two with mitral arcade, two with supravalvular mitral stenosis with hypoplastic mitral valve, and one with parachute mitral valve. Included in the group of patients with mitral regurgitation were 12 with atrioventricular canal defect, six with chordal and leaflet defects, one with Marfan's syndrome, and one with bacterial endocarditis. Prostheses included 12 Bj?rk-Shiley (17 mm), seven St. Jude Medical (19 mm in four, 21 mm in three), five stent-mounted dura mater valves (12 mm to 16 mm), and one porcine xenograft (19 mm). In four patients the valves were placed in the left atrium in a supraannular location. There were nine operative (atrioventricular canal defect seven, mitral regurgitation two) and five late (atrioventricular canal defect four, mitral stenosis one) deaths, giving actuarial 1- and 5-year survival rates of 52% and 43%, respectively. All 6 patients with tissue valves died; the four with supraannular mitral valve replacement survived. Since 1983 operative mortality has been reduced to 0% (70% confidence limits 0% to 24%). Nine patients required a second mitral valve replacement for prosthetic stenosis 5 to 69 (mean 30) months after the original mitral valve replacement (one operative death). Because of improvements in repair of atrioventricular canal defect in infancy, the need for mitral valve replacement at atrioventricular canal defect repair has decreased. Although valvuloplasty has been advocated for repair of congenital mitral valve disease and is applicable in some infants with mitral regurgitation, mitral valve replacement is frequently unavoidable for congenital mitral disease and can now be accomplished at a low operative risk, even when the prosthesis has to be positioned supraannularly.     

Surgical repair of double-orifice of the mitral valve in cases with an atrioventricular canal defects

OBJECTIVE: A Double-orifice in the mitral valve is an uncommon congenital cardiac lesion which occurs as an isolated anomaly or in association with other cardiac malformation. This report deals with our surgical experience of a double-orifice of the mitral valve in cases with an atrioventricular canal defect. PATIENTS AND METHODS: From 1991 through 1999, ten patients were diagnosed to have a double-orifice of the mitral valve at Shizuoka Children's Hospital. Each patient had associated major cardiac malformations, among which atrioventricular canal defect underwent surgical management, with five of these undergoing complete correction with or without previous pulmonary artery banding. Of these 10, the five cases were enrolled in this study. Two of these had a complete type, and the other three had a partial type. The cleft in the left-sided atrioventricular valve was closed partially in four and left untouched in one. Bridging tissue, when present, was left intact. There was no regurgitation from any accessory orifice and no repair for an accessory orifice was needed. RESULT: There was no late death and no replacement of the valve with prosthesis. During follow-up ranging from 1 to 4 years, none of the patients developed severe stenosis or progressive regurgitation in the left-sided atrioventricular valve. CONCLUSION: Meticulous surgical management of a double-orifice in the mitral valve in association with atrioventricular canal defect an achieve an acceptable midterm result without developing severe dysfunction in the left-sided atrioventricular valve.     

Surgical treatment of double-orifice mitral valve in atrioventricular canal defects. Experience in 25 patients

Double-orifice mitral valve is an uncommon but surgically important condition. The experience in 25 cases of double-orifice mitral valve associated with atrioventricular canal defects was reviewed. This constituted 4.3% of the 581 cases of atrioventricular canal defects operated upon between 1961 and July, 1984. The combined mitral orifice area ranged from 85% to 91% of normal in those patients whose valves were sized intraoperatively. Ten associated cardiac defects were repaired in six patients. Of 23 patients having cleft mitral valve, 21 had partial closure of the cleft. There was one operative death (4.0%), which occurred early in the series in a patient in whom the tissue bridge was severed and massive mitral regurgitation resulted. In the remaining 24 patients the tissue bridge was left intact, and all survived operation. No patient had clinically significant mitral stenosis during a follow-up of 1 to 14 years (mean 4.9 years). Two patients (8%) developed progressive mitral regurgitation and required mitral valve replacement 3 and 11 years postoperatively. One of these patients died and a second death occurred suddenly 2 years following operation. All survivors are in Functional Class I or II. The noncleft orifice of a double-orifice mitral valve usually is competent and rarely requires closure. The cleft, because it constitutes a type of parachute (single papillary muscle) valve, should be closed partially so as to relieve valve incompetence without causing undue stenosis. The incidence of late development of mitral regurgitation is similar to that of atrioventricular canal without double-orifice mitral valve. Repair of atrioventricular canal associated with double-orifice mitral valve can be achieved with a low operative mortality and excellent late results.     

Surgical treatment of atrioventricular canal defect.

Fifty-nine patients with congenital anomalies of the atrioventricular canal underwent operation and all survivors were followed up. In 42 patients with partial atrioventricular canal defects, ten had preoperative congestive heart failure. Three, or 7.1%, died of endomyocardial fibroelastosis, high pulmonary vascular resistance, and severe mitral regurgitation. A fourth patient later died of Wolff-Parkinson-White syndrome and fibrilation. Reoperations in five patients were all successful. No patients had persistent atrioventricular blocks, and all patients are asymptomatic. Two of these subjects continue to receive digoxin therapy, and one of them is believed to have substantial mitral insufficiency. Of the 17 patients who had complete atrioventricular canal defects, 13 had a divided common anterior leaflet attached to the septum by chordae tendineae, and four had undivided and unattached anterior leaflets. Two had previously undergone pulmonary banding, and nine were treated for congestive heart failure. Six died after operation. There were no reoperations. No patient presently has required a pacemaker. Two subjects have persistent cardiomegaly.     

Unusual mitral valve abnormalities complicating surgical repair of endocardial cushion defects

A review of 155 cases of surgically repaired endocardial cushion defects revealed 16 patients (10%) with additional unusual mitral valve abnormalities that complicated the surgical procedure. Eight patients had accessory mitral valve tissue that connected the anterior and posterior leaflets to form a double-orifice valve (Group I). In four (50%), the lesion was associated with intermediate atrioventricular canal and small left ventricle; all four died following repair. In the other four, it was associated with ostium primum defect; all survived and are well. A single papillary muscle in the left ventricle was present in six patients (Group II). Two had intermediate atrioventricular canal and both died postoperatively. The other four had complete endocardial cushion defect and three are well following the operation. Perforation of the valve leaflets was present in two patients with ostium primum (Group III). Both patients are well postoperatively. Modification of the surgical technique is required to effect satisfactory repair. The bridge connecting the posterior and anterior leaflets of the mitral valve should be left undisturbed. Otherwise, severe regurgitation may result. In patients with single papillary muscle and complete atrioventricular canal, repair may be accomplished by borrowing from the tricuspid portion of the anterior leaflet, rotating that part posteriorly, and partially closing the cleft. Small perforations of the mitral leaflet do not require closure and do not result in regurgitation. Echocardiographic and angiographic delineation of these abnormalities and thorough intraoperative exploration are important in avoiding pitfalls at the time of repair.     

Partial atrioventricular canal defect: the early and late results of operation

Thirty-five patients underwent repair of a partial atrioventricular canal defect and have been followed for a mean of 8 years. Seventeen patients had either moderate or severe mitral incompetence prior to operation. The mitral valve was treated as a bileaflet structure. The cleft in its anterior leaflet was closed in each patient in whom the valve was incompetent. The septal defect was closed with pericardium, and the coronary sinus was left on the right atrium. Two patients required reoperation, 1 to close a recurrent septal defect and 1 to replace a mitral valve that had been competent for the first several years after the initial repair. All 33 survivors remain in New York Heart Association Functional Class I. Nineteen patients have no mitral incompetence, and the remaining 14 have only mild incompetence. These data demonstrate that excellent late results from repair of partial atrioventricular canal are possible when the mitral valve is managed as a bileaflet structure.     

二尖瓣脱垂并关闭不全的外科修补

目的：总结二尖瓣脱垂的外科修复经验,方法：对44例二尖脱垂患者的临床资料进行回顾分析。44例患者中风湿性2例,非风湿性42例（22例合并先天性心脏病）,关不全中度24例,重度20例,腱索断裂或缺如12例,腱索过长32例,其中多根腱索过长6例,治疗行腱索移植10例,健索缩短25例（多根腱索短6例）,人工腱索1例,瓣叶折叠3例,瓣叶切除5例,同时行瓣裂缝合8例,瓣环成形28例（后环缝缩14例）,结果：结果：全组无手术死亡病例,1例风湿性患者术后1个月发生左心房血栓再次手术行瓣膜替换,二尖瓣功能正常34例（77．8％）,基本正常6（13．6％）,残留轻至中度关闭不全3例（6．8％）,随访1－18例（平均6．5年）,效果良好,结论：外科修复治疗二尖瓣脱垂是一种安全有效的手术方法。