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     

儿童完全性房室通道外科治疗16例

１６例完全性房室通道男女各８例，年龄８月～８岁，体重６～２０ｋｇ．中、重度肺高压各６例。ＲａｓｔｅｌｌｉＡ型１４例，Ｂ及Ｃ型各１例。均行外科治疗，早期死亡１例，医院死亡率６％；晚期死亡２例。存活１３例中，１２例（９２．３％）随访２月～４年，恢复情况良好。强调本病须尽早手术治疗、术中牢固修补ＶＳＤ和原发孔ＡＳＤ及恢复二尖瓣功能的重要性。对术后肺高压处理特点进行讨论。     

先天性心脏病术后完全性房室传导阻滞的防治

目的探讨１７５４例先天性心脏病直视术后１０例完全性房室传导阻滞（ＣＡＶＢ）的发生原因和转归，提出正确的防治方案。方法手术中立即发生ＣＡＶＢ８例；其中６例立即再次阻断主动脉重新修补，２例使用心外膜起搏和异丙肾上腺素处理，其中１例术后第７天再次手术重新修补缺损；手术后３～４天由于ＣＡＶＢ发生心源性昏厥２例，均安置临时心内膜起搏器。结果全组患者无住院死亡，全部康复出院未留置永久性心内膜起搏器。结论ＣＡＶＢ的发生与房间隔缺损（ＡＳＤ）、室间隔缺损（ＶＳＤ）的位置和手术操作有关；正确认识房室传导组织的解剖是防止损伤的关键，术中一旦发生ＣＡＶＢ应果断拆除原修补缝线，重新修补缺损；术后安置心外膜或心内膜临时起搏器对防止心源性昏厥非常有效。     

完全性房室隔缺损的外科治疗

目的 总结完全性房室隔缺损的手术方法和临床经验，以提高手术疗效。方法 27例完全性房室隔缺损患者，年龄88天～24岁。合并右心室双出口、肺动脉瓣狭窄和镜像右位心1例，动脉导管未闭1例，继发孔型房间隔缺损1例，左上腔静脉残留2例，重度肺动脉高压13例，中度肺动脉高压14例。手术均采用双补片法修补，室间隔缺损采用Gore—Tex补片修补，房间隔缺损采用自体心包补片修补。结果 术后早期死亡2例，其中1例死于脑出血，1例死于右侧心力衰竭。所有患者术后均为窦性心律，无完全性房室传导阻滞。随访23例，随访时间2个月～10年，均为窦性心律，无死亡。复查心脏彩色超声心动图提示：二尖瓣轻度反流6例，中度反流2例，心功能均较术前增强。结论 完全性房室隔缺损患者早期行手术治疗，防止肺血管阻塞性病变的发生、发展，术中注意防止左心室流出道狭窄，矫治二尖瓣反流是提高手术疗效的关键。     

主动脉瓣上狭窄的外科治疗

报告１３例主动脉瓣上狭窄（ｓｕｐｒａｖａｌｖｕｌａｒａｏｒｔｉｃｓｔｅｎｏｓｉｓ，ＳＶＡＳ）病例的治疗。其中局限型１１例，弥漫型２例。合并的心血管畸形包括：主动脉瓣狭窄５例，主动脉瓣反流２例，主动脉瓣环发育不全２例，冠状动脉畸形６例，动脉导管未闭１例，多发肺动脉狭窄１例，肺动脉瓣上隔膜１例，头臂动脉畸形５例。１３例病人中，９例行狭窄解除术，１例用同种升主动脉行心尖至胸主动脉架桥术，１例单纯动脉导管结扎术，２例未手术。结果手术早期死亡２例，晚期死亡１例。作者认为ＳＶＡＳ手术治疗的要点为：（１）充分解除主动脉瓣上狭窄；（２）避免一切影响冠状动脉灌注的因素；（２）妥善处理并存的主动脉瓣病变。     

活性同种带瓣主动脉补片重建法乐四联症右室流出道

为评价活性同种主动脉（ＣＶＡＨ）补片重建法乐四联症（ＴＯＦ）右室流出道（ＲＶＯＴ）的临床效果。以保留无冠瓣及二尖瓣大瓣的ＣＶＡＨ重建ＲＶＯＴ４０例。关胸前测定各心腔压力并行彩色多普勒超声随访。结论：关胸前测压，ＣＶＡＨ组与对照组右室收缩压／左室收缩压（ＲＶＳＰ／ＬＶＳＰ）分别为０．４９±０．０９和０．６２±０．１３（ｔ＝４．８０，Ｐ＜０．００１）；ＲＶＳＰ分别为６．３０±１．３６ｋＰａ和７．８８±２．３４ｋＰａ（ｔ＝３０．７７，Ｐ＜０．００１）；右室—肺动脉压力阶差（ＲＶ—ＰＡＰＧ）分别为０．９７±０．７９ｋＰａ和３．２９±１．９７ｋＰａ（ｔ＝６．６９，Ｐ＜０．００１）；中心静脉压（ＣＶＰ）分别为１．３２±０．２４ｋＰａ和２．０６±０．５１ｋＰａ（ｔ＝８．２１，Ｐ＜０．００１）。ＣＶＡＨ组血流动学力效果明显优于对照组。ＣＶＡＨ组手术死亡１例（２．５％），余３９例全部随访２８．３±１８．１（６～６６）个月，无远期死亡，心功能恢复优良。多普勒超声显示术后肺动脉反流轻微，ＣＶＡＨ无失功及钙化。结论：带瓣ＣＶＡＨ是理想的ＲＶＯＴ重建材料，血流动力学效果优良，可以最大限度加宽ＲＶＯＴ和减轻肺动脉反流。     

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。     

先天性永存动脉干的外科治疗

报道１９８９年１月至１９９３年１２月外科治疗先天性永存动脉干６例。按ＶａｎＰｒａａｇｈ分型，Ｉ型２例、ＩＩ型４例。Ｉ型中伴主动脉弓中断１例，按Ｃｅｌｏｒｉａ等分类为Ａ型。伴其它心内畸形３例。手术在中度低温体外循环下进行，其中１例采用深低温停循环技术处理主动脉弓中断。手术５例成功，随访７个月～４年８个月，疗效良好。１例术后肺动脉高压并发感染，自动出院后失访。作者主张永存动脉干一旦确诊宜早期手术。并对Ｉ型、ＩＩ型的手术方法，肺动脉高压的治疗作了分析探讨。     

矫正型大动脉转位的手术纠治

目的回顾性分析１７例矫正型大动脉转位的手术纠治，通过两种不同方法的比较，以提高手术成功率，防止完全性房室传导阻滞的发生。方法室间隔缺损修补：１０例经右心房二尖瓣或解剖左心室切口，缝针经室间隔缺损置于室间隔左侧面；７例经解剖右心室切口缝针直接缝于室间隔左侧面。结果经解剖左心室修补的完全性房室传导阻滞发生率为４０％，而经解剖右心室的发生率为１４％。１４例伴不同程度的肺动脉流出道梗阻，除１例作瓣交界切开，余１３例均采用同种带瓣主动脉管道连接。手术死亡２例，死亡率１１．７６％。结论矫正型大动脉转位的室间隔缺损修补经解剖右心室修补为好，肺动脉高压是手术死亡的主要原因     

心内直视术中鱼精蛋白毒性反应2例

例１女，６岁。室间隔缺损（ＶＳＤ）并肺动脉高压。１９４年１１月在体外循环下手术。ＶＳＤ为膜周型，直径１．５ｃｍ，补片修补。术中阻断循环４０分钟，开放循环后心跳有力，平均动脉压１０ｋＰａ（１ｋＰａ＝７．５ｍｍＨｇ），经锁骨下静脉给予鱼精蛋白（批号：９３...     

Intraoperative evaluation of atrioventricular septal defect repair by color flow mapping echocardiography

Despite improving survival rates after repair of atrioventricular septal defect, many patients require reoperation because of postoperative heart failure. We used intraoperative color flow mapping echocardiography to assess the results of surgical repair of atrioventricular septal defect in 19 consecutive patients and compared those findings with results three to five days and 3 to 11 months after repair. There was close correlation between intraoperative and postoperative color flow mapping echocardiography in estimating the presence and severity of left atrioventricular valve regurgitation. All patients survived surgical repair, but in 4 (21%), postoperative congestive heart failure due to left atrioventricular valve regurgitation developed. The need for reoperation was significantly correlated with the severity of left atrioventricular valve regurgitation (r = 0.68) as estimated by intraoperative echocardiography and preoperative aberrancies in the atrioventricular valve (r = 0.68). Age, weight, additional congenital heart disease, preoperative pulmonary vascular resistance, preoperative atrioventricular valve regurgitation, and postoperative mean pulmonary arterial or left atrial pressure were not significantly correlated with the need for reoperation. Intraoperative color flow mapping echocardiography can accurately predict the development of early postoperative heart failure and subsequent reoperation after surgical repair of atrioventricular septal defect.     

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。     

Long term follow-up of atrioventricular valve function after repair of atrioventricular septal defect.

To elucidate the factors which are associated with early and late operative results of atrioventricular septal defects, 102 consecutive patients who underwent reparative operation of atrioventricular septal defects (AVSD) in our institution since 1968 were studied. Our operative technique was basically the same through this period. That is the two patch method in its complete form (the so-called Shirotani's method) and preferential use of cleft closure supplemented with Kay-Reed-Wooler type annuloplasty. Early mortality (< 30 days) rate was 18.6%. More than 80% of the early deaths were not related to atrioventricular valve malfunction. Non-complete closure of the cleft, high preoperative pulmonary vascular resistance, deficient atrioventricular valve, and complete form showed independent correlations with early mortality in multiple logistic regression analysis. For operative survivors, event-free survival curves, for atrioventricular valve related reoperations were drawn for various factors. Two late deaths and 3 late atrioventricular-valve-related reoperations occurred. The event-free actuarial survival for operative survivors at 5, 10, and 20 years were 97.0%, 89.4%, and 89.4%, respectively. The survival analysis revealed that preoperative high pulmonary vascular resistance, preoperative severe atrioventricular regurgitation, and preoperative large cardiothoracic ratio in chest radiogram were related with late event occurrence. Higher early mortality in our series may be attributable to relatively advanced pulmonary vascular occlusive disease rather than post repair atrioventricular valve malfunction. On the other hand, our late results were rather good. We concluded that the Shirotani's method and preferential use of cleft closure supplemented with Kay-Reed-Wooler type annuloplasty was effective for most of atrioventricular valves in atrioventricular septal defects. Early surgical intervention before pulmonary vascular disease progression or atrioventricular valve regurgitation development is also important.     

Surgically created double-orifice left atrioventricular valve: a valve-sparing repair in selected atrioventricular septal defects

OBJECTIVES: Some features of the left atrioventricular valve (large mural leaflet, dystrophic tissue) represent a challenge for repair of atrioventricular septal defects without postoperative regurgitation. A retrospective study was conducted to evaluate the results of surgically creating a double-orifice left atrioventricular valve in such circumstances. Clinical results were analyzed according to valvular and subvalvular left atrioventricular valve measurements in pathologic specimens with atrioventricular septal defects. METHODS: Among 157 patients operated on for atrioventricular septal defect since October 1989, 10 patients underwent primary repair (n = 8) or reoperation (n = 2) by this procedure. Median age at repair was 3.3 years (0.1-33 years). Anatomic types were complete (n = 3), intermediate (n = 5), and partial (n = 2). Preoperative moderate to severe left atrioventricular valve regurgitation was present in 6 patients. After the repair (two-patch technique in complete atrioventricular septal defect, cleft closed in each case), these 10 patients were found to have moderate to severe residual regurgitation not amenable to repair by annuloplasty. The top edge of the mural leaflet was anchored to the facing free edge of the cleft. RESULTS: No hospital death or morbidity was observed. Left atrioventricular valve regurgitation was absent or trivial (8 patients) and mild (2 patients). Color-coded echocardiography did not show significant left atrioventricular valve stenosis. The mean diastolic pressure gradient across the left atrioventricular valve was 3.2 +/- 1.1 mm Hg (1.4-4.5 mm Hg). At a median follow-up of 72 months (6-91 months), there was 1 late death, unrelated to left atrioventricular valve malfunction, due to pulmonary vascular obstructive disease. Left atrioventricular valve regurgitation did not increase over time, except in 1 patient in whom regurgitation recently progressed from mild to moderate. At rest, the mean diastolic pressure gradient across the left atrioventricular valve was 3.8 +/- 2.9 mm Hg (1.5-11.2 mm Hg). One child had an early moderate stenosis without pulmonary hypertension. Studies on pathologic specimens (n = 34) indicated that long chordal lengths and large mural leaflet size are essential independent anatomic features to assess its feasibility. CONCLUSIONS: Surgical creation of a double-orifice left atrioventricular valve is an effective additional procedure for repair of atypical cases of atrioventricular septal defect. The operation may decrease the need for reoperation or left atrioventricular valve replacement.     

Annuloplastic reconstruction for common atrioventricular valvular regurgitation in right isomerism

Two patients who had common atrioventricular valvular regurgitation associated with right isomerism, univentricular heart of the right ventricular type, transposition of the great arteries, pulmonary stenosis, and both systemic and pulmonary venous anomalies underwent common atrioventricular valvular annuloplasty. They also underwent bilateral, bidirectional cavopulmonary shunt. A Carpentier's ring was used in patient 1 and a polytetrafluoroethylene tube was used in patient 2 to reduce the diameter of the common atrioventricular annuli. Postoperative catheterization confirmed complete elimination of regurgitation of the common atrioventricular valve in both patients.     

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.     

合并单侧肺动脉缺如先天性心脏病的外科治疗

9例合并单侧肺动脉缺如的先天性心脏病,其中7例为法乐四联症合并单侧肺动脉缺如,系重症先天畸形。手术均采用带瓣的补片疏通流出道。围术期处理以纠治低心输出量为重点。     

Surgical Treatment of Acute Aortic Regurgitation in Infective Endocarditis

During a six-year period 15 consecutive patients with isolated aortic regurgitation due to infective endocarditis were encountered. None had prior significant aortic valve disease. Elective valve replacement was performed in 13 patients; emergency operation was needed in only 1 patient because of intractable pulmonary edema. One patient died suddenly from acute heart block while undergoing medical treatment.Preoperative cardiac catheterization studies in 10 of the 14 patients revealed gross elevations of left ventricular end-diastolic pressure, pulmonary hypertension, depressed cardiac output, and 3 to 4+ aortic regurgitation. There was 1 early and 1 late postoperative death, both due to systemic embolism, yielding an overall surgical mortality of 14%. After a mean follow-up of 18 months, 10 of the 11 patients are in New York Heart Association Functional Class I.Most patients with acute aortic regurgitation secondary to infective endocarditis have clinically observable congestive heart failure and will eventually require valve replacement. If congestive heart failure can be stabilized by a medical regimen, a course of antibiotic therapy can be administered and elective valve replacement can be performed. The time taken for preoperative antibiotic treatment is not associated with irreversible myocardial damage sufficient to influence the results of operation.     

Complete repair of atrioventricular septal defect

We report our experience with 103 consecutive children who underwent repair of complete atrioventricular septal defect between 1971 and 1990. Ninety-one patients were less than 18 months old (mean age, 6.2 months; mean weight, 5.8 kg) and were repaired using deep hypothermia and circulatory arrest. There were 15 perioperative deaths. Twelve patients were older (mean age, 40.2 months; mean weight, 18.9 kg) and were repaired using moderate hypothermia and cardiopulmonary bypass. There were two perioperative deaths. Repairs were performed with the single-patch technique. Four younger patients required repeat repair to control residual mitral regurgitation. Two of the older children required late reoperation to replace one or both atrioventricular valves. Three younger children underwent pulmonary artery banding initially; 1 died after complete repair. Three older children underwent initial pulmonary artery banding; 2 died at definitive repair, and the survivor required pulmonary artery reconstruction, which was repeated subsequently. Since 1977 our policy has been to perform primary definitive repair whenever possible. Two patients died late from unrelated causes. At the most recent follow-up the majority of patients had no or minimal symptoms. We continue to advocate primary definitive repair whenever possible using the single-patch technique in symptomatic patients with complete atrioventricular septal defect.