法洛四联症合并完全房室间隔缺损手术方法探讨

目的探讨法洛四联症合并完全房室间隔缺损(TOFAVSD)心内矫治的手术方法和效果。方法1985年1月至2004年10月,TOFAVSD心内矫治术16例中男7例,女9例;年龄2～16岁,平均(8.1±3.8)岁。采用右房、右心室纵切口,前7例采用3块补片;后9例用2块补片修复房室间隔缺损,左侧房室瓣裂隙采用间断缝合,右心室流出道用跨瓣补片加宽。结果术后早期死亡4例,前7例死亡3例;后9例死亡1例。死因为严重低心排血量综合征3例,灌注肺1例。8例随访2个月至13.5年。心功能I或II级,无临床症状。结论采用右房、右心室纵切口,2块补片修复房室间隔缺损,常规间断缝合左侧房室瓣裂隙,跨瓣补片加宽右室流出道可取得较良好的疗效。     

完全性房室间隔缺损合并法乐四联症的手术矫正

目的　报告完全性房室间隔缺损合并法乐四联症的外科治疗。　方法　 6例患者 ,年龄 3～ 9岁 ,均施行手术根治 ,经右房 右室切口修补VSD ,房室间隔缺损采用两块补片技术修复 ,左房室瓣裂隙的修复均采用间断缝合 ,右室流出道均用带单瓣的补片跨瓣加宽。　结果　全组 6例术后出现低心排综合征、多器官衰竭、灌注肺并发症各 1例 ,早期死亡 1例为多器官衰竭者 ,5例长期存活。术后随访 6个月～ 5 5年 ,1例有轻度左房室瓣关闭不全。心功能Ⅰ级 4例 ,Ⅱ级 1例。　结论　完全性房室间隔缺损合并法乐四联症可应用 2块补片法行手术根治 ,左房室瓣瓣裂的常规修补可降低其关闭不全的发生率 ,大部分患者术后心功能可获得良好的改善。     

部分型房室间隔缺损二尖瓣处理策略

目的总结部分型房室间隔缺损（PAVSD）患者外科手术的近远期疗效,探讨二尖瓣处理方法。方法回顾性分析1990年1月至2008年12月于第二军医大学长海医院行外科治疗PAVSD患者118例的临床和随访资料,男51例,女67例;年龄7个月~62岁（28.5±12.6岁）。均有原发孔型房间隔缺损和不同程度的二尖瓣前瓣裂隙,其中二尖瓣中重度反流78例,三尖瓣中重度反流56例。手术均在中度低温体外循环下进行。术后通过门诊和电话随访。结果术后早期死亡2例,病死率1.69%。发生其他严重并发症为再次气管内插管5例,左心辅助1例,Ⅲ°房室传导阻滞1例。术后随访103例,随访时间3个月~18年（91.2±25.6个月）。再次手术8例,距第一次手术时间为5 d~18年（10.5±5.6年）。再次手术死亡1例。随访生存的102例患者生活质量均有明显改善。二尖瓣无反流70例,微量和轻度反流17例,中度4例。行二尖瓣置换术患者瓣膜功能良好,无抗凝相关并发症。心房颤动19例,Ⅰ°房室传导阻滞21例,频发性房性早搏4例。结论二尖瓣修复是PAVSD矫治手术成功的关键,应综合应用二尖瓣成形修复的方法,尽量消除二尖瓣反流;术后二尖瓣再反流是再次手术的主要原因,对中重度二尖瓣反流患者应定期随访和及时手术。     

改良单片法矫治儿童完全型房室间隔缺损单中心应用

目的总结应用改良单片法(modified single-patch,MSP)矫治儿童完全型房室间隔缺损(complete atrioventricular septal defect,CAVSD)的相关经验。方法回顾性分析2009年6月至2017年12月间在我中心采用MSP技术行CAVSD双心室矫治术141例患儿的临床资料,其中男62例、女79例,手术时中位年龄6(3,11)个月,中位体重5.8(4.5,7.0)kg。Rastelli分型:A型116例,B型14例,C型11例。合并Down’s综合征15例。记录体外循环时间、主动脉阻断时间、房室瓣反流等相关临床数据。结果术后17例患者出现重度左侧房室瓣反流(left atrioventricular valve regurgitation,LAVVR),6例患者出现重度右侧房室瓣反流(right atrioventricular valve regurgitation,RAVVR)。末次随访5例患者出现重度LAVVR,1例患者出现重度RAVVR。随访期间左室流出道梗阻(left ventricular outflow tract obstruction,LVOTO)1例,末次随访无LVOTO发生。早期死亡5例,随访死亡2例。再手术患者12例,距首次手术的中位间隔时间为268(8,1270)d。结论利用MSP技术外科矫治儿童CAVSD的预后良好,改善了患者术后死亡率和房室瓣反流程度。     

完全型房室间隔缺损的外科治疗

目的　总结完全型房室间隔缺损外科治疗的经验。方法　16例完全性房室间隔缺损患儿,平均年龄(1.2±0 .9)岁,平均体重(6.8±3 )kg。其中10例伴有Down综合征,1例合并法洛四联征。术前超声心动图显示房室瓣轻度反流12例,中度反流3例,重度反流1例。行单片法修补10例,双片法修补6例。结果　术后恢复顺利,无围手术期死亡。除1例患儿于出院4个月后因肺部感染合并心衰死亡外,余随访0 .3～5 .2年,经超声心动图检查显示房室瓣功能良好,未见明显反流。结论　完全型房室间隔缺损患者早诊断,早手术,可获良好疗效。     

房室间隔缺损的外科治疗

目的总结43例房室间隔缺损的手术方法和临床疗效。方法回顾总结2008-01—2015-12间43例房室间隔缺损矫治手术的临床资料。结果本组患者中术后6个月内死亡2例,其中1例死于重度低心排出量综合征,1例死于重度肺部感染。其余患者均获随访6个月~7 a,无远期死亡。2例二尖瓣中度反流患者反流加重。结论细致的瓣膜成形手术是保证治疗效果的关键,避免残余漏和传导阻滞对获得较好预后意义重大。     

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。     

法洛四联症合并完全性房室隔缺损的外科治疗

目的探讨法洛四联症合并完全性房室隔缺损外科治疗经验。方法回顾性分析9例患者的临床资料,其中完全性房室隔缺损A型3例,C型6例;均有右心室流出道漏斗部狭窄,6例伴明显的肺动脉瓣及瓣环狭窄。术前共同瓣轻度反流7例,中度反流2例。1例行中央体肺分流术,1例行心外管道全腔静脉-肺动脉吻合术,其余7例均行一期根治手术,其中跨肺动脉瓣环5例。结果9例患者死亡3例;1例婴儿术后2d脱机,后因低氧再次插管呼吸机辅助呼吸40d脱机,5例在术后1d脱机,呼吸循环功能良好,顺利康复出院。随访1个月-4年,4例有轻度二尖瓣反流,无残留心脏畸形。结论法洛四联症合并完全性房室隔缺损手术的关键在于确切修补室间隔缺损、完善修复二尖瓣、防止左心室流出道狭窄及Ⅲ°房室传导阻滞。     

部分性房室管畸形外科治疗及疗效探讨

目的 探讨外科治疗部分性房室管畸形的手术方法及其疗效。方法 ４８例部分必室管畸形患者均在体外循环心内直视术下缝合二尖瓣大瓣裂缺及修补房间隔缺损,其中９例同时行瓣膜成形手术。结果 术后早期（３０天内）死亡２例,死亡率４．１７％,发生Ⅲ度房室传导阻滞２财次手术４例,术后３９例随访３个月～１２年,平均随访９年。结论经随访,手术后 瓣无反流或少量反流者长期疗效良好,中等量以上反流者长期疗效差。     

房室隔缺损的外科治疗

目的总结手术治疗房室间隔缺损的经验.方法回顾1991年5月至2002年9月接受手术修补的30例房室隔缺损患者的病例资料,其中部分型23例,完全型5例,过渡型2例,分别行房、室间隔缺损补片修补术,二尖瓣和/或三尖瓣缝合矫治术.结果术后死亡2例,1例为肺动脉高压,死于重度低心排血量综合征；另1例术后7天死于颅内出血.安置永久性心脏起搏器1例,长期存活28例,随访9个月～12年,临床效果良好.结论充分认识房室隔缺损的传导组织解剖特点,是防止心脏传导阻滞的基础,根据瓣膜畸形的特点决定手术方法是提高手术效果的关键.     

A novel repair for patients with atrioventricular septal defect requiring reoperation for left atrioventricular valve regurgitation.

OBJECTIVE: Left atrioventricular valve regurgitation (LAVVR) is the most frequent indication for reoperation following atrioventricular septal defect (AVSD) repair. We estimate from our experience that within 10 years of initial repair, 14% of patients undergoing repair of atrioventricular septal defect (AVSD) require reoperation for this complication. We have developed a novel leaflet augmentation technique for LAVVR which may avoid failure of conventional repair and/or the need for valve replacement. METHOD: The novel technique consists of insertion of a glutaraldehyde-treated autologous pericardial patch to augment the bridging leaflets of the atrioventricular valve. We describe the outcome of eight patients in whom this technique was used and compared them to 68 other patients with AVSD undergoing reoperation for LAVVR by either conventional repair (n=54) or valve replacement (n=14). RESULTS: There were no early deaths or major complications following patch repair. The mean follow-up is 2.3 years (range 1-8.5 years) during which there were no late deaths. Two patients underwent reintervention at 3.5 and 5 years after patch repair for LAVVR and were successfully rerepaired. Mild residual LAVVR was seen at last echocardiography in six patients and mild to moderate in two. These results compare favorably with the 68 patients who underwent conventional surgery. The 3-year freedom from reoperation was 86% for both repair groups. Dysplastic valve tissue appears to be a major risk factor for failure of conventional repair or for valve replacement. Failure of conventional valve repair led to valve replacement in six of seven patients. CONCLUSIONS: For patients with late LAVVR after AVSD repair, pericardial leaflet augmentation is durable and may avoid failure of conventional repair or valve replacement in patients with dysplastic valves.     

Results of Left Atrioventricular Valve Reoperations Following Previous Repair of Atrioventricular Septal Defects

Abstract   Objectives: We report results of left atrioventricular valve reoperations (LAVVR) following atrioventricular septal defect (AVSD) repair and examine variables predictive of outcome. Methods: Multiple demographics and operative variables were analyzed to determine factors affecting survival and reoperation. Results: Forty patients following partial (n = 9) or complete (n = 31) AVSD repair underwent 47 LAVVR (1992-2005). Median age was 0.87 years (24 days-7.7 years) at initial AVSD repair and 3.15 years (84 days-13.6 years) at subsequent LAVVR with median interval between AVSD repair and LAVVR of 1.76 years (1 day-12.9 years). First LAVVR included repair (n = 20) or replacement (n = 20). Operative mortality was 10% and five-year survival was 76 ± 6%. Significant risk factors were complete AVSD (p < 0.001), valve replacement (p < 0.001) for early death, and young age at time of LAVVR (p = 0.03) for late death. Five-year freedom from LAVV re-intervention was 100% for replacement versus 55 ± 13% for repair (p = 0.006). Overall, ejection fraction increased to 61 ± 3% versus 42 ± 2% preoperatively (p < 0.01), and left-ventricle end-diastolic dimension Z-score decreased to 0.05 ± 0.36 versus 3.1 ± 0.3 preoperatively (p < 0.01). Eighty-seven percent of children were in New York Heart Association class I/II at latest follow-up. Conclusions: LAVVR results in significant clinical improvement and lasting recovery in ventricular chamber function and size. Valve repair offers survival advantage and should be aggressively attempted; however, it is only achievable in 50% of cases. Valve replacement is necessary in cases associated with complex LAVV morphology or following repair failure. At intermediate follow-up, patients continue to be at risk of major valve-related morbidity, requirement for re-intervention, and cardiac death. (J Card Surg 2010;25:74-78)     

Double-orifice repair for left atrioventricular valve regurgitation in atrioventricular septal defect: report of two cases

We describe two patients who successfully underwent a surgically created double-orifice repair using the edge-to-edge repair for residual left atrioventricular valve (LAVV) regurgitation in an atrioventricular septal defects (AVSD). Both patients had previously received patch closure of the AVSD and partial closure of a cleft of the LAVV. Preoperatively, echocardiography showed a wide open cleft and remarkable dilatation of the LAVV annulus. Doppler study revealed severe regurgitation through the cleft and the central portion of the LAVV orifice and no intracardiac shunt. Postoperative echocardiography showed a remarkable decrease of the AV valve regurgitation to none or trivial levels without stenosis of the LAVV in both patients. Among several valve-sparing techniques, our experience suggests that the surgically created double-orifice repair is one of the most effective reparative procedures for LAVV regurgitation in AVSD.     

Reoperation for Left Ventricular Outflow Tract Obstruction After Repair of Atrioventricular Septal

Left ventricular outflow tract obstruction (LVOTO) is an important source of morbidity and mortality after repair of atrioventricular septal defect (AVSD). The intrinsic anatomy of the left ventricular outflow tract in AVSD is complex and predisposes to the development of LVOTO. LVOTO after repair of AVSD usually involves multiple levels and sources of obstruction, and surgical intervention must address each component of the obstruction. This includes fibromuscular obstruction, septal hypertrophy, and valve related sources of obstruction. Special attention is also directed to the anterolateral muscle bundle of the left ventricle, a well defined but under recognized feature of the left ventricular outflow tract in AVSD. It is present in all patients with AVSD, and resection of a hypertrophic anterolateral muscle bundle of the left ventricle should be incorporated in all operations for LVOTO after repair of AVSD. LVOTO after repair of AVSD has several unique features that must be taken into consideration to maximize outcome after surgical intervention. These include anatomic factors, technical aspects of surgical intervention, and proper selection of the operation used for relief of LVOTO.     

Subaortic Stenosis in Adult Patients With Atrioventricular Septal Defect

BackgroundPatients with atrioventricular septal defects (AVSD) are at risk for development of subaortic stenosis throughout their lifetime. The early and midterm outcomes of adults with AVSD undergoing primary operation or reoperation for subaortic stenosis remain unknown.MethodsAll patients aged 18 years or more with partial or complete AVSD who underwent operation for subaortic stenosis at our institution from 1992 to 2020 were retrospectively reviewed.ResultsNineteen patients were identified: 15 patients with partial AVSD (79%); 3 (16%) with complete AVSD; and 1 (5%) with transitional AVSD. Fifteen patients (79%) had previously corrected AVSD (median 8 years; interquartile range, 3.6-23.1) and 7 (37%) had previous repair of subaortic stenosis. The mechanism for obstruction included subaortic membrane (n = 19, 100%); septal hypertrophy (n = 11, 58%); anomalous papillary muscle, chordae, or left atrioventricular valve tissue (n = 9, 47%); and tunnel obstruction (n = 5, 26%). All patients underwent transaortic membrane resection, and septal myectomy was done in 18 patients (95%). There was no early mortality. During follow-up (median 8.3 years, maximum 28), survival was 100% at 5 years and 95% at 10 years. One patient required reintervention for subaortic stenosis 15 years after the operation at our institution.ConclusionsSurgical correction of subaortic obstruction in adult patients with AVSD can be accomplished with low morbidity and mortality. Subaortic stenosis can appear late after the initial repair of AVSD, and these patients remain at risk for recurrence after resection.     

Modified single-patch repair for atrioventricular septal defects results in good functional outcomes in the absence of deep ventricular septal defects

ObjectivesWe compared 2-patch repair (TP) with modified single-patch repair (MSP) for complete atrioventricular septal defects and evaluated their effect on the left atrioventricular valve (LAVV) competence. We also identified risk factors for unfavorable functional outcomes.MethodsThis retrospective study included 118 patients with complete atrioventricular septal defects who underwent intracardiac repair from 1998 to 2020 (MSP: 69; TP: 49). The median follow-up period was 10.4 years. The functional outcome of freedom from moderate or greater LAVV regurgitation (LAVVR) was estimated using the Kaplan–Meier method.ResultsThe hospital mortality was 1.7% (2/118) and late mortality was 0.8% (1/118). Eight patients required LAVV-related reoperation (MSP: 4; TP: 4) and none required left ventricular outflow tract-related reoperation. In the MSP group without LAVV anomaly, the receiver operating characteristic curve analysis revealed that the ventricular septal defect (VSD) depth was strongly associated with moderate or greater postoperative LAVVR, with the best cutoff at 10.9 mm. When stratified according to the combination of intracardiac repair type and VSD depth, the MSP-deep VSD (VSD depth >11 mm) group showed the worst LAVV competence among the 4 groups (P = .002). According to multivariate analysis, weight <4.0 kg, LAVV anomaly, and moderate or greater preoperative LAVVR were independent risk factors for moderate or greater postoperative LAVVR, whereas MSP was not a risk factor.ConclusionsPostoperative LAVVR remains an obstacle to improved functional outcomes. MSP provides LAVV competence similar to TP unless deep VSD is present. The surgical approach should be selected on the basis of anatomical variations, specifically VSD depth.     

The left atrioventricular valve in partial atrioventricular septal defect: management strategy and surgical outcome.

OBJECTIVE: To test the hypothesis that in patients with a partial atrioventricular septal defect (PAVSD) and a competent left atrioventricular valve (LAVV), sutures should be placed across the line of apposition of the superior and inferior bridging leaflets, septal commissure (SC), to prevent the development of regurgitation. Outcome of surgery and risk factors for the need for LAVV reoperation of patients with mild or no LAVV regurgitation (LAVVR) were evaluated. BACKGROUND: Controversy over management of the LAVV in PAVSD. METHOD: One hundred and forty seven children with PAVSD underwent surgical repair at the Royal Brompton Hospital between January 1983 and December 1999. Of this group, 21 (16.7%) had LAVVR of sufficient severity to require surgical intervention and were therefore excluded from analysis. The median age and weight at repair of those with mild or no LAVVR was 4.1 years and 15.4 kg. One hundred and eight had normal chromosomes, 13 Down syndrome and five other syndromes. The interatrial communication was closed using a pericardial patch in 62.7% and with synthetic material in the remainder. Intraoperative testing of LAVV competence was undertaken using saline injection into the left ventricle. In 80.9%, sutures were placed across the line of apposition of the left sided superior and inferior bridging leaflets partially to close the SC (sometimes incorrectly named the mitral valve cleft). RESULT: The overall hospital mortality was 3.2% (95% confidence interval (CI) 1, 8.4%), which did not differ statistically in the last 20 years. No specific risk factors for early death were identified. Eleven patients (8.7%, 95% CI 4.7, 15.4%) required reoperation, 10 for LAVV repair and 1 resection of subaortic stenosis. Univariate analysis of risk factors for LAVV reoperation were low weight, relatively small size LAVV, the presence of a small preoperative interventricular interchordal communication and duration of ventilation. Ten (9.8%) of 102 patients in whom SC was sutured required LAVV reoperation but none for 24 in whom the commissure was left alone. CONCLUSION: The hypothesis that in the absence of preoperative LAVVR it is necessary to place sutures in the SC has not been proven. We consider that in addition to preoperative cross sectional echocardiographic assessment of LAVVR intraoperative evaluation of LAVV function allows discrimination between those valves where sutures to the septal commissure are necessary and those where the valve can be left undisturbed.     

Atrioventricular septal defect complicated by right ventricular outflow tract obstruction. Analysis of risk factors regarding surgical repair

Eighteen patients with complete atrioventricular septal defect (AVSD) were found to have either associated tetralogy (n = 15) or pulmonary atresia (n = 3). Their pre- and post-operative course was reviewed focusing attention on risk factors for complete repair. These included right ventricular (RV) hypoplasia, identified by cineangiographic volume determinations in two cases (11%) and inadequate pulmonary artery size in one patient. In the 2 cases with RV hypoplasia and in one case, in whom all chordae of the AV valve were connected to a single right ventricular papillary muscle (AVSD type Rastelli B), complete repair was not feasible. In addition, angiography revealed coronary artery variations in 5/18 (28%), which did not preclude surgical repair. Palliative surgery was undertaken in 10 patients and complete repair carried out in five. Six of 18 patients died, 2 among those after complete repair.     

Repair of complete atrioventricular septal defects in patients weighing less than 5 kg

BACKGROUND: The aim of this study was to evaluate the impact of weight less than 5 kg at operation on mortality and morbidity in patients with atrioventricular septal defect (AVSDc) undergoing total correction. METHODS: Between January 1990 and December 2002, 190 consecutive patients with AVSDc underwent total biventricular correction. They were divided into two groups: group I (n = 64 patients weighing < 5 kg) and group II (n = 126 patients weighing > 5 kg). Associated major cardiac malformations were found in 49 (25.8%) patients. Associated left atrioventricular valve (LAVV) malformations were found in 35 (18.4%) patients. The mean follow-up time was 4.1 +/- 2.9 years (range 2 months-10.7 years). RESULTS: The in-hospital mortality in group I was 7.8% (5 patients) versus 8.7% (11 patients) in group II (p = 0.95). Major associated cardiac malformations (p < 0.001) and pulmonary hypertension (p = 0.006) were found to be strong predictors for poor postoperative survival. At discharge the mean LAVVR grade in group I was 1.45 +/- 1.2 versus 1.2 +/- 1 in group II (p = 0.13). The actuarial overall survival rates at 1, 3, 5, and 7 years were 96.5%, 92.5%, 91.5%, and 89% respectively and the actuarial overall reoperation free survival rates at 1, 3, 5, and 7 years were 95%, 87%, 84%, and 73%. Twenty-three patients underwent reoperation due to severe left atrioventricular valve regurgitation (LAVVR). Strong predictors for overall reoperation free survival were the operation year before 1995 (p < 0.001), postoperative LAVVR greater than or equal to 2 (p = 0.006), major associated cardiac malformations (p = 0.00034), associated LAVV malformations (p = 0.0044), and non or partial LAVV cleft closure (p = 0.012). The actuarial survival rates between patients weighing less than 5 kg versus patients weighing more than 5 kg were similar (p = 0.51); instead the overall reoperation free survival was significantly lower in patients weighing less than 5 kg (p = 0.022) according to the log-rank test. Weight less than 5 kg (p = 0.023, beta = -0.6) was one of the predictors for reoperation due to severe LAVVR in this series. CONCLUSIONS: We may conclude that in the current era repair of AVSDc can be carried out successfully in patients less than 5 kg, however, weight less than 5 kg at initial complete repair seems to be a predictor for late reoperation due to LAVVR. Suture separation at the cleft site or between the leaflets of the newly created mitral valve and the patch remain the main causes of postoperative LAVVR in patients weighing less than 5 kg.