食管超声心动图评价围术期儿童法洛四联症的右室流出道

目的 运用经食管超声心动图（TEE）对儿童法洛四联症（TOF）的右室流出道（RVOT）情况进行围术期评价。方法 32例TOF病儿体外循环前后分别进行TEE检查，年龄4个月～15岁8个月，平均4．0岁。结果 术前32例均TEE诊断为TOF。31例行根治术，1例行B-T分流术。RVOT扩大补片15例；跨肺动脉瓣环补片（TAP）16例，包括4例切除肺动脉瓣，2例带瓣移植物重建RVOT。术后TEE诊断RVOT残余梗阻5例（16．1％），与是否采用TAP无关（X^2=1．115，P=0．291）。肺动脉反流（PR）16例（51．6％），采用TAP比扩大补片术后更易出现PR（X^2=14．212，P=0．000）。保留原肺动脉瓣可减轻术后PR程度（X^2=5．915，P=0．015）。带瓣移植物重建RVOT可减少术后PR（X^2=8．163，P=0．004）。结论 术前TEE可明确诊断TOF，术后TEE可评价不同手术方法对于RVOT的效果。     

Simultaneous enlargement of the pulmonary annulus and the pulmonary cusp with autologous pericardium in right ventricular outflow tract reconstruction

BACKGROUND: Surgical repair of obstructive lesions of the right ventricular outflow tract (RVOT) commonly creates pulmonary valve incompetence, which continues to stimulate research for the optimal materials and surgical techniques to reconstruct RVOT. In this study, we present the early results with simultaneous enlargement of the pulmonary annulus and the pulmonary cusp with a transannular patch of autologous pericardium in RVOT reconstruction. PATIENTS AND METHODS: From January 2003 to December 2005, the surgical technique of simultaneous enlargement of the pulmonary annulus and the pulmonary cusp was used in 32 patients who had complex congenital heart anomalies with pulmonary artery hypoplasia. The functional status of the patients was followed up in the cardiologic clinic of our institute. The motion of the newly constructed valve and the degree of pulmonary insufficiency were evaluated by echocardiography before discharge and at 2-6 months, 12 months, and 36 months postoperatively. RESULTS: Early death occurred in one patient (3.1%). Postoperative complications occurred in six patients but they recovered uneventfully. During the follow-up, 28 of 31 operative survivors were in New York Heart Association functional class I without medication and the other three were in class II. Seventeen patients had no or trivial pulmonary regurgitation; mild regurgitation was present in 12 patients, and moderate regurgitation was seen in 2 patients. None of these patients needed reoperation and echocardiography showed good motion of the reconstructed valve. CONCLUSIONS: The surgical technique of simultaneous enlargement of the pulmonary annulus and the pulmonary cusp with a transannular patch of autologous pericardium is a safe, reliable, and effective way for RVOT reconstruction. Satisfactory early results have been achieved; however, long-term follow-up is necessary to determine the true value of this technique.     

右心室流出道的处理方法与法洛四联症矫治术后右心室功能的相关分析

目的 评价法洛四联症(TOF)右心室流出道(RVOT)处理方法与矫治术后右心功能变化的关系,以期提高矫治手术的中、远期效果.方法 2003年1月至2006年1月,125例TOF患儿接受矫治手术,其中男66例,女59例;手术年龄1～5岁,平均(3.4±1.1)岁;体质量7～26 kg,平均(15.2±5.7)kg.对照组选择20例同龄健康儿童.RVOT处理方法包括跨肺动脉瓣环补片加宽(M1组)67例,右心室漏斗部补片加宽(M2组)6例,经右心室切口非补片修复(M3组)18例,经右心房-肺动脉切口非补片修复(M4组)34例.采用脉冲多普勒(PDE)和组织多普勒超声心动图(TDI)评价右心室功能,选择三尖瓣环收缩期移位(TAPSE)、右心室心肌作功指数(MPI)作为右心室收缩功能指标,测定的三尖瓣瓣环处舒张早期峰速(Em),舒张晚期峰速(Am),计算Em/Am和E/Em比值作为右心室舒张功能指标.计量资料采用单因素方差分析,单因素分析有统计学意义的变量纳入logistic回归分析.计数资料采用x2检验.结果 术后5年随访检查心脏超声发现,M1和M2组的MPI和E/Em显著高于M3和M4组.单因素分析显示,术前Nakata指数、既往分流手术、手术方式、肺动脉瓣反流和术后QRS时间是影响术后MPI的危险因素;术前Nakata指数、手术方式、肺动脉瓣反流和术后QRS时间是影响术后E/Em的危险因素.二元logistic逐步回归分析结果显示,手术方式和术后QRS时间是影响术后MPI的独立因素;术后肺动脉瓣反流是影响术后E/Em的独立因素.结论 TOF术后右心室功能降低与RVOT处理方法有关,舒张功能的降低与术后肺动脉瓣的反流有明显的相关性,收缩功能的降低与手术中应用补片加宽右心室流出道和术后的QRS时间有明显的相关性.心脏超声多普勒技术在测定TOF术后右心室功能和评价术后中、长期疗效有重要的指导意义.     

法乐四联症右室流出道心肌超微结构特点及其临床意义

目的 研究不同年龄组法乐四联症（TOF）右室流出道心肌超微结构特点。方法 取31例TOF右室流出道心肌组织，按年龄分为1岁以下组、1～3岁组和3岁以上组，取12例其他心脏病患者右室流出道心肌作为对照组，分别用光学显微镜和透射电镜对心肌组织进行超微结构的观察。结果 1～3岁TOF组和3岁以上TOF组心肌肌原纤维排列方向紊乱，同向性丧失，大量小线粒体增生堆积，分布不均匀，基质密度增加，z线粗细不等、缺失、断裂；1岁以下TOF组超微结构改变较轻微。对照组可见心肌结构正常。结论 TOF右室流出道存在许多心肌超微结构异常的心肌细胞，TOF病人应尽量在1岁以内进行手术，可避免心肌超微结构变化对心功能的不良影响。     

Late results of total correction of tetralogy of Fallot in adults

Between 1966 and 1986, 30 patients underwent total correction of the tetralogy of Fallot. Preoperative presenting features were: dyspnoea on exertion, clubbing, cyanosis and polycythaemia. Twenty-six patients had one or more palliative procedures prior to definitive repair. Preoperatively, all patients had a significant gradient across the right ventricular outflow tract (mean gradient 70 ± 46 mmHg). Peak right ventricle to left ventricle systolic pressure ratio (pRV/LV) was 0.9 ± 0.2. A functioning Blalock Taussig shunt was ligated in 11 patients prior to the institution of cardiopulmonary bypass. All patients had a patch closure of the ventricular septal defect. An additional muscle bundle resection from the right ventricular outflow tract was performed in 15, pulmonary valvotomy in 6 and enlargement of the right ventricular outflow tract in 2 patients. There was a significant fall in pRV/LV ratio postoperatively (P < 0.05). There were 3 early and 2 late deaths. Mild right ventricular outflow tract obstruction has persisted in all survivors. Four patients have remained on antiarrhythmic drugs. Long term results after definitive repair were satisfactory in this group of adult patients who have survived due to palliative procedures performed during childhood.     

Triple valve replacement late after tetralogy of Fallot repair: Report of a case

Right ventricular failure after repair of tetralogy of Fallot (TOF) is associated with late mortality and morbidity. We report a case of tricuspid and mitral regurgitation with severe pulmonary regurgitation (PR) diagnosed 31 years after repair of TOF. The patient, a 48-year-old woman, presented with severe ventricular arrhythmia and exercise intolerance. She was treated successfully with triple valve replacement, following which her symptoms improved dramatically. She has remained well for 2 years postoperatively.     

Right ventricular outflow tract reconstruction for primary pulmonary artery sarcoma

Primary pulmonary artery sarcoma is extremely rare and a lethal tumor. Complete resection of the tumor and following adequate reconstruction is the only hope for cure and prolonged survival. We report a case of successful surgical excision of primary pulmonary artery sarcoma involving pulmonary trunk, pulmonary valve and right ventricular outflow tract wall, which were replaced with prosthetic valved conduit Postoperative histopathological diagnosis was leiomyosarcoma. The patient is well without any evidence of recurrence at 10 months after surgery.     

Realignment of the ventricular septum using partial direct closure of the ventricular septal defect in Tetralogy of Fallot

Objective: The aim is to describe our technique of partial direct closure of the ventricular septal defect (VSD) in Tetralogy of Fallot (TOF), and assess its influence on the realignment and remodeling of the left ventricular outflow tract. Methods: Between 2004 and 2010, 32 non-consecutive patients with TOF underwent a direct or partial direct closure of VSD. Median age and weight were 5.2 months and 6.7 kg, respectively. An approach through the right atrium was used in 30 patients and through the infundibulum in two patients. The conal septum was mobilized by transecting the hypertrophic trabeculae to facilitate the approximation of the VSD. The membranous part of the VSD was closed (in the later part of the series) with a small xenopericardial patch to avoid tension on the suture line traversing the area of risk to the bundle of His. Follow-up was complete, with a median duration of 46.9 (range 12–75.3) months. Results: The VSD could be closed successfully in all patients. A residual VSD was partly responsible for one early postoperative re-operation. There were no early or late deaths. At follow-up, all patients were in sinus rhythm. Three patients showed a small residual VSD. Thirty patients had none, one showed trivial, and one had mild aortic regurgitation. The left ventricular outflow showed a good realignment of the ventricular septum in all the patients. Conclusions: Partial direct closure of the VSD corrects the primary defect in TOF, that is, the malalignment of the septum. It results in a straight, wide open left ventricular outflow tract and brings better support to the aortic root.     

自制带单瓣管道重建右心室-肺动脉连接治疗复杂先天性心脏病

目的总结自制带单瓣管道重建右心室-肺动脉连接治疗复杂先天性心脏病的经验,探讨术中操作要点,评估该疗法的近中期结果。方法回顾性分析我科2006年1月至2018年6月利用自制带单瓣管道外科治疗的65例复杂先天性心脏病患者的临床资料。其中男42例、女23例,年龄5~23(9.9±4.2)岁,体重15~65(26.2±9.9)kg。根据所使用带单瓣管道将患者分为两组:带单瓣人工血管组19例,带单瓣牛心包管组46例。其中室间隔缺损(VSD)合并肺动脉闭锁(PA)48例,矫正性大动脉转位合并肺动脉狭窄10例,法洛四联症合并单支冠状动脉畸形5例,右室双出口合并肺动脉狭窄及单支冠状动脉畸形2例。结果术后早期死亡2例,均为VSD/PA行人工血管外管道根治患者。术中测右室流出道压差:人工血管组13~37(25.2±4.9)mm Hg,牛心包管组5~23(10.5±3.3)mm Hg。出院时心脏彩超显示外管道内无明显反流。术后失访1例,随访时间8个月至13年。随访期间,三尖瓣中度反流5例,管道内中度反流32例,右室流出道重度梗阻7例,中度梗阻11例,轻度梗阻25例。无中晚期死亡。心功能分级(NYHA)Ⅰ~Ⅱ级,活动耐量良好。结论自制带单瓣外管道重建右心室-肺动脉连接临床效果良好,牛心包管道术中流出道压差小于人工血管,我们更推荐牛心包管用于低龄、低体重患者。     

Prevalence and optimal management strategy for aortic regurgitation in tetralogy of Fallot

Objective: Aortic regurgitation (AR) in the tetralogy of Fallot (TOF) is not frequent, but when present it impacts significantly on surgical management. Furthermore, the incidence of late AR development has been increasing, along with surgical interest in current practices. Methods: Pre- and post-operative studies on 427 patients (TOF, 374; TOF/PA (TOF with pulmonary atresia), 53) who survived corrective operation were reviewed. AR (mild) was detected in 28. Results: Nine had AR preoperatively, while 25 (including six with preoperative AR) exhibited AR post-operatively. In the 19 who developed AR post-operatively, the aortic root diameter (AoRoD) and indexed AoRoD (%AoRoD) were 42±11 mm and 166±36%, increased from the preoperative values of 30±10 mm and 149±24%. AR-free rate at 20 years was 95.1% of all cases studied, 84.3 vs 96.5% in TOF/PA vs classic TOF (P<0.0001), and 82.2 vs 97.0% in bulboventricular VSD vs infracristal VSD (P<0.0001). Older age at repair, and bulboventricular VSD were identified as risk factors for the progression of AR. Aortic valvuloplasty (AVP; n=5) or replacement (AVR; n=4) was performed nine times in eight patients before (n=1), during (n=4), or late after TOF repair (n=4); all showed improvement of NYHA class. Survival- and reoperation-free survival curves showed no significant difference between patients with or without AR. Conclusions: After repair of TOF, careful observation for a late progression of AR is needed for the optimal timing of surgical intervention, especially in patients who repaired at higher age with a dilated aortic root or in patients with bulboventricular VSD.     

Pulmonary reinterventions after complete unifocalization and repair in infants and young children with tetralogy of Fallot with major aortopulmonary collaterals

Background

Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit.

法洛四联症矫治术后远期肺动脉瓣关闭不全的治疗进展

随着外科、麻醉、灌注及围术期处理技术的不断发展,法洛四联症(TOF)行常规矫治手术的手术死亡率低于2%～3%,而远期预后越来越受到关注,其中远期肺动脉瓣反流是困扰心脏外科医师和影响患者预后的重要因素;由此带来的右心室扩张、右心室功能不全、致死性心律失常等直接影响患者的生存质量和术后远期生存率。目前,对肺动脉瓣反流的治疗方法包括肺动脉瓣置换术(PVR)、介入治疗、镶嵌治疗等,PVR是治疗肺动脉瓣反流的主要方法,该方法能明显改善患者的心功能和临床症状,延长患者的生存时间;介入治疗因不需要行再次开胸手术,减少了手术创伤和手术风险,而且能改善右心室压力、肺动脉瓣反流程度,使许多患者受益。我们对TOF矫治术后远期肺动脉瓣反流的病理生理改变、PVR的手术指征、肺动脉瓣反流临床治疗进展和早期防治进行综述。     

Long-term outcomes of expanded polytetrafluoroethylene conduits with bulging sinuses and a fan-shaped valve in right ventricular outflow tract reconstruction

Objective

Various types of conduits are available for right ventricular outflow tract (RVOT) reconstruction. We have developed an expanded polytetrafluoroethylene (ePTFE) conduit with bulging sinuses and a fan-shaped ePTFE valve. This study summarized the results of a multicenter study evaluating the valved ePTFE conduit.

Right ventricular myxoma in a patient with tetralogy of Fallot

INTRODUCTION

Cardiac myxoma is the most common primary cardiac tumour in adulthood and may present in the context of Carney''s complex.

PRESENTATION OF CASE

A 32-year-old male with a history of repaired tetralogy of Fallot in childhood was admitted with severe pulmonary valve regurgitation and a mobile mass in the right ventricle. The patient underwent pulmonary valve replacement and mass excision. Pathology examination showed myxoma.

DISCUSSION

In the majority of cases myxomas originate in the atria, nevertheless they can also be found in a ventricular cavity. Myxoma is a prevalent feature of Carney''s complex, an inherited, autosomal disease, characterised by multiple tumours in several organs. Tetralogy of Fallot has also been described in association with Carney''s complex.

CONCLUSION

Coexistence of tetralogy of Fallot with a cardiac ventricular myxoma in a patient not affected from Carney''s complex or other familial syndrome.     

实时三维超声心动图评价法洛四联症患者手术前后右心室收缩功能

目的应用实时三维超声心动图（RT-3DE）评价法洛四联症（TOF）患儿术前及术后右心室收缩功能变化。方法应用RT-3DE测量29例TOF患儿术前及术后1周、6个月的右心室舒张末期容积（RVEDV）、右心室收缩末期容积（RVESV）、右心室每搏输出量（RVSV）及右心室射血分数（RVEF）。结果 29例TOF患儿中,12例术前RVEF低于50%（12/29,41.38%）。术后1周RVESV较术前增加,RVEF较术前减小,术后6个月RVEDV、RVESV较术前增加,RVEF值较术前减低（P均〈0.05）。随着心功能降低,三维右心室容积曲线（RVVC）逐渐低平、收缩峰值后移。结论 RT-3DE可准确、定量评价TOF患儿术前、术后右心室功能变化。     

Konno-Rastan手术治疗儿童复杂性多水平左心室流出道梗阻

目的 总结Konno-Rastan手术治疗复杂多水平左心室流出道梗阻的疗效、并发症和常见失误及预防.方法 1996年1月至2012年8月,13例患儿行主动脉根部及左心室流出道扩大、人工机械瓣膜主动瓣置换(Konno-Rastan术).男8例,女5例;年龄5～13岁;体质量12～51 kg,中位值21 kg.诊断先天性主动脉瓣狭窄8例,先天性主动脉瓣狭窄合并主动脉瓣上狭窄3例,先天性主动脉瓣狭窄合并室间隔缺损、主动脉缩窄及右心室流出道狭窄1例,先天性主动脉瓣狭窄、主动脉瓣球囊扩张术后再狭窄1例.患儿均合并继发性室间隔增厚、左心室流出道梗阻.主动脉瓣环直径12.0 ～ 16.4 mm,术前跨主动脉瓣压差90～ 151 mm Hg(1 mm Hg =0.133 kPa).置入St.Jude AG19号机械瓣膜8例,AG17号5例.结果 无手术死亡.呼吸机辅助4～74 h,中位时间6h;ICU停留1～6天.1例术后第3天出现突发性晕厥,确诊为Ⅲ度房室传导阻滞,植入永久性心外膜起搏器,术后7天恢复窦性心律,传导功能正常,将起搏器设定为60次/min,VVI模式备用.2例术后3个月复查发现新生心室水平残余分流,其中1例因反复心功能衰竭再次手术,修补残余分流,术后心功能状态良好,无残余心内畸形;1例因无明显症状,门诊随访,术后3年患类风湿,感染性心内膜炎及肾病综合征,拟择期行残余分流修补术.其余患儿无不适症状.全部患儿随访1 ～78个月,终生服用华法林抗凝,控制国际标准比值(INR) 1.8 ～2.5.随访期内无死亡,未发现人工瓣膜相关性并发症发生.完全性房室传导阻滞发生率为7.7％;残余分流发生率为15.4％;术后早期感染性心内膜炎发生率为0,远期发生率为7.7％.结论 Konno-Rastan手术可以有效地治疗患儿复杂的多水平左心室流出道梗阻,但由于手术操作复杂,可导致较高比例的手术并发症;完善手术操作是提高手术成功率的关键因素之一.     

小儿永存动脉干右室流出道重建

目的 总结小儿永存动脉干(PTA)右室流出道重建治疗经验.方法 2000年1月至2007年12月共行PTA根治手术治疗43例,男26例,女17例.年龄1.5个月～3.8岁;体重3.2～23.0kg.Ⅰ型、Ⅱ型和Ⅲ型PTA分别为26例、11例和6例.18例PTA的肺动脉直接下拖至右室流出道切口上缘相吻合,前壁再用心包补片扩大;8例用Homograft管道、14例用牛颈静脉管道连接远端肺动脉和右心室,重建右室流出道;3例Ⅱ型者,肺动脉后壁用左心耳壁与右室流出道上缘做吻合,前壁再用心包补片扩大.结果 术后所有病儿均生存,5例表现为右心功能不全,2例右肺动脉(RPA)压差37.5～47.3 mm Hg(1mm Hg=0.133kPa),术后17d恢复至35.3mm Hg以下.左心室流出道阶差均小于20.3mm Hg.残余VSD(2mm)1例,乳糜胸1例.5例病儿肺血管阻力高,吸一氧化氮(NO)治疗7d左右后好转.随访3个月～3年,2例病儿RPA残余压差24.0～29.3 mm Hg,均无明显右心室或左心室流出道梗阻.结论 肺总动脉后壁直接与右心室切口作吻合重建PTA的右室流出道,早期和远期效果良好.牛颈静脉的带瓣管道的应用,解决了小尺寸同种带瓣管道来源不足的问题,操作简便.