法乐四联症矫治术后右心室流出道瘤的临床分析

目的:探讨法乐四联症(TOF)矫治术后右心室流出道(RVOT)瘤形成的原因、对心功能的影响及诊治结果.方法:回顾性分析我院2002- 01至2010- 06间14例TOF术后RVOT瘤及伴随病变的初次手术方法、心功能状态和治疗结果.结果:因TOF矫治术后残余心内畸形诊治患者中,合并RVOT瘤者14例,年龄(5.6±3.7)岁,距初次手术(3.4±3.6)年.12例合并肺动脉狭窄,其中9例初次修补手术时未跨环补片,3例虽跨环补片但残余重度狭窄.4例合并中-重度肺动脉瓣反流,初次手术均用自体心包跨环补片,其中3例同时合并肺动脉高压.心功能NYHA (2.7±0.7)级.12例再次外科手术,术中切除RVOT瘤并重建RVOT.合并肺动脉狭窄者,予跨环补片加宽.2例重度肺动脉瓣反流分别予带单瓣的同种主动脉跨环补片、植入牛颈静脉管道.围术期死亡1例.术后随访(1.9±2.1)年,NYHA心功能均Ⅰ~Ⅱ级,无再次手术病例.结论:TOF术后RVOT瘤的形成与初次手术方式有关.RVOT瘤促使TOF术后早期出现心功能不全.及时手术重建RVOT和矫治伴随病变有利于心功能恢复.     

法洛四联症根治术后再次手术56例原因分析

目的:总结法洛四联症(TOF)根治术后再次手术的原因及其相关处理。方法 :2007年1月至2013年1月间,我院共对56例TOF根治术后的患者进行再次手术治疗,对其进行回顾性研究,分析再次手术的原因和处理方式。再次手术的原因包括:室间隔缺损残余分流、右心室流出道梗阻/肺动脉狭窄、三尖瓣反流、肺动脉瓣反流、右心室流出道瘤样扩张以及主动脉瓣反流等。结果:全组无死亡病例。中位住院天数26.2 d,中位ICU时间12.5 d。再次手术的效果满意。结论:提高手术技术、采用适当的心脏切口以及避免单纯使用新鲜的自体心包片,加宽右心室流出道等,可减少再次手术的机会。对这类患者加强随访,并对相关并发症进行积极处理。关于TOF的治疗策略,需要更进一步的临床研究。     

法乐氏四联症外科治疗进展

法乐氏四联症(TOF)是最常见的紫绀型先心病,包括四种病变,即右室流出道(RVOT)梗阻、室间隔缺损(VSD)、主动脉右跨和右心室肥大。其中最基本的畸形为RVOT梗阻和VSD。对TOF自然病史的研究表明,多数患者幼年夭折,     

室间隔缺损修补后残余漏的外科治疗(附18例报告)

目的探讨先天性室间隔缺损(VSD)修补后残余漏的手术疗效及预防VSD残余漏的要点.方法回顾分析了我院19年内收治的VSD修补术后发生残余漏再次手术18例,均为先天性膜部VSD,首次手术中缺损直接缝合修补者4例(直径＜1.0cm),1例术中停体外循环后发现右室表面震颤,立即再次修补,3例术后早期分别因血红蛋白尿、严重心脏杂音及心功能不良再次手术.首次手术中缺损采用补片修补(直径＞1.0cm)14例术后晚期(＞6个月)再次手术,除一例仅因心脏杂音外,其余均因有程度不同的临床症状及x线胸片示心影大、肺血多,ECG示左室肥厚等表现和超声心动图示VSD残余分流明显.结果2例死亡,2例仍存在残余漏,14例痊愈.结论良好的术野显露、准确的手术操作及丰富的手术经验是防止VSD修补后残余漏的主要因素.再次手术应严格掌握指征,手术的关键在于避免损伤性大出血及准确地修补残余漏.     

室间隔缺损术后残余漏

室间隔缺损 (VSD)是常见的先天性心脏病 ,可单独发生 ,也可以是法洛四联症 (TOF)、大动脉转位等复杂畸形的一部分。随着体外循环技术及手术技巧的提高 ,VSD修补术的死亡率目前已很低 ,但仍存在不少并发症 ,其中残余漏是主要并发症之一。严重残余漏可造成血液破坏、心功能衰竭、心内感染等 ,甚至危及生命。因此 ,临床上对 VSD术后残余漏应积极采取措施进行预防及治疗。本文将国内外近年来对残余漏病因、预防、诊治等方面的研究综述如下。1　残余漏的好发部位残余漏多发于三尖瓣前、隔瓣基底周围 ,这可能与该区域邻近主动脉瓣和传导束等…     

法洛四联症右室流出道疏通的临床经验

目的 探讨法洛四联症（Tetralogy of Fallot,TOF）根治术中右心室流出道疏通的最佳直径。方法 回顾性分析2011年1月至2016年12月我院心脏外科行TOF根治术的儿童病例308例,2011年1月至2013年12月201例患儿（A组）均采取右心室流出道疏通直径等同于标准肺动脉瓣环直径;2014年1月至2016年12月107例患儿（B组）采取右心室流出道疏通直径大于标准肺动脉瓣环直径3 mm。所有出院患者术后随访12~36个月。结果 A组有17例患者术后出现右心室流出道残余梗阻致低心排血量综合征（Low Cardiac Output Syndrome,LCOS）,多普勒测右心室流出道流速3.1~4.8（3.8±0.4）m/s,跨肺动脉瓣压差均大于50 mm Hg,其中6例右心室流出道流速超过4.5 m/s,再次手术行右心室流出道疏通术;术后早期死亡5例,随访余12例未发现右心室流出道残余梗阻进一步发展,且均较术后早期明显改善。B组术后无右心室流出道残余梗阻,跨肺动脉瓣压差均小于50 mm Hg,术后早期死亡2例。随访两组出院患者无三尖瓣、肺动脉瓣大量反流,生长发育良好,心功能均达Ⅰ~Ⅱ级。结论 TOF右心室流出道疏通直径等同于标准肺动脉瓣环直径造成术后残余梗阻发生率较高,大于标准肺动脉瓣环直径3 mm可有更好的疗效。     

法洛四联症根治术后低心排血量综合征患者的临床分析

目的探讨法洛四联症(tetralogy of Fallot,TOF)患者一期根治术后早期低心排血量综合征(low cardiac output syndrome,LCOS)发生的原因及处理方法。方法 2015年12月至2017年12月期间高州市人民医院心外科行TOF一期根治术患者共167例,其中47例术后出现LCOS,回顾性分析此组患者的临床资料。结果 LCOS原因分析:术后血容量不足17例,右心室流出道残余梗阻11例,严重心律失常9例,左心室功能衰竭8例,心包压塞2例。处理方法:LCOS患者均使用血管活性药物,根据血流动力学参数,调整循环功能状态;其中行腹膜透析术16例,透析(3.5±1.4)d;跨肺动脉瓣压差50 mmHg(1 mmHg=0.133 kPa)的1例患者围术期重返手术室再次体外循环下疏通右心室流出道,2例再次手术行房间隔造孔术。4例体肺侧支3 mm且存在灌注肺的患者送介入室行侧支封堵;1例室间隔残余缺损4 mm的患者于围术期行缺损修补术。2例心包压塞患者其中1例再次开胸探查止血,1例更换引流管。死亡8例(17.0%),因多脏器功能障碍综合征死亡5例,重症感染死亡2例,完全性房室传导阻滞死亡1例。余39例患者康复出院。结论严格把握手术适应证,完善手术技术,注重心肌保护,术后加强监护,积极寻找LCOS病因并及时纠正,必要时二次手术,可提高LCOS治愈率。     

法洛四联症根治术后残余畸形的相关因素研究

目的:分析影响法洛四联症(TOF)根治术后残余畸形发生的相关因素。方法:回顾性分析我院2013年至2017年,行TOF根治术并有随访资料的200例患者,观察术后近、中期残余畸形的发生情况,包括室间隔残余分流、残余肺动脉狭窄、肺动脉瓣反流。统计分析术后残余畸形的发生与随访时间、手术年龄、手术方式、主动脉骑跨率、右心室流出道内径、室间隔缺损部位、室间隔缺损大小、肺动脉发育等因素的关系。结果:2013至2017年在我院接受TOF根治术患者随访时间3～30个月(中位数24个月)。随着随访时间的延长,室间隔残余分流的发生逐渐减少,残余肺动脉狭窄、肺动脉瓣反流的发生逐渐增加。有轻度及以上肺动脉瓣反流的患者中,行跨瓣环手术占60.9%;无肺动脉瓣反流的患者中,行跨瓣环手术的占30.8%,二者差异有统计学意义(P0.05)。手术年龄、手术方式、主动脉骑跨率、右心室流出道内径、室间隔缺损部位、室间隔缺损大小、肺动脉发育等情况,在术后近、中期随访中,对室间隔残余分流和残余肺动脉狭窄的发生,差异无统计学意义。结论:全组患者术后无严重残余畸形发生。随着随访时间的延长,室间隔残余分流的发生逐渐减少,残余肺动脉狭窄、肺动脉瓣反流的发生逐渐增加。轻度及以上的肺动脉瓣反流多发生于行跨瓣环手术的患者。     

杂交技术在法洛四联症中的应用

目的:总结杂交技术镶嵌治疗儿童法洛四联症(TOF)的经验。方法:外科术前介入治疗:3例有巨大体肺侧支(APCAs)的重症TOF在根治术前予以侧支血管堵塞术。外科术后镶嵌治疗:6例TOF根治术后残余分流,其中4例残余膜周部室间隔缺损,1例残余左室右房通道室间隔缺损,1例残余房间隔缺损,分别予以经导管残余心脏缺损封堵术。结果:3例有巨大APCAs的重症TOF在根治术前予以侧支血管堵塞术后随即进行外科手术,皆获得满意效果。6例TOF根治术后残余分流者行经导管封堵术封堵成功,随访无残余分流及心脏瓣膜异常,未出现心律失常。结论:杂交技术镶嵌治疗伴有巨大APCAs及术后存在残余分流的TOF安全、有效。     

婴幼儿法洛四联症1期根治术时机选择

目的:评价婴幼儿法洛四联症(TOF)早期根治术后效果。方法:2002年1月至2006年6月,我科共手术治疗<36个月婴幼儿TOF 87例,年龄(2~36)个月,平均(17.2±5.6)个月:<6个月14例(16.1%)、7~12个月34例(39.1%)、13~36个月39例(44.8%)。比较不同年龄组手术病死率、围手术期处理、术后并发症及随访结果。结果:2例(2.3%)死于术后低心排出量综合征,1例(1.2%)死于术后心律失常。不同年龄组术后并发症差异无统计学意义。术后6个月以下患儿更多需要升压药维持,但术后呼吸机通气时间、ICU停留时间、住院时间差异无统计学意义。回访时间2.0~6.0年,平均(3.5±1.6)年,56例(66.7%)例患儿存在不同程度肺动脉瓣反流,2例(2.3%)残余右心室流出道梗阻,3例(3.4%)室缺残余分流再次手术矫治。结论:对<6个月婴儿TOF一期根治手术病死率和术后并发症低,手术年龄不影响术后效果。     

Complete atrioventricular septal defect with tetralogy of Fallot: diagnosis and management.

OBJECTIVE--To report recent experience of patients with complete atrioventricular septal defect and tetralogy of Fallot, with emphasis on anatomical features, diagnosis, and management. DESIGN--Case notes were reviewed and patients were assessed at follow up by clinical examination and cross sectional and Doppler echocardiography. SETTING--Tertiary cardiothoracic referral centre. PATIENTS--Between 1987 and 1992 13 patients with atrioventricular septal defect and tetralogy of Fallot (12 with concordant and one with double outlet ventriculoarterial connections) underwent surgery; 10 underwent complete intracardiac repair. 11 patients had Down's syndrome. The complete diagnosis was established preoperatively by cross sectional echocardiography in all but one patient. A tri-leaflet left atrioventricular valve as seen in parasternal short axis views was the diagnostic feature of atrioventricular septal defect, with tetralogy of Fallot diagnosed from the presence of anterocephalad deviation of the outlet septum producing subvalvar pulmonary stenosis as seen in subcostal right anterior oblique views. INTERVENTIONS--Total correction consisted of closure of the atrioventricular septal defect by a combined right atrial and ventricular approach, reconstruction of the atrioventricular valves, and relief of the obstruction within the right ventricular outflow tract. Separate patches were used to close the atrial and ventricular septal defects. Modified Blalock-Taussig shunts were performed in three patients, who await intracardiac repair. Surgical correction was carried out at mean (range) age of 5 (2 to 15) years. MAIN OUTCOME MEASURES--Diagnostic methods, surgical results, and functional state after complete correction. RESULTS--The presence of an atrioventricular septal defect was missed preoperatively in one patient with tetralogy of Fallot. The characteristic goose neck deformity on the left ventriculogram was not present and the tri-leaflet nature of the left atrioventricular valve was not sought on echocardiography. Of the 10 patients who underwent complete repair, nine are alive and one died 34 days after operation with adult respiratory distress syndrome. Examination at necropsy showed an excellent surgical correction. Mean (range) follow up was 23 (8 to 48) months. All nine patients are alive and well (New York Heart Association Class 1). CONCLUSION--Accurate diagnosis and staged management with improved surgical techniques have lowered mortality of this complex combination of cardiac defects. The current policy of this group is to recommend a systemic to pulmonary arterial shunt procedure for symptomatic children younger than 2 years and total correction in older children.     

双心室矫正法洛四联症合并完全性房室间隔缺损的手术技巧

目的:评价双心室矫治法洛四联症合并完全性房室间隔缺损(TOF/CAVSD)的效果。方法:2007年1月至2012年12月,共行TOF-CAVSD解剖矫正术16例,年龄1~13岁,平均3.3岁,体质量7~36kg,平均9.1kg,其中6例为Down综合征;16例均采用右心房、右心室纵切口,二片法补片修补房室间隔缺损,右心室流出道加宽补片或外管道。结果:术后早期无死亡,无因为室缺残余分流、左侧房室瓣反流和右心室流出道梗阻而再手术。随访6个月至5年,无晚期死亡及再手术;NYHA心功能Ⅰ级或Ⅱ级。结论:双心室法矫正TOF-CAVSD效果良好。     

Echocardiographic and morphological correlations in tetralogy of Fallot.

AIMS: Our aim was to clarify the location and structure of the outlet septum relative to the free-standing subpulmonary infundibulum in the setting of tetralogy of Fallot and to examine its relationship to the other components of the subpulmonary outflow tract, determining their potential influence on clinical outcome. METHODS AND RESULTS: We studied prospectively 41 patients with tetralogy of Fallot (mean age 14 +/- 10.9 months) prior to surgical repair, and compared them with 15 patients undergoing closure of a ventricular septal defect associated with malalignment of the outlet septum but no subpulmonary infundibular stenosis (Eisenmenger ventricular septal defect), and 20 healthy controls. We also examined available autopsied hearts from cases with uncorrected tetralogy of Fallot (8) and Eisenmenger ventricular septal defect (13). Data were indexed for body surface area, and diameter of the tricuspid valve, respectively. The overall length of the subpulmonary infundibulum, including the extent of the muscular outlet septum, was significantly greater for patients with tetralogy of Fallot compared to normals (2.34 +/- 0.6 vs 1.46 +/- 0.34 cm/BSA0.5, P<0.001), whereas the difference between those with tetralogy of Fallot and an Eisenmenger ventricular septal defect was confined to the degree of narrowing of the subpulmonary outlet (0.43 +/- 0.22 vs 2.17 +/- 0.64 cm/BSA0.5, P<0.001). Within the tetralogy of Fallot group, there were linear relationships between deviation of the outlet septum (r= -0.61, P<0.005) and the diameter of the pulmonary valvar orifice (r=0.75, P<0.001), suggesting that growth of the pulmonary arteries may be related to this feature. When patients requiring a transannular patch as part of their surgical repair were compared with those not needing this procedure, differences were found in the diameter of the pulmonary valvar orifice and the pulmonary trunk, but not in the dimensions of the outlet septum. CONCLUSION: The position of the outlet septum in relationship to the remainder of the muscular subpulmonary infundibulum represents a hallmark of tetralogy of Fallot, permitting its differentiation from Eisenmenger ventricular septal defects and normal hearts.     

Reoperation following total repair of congenital heart disease

For the past 25 years we have performed 61 reoperations in 58 patients with congenital heart disease who previously underwent total correction for the diagnosed ventricular septal defect, tetralogy of Fallot, atrioventricular septal defect, atrial septal defect, total anomalous pulmonary venous connection (TAPVC), and other involvement for 27, 11, 5, 4, 4, and 7 patients, respectively. Causes of reoperation included 43 events of valvular lesion, 29 of residual septal defect, 5 of conduit failure, 5 of venous obstruction, 3 of hemolysis, 3 of infective endocarditis, and 2 of prosthetic valve dysfunction. Although 5 early and 4 late deaths were encountered, the remaining 49 patients (3 in New York Heart Association (NYHA) Class II and 46 in NYHA class I or II) survived the reoperation. The patients were followed up by echocardiography and chest X-ray examination, and reoperation was indicated under the following conditions: moderate to severe valvular insufficiency; residual defect causing the shunt ratio to be over 30% or a systolic pulmonary pressure over 50 mmHg; or a high pressure gradient over 50 mmHg across the right ventricular outflow tract. Early timing for reoperation seemed to be important in patients with pulmonary venous obstruction, prosthetic dysfunction, severe hemolysis and infective endocarditis.     

Congenital heart disease in Down's syndrome patients: a decade of surgical experience

Patients with Down's syndrome represent a significant subset of patients with congenital heart disease. Fifty-five patients with Down's syndrome have undergone surgical treatment for congenital heart disease at our institution in the past decade. Twenty-six had atrioventricular canal, 11 had ventricular septal defect, 7 had secundum atrial septal defect, 7 had tetralogy of Fallot, 3 had primum atrial septal defect and 1 patient had double outlet right ventricle. The thirty day mortality following operative intervention was 16.4%. Mortality was highest for tetralogy of Fallot followed by atrioventricular canal and ventricular septal defect. Long term mortality for all lesions was 27.3% over our follow-up period which averaged 33 months. Thirty day mortality compared similarly to previous reports of surgically treated Down's syndrome patients. When compared to our patients without Down's syndrome, the Down's population did not exhibit an increased risk for surgical treatment of congenital heart disease.     

婴幼儿先天性心脏病的外科手术与围术期处理

目的总结婴幼儿先天性心脏病的外科手术经验。方法自2000年1月至2004年12月共为133例,年龄5￣36个月,体重6￣15kg的先天性心脏病患儿施行外科手术治疗。全组非体外循环动脉导管结扎术29例;体外循环手术104例,包括室间隔缺损、继发孔房间隔缺损、肺动脉瓣狭窄、部分型房室间隔缺损、部分型肺静脉异位引流、法乐四联症、房间隔缺损并右室流出道梗阻和其他复合畸形。结果全组术后住院死亡6例,死亡率4.5%。术后主要并发症包括低心排综合征、呼吸衰竭、心律失常、肺部感染、出血等。死亡原因包括重度低心排综合征、严重室性心律失常、肺动脉高压危象等。结论心外科、心儿科、麻醉科等人员的密切配合,心内、外科镶嵌治疗成为婴幼儿心脏外科手术成功的重要保证。     

保留隔、壁束技术在法洛四联症手术矫正中的应用(附193例报告)

目的：探讨保留隔、壁束技术在法洛四联症手术矫正中的价值。方法：右室流出道纵切口，环形狭窄只切除狭窄环；管形狭窄或右室前壁极度肥厚者，将切口两侧心肌自前向后斜形切除修剪，完全保留隔、壁束和室上嵴。均行右室流出道加宽。结果术后早期死亡l例。余右／左室压比均在0．5以下，全部治愈出院，随访效果满意。结论：法洛四联症手术矫正保留隔、壁束技术，可有效的保护右心室功能、预防室缺残余漏、避免完全性右束支传导阻滞。     

Tetralogy of Fallot with restrictive ventricular septal defect by accessory tricuspid leaflet tissue

In tetralogy of Fallot septal defect is usually large because of malalignment of outlet septum, restrictive defect has been reported rarely. We present a case of tetralogy of Fallot with accessory tricuspid leaflet tissue restricting ventricular septal defect. The report includes echocardiographic and catheter images of this rare presentation of tetralogy of Fallot.     

Echocardiographic diagnosis of tetralogy of Fallot

The echocardiographic features of tetralogy of Fallot were defined in 25 patients with this malformation proved by cardiac catheterization. The echocardiographic characteristic that was present in all patients and that was most sensitive was the abrupt ending of the interventricular septal echoes with the aorta overriding the ventricular septal defect. The following additional echocardiographic features were frequently demonstrated in these 25 patients: right ventricular enlargement (20 patients), hypertrophy of the interventricular septum (20 patients), diminution of the right ventricular outflow tract (21 patients) and widening of the aorta (24 patients). The suggestion is made that the most specific echocardiographic pattern of tetralogy of Fallot is the finding of several echocardiographic abnormalities rather than the single feature of aortic overriding. Recognition of the altered anatomic relation coupled with a complete echocardiographic evaluation of all cardiac structures is a reliable means of diagnosing tetralogy of Fallot.     

Spectrum of congenital heart disease in Malta. An excess of lesions causing right ventricular outflow tract obstruction in a population-base study

Aims To quantify birth prevalence and spectrum of congenital heartdisease in the Island population of Malta, and compare theserates with previous studies. Methods All patients diagnosed as having congenital heart disease byechocardiography, cardiac catheteriz-ation, surgery or post-mortemby 1 year of age between 1990–1994 were included. Therewere 231 cases of live born congenital heart disease with abirth prevalence of 8·8/1000 live births. The commonestlesions were ven-tricular septal defect, pulmonary stenosisand tetralogy of Fallot. The rates of individual lesions werecompared with two recent epidemiological studies with similarmethodologies. Results Although the overall birth prevalence of congenital heart diseasewas similar in three studies, significantly higher rates ofpulmonary stenosis, tetralogy of Fallot and double outlet rightventricle were found in Malta, all of which predispose to rightventricular outflow tract obstruction. In contrast, there werelower rates of lesions causing left ventricular outflow tractobstruction. A higher rate of ventricular septal defect wasalso found. The Maltese gene pool may contain an inherent predispositiontowards lesions causing right ventricular outflow tract obstruction.