单心室合并完全性肺静脉异位引流的外科治疗

目的:总结单心室合并完全性肺静脉异位引流(TAPVC)的外科治疗效果。方法:入选2008-09至2016-03期间,我院手术治疗的单心室合并TAPVC患儿25例,年龄5天~14岁,体重3.3~49.5kg,心上型TAPVC 22例,心下型TAPVC 1例,混合型TAPVC 2例;合并完全性房室间隔缺损17例,三尖瓣闭锁2例,二尖瓣闭锁2例;中度以上房室瓣反流4例,肺动脉闭锁5例,肺静脉梗阻9例。结果:院内早期死亡5例,死亡率20%(5/25),死亡原因均为低心排血量综合征。存活20例患儿随访1~65个月,7例再次手术,其中5例完成二期改良Fontan术,1例因重度房室瓣反流行房室瓣置换术,另1例因肺静脉梗阻及重度房室瓣反流行肺静脉狭窄矫治+房室瓣成形术;余13例患儿未再次手术。20例出院患者随访期间3例死亡,死亡原因分别为低心排血量综合征、蛋白丢失性肠病和神经系统损伤。余存活患儿一般情况好,未发现残余肺静脉梗阻。结论:单心室合并TAPVC常合并右心房异构,心内畸形复杂,手术治疗仍具有较高死亡率。     

改良Fontan手术治疗功能单心室合并左异构的早中期临床结果

目的:总结分析改良Fontan手术治疗功能单心室合并左异构的早期及中期临床结果。方法:回顾性纳入中国医学科学院阜外医院自2009年1月至2016年1月完成的改良Fontan手术治疗功能单心室合并左异构患者19例,依据是否分期完成改良Fontan手术,分为一期手术组(n=9)和分期手术组(n=10),对比分析两组患者术后早期及中期的临床结果。结果:19例患者中,男性13例(68.4%),女性6例(31.6%)。分期手术组患者行第一期Glenn手术时的平均年龄为(1.87±1.56)岁;两组患儿行Fontan手术时的平均年龄为(5.09±2.01)岁。住院期间死亡1例,为分期手术组患者;平均随访时间4.2(1~9)年,随访期间无死亡患者。两组患者术前在性别、年龄、身高、体重、心室形态、房室瓣反流及上腔静脉形态、肺动脉发育和平均肺动脉压力等方面的差异均无统计学意义(P均>0.05);术中体外循环时间、阻断时间、管道建立方式及术后住院时间的组间差异也均无统计学意义(P均>0.05)。在重症监护病房停留时间、机械辅助通气时间及术后血浆用量、胸腔引流量、胸腔引流时间、腹腔积液发生率等方面,一期手术组均优于分期手术组,差异均有统计学意义(P均<0.05)。两组患者术后均出现进行性血氧饱度下降,各有1例患者经皮血氧饱和度<85%,组间差异无统计学意义(P>0.05)。结论:与分期Fontan手术相比,一期Fontan手术治疗年龄偏大的功能单心室合并左异构患者具有良好的围术期结果,两种手术策略在早期和中期疗效方面无明显差异,术后均会出现氧饱和度进行性下降的问题,需要远期随访。     

肝静脉直接入心房的(功能)单心室患者的改良Fontan术式及早中期疗效

目的:总结肝静脉直接入心房(SHV)的(功能)单心室患者的改良Fontan术式及早中期临床结果。方法:回顾性分析2009-01至2015-12阜外医院对39例SHV的(功能)单心室患者施行改良Fontan术的临床资料。男26例(66.7%),平均年龄(6.98±3.62)岁,平均体重(20.79±9.66)kg,术前平均肺动脉压(m PAP)9.77±2.51(4~15)mm Hg(1mm Hg=0.133k Pa)。依据SHV患者是否合并下腔静脉与心尖同侧(ACJ),分为SHV组(n=29)和SHV+ACJ组(n=10)。对术后早期(术后3个月)及中期(5年)进行临床结果分析。结果 :两组患儿的临床资料比较差异无统计学意义(P0.05)。39例患者中,一期手术20例,二期手术19例(18例为Glenn术后,1例为B-T术后)。选择的改良Fontan术式主要采取外管道—共同开口术式(30例,76.9%)。19例患者建立体外循环并阻断主动脉,体外转流时间72~446 min,平均(189.74±84.96)min,主动脉阻断26~171min,平均(89.05±43.96)min;另19例患儿并行循环,体外转流时间60~209 min,平均(109.99±98.86)min。术后早期因并发症再次手术10例。术后院内死亡3例,死亡率7.7%。36例患儿平均随访时间3.90(0.83~7.17)年,35例心功能为Ⅰ级,1例为Ⅱ级,5年生存率100%。结论 :SHV的(功能)单心室患者行改良Fontan术后早中期随访结果良好。而改良Fontan术式的选择应依据下腔静脉与肝静脉间的距离以及是否合并ACJ。     

137例婴幼儿完全性肺静脉异位引流各年龄段临床特点和外科疗效分析

目的:探讨婴幼儿完全性肺静脉异位引流(TAPVC)各年龄段的临床特点和外科治疗效果.方法:2000-01至2006-12 诊断TAPVC并在我院进行手术治疗的婴幼儿(除外合并法乐四联症、右心室双出口、完全性房室通道、单心室等复杂畸形的患儿)共137例.分为≤28天新生儿组(组1,n=7)、＞28天～≤6个月组(组2,n=70)、＞6个月～≤1岁组(组3,n=26)、＞1岁～≤3岁组(组4,n=34)4个年龄组.137例患儿伴房间隔缺损119例,卵圆孔未闭18例,动脉导管未闭27例,室间隔缺损2例,三尖瓣关闭不全103例.合并重度肺动脉高压50.4%(69/137).其中≤6个月患儿(组1、组2)占71.0%(49/69).心上型49.6%(68/137),心内型37.2%(51/137).心下型1.5%(2/137),混合型11.7%(16/137).合并肺静脉狭窄18.2%(25/137),其中≤6个月患儿(组1、组2)占88.0%(22/25).结果:手术死亡率11.7%(16/137),组1和组2占87.5%(14/16).主要死亡原因为肺部感染和低心排综合征.有随访资料99例,随访率81.8%(99/121),随访期限2～86个月,平均(25.5±22.3)个月.晚期死亡9例,均为组1和组2患儿,晚期死亡率9.1%(9/99).结论:年龄＞6个月的TAPVC患儿可获得满意的外科治疗效果,而年龄≤6个月尤其是新生儿患儿病情重,合并限制性房间隔缺损、肺静脉狭窄和重度肺动脉高压比例高,往往需要急诊手术,手术及远期死亡率较高.     

非体外循环下复杂先天性心脏病的姑息手术

目的:探讨非体外循环下复杂先天性心脏病姑息手术适应证及处理要点。方法:2008-01-2010-06对26例复杂先天性心脏心病患儿实行非体外循环下姑息手术,其中肺动脉环缩术5例,改良体肺分流术6例,双向腔肺分流术14例,双向腔肺分流+改良体肺分流术1例;其中功能单心室9例,法洛四联症肺动脉闭锁6例,右室双出口4例,三尖瓣闭锁3例,完全性大动脉转位2例完全心内膜垫缺损并肺动脉闭锁1例,Ebstein畸形1例。结果:死亡4例,22例患者顺利出院,死亡率15.4%;死亡原因包括低氧血症及心功能衰竭、肺部感染合并心功能衰竭;肺动脉环缩术、改良体肺分流术、双向腔肺分流术后平均血氧饱和度分别为(81.8±2.4)%、(90.0±3.2)%、(86.4±4.1)%;术后并发症包括肺部感染3例,胸腔积液7例,上腔静脉综合征1例。术后随访2个月～1年,无远期死亡,心功能NYHAⅠ级7例,NYHAⅡ级15例。结论:非体外循环下姑息治疗是复杂先天性心脏病的有效治疗手段,特别适用于有体外循环高危因素患者,手术适应证的正确把握、术中术后合理的处理是降低死亡率的关键。     

新生儿完全性肺静脉异位连接外科治疗的临床分析

目的:评估外科手术治疗新生儿完全性肺静脉异位连接(TAPVC)的近中期临床疗效。方法:2010-11至2014-01,我院确诊为TAPVC的新生儿20例(其中男性15例,女性5例)均接受外科手术治疗。平均手术年龄(10.2±4.8)d,体重(3.2±0.4)kg。所有患儿术前均行超声心动图检查,部分患儿行心脏计算机断层摄影术(CT)检查。术后1个月、3个月、6个月、12个月及24个月定为临床随访时间,复查以超声心动图为主,必要时复查心电图及X线胸片,怀疑肺静脉梗阻者加做心脏CT检查。结果:全组无手术早期死亡,晚期死亡1例。平均体外转流时间(83.7±255)min,平均主动脉阳断时间(41.0±19.4)min。术后中位机械通气时间4d(范围2~128 d),中位正性肌力药物支持时间3 d(范围2~128 d),中位住院时间21 d(范围13~128 d)。19例存活者中平均随访(19.5±11.8)个月,其中2例术前有双侧肺静脉发育不良仍伴重度肺动脉高压、心脏功能Ⅲ级,其余17例恢复良好,心脏功能Ⅰ~Ⅱ级。结论:外科手术治疗新生儿TAPVC近中期疗效满意,伴双侧肺静脉发育不良者预后不佳。     

完全性肺静脉异位引流矫治术后肺静脉梗阻的治疗探讨

目的:总结完全性肺静脉异位引流(TAPVC)矫治术后肺静脉梗阻的治疗经验。方法:回顾性分析2011-01至2015-12于青岛市妇女儿童医院心脏中心施行TAPVC矫治术后发生肺静脉梗阻16例患儿的临床资料,其中男性10例。所有患儿均出院前、术后1个月、3个月、6个月、12个月、24个月回院复诊,包括经胸心脏超声多普勒、心电图、X线胸部正侧位片。以经胸心脏超声多普勒检查肺静脉流速2 m/s为梗阻标准,诊断肺静脉梗阻。结合患儿临床表现(反复心力衰竭、生长发育迟缓)确定二次手术时机。二次手术采用sutureless缝合技术和常规补片扩大技术。结果:术后发生肺静脉狭窄16例患儿无失访,其中7例吻合口周围狭窄(混合型1例,心下型3例,心上型2例,心内型1例);7例单支肺静脉狭窄,2例双支肺静脉狭窄,无三支及以上肺静脉狭窄。按术前Darling类型分:混合型2例,心下型5例,心上型5例,心内型4例。11例(68.8%)术后肺静脉狭窄流速增快发生在术后3~6个月。5例二次手术患儿中,4例采用sutureless缝合技术,1例采用常规补片扩大技术,均为吻合口周围梗阻狭窄,2例死亡,3例患儿随访中。结论:TAPVC矫治术后吻合口周围狭窄为主要二次手术适应证,并且需早期即行手术。     

完全性肺静脉异位引流患儿临床及尸检分析

目的 :探讨儿童完全性肺静脉异位引流 (TAPVC)的临床及尸检特征性改变。方法 :回顾性总结我院 1980～ 2 0 0 0年间收治的 2 3例TAPVC患儿的临床表现、心电图、胸片、心脏超声、心导管检查、手术及尸检资料。结果 :2 3例TAPVC患儿中属心上型 17例 (占 74 % ) ,心内型 6例 (占 2 6 % ) ,其中伴有动脉导管未闭 (PDA) 6例 ,房间隔缺损 (ASD) 11例 ,卵圆孔未闭 5例 ,室间隔缺损 (VSD) 2例 ,二尖瓣发育不良 1例。 2 3例患儿中 5例经内科保守治疗后好转出院 ,4例放弃治疗 ;3例施行纠治术 ,2例痊愈出院 ,1例死亡 ;死亡 12例 (5 2 % ) ,年龄均 <6个月 ,其中 10例尸检年龄为 2 4d～ 4个月 ,均发现有肺静脉梗阻性病变。结论 :TAPVC在先心病中并不少见 ,常伴发一些临床综合征及其他心脏畸形 ,大多在婴幼儿即有症状 ,本组病例中 5 2 %死于 6个月以内 ,特别是患肺血管梗阻性病变者 ,多于数日或 3～ 4个月内死亡 ,极易误诊为新生儿肺炎 ,尸检中才得以证实。心脏超声、心导管及选择性心血管造影术是常用的有效确诊手段     

完全性肺静脉异位引流的外科治疗体会(附16例报告)

完全性肺静脉异位引流 ( TAPVC)为一种少见的先天性心脏畸型 ,发病率占先天性心脏病的 1 %～3% ,自然病程预后极差 ,其中 5 0 %于生后 3个月内死亡 ,80 %于 1岁以内死亡 ,存活至 1岁以上的患儿常合并较大的房间隔缺损。 1 995年 6月至 2 0 0 2年 6月 ,我院共实施 TAPVC矫正术 1 6例 ,取得较好的效果。现总结报告如下。1　资料与方法1 .1　临床资料　本组 TAPVC患儿 1 6例 ,男 7例 ,女 9例 ;年龄 3个月至 6岁 ,平均 1 .2岁。均经心脏超声和多普勒检查明确诊断 ,1例行心导管检查和心血管造影。按 Darling分类法均为心上型 ,均合并房间…     

成人功能性单心室的一期Fontan手术治疗

目的:总结和分析成人功能性单心室Fontan手术治疗的早、中期结果。方法: 自2001年11月~2010年11月,共15例成人功能性单心室患者行一期Fontan手术治疗,术前心功能NYHA Ⅱ级4例,NYHA Ⅲ级11例;手术适应证为肺动脉发育良好,肺动脉平均压≤15 mmHg;手术方法为心外管道法Fontan手术,6例行人工血管和右心房开窗吻合,随访3月～6年。结果: 围术期死亡2例,术后平均拔除气管插管时间11 h（6～576 h）,平均住院时间13 d（7～44 d）,平均中心静脉压13 mmHg（7～16 mmHg）,平均动脉血氧饱和度96％（87%～99％）;12例患者心功能得到明显改善,4例心功能NYHA Ⅰ级,8例心功能NYHA Ⅱ级,1例心功能仍为NYHA Ⅲ级。结论: 一期心外管道法Fontan手术治疗成人功能性单心室手术早期和中期疗效良好。     

Risk factors in the Glenn bidirectional shunt as an intermediate procedure before Fontan correction

OBJECTIVE: The bidirectional Glenn shunt is the most common palliation before the Fontan repair, especially in high-risk patients. We studied the influence of certain risk factors in bidirectional Glenn results, with and without an additional source of pulmonary blood flow. METHODS: Between 1993 and 1998 twenty patients (6-53 months of age) underwent a bidirectional Glenn shunt as the intermediate repair for the Fontan procedure. Diagnoses were: 7 cases of double inlet single ventricle, 4 of tricuspid atresia, 3 of unbalanced AV septal defect, 4 of mitral atresia, 1 hypoplastic left heart syndrome and 1 TGA with hypoplasia of the right ventricle. 17 patients had undergone previous operations. Mean preoperative arterial oxygen saturation was 78.5%. In 6 patients an auxiliary source of pulmonary blood flow was added. RESULTS: Hospital mortality was 15%. In 4 patients the bidirectional Glenn failed. By univariate analysis low weight, preoperative functional status and high pulmonary pressure were factors associated with early death. In bidirectional Glenn failure only the duration of ventilatory support was significant. By multivariate analysis, preoperative functional status and pulmonary pressure were significant. Mean postoperative arterial oxygen saturation at a mean follow-up of 10 months was 84%. CONCLUSIONS: Bidirectional Glenn shunt is an effective and low-risk palliation for patients with univentricular hearts. Only low weight and high pulmonary pressure were significant in hospital mortality, and we advise the association of an additional source of pulmonary blood flow in these patients at the start. Early extubation provides correct performance of the shunt.     

Outcome of single ventricle and total anomalous pulmonary venous connection

Management of patients with single-ventricle physiology is significantly affected by anomalies of pulmonary venous return at all stages, whether primary palliation, bidirectional Glenn shunt, or completion of Fontan circulation. We treated 25 patients with pulmonary venous anomalies and single ventricle by staged palliation, from June 1996 to May 2005. Visceral heterotaxy with atrial isomerism was present in 19 of them. Primary palliation with a systemic-to-pulmonary artery shunt was undertaken in 15 patients. There were 5 early deaths, of which 4 were due to obstruction of pulmonary venous return. A bidirectional Glenn shunt was constructed in 17 patients including 10 who had it as a primary palliative procedure. There were 7 early deaths after the bidirectional Glenn procedure; only one was due to pulmonary venous obstruction. Five patients attained completion of the Fontan procedure. There was one early death after the Fontan operation. Anomalous pulmonary venous return can significantly complicate the management of patients with single ventricle, with an impact on survival in early infancy. Palliation with the aim of instituting extracardiac conduit Fontan circulation allows greater latitude and more streamlined management.     

Discontinuous Pulmonary Arteries Do Not Preclude Good Fontan Outcomes

Objective. Discontinuous pulmonary arteries are believed to portend poor outcomes for a single ventricle palliation leading to Fontan's operation. This is a single institutional review of patients with single ventricle and discontinuous pulmonary arteries who underwent pulmonary artery centralization as part of staged surgical palliation. Design. The study is a retrospective case series. Patients. From November 1997 to December 2005, 12 centralization procedures were performed on 12 single ventricle patients with discontinuous pulmonary arteries. The diagnoses at surgery were as follows: heterotaxy 67%, pulmonary atresia 75%, a single morphologic right ventricle 58%, a single morphologic left ventricle 33%, and functional single ventricle with atrial situs inversus 8%. Outcome Measures. The outcome was assessed by hospital survival, actuarial survival, and New York Heart Association (NYHA) classification at follow-up. Results. The overall actuarial survival following centralization is 100% (95% confidence interval = 0.698 to 1). Seventy-five percent of the patients have undergone a Fontan procedure. Median McGoon ratio pre-Fontan = 1.65 (range: 1–2.1). Median follow-up after Fontan = 4.4 years (range: 1.2–9 years). Overall actuarial survival following Fontan is 100% (95% confidence interval = 0.428–0.911). Following the Fontan, there have been no thromboembolic complications, protein-losing enteropathy, nor Fontan takedowns. One hundred percent of the Fontan patients are NYHA class I. Conclusions. This experience indicates that a resuscitative strategy for discontinuous pulmonary arteries can result in good outcomes after the Fontan procedure. The presence of discontinuous pulmonary arteries in patients with single ventricle physiology should not preclude a management strategy with the goal of Fontan candidacy.     

Fontan procedure--indication and clinical results

Twenty-six patients with tricuspid atresia (15), univentricular heart (7), and single ventricle (4) underwent 27 Fontan or modified Fontan procedures between 1975 and 1981. The age of the patients varied between 4 and 26 years. Twenty patients had had a total of 33 palliative operations prior to correction. The original Fontan procedure was performed in 10 patients from 1975 to 1977. According to the various anatomical findings modifications of the Fontan procedure, such as direct anastomosis or implantation of a valveless conduit, were introduced in 1977. Early mortality among all the patients was 22% (6 patients died). Three deaths occurred in the initial period 1975 to 1977. Among the last 20 patients (1978 to 1981) there were 3 early deaths. Three patients with single ventricle survived, one died due to pulmonary failure. There were 2 late deaths (sepsis, sudden cardiac death). Postoperative cardiac catheterization performed in 17 patients revealed excellent results in 13 patients; the remaining 4 displayed diminished arterial oxygen saturation, three of them had Glenn palliation prior to corrective surgery. Postoperative right atrial mean pressure varied from 10 to 23 mmHg. The left ventricular parameters were within the normal range.     

Early and late results of the modified Waterston shunt with PTFE grafts for palliation of complex congenital cyanotic heart disease in neonates.

During a 12 year period from 1978 to 1989, 35 infants under 4 weeks of age underwent palliative surgery for complex congenital cyanotic heart disease with a short (1-1.5 cm) PTFE graft between the ascending aorta and the right pulmonary artery (modified Waterston shunt). Twenty-three infants had pulmonary atresia and 14 had severe pulmonary stenosis. Underlying cardiac lesions were tetralogy of Fallot (n = 11), single ventricle (n = 7), transposition complexes (n = 6), and intact ventricular septum and hypoplastic right heart syndrome (n = 13). There were 4 early deaths (10.7%) in the entire series, 2 of which were shunt related. Three of the 4 occurred during our initial experience with this shunt in 1978 and 1979. They led to the modified Waterston shunt being abandoned for 3 years in favor of other shunt procedures. Since 1983 one early death occurred in 28 infants (3.5% mortality) with no death in the latest 26 patients. All patients were followed up between 6 and 108 months. There were 4 late deaths, one of which was shunt related. We observed a significant difference in the shunt patency rate between 4 and 5 mm grafts: palliation was adequate after 2 years in 52% of the patients when a 4 mm graft was used and in 89% of the 5 mm graft group (p less than 0.005). Reshunting was necessary in 7 infants between 5 and 60 months after primary surgery. Recatheterization was performed in 17 infants for suspected shunt failure (n = 6) or diagnostic reasons (n = 11).(ABSTRACT TRUNCATED AT 250 WORDS)     

Surgical evaluation of the modified Fontan procedure.

From 1980 to 1990 152 patients underwent Fontan operation at our institution. The following patient groups were identified: 1. patients with tricuspid atresia (n = 82, 54.0%); 2. patients with single ventricle (n = 31, 20.3%); 3. patients with a wide variety of non correctable, complex cardiac malformations (n = 39, 25.7%). In 27.0% of the patients a primary Fontan operation was performed. 45.0% of the patients received a previous shunt to increase pulmonary blood flow and in 29.4% of the patients a pulmonary artery band was placed to reduce pulmonary flow. Overall mortality was not significantly different in patients with previous palliative procedures (19.4%, n = 18) as compared to 17.4% (n = 6) in patients with primary Fontan operation. Risk of death was high in the group with complex cardiac malformations (28.2%, n = 11) and in patients with single ventricle (19.4%, n = 6). Early mortality was considerably less in patients tricuspid atresia (8.5%, n = 7). Postoperatively patients with incorporation of the residual right ventricular chamber and pulmonary valve (Fontan-Bjoerk) showed a significant (p less than 0.05) lower incidence of pleural effusion as compared to patients with other modifications of the Fontan procedure. Actuarial survival rate of all patients is 83.8 +/- 3.1% (mean +/- SEM) at ten years. The modified Fontan procedures are providing an accepted surgical method for patients with otherwise non correctable cardiac malformations.     

Recurrent Exacerbations of Protein-losing Enteropathy after Initiation of Growth Hormone Therapy in a Fontan Patient Controlled with Spironolactone

Protein-losing enteropathy (PLE) is a rare, but serious complication in single ventricle patients after Fontan palliation, and is associated with a 5-year mortality of 46%. We describe a patient with PLE after Fontan palliation who achieved remission with high-dose spironolactone (an aldosterone antagonist), but had three exacerbations each temporally correlated with the use of growth hormone (an aldosterone agonist). Because of the opposing mechanisms of action of these two medications, caution might be indicated when using growth hormone for patients with PLE who are successfully treated with spironolactone.     

A novel technique for creation of a systemic-to-pulmonary arterial shunt

A systemic-to-pulmonary arterial shunt is still widely used for palliation of some neonates with cyanotic congenital cardiac lesions. This procedure, however, is well known to be associated with some degree of morbidity and mortality. To reduce the incidence of iatrogenic pulmonary arterial deformities, we have devised a new and simple technique to create the shunt using a partial sternotomy, and have used our technique in 10 neonates with cyanotic cardiac malformations. All but one of our patients survived, and an early reoperation was needed in only one further patient for revision of the shunt. Successful bidirectional Glenn procedures were performed in five of the nine surviving patients within 18 months of the initial procedure. We believe that our technique provides superior palliation by permitting equal enlargement of the right and left pulmonary arteries, and thus facilitates subsequent completion of the Fontan circulation.