应用系列改良Fontan手术治疗复杂先天性心脏病

目的探讨系列改良Fontan手术的特点和治疗复杂先天性心脏病的效果，进一步提高对其临床应用的认识。方法回顾性分析1992年9月～2006年6月期间77例复杂先天性心脏病患者行改良Fontan手术治疗的临床资料，其中行右心房肺动脉吻合术21例，心房内板障或管道全腔静脉-肺动脉连接术28例，心外人工管道全腔静脉-肺动脉连接术24例，自体右房壁管道全腔静脉肺动脉连接术2例，自体带蒂心包心外管道全腔静脉-肺动脉连接术1例，主肺动脉与下腔静脉吻合全腔静脉-肺动脉连接术1例。结果术后早期死亡5例，其中死于心力衰竭3例，突发心律失常1例，脑出血1例。再次手术1例，术后早期生存率93．5％（72／77），手术成功率92．0％。左心室舒张期末内径（LVEDD）较术前减小（52．5±7．8mm vs．62．5±11．0mm，P=0．013），左心室射血分数（LVEF）较术前增加（68．5％±4．0％vs ．62．0％±4．5％，P=0．032）。随访63例，随访率87．5％（63／72），随访时间1～15年。随访期间死亡4例，远期再次手术1例，远期生存率88．3％，手术成功率86．0％。结论在复杂先天性心脏病的治疗中，改良Fontan手术有良好的疗效；同时根据具体解剖结构可选择不同的手术方式。     

非体外循环下改良全腔静脉-肺动脉连接术

目的：探讨非体外循环下行改良全腔静脉－肺动脉连接手术治疗功能性单心室技术。方法：3例功能性单心室病儿在非体外循环下施行了改良全腔静脉－肺动脉连接手术。术中先作上、下腔静脉插管并与右房插管连接，静脉血分流入右房，然后置右肺动脉侧壁钳，上腔静脉远心端与右肺动脉上缘行端侧吻合，吻合口尽量偏左；下腔静脉通过心外管道与右肺动脉下缘连接，吻合口尽量偏右。最后缝扎肺动脉口。结果：术后无早、晚期死亡，仅1例发生低心输出量综合征及胸腔渗出。动脉血氧饱和度0．94－0．97。术后随访1－3个月，心功能I级2例，Ⅱ级1例。结论：非体外循环下行改良全腔静脉－肺动脉连接术，是治疗功能性单心室的有效技术，可取得良好的手术效果。     

改良全腔静脉-肺动脉连接术治疗功能性单心室

目的 为了进一步改善全腔静脉－肺动脉连接手术的效果,探讨其经典手术方式的改良方法。方法 对１３例功能性单心室施行了改良全腔静脉－肺动脉妆手术 上腔静脉远心民右肺动脉上缘作端侧吻合,吻合口尽量偏左,近心端与右肺动脉下缘吻合,吻合口尽量偏右。其中５便在心脏不停跳下用Ｇｏｒｅ－Ｔｅｘ心外管道连接下腔静脉与右肺动脉下缘,８例采用心房内隧道。结果 术后早期死亡１例（７．７％）,１２例（９２．３％）存活者术后     

改良Fontan手术治疗复杂先天性心脏病

目的 总结改良Fontan手术治疗复杂先天性心脏病的临床经验.方法 1996年11月～2005年5月,采用改良Fontan手术纠治124例复杂先天性心脏病(病种包括三尖瓣闭锁、单心室、右心室双出口、大动脉错位、肺动脉闭锁、矫正型大动脉转位、右心室发育不良等)患者,手术年龄7.6±5.5岁.常温非体外循环下手术19例,体外循环下手术105例.右心房-肺动脉连接17例,右心房-右心室连接19例,全腔静脉-肺动脉连接术(TCPC)88例.23例行分期手术.结果 术后早期(术后30d)死亡17例(13.7%),其中行右心房-肺动脉连接者死亡率为23.5%(4/17),行右心房-右心室连接者死亡率为15.8%(3/19),行TCPC者死亡率为11.4%(10/88),同期预留或术后开窗手术死亡率为14.6%(6/41),分期手术患者死亡率为8.7%(2/23).死亡原因低心排血量、多器官功能衰竭和心室颤动等.术后早期并发症发生率为16.9%(21/124),主要为胸腔积液、心律失常、心包积液和低心排血量综合征等.术后随访89例,随访时间6～65个月.远期再住院率6.5%,再手术率0.9%.3例出现再发性胸腔积液,3例出现心包积液,1例出现下腔静脉梗阻,均经相应的治疗后治愈.其余患者心功能恢复好.结论 改良Fontan手术是治疗复杂先天性心脏病中功能性单心室的最佳手术方案;房间隔开窗可明显提高术后早期疗效,减少渗出.     

改良Fontan手术的危险因素分析

目的 探讨改良Fontan手术后早期死亡的危险因素和手术适应证。方法 统计分析154例改良Fontan手术病人的17个围手术期指标与手术结果。结果 术后早期死亡37例，死亡率24.0%。单因素分析结果表明，术前McGoon比值≤1.8，术前房室瓣存在反流，手术方式，术后右房压≥20mmHg,心律失常，严重低心输出量综合征是手术早期死亡的高危因素，多因素Logistic逐步回归分析结果显示，手术方式（右房与右室连接，右房与肺动脉连接），术后右房压和术后严重低心输出血量综合征与术后早期死亡有关。结论 术前严格选择心室功能和肺动脉发育好，无明显房室瓣反流的病例，采用全腔静脉与肺动脉连接术式，术后加强监护是预防和降低术后早期死亡的有效措施。     

心外管道全腔静脉-肺动脉连接术在复杂先天性心脏病外科治疗中的应用

目的总结心外管道全腔静脉-肺动脉连接术治疗复杂先天性心脏病的应用经验及其治疗效果。方法回顾性分析2006年9月至2012年12月间广州军区广州总医院心脏外科中心52例行心外管道全腔静脉-肺动脉连接术患者的临床资料。12例行一期心外管道全腔静脉-肺动脉连接术,40例为双向Glenn手术后行二期心外管道全腔静脉-肺动脉连接术。分析所有患者的临床资料,并比较两种手术方式患者的死亡率、并发症发生率、住院时间、住重症监护室（ICU）时间、机械辅助通气时间、动脉血氧饱和度的改善情况等。结果围术期死亡2例,死亡率3．8％。其中1例术后因严重低心排血量综合征死亡,1例术后因多器官功能衰竭死亡;50例治愈出院。二期心外管道全腔静脉．肺动脉连接术患者（40例肌械辅助通气时间、住ICU时间、住院时间明显短于一期心外管道全腔静脉-肺动脉连接术患者（12例）,但两种手术方式患者术后并发症发生率及术后动脉血氧饱和度（二期手术与一期手术比较：93％±3％vs．94％±3％）、死亡率（二期手术与一期手术比较：2．5％vs．8．3％）差异均无统计学意义（P〉0．05）。随访45例（90％）,随访时间6～52个月,随访期间无死亡。术后3个月,存活患者心功能均为Ⅰ～Ⅱ级,心脏彩色超声心动图显示：腔静脉肺动脉吻合口血流通畅。结论心外管道全腔静脉．肺动脉连接术血流动力学更符合生理血流动力学特点,手术操作简捷,是不能进行双心室治疗时的有效手术术式;分期心外管道全腔静脉-肺动脉连接术较一期心外管道全腔静脉-肺动脉连接术手术适应证广泛,术后恢复较好,更易推广。     

功能性单心室的外科治疗

目的 为了使更多的先天性心脏病患者得到纠治，回顾性总结手术治疗功能性单心室的临床经验。方法 手术纠治127例功能性单心室患者，年龄7个月－12岁，体重7．5－39kg，其中丰唐手术（Fontan operation)72例，半Fontan术3例，双向上腔静脉肺动脉吻合术51例，肺动脉环缩术1例。结果 早期Fontan术19例，死亡11例；改良Fontan术53例，死亡10例；半Fontan术死亡1例；双向上腔静脉肺动脉吻合术死亡4例；总手术死亡率20．5％。结论 功能性单心室必须早期得到纠治，控制肺动脉血流，预防严重缺氧。双向上腔静脉肺动脉吻合术能减少功能性单心室的容量负荷，保持足够的心排血量。改良Fontan术是功能性单心室的最佳手术方案。     

改良侧通道Fontan手术在小儿复杂先天性心脏病中的应用

目的总结改良侧通道Fontan手术纠治小儿危重复杂先天性心脏病的经验,探讨该手术方法的优越性。方法自1999年3月至2008年8月我院共对86例复杂型心内畸形患者施行心内板障侧通道Fontan手术,男47例,女39例;年龄1.9～11.5岁,平均年龄4.7岁;体重8.6～52.0kg,平均体重17.0kg。病种为无脾综合征33例,多脾综合征17例,三尖瓣闭锁（TA）11例,房室连接不一致的右心室双出口（DORV）11例,完全性大动脉错位（D-TGA）合并肺动脉狭窄8例,矫正性大动脉错位（cTGA）5例,Ebstein’畸形1例。术前分别行单侧双向上腔静脉肺动脉吻合术（BSCPA）,双侧双向上腔静脉肺动脉吻合术和半-Fontan手术（hemi-Fontan opertiong）;二次手术时间间隔0.7～7.8年（3.6±2.9年）。术中采用心内板障侧通道Fontan手术（LT组,47例）和改良心内板障侧通道Fontan手术（M-LT组,39例）方法连接下腔静脉的血引流入右肺动脉,部分完成二期改良Fontan手术。结果两组共死亡7例（9%）,LT组死亡5例,M-LT组死亡2例,差异无统计学意义（χ^2=0.865,P=0.448）。在分期改良Fontan手术中,M-LT组患者术前行BSCPA术明显多于LT组。术后仍有22例患者发生低心排血量综合征,其中肾功能受损导致无尿而行腹膜透析13例,透析2～5d后尿量恢复。术后LT组的心律失常患者明显多于M-LT组（χ^2=8.763,P=0.003）,置胸腔引流管时间LT组明显长于M-LT组（t=2.970,P=0.003）。门诊随访3个月～8年,无1例死亡。M-LT组随访33例（85%）,LT组随访39例（83%）,均未出现严重的并发症,患者活动能力明显改善。结论改良侧通道Fontan手术有一定的优越性,不失为一种提高手术成功率、减少术后并发症的改良方法。     

双向上腔静脉肺动脉吻合术

双向上腔静脉肺动脉吻合术(bidirectional superior cavopulmonary anastomosis,BCPA)是应用于单心室纠治中的姑息术式,主要形式有双向Glenn分流术和半Fontan手术.适应证包括作为全腔静脉-肺动脉连接术的姑息形式,部分双心室修补或1 1/2心室修补及复杂先天性心脏病手术的辅助步骤.目前对年龄的选择、术后肺动脉的生长发育、额外肺血流是否保留、侧枝血管的形成及转换全腔静脉-肺动脉连接术的时机等问题仍值得进一步研究.     

心外管道全腔静脉肺动脉连接术

心外管道全腔静脉肺动脉连接术 (totalextracardiaccavopulmonaryconnection ,TECPC)是对全腔静脉肺动脉连接术的改进。 1999年 10月至 2 0 0 2年 9月 ,我们应用心外全腔静脉肺动脉连接术纠治了 11例复杂先天性心脏病 ,现报道如下。资料和方法　全组男 6例 ,女 5例 ;年龄 4～ 16岁。术前均有明显口唇及四肢末梢发绀。心功能II～III级。心胸比率 0 5 2～ 0 6 1。心电图均示窦性心律 ,右心室肥大。术前均经超声心动图、心导管和心血管造影确诊 ,其中单心室 4例 ,完全性大动脉转位 3例 ,右室双出口 2例 ,三尖瓣闭锁 2例。全组病人均有肺动…     

心外管道全腔静脉-肺动脉连接术治疗复杂先天性心脏病

目的总结心外管道全腔静脉-肺动脉连接术（ECTCPC）治疗复杂先天性心脏病的临床经验,并就手术适应证、手术方法及手术效果进行讨论。方法1998年6月至2006年12月,68例先天性心脏复杂畸形的患者接受了ECTCPC。包括单心室伴有大动脉转位、肺动脉瓣狭窄45例：三尖瓣闭锁、右心室发育不良19例;三尖瓣下移畸形并右心室发育不良4例。其中合并永存左上腔静脉6例,双向Glenn术后行全腔静脉-肺动脉连接术18例（其中包括单心室、肺动脉闭锁、左肺动脉狭窄双向Glenn术后1例）,单心房、单心室、心上型完全性肺静脉异位引流、多发粗大体肺侧支1例。全组采用体外循环下手术共57例,其中8例患者因需要矫正心内畸形在主动脉阻断下手术外,其余49例均在全身麻醉并行体外循环心脏跳动下进行;非体外循环下手术11例。结果术后早期死亡2例,病死率为2．9％。其中1例死于术后反复肺内出血,1例死于上消化道反复大出血。66例痊愈出院,术后随访1个月至8年,无晚期死亡。所有患者症状消失,血氧饱和度90％～96％,恢复良好。结论ECTCPC方法简便易行,术后并发症较少,效果好,较其他术式有较大优点。     

Total Extracardiac Right Heart Bypass Using a Polytetrafluoroethylene Graft

A bstract Background : With regard to hemodynamics and late arrhythmias, total cavopulmonary connection has been accepted as a superior technique as compared to Fontan type procedures. However, intra-atrial baffles for lateral tunnel or conduit remain construction retain some similar disadvantages. Patients and Methods : As an alternative to total cavopulmonary connection, total extracardiac right heart bypass using a polytetrafluoroethylene tube for the inferior vena cava to pulmonary artery connection may obviate some problems. Five patients with complex heart disease necessitating one ventricle repair underwent this procedure successfully. Results : Aortic cross-clamp time ranged from 0 to 24 minutes (mean = 15.8 min). No case required takedown or an additional step. Although the follow-up periods have been relatively short (mean = 19 months), all patients are well and no arrhythmic event or thromboembolic episode has occurred. Conclusions : As a simple, safe, and reproducible procedure, total extracardiac right heart bypass is an alternative to Fontan or total cavopulmonary connection procedure.     

Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience

BACKGROUND: Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. METHODS: Thirty-one patients (19.9 +/- 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. RESULTS: There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. CONCLUSIONS: Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.     

全腔静脉-肺动脉连接术的回顾与展望

Fontan手术迄今仍是治疗单心室一类复杂先天性心脏病的主要手段。Fontan手术方法从1968年问世以来有许多改进,早期应用的心房-肺动脉连接术已为全腔静脉-肺动脉连接术所取代。通过对心房内隧道和心外管道全腔静脉-肺动脉连接术的中、晚期手术疗效进行比较分析,心外管道优点更多。然而,由于缺乏右心室的泵血功能,晚期循环衰竭最终都难以避免。当Fontan循环衰竭药物治疗无效时,惟一的选择是心脏移植,但后者供体来源缺乏。目前正研究开发的,旨在＂双心室化＂单心室Fontan循环的腔-肺机械辅助装置,有很好的前景。近年来胎儿心脏病诊疗技术的进展也拓宽了治疗复杂先天性心脏病的径路,经导管扩张严重主动脉瓣狭窄防止发展为左心室发育不全综合征,以增加双心室修复的机会;或对不能根治的复杂先天性心脏病患者终止妊娠,以降低此类复杂先天性心脏病的出生率等。我们结合文献对Fontan手术的方法进行回顾和展望。     

Presurgical Evaluation of Fontan Connection Options for Patients With Apicocaval Juxtaposition Using Computational Fluid Dynamics

Apicocaval juxtaposition (ACJ) is a rare congenital heart defect associated with single ventricle physiology where optimal positioning of the Fontan conduit for completion of total cavopulmonary connection (TCPC) is still controversial. In ACJ, the cardiac apex is ipsilateral with the inferior vena cava (IVC), risking kinking and collapse of the Fontan conduit at the apex of the heart. The purpose of this study is to evaluate two viable routes for Fontan conduit connection in patients with ACJ, using computational fluid dynamics. Internal energy loss evaluations were used to determine contribution of conduit curvature to the energy efficiency of each cavopulmonary anastomosis configuration. This percentage of energy loss contribution was found to be greater in the case of a curved extracardiac conduit connection (44%, 4.1 mW) traveling behind the ventricular apex, connecting the IVC to the left pulmonary artery, than the straighter lateral tunnel conduit (6%, 1.4 mW) installed through the ventricular apex. In contrast, net energy loss across the anastomosis was significantly lower with extracardiac TCPC (9.3 mW) in comparison with lateral tunnel TCPC (23.2 mW), highlighting that a curved Fontan conduit is favorable provided that it is traded off for a superior cavopulmonary connection efficiency. Therefore, a relatively longer and curved Fontan conduit has been demonstrated to be a suitable connection option independent of anatomical situations.     

Modified fontan operation

From November 1988 to October 1991 30 patients underwent a total extracardiac right heart bypass for complex cardiac anomalies by means of bidirectional cavopulmonary anastomosis and interposition of a conduit from the inferior vena cava to the pulmonary artery. Mean age at surgery was 6.4 years and mean weight 19.2 kg. There was 1 hospital death (3%) due to a borderline indication for a Fontan operation. 2 patients had further surgery: In 1 the repair was taken down due to the stenosis of the left pulmonary artery and the patient was left with a bidirectional cavopulmonary anastomosis only, the second patient required a revision of the cavopulmonary anastomosis due to a stenosis of the superior vena cavaright pulmonary artery junction. There were no late deaths and the survivors are in good clinical condition a mean of 15.1 months after the operation. We propose this technique as an alternative surgical option in candidates for a Fontan operation in whom atrial septation is hazardous including those with 1) hypoplasia or atresia of the left atrio-ventricular valve, 2) common atrioventricular valve, 3) anomalies of systemic and/or pulmonary venous return, or 4) auricular juxtaposition.     

Computational fluid dynamics in the evaluation of hemodynamic performance of cavopulmonary connections after the Norwood procedure for hypoplastic left heart syndrome

OBJECTIVE: Computational fluid dynamics have been used to study the hemodynamic performance of surgical operations, resulting in improved design. Efficient designs with minimal energy losses are especially important for cavopulmonary connections. The purpose of this study was to compare hydraulic performance between the hemi-Fontan and bidirectional Glenn procedures, as well as the various types of completion Fontan operations. METHODS: Three-dimensional models were constructed of typical hemi-Fontan and bidirectional Glenn operations according to anatomic data derived from magnetic resonance scans, angiocardiograms, and echocardiograms. Boundary conditions were imposed, and fluid dynamics were calculated from a mathematic code. Power losses, flow distribution to each lung, and pressures were measured at three predetermined levels of pulmonary arteriolar resistance. Models of the lateral tunnel, total cavopulmonary connection, and extracardiac conduit completion Fontan operations were constructed, and power losses, total flow distribution, vena caval and pulmonary arterial pressures, and flow distribution of inferior vena caval return were calculated. RESULTS: The hemi-Fontan and bidirectional Glenn procedures performed nearly identically, with similar power losses and nearly equal flow distributions to each lung at all levels of pulmonary arteriolar resistance. However, the lateral tunnel Fontan procedure as performed after the hemi-Fontan operation had lower power losses (6.9 mW, pulmonary arteriolar resistance 3 units) than the total cavopulmonary connection (40.5 mW) or the extracardiac conduit (42.9 mW), although the inclusion of an enlargement patch toward the right in the total cavopulmonary connection was effective in reducing the difference (10.0 mW). Inferior vena caval flow to the right lung was 52% for the lateral tunnel, compared with 19%, 30%, 19%, and 15% for the total cavopulmonary connection, total cavopulmonary connection with right-sided enlargement patch, extracardiac conduit, and extracardiac conduit with a bevel to the left lung, respectively. CONCLUSIONS: According to these methods, the hemi-Fontan and bidirectional Glenn procedures performed equally well, but important differences in energy losses and flow distribution were found after the completion Fontan procedures. The superior hydraulic performance of the lateral tunnel Fontan operation after the hemi-Fontan procedure relative to any other method may be due to closer to optimal caval offset achieved in the surgical reconstruction.     

New approach to the surgical management of pulmonary arteriovenous malformations after cavopulmonary anastomosis

The development of pulmonary arteriovenous malformations after cavopulmonary bypass in patients with congenital heart disease is well documented. We report successful management of pulmonary arteriovenous malformations after cavopulmonary bypass in a patient with an interrupted inferior vena cava (IVC) and multiple hepatic veins utilizing an extracardiac conduit from the hepatic veins to the hemiazygous continuation of the interrupted IVC. This technique, performed without circulatory arrest or an atriotomy, may limit morbidity associated with intracardiac procedures in patients with single ventricle morphology. Furthermore, this case suggests an alternative technique for completion Fontan in patients with an interrupted IVC and multiple hepatic venous drainage.     

Extracardiac total cavopulmonary connection using a Y-shaped graft

We report on 17-year-old Fontan candidate with a seerely distorted central pulmonary artery (PA) who underwent a successful extracardiac total cavopulmonary connection using a Y-shaped bifurcated graft. A nonanatomic pathway from the inferior vena cava to the left PA was constructed and positioned anterior to the ascending aorta. The other arm was used as a conduit between the inferior vena cava and the right PA. All procedures were performed under temporary venous bypass without cardiopulmonary bypass.     

Extracardiac Fontan operation without cardiopulmonary bypass

AIM: The avoidance of cardiopulmonary bypass and aortic cross-clamping in patients possessing single ventricular physiology has potential advantages including preservation of ventricular and pulmonary functions; early extubation, decreased incidence of pleural effusions and decreased requirement of inotropic agents and blood products. In this study, we assessed the postoperative outcome of patients who have undergone extracardiac Fontan operation performed without cardiopulmonary bypass. METHODS: Between March 1999 and August 2002, 10 consecutive patients (6 males and 4 females) underwent extracardiac Fontan operation without cardiopulmonary bypass. The age of patients ranged between 1.5 to 12 (5.2+/-3.1) years. All the patients requiring any intracardiac intervention were excluded from the study. Previous operations of the patients were modified Blalock-Taussig shunt procedure in 3 patients, bidirectional cavopulmonary shunt operation in 2 patients and pulmonary arterial banding in 1 patient. All operations were performed without cardiopulmonary bypass. Bidirectional cavopulmonary anastomosis was performed by using a transient external shunt constructed between the superior vena cava and right atrium. An appropriate sized tube graft was anastomosed to the inferior surface of right pulmonary artery. Finally, inferior vena cava to tube grafts anastomosis was performed with the aid of another external shunt constructed between inferior vena cava and right atrium. During the procedure central venous pressure, blood pressure and arterial oxygen saturation levels were continuously monitored and recorded. RESULTS: The mean intraoperative Fontan pressure was 16.1+/-2.75 mmHg. Intraoperative fenestration was required in 4 patients with a Fontan pressure above 18 mmHg. There were no intra and postoperative deaths. Three patients required mild doses of inotropic support during the postoperative period. All patients were weaned off mechanical ventilation within 24 h. The mean arterial oxygen saturation raised from 74.5+/-4.2% to 93.5+/-2%. Arterial oxygen saturation was 95+/-0.6% in 6 patients without fenestration and 91.2+/-0.5% in 4 patients with fenestration (P=0.001). All patients were in sinus rhythm postoperatively. Only 2 patients required blood transfusion. Two patients suffered from prolonged pleural effusion (more than 7 days). The mean intensive care unit and hospital stay periods were 3.3+/-1.5 and 15.4+/-5.3 days, respectively. CONCLUSIONS: The extracardiac Fontan operation performed without cardiopulmonary bypass provides good results in short and midterm follow-up periods with improved postoperative hemodynamics.