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。     

心外管道全腔静脉肺动脉连接术

目的 报告心管道全腔静脉肺动脉连接术（TECPC）应用经验。方法 横断上腔静脉与右肺动脉端侧吻合,切断下腔静脉前壁,保留原位吻合口,将下腔静脉通过人工血管与主肺动脉吻合,共治疗9例复杂性先天性心脏病,其中1例单心室改良Fontan术后4年频发室上性心动过速而改行TECPC。结果 全组手术后均生存。3例术后出现胸腔积液、乳糜胸并发症、均治愈。血流动力学指标满意,术后随访心功能Ⅰ-Ⅱ级,无心律紊乱发生。结论 TECPC手术操作简单,并发症少,适应证广泛,优于改良Fontan手术和传统的全腔静脉肺动脉连接术。     

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。     

非体外循环双向Glenn分流术治疗紫绀型复杂先天性心脏病

目的总结非体外循环下行双向Glenn分流术治疗紫绀型复杂先天性心脏病的临床经验,以提高手术疗效。方法2001年5月至2007年5月,56例患者在非体外循环下行双向Glenn分流术,术后采用电话和信件问卷方式对患者进行随访。结果术后早期死亡3例,死于低心排血量综合征。术后肺动脉压较术前增高（16．3±3．2mm Hg vs．12．4±2．1mm Hg,P〈0．05）;术后动脉血氧饱和度较术前提高（91％±5％ vs．74％±10％,P〈0．05）。随访39例,随访时间3个月～6年．失访14例。随访期间死亡2例,其中1例出院后即死于心力衰竭,1例于3年后死于肺气肿。37例生存患者的5年生存率为95％（37／39）,32例紫绀有所减轻,21例患者于术后3～6个月行心电图、彩色超声心动图检查,腔静脉肺动脉吻合口均通畅,肺动脉压为17．3±1．8mm Hg。2例患者于术后2年行全腔静脉-肺动脉连接术,二期手术后效果满意。结论非体外循环下双向Glenn手术安全、可靠,是一种治疗难以解剖根治或一期生理矫治的紫绀型复杂先天性心脏病患者的较好术式。     

全腔肺动脉连接术在复杂先天性心脏病中的应用

许多复杂先天性心脏病（先心病），尤其是合并单心室发育不良、一组房室瓣等病儿往往只能接受Fontan类手术，目前应用最多的是双向Glenn术和全腔肺动脉连接术。全腔肺动脉连接术（TCPC）是完全性生理矫正，在我院占同期Fontan类手术的58．1％（25143例）。现总结报道2001年8月至2005年5月25例复杂先心病病儿在我院行全腔肺动脉连接术的治疗体会，报道如下。     

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。     

全腔静脉肺动脉连接术的疗效与术式评价

目的 探讨全腔静脉肺动脉连接术（TCPC）的外科治疗效果并对相关术式进行评价。方法 1998年2月至2006年6月，对51例复杂先天性心脏病病儿采用TCPC予以纠治，15例采用心内隧道术式、29例采用心外管道术式、7例采用主肺动脉与下腔静脉直接吻合术式。结果 手术死亡4例（7.8％）。此外，术后低心排出量综合征6例（12．8％）、心律失常14例（29．8％）。80．1％术后24h内撤离呼吸机。术后48．9％留置胸引流管超过7d。随访1个月至8年，9例（19．1％）术后6个月后仍需长期或间断服用强心、利尿剂，7例（14．7％）有房性心律失常。结论 全腔静脉肺动脉连接术可取得满意的外科治疗效果。应用主肺动脉下腔静脉直接吻合术式，在部分病例中可望成为理想术式。     

全腔静脉-肺动脉连接术的临床应用

目的探讨全腔静脉-肺动脉连接术（TCPC）的手术方法，总结其临床应用经验。方法回顾分析2004年11月～2006年8月我科施行心内隧道TCPC和心外管道TCPC治疗21例复杂紫绀型先天性心脏病患者的临床资料，比较两种术式间术前、术后的临床指标。结果全组共死亡2例，1例死于术后反复发生心室颤动，1例死于低心排血量综合征。术后发生并发症16例，其中胸腔积液或心包积液7例，乳糜胸5例，经行胸腔闭式引流或胸腔穿刺后治愈；心律失常、肺部感染各1例，均经保守治疗治愈。19例生存患者术后紫绀均得到有效改善；除心内隧道TCPC平均手术时间（288．5min vs．217．1min，P〈0．05），呼吸机平均使用时间（9．63h vs 65．8h，P〈0．05）长于心外管道TCPC外，两种手术方式术后中心静脉压（CVP）、血红蛋白（HGB）、红细胞压积（HCT）、动脉血氧饱和度（SaO2）、平均肺动脉压（MPAP）、上腔静脉吻合口压差（SVCPG）、下腔静脉吻合口压差（IVCPG）等均差异无统计学意义。随访16例，随访时间2个月～2年，无死亡患者。超声心动图、胸部x线片复查结果满意。结论TCPC是治疗复杂紫绀型先天性心脏病的有效方法，两种手术方法的疗效相似，但各有利弊，对婴幼儿患者选择心内隧道TCPC较为合适，对年龄较大的患者选择心外管道TCPC为佳。     

二期Fontan术治疗小儿复杂先天性心脏病

目的总结二期Fontan术纠治小儿危重复杂先天性心脏病治疗经验。方法28例复杂心内畸形病儿进行二期Fontan术。年龄3．0～16．5岁,平均（7．3±3．8）岁;体重13．5～61．0 kg,平均（21．0±5．5）kg。主要为无脾综合征、多脾综合征、三尖瓣闭锁（TA）及房室连接不一致的右室双出口（DORV）等。一期分别行单侧Glenn、双侧Glenn、半Fontan术。两次手术间隔0．8～7．3年,平均（3．9±2．8）年,其中5例在Glenn术前行体肺动脉分流术。术前均行二维多普勒超声检查,23例行心导管和心血管造影检查。术中采用4种不同的连接方法将下腔静脉的血引流入右肺动脉,完成二期的全腔肺血管连接术（TCPC）。结果术后死亡4例（14．2％）。虽然采取综合措施降低肺血管阻力和增加回心血量,术后仍有12例发生低心输出量综合征,其中肾功能受损导致无尿而行腹膜透析术8例。2例右房和腔静脉内有血栓形成,再次进胸手术取栓后好转。吸入空气的动脉血氧饱和度在0．89～0．95。门诊随访3个月～2年,无死亡。无慢性渗出、蛋白丢失肠病等并发症。结论分期TCPC术可放宽对复杂先天性心脏病手术指征,并能增加手术成功率。分期TCPC术中心外管道的应用有许多优点。     

永存动脉干纠治术中右心室流出道重建方式的选择

目的比较采用自体肺动脉和外管道连接右心室的两种不同手术方法，以选择更佳的手术方案。方法2000年2月至2006年9月，我院共收治23例永存动脉干患者，手术年龄为1．5～63．3月龄。根据右心室切口与肺动脉连接的方法不同分为两组，组Ⅰ：18例，采用自体肺动脉连接右心室；组Ⅱ：5例，采用外管道连接右心室至肺动脉。3例合并主动脉弓中断。采用Kaplan—Meier法分析两组患者的早期生存情况、术后生存时间、再手术情况，采用配对t检验或团体t检验分析患者远期肺动脉生长情况以及心功能的变化。结果术后早期死亡2例。术后随访17例，随访时间2．14±1．97年（32．00d～6．95年），随访中无死亡。总生存率为91．30％（21／Z3），生存时间的95％可信区间为5．55～7．15年。1例因术后右室流出道梗阻（RVOTO）再次手术。组Ⅰ生存率为94．44％（17／18），组Ⅱ为80．00％（4／5）。术后组Ⅰ主肺动脉与右心室吻合口直径早期为1．01±0．26cm，远期为1．32±0．45cm（P=0．019）；组Ⅱ术后主肺动脉与右心室吻合口直径早期为1．46±0．77cm，远期1．26±0．21cm（P=0．581）；两组吻合口、左右肺动脉开口处血流速度变化差异无统计学意义（P〉0．05）。组Ⅱ近远期左、右肺动脉开口直径均大于组Ⅰ。组Ⅰ早期左心室射血分数（LVEF）为62.82％，远期为69．87％（P=0．026）；组Ⅱ早期LVEF为58．17％，远期为64．00％（P=0．029），两组患者术后远期心功能均较术后近期明显改善。无因动脉干瓣膜反流而再手术者。结论永存动脉干手术成功率高，术后生存率及随访结果满意。采用自体肺动脉重建右心室流出道，使吻合口具有生长性，术后肺动脉及分叉处梗阻率低，术后心功能良好。术后早期死亡与合并主动脉弓中断及动脉干瓣膜反流有关。     

The modified Fontan procedure: early and late results in 132 adult patients

OBJECTIVE: The modified Fontan procedure, usually performed in children, is used for the treatment of anomalies with a single functional ventricle. We reviewed our experience with the modified Fontan procedure performed in the adult patient. METHODS: Between October 1973 and May 2001, the modified Fontan procedure was performed on 132 adult patients (74 men, 58 women). Median age was 23 years (range, 18 to 53 years). Diagnoses included tricuspid atresia in 34 patients (26%), double-inlet left ventricle in 48 (36%), and complex lesions in 50 (38%). The majority of patients (89%) had at least one prior palliative procedure; the most common procedures were Blalock-Taussig shunt in 85 patients and Glenn anastomosis in 31. RESULTS: Operations included an atriopulmonary connection in 74 patients, lateral tunnel in 27, intra-atrial conduit in 14, right atrium-to-right ventricle in 9, extra-cardiac conduit in 3, and other in 5. Overall early mortality was 8.3%. Mortality was 6.5% for operations performed after 1980. This is comparable to the mortality of the modified Fontan procedure performed in children during the same time interval at our institution. All 7 of the early deaths since 1980 occurred in the complex lesion group. Morbidity included prolonged pleural effusion in 36 patients, atrial arrhythmias in 25, reoperation for bleeding in 13, permanent pacemaker in 8, and stroke in 2. Mean follow-up was 9.1 years with a maximum of 21.2 years. Actuarial survival for early survivors was 89% (84,95), 75% (67,84), and 68% (58,79) at 5, 10, and 15 years, respectively. Freedom from late reoperation was 89% (83,95), 85% (78,93), and 80% (70,91) at 5, 10 and 15 years, respectively. The majority (90%) of present survivors were New York Heart Association class I or II at follow-up. CONCLUSIONS: In properly selected adult patients with functional single ventricle, the modified Fontan procedure can be performed with early mortality similar to younger patients. Early mortality is more likely with complex lesions. The majority of late survivors have a good quality of life.     

Histomorphometric analysis of pulmonary vessels in single ventricle for better selection of patients for the Fontan operation

OBJECTIVE: In cases of single-ventricle physiology, the Fontan procedure often fails even when the usual selection criteria are strictly respected. We analyzed specimens from intraoperative open lung biopsies performed on 40 patients with single-ventricle physiology who were considered to be good candidates for the Fontan procedure. Histomorphometric study was performed to determine histologic factors predictive of failure of the Fontan procedure. METHODS: Histomorphometric studies were performed on samples from 40 patients aged 6 months to 23 years with single-ventricle physiology, either tricuspid atresia (n = 14) or univentricular heart (n = 26). The preoperative pulmonary arterial pressure was 18 mm Hg or less in 35 cases and greater than 18 mm Hg in 5 cases. Eighteen patients underwent total cavopulmonary connection, 16 patients underwent partial cavopulmonary connection, and 6 underwent a palliative procedure, as determined according to clinical and hemodynamic findings. RESULTS: Lung biopsy specimens from all 5 patients with pulmonary arterial pressure greater than 18 mm Hg appeared abnormal, whereas they appeared abnormal only 51% of the time in the low pulmonary arterial pressure group. The most frequent histologic abnormality observed was extension of smooth muscle cells in the wall of distal intra-acinar pulmonary arteries. Of the 18 patients who underwent the Fontan procedure, 9 had normal distal pulmonary arteries and good surgical results (except 1 with the Fontan circulation taken down for an anatomic reason). The remaining 9 had thick-walled distal intra-acinar pulmonary arteries with poor results of the Fontan procedure, and 6 died. The mean percentage wall thickness of small intra-acinar pulmonary arteries was significantly greater among the patients with bad results than among those with good results of the procedure (P <.01). CONCLUSIONS: Lung biopsy specimens were abnormal in 51% of patients with low pulmonary arterial pressure, there was no relationship between preoperative pulmonary arterial pressure and outcome, and extension of muscle in peripheral arteries was always present in cases of failure of the Fontan procedure. Histomorphometric study is therefore a useful adjunct to the usual selection criteria for surgical decision making in cases of single-ventricle physiology.     

Extending the limits for modified Fontan procedures

During the early development of atriopulmonary anastomotic operations (Fontan-Kreutzer), a number of physiologic and anatomical limits were proposed by the Fontan group as selection criteria. Among 167 consecutive patients undergoing modified Fontan procedures from 1973 through 1985, 109 (65%) patients exceeded one or more of the original selection criteria in areas of age, anomalies of systemic or pulmonary venous connection, pulmonary artery distortion, and pulmonary artery pressure. Twenty-six patients had a mean pulmonary artery pressure greater than 15 mm Hg, with 16 operative survivors (62%). Nineteen patients had anomalies of systemic and/or pulmonary venous connection, and 16 survived (84%). There were 44 patients under the age of 4 years, and 26 survived (59%). Twenty-five patients were older than 15 years, and 23 (92%) survived the Fontan procedure. Pulmonary artery distortion, relating to prior palliative operations, was found in 34 patients. Seventeen of these 34 survived a modified Fontan procedure (50%). Twenty-six patients had a pulmonary arteriolar resistance more than 2 Wood units times square meter, and 14 survived (54%), whereas 81 of 93 with a pulmonary arteriolar resistance of less than 2 U X m2 survived (87%). Multivariate analysis showed that pulmonary arteriolar resistance and pulmonary artery distortion had a significant, negative impact on survival, but age and anomalies of systemic and/or pulmonary venous connection did not. Pulmonary artery pressure was not an independent predictor of outcome. The results show that the original criteria may be exceeded in the areas of age and anomalies of pulmonary or systemic venous connection. Pulmonary artery pressure alone should not contraindicate a Fontan procedure if pulmonary arteriolar resistance is low. Pulmonary artery distortion from a prior palliative operation and elevated pulmonary arteriolar resistance increase the risk of a Fontan procedure.     

Thromboembolic complications after Fontan procedures: comparison of different therapeutic approaches

BACKGROUND: Although patients after Fontan procedure have a high incidence of thromboembolic complications, anticoagulant therapy is not handled uniformly. We analyzed the frequency and clinical relevance of thromboembolism after Fontan procedure and compared different therapeutic approaches. METHODS: From 1986 to 1998, 101 patients (mean age, 7.3 +/- 8.1 years) underwent Fontan type procedure (modified Fontan, n = 40; total cavopulmonary connection, n = 61). In 85 of 87 survivors, transthoracic echocardiography was performed; and in 31 transesophageal echocardiography and/or angiography was performed. Mean follow-up was 5.7 +/- 3.5 years. Three groups with different anticoagulant regimen were compared: group I without medication (n = 45), group II with acetylsalicylic acid therapy (n = 14) and group III with Coumadin (n = 26). RESULTS: Thromboembolic events occurred in 13 of 85 patients (15.3%; 3.3 events/100 patient-years). Type of operation as well as other known risk factors had no influence on the rate of thromboembolism. Within the first postoperative year, seven of 13 events occurred. A second peak developed beyond 10 years of follow-up. Patients benefit significantly from Coumadin compared with those who did not receive any medication, with similar results in the entire population and the subgroup of patients with total cavopulmonary connection (log-rank, p = 0.031 and p = 0.033, respectively). With 4.2 events/100 patient-years, the cumulative event rate was substantially higher in group I than with 1.6 in group II and with 1.1 in group III. No relevant bleeding complications occurred. CONCLUSIONS: Thromboembolism is frequent after Fontan procedure with a peak during the first postoperative year and another peak beyond 10 years of follow-up. Coumadin is the most effective prophylactic therapy in preventing thromboembolism. Therefore, we suggest initial oral anticoagulation therapy in patients with Fontan type operation.     

Physiological rationale for a bidirectional cavopulmonary shunt. A versatile complement to the Fontan principle

The original Fontan procedure included a classic superior vena cava-to-right pulmonary artery (Glenn) shunt. Subsequent experience demonstrated that this anastomosis was not essential and was an unnecessary commitment of the larger right pulmonary circulation to the smaller blood volume of the superior vena caval return. With application of the Fontan principle to more complex cardiac malformations, there has been a reconsideration of possible benefits of a cavopulmonary shunt in selected patients. A modified shunt from the divided end of the superior vena cava to the side of the undivided right pulmonary artery utilized in 21 patients is described. This shunt is designed to allow bidirectional pulmonary arterial distribution of both superior vena caval inflow and right atrial outflow after completion of the Fontan procedure. Twelve patients had the bidirectional shunt performed prior to a Fontan operation; five of these had a subsequent atriopulmonary connection and seven await operation. Eight patients had construction of this shunt at the time of their Fontan procedure. One patient had a bidirectional shunt constructed following atriopulmonary anastomosis to help relieve right atrial outflow obstruction. Two patients with univentricular heart undergoing simultaneous Fontan procedure and a bidirectional shunt died while in the hospital. The remaining 19 patients have been followed up for 2 months to 9 years with one late sudden death at 9 years. There have been no bidirectional cavopulmonary shunt failures, stenoses, kinks, or recognized pulmonary arteriovenous malformations. Postoperatively, eight patients had assessment of pulmonary distribution of shunt blood flow by angiography. Seven of these patients were also evaluated by radionuclide angiography. Superior vena caval blood flow via the bidirectional cavopulmonary shunt tended to be greater to the right lung, but bilateral pulmonary flow was documented in all but one patient. After Fontan operation, six of seven patients tested also demonstrated bilateral distribution of atriopulmonary flow. We concluded from our experience that this modified shunt provides excellent relief of cyanosis, allows bidirectional pulmonary distribution of both superior vena caval return and also the right atrial blood flow after atriopulmonary connection, and may be done before, with, or after a Fontan procedure and is compatible with all currently recommended modifications. Perioperative hemodynamic adjustments to the Fontan procedure may be improved by reducing atrial volume, and this may also be of potential benefit in the long-term adaptation to Fontan physiology by minimizing atrial distention.     

Functional status in adolescents and adults with Fontan circulation

OBJECTIVE: We determined functional status in adolescents and adults with Fontan circulation. METHODS: Functional status was studied in 25 patients surviving more than 2 years after the definitive procedure and currently no younger than 18 years old. Age at operation was 2 to 44 years old, and follow-up was 12 +/- 5 years. To achieve Fontan circulation, atriopulmonary connection was used in 14 patients, and total cavopulmonary connection in 11 patients. RESULTS: One patient undergoing atriopulmonary connection died suddenly 6 years after the Fontan procedure due to pulmonary thromboembolism. New York heart association functional status was class I in 23, and class II in 2, at the latest follow-up. Catheterization done 6.5 +/- 6.8 years after the Fontan procedure showed that systemic venous pressure was statistically higher (p = 0.019) in the atriopulmonary connection group (13 +/- 3 mmHg) than in the total cavopulmonary connection group (10 +/- 3 mmHg). Exercise tests in 19 patients showed reduced tolerance in all, with maximal oxygen intake being 24.4 +/- 5.1 ml/kg/min. Serum glutamic oxaloacetic transaminase and glutamic pyruvic transaminase were elevated above normal in 11 (44%). Arrhythmia was noted over longer terms in 4 patients undergoing atriopulmonary connection; in 3, atriopulmonary connection was converted to total cavopulmonary connection, and surgical intervention for atrial arrhythmia was successful. CONCLUSION: Although functional status in adolescents and adults with Fontan circulation was good, arrhythmia and liver dysfunction in such subjects could lead to morbidity.     

Factors influencing early and late outcome following the Fontan procedure in the current era. The 'Two Commandments'?

OBJECTIVE: This study was undertaken to identify the factors affecting early and late outcome following the Fontan procedure in the current era. We have examined whether conventional selection criteria, the 'Ten Commandments', are still applicable in the current era. MATERIALS AND METHODS: Between January 1988 and July 2004, 406 patients underwent a modified Fontan procedure at a median age of 4.7 years (IQR, 3.8-7.1 years). The single functional ventricle was of left (n=241, 59%) or right ventricular morphology (n=163, 40%). The modified Fontan procedure was performed using an atriopulmonary connection (n=162, 40%) or total cavopulmonary connection (TCPC) involving a lateral atrial tunnel (n=50, 12%) or extracardiac conduit (n=194, 48%). They were fenestrated in 216 patients (53%). RESULTS: The early mortality was 4.4% (n=18) and four other patients required takedown of the Fontan circulation. On multivariable analysis, early outcome was adversely influenced by two factors (p<0.05): preoperative impaired ventricular function and elevated pulmonary artery pressures. Two risk models were constructed for early outcome based on preoperative and predictable operative variables (Model 1) and all preoperative and operative data (Model 2). Both models were calibrated across all deciles (p=0.83, p=0.25) and discriminated well. The area under the ROC curve was 0.85 and 0.89, respectively. There were 21 late deaths, 1 patient required late takedown of the Fontan circulation and 3 required orthotopic cardiac transplantation. Actuarial survival was 90+/-2%, 86+/-2% and 82+/-3% at 5, 10 and 15 years, respectively. Multivariable analysis identified that outcome was influenced by preoperatively impaired ventricular function, elevated preoperative pulmonary artery pressures and an earlier year of operation. The freedom from reintervention was 83+/-4%, 76+/-4% and 74+/-8% at 5, 10 and 15 years, respectively. Additional risk factors for reintervention were right atrial isomerism and preoperative small pulmonary artery size. CONCLUSIONS: Late outcome of the Fontan circulation is encouraging. Ventricular morphology, surgical technique and fenestration do not appear to influence early or late outcome. Preoperatively impaired ventricular function and elevated pulmonary artery pressures have an adverse influence on both early and late outcome. Reintervention is common, with small preoperative pulmonary artery size being an additional risk factor.     

56例单心室外科治疗经验

总结单心室外科治疗经验。方法回顾性分析了接受外科治疗的５６例单心室病例结果：全组手术死亡率１４．２％,其中Ｆｏｎｔａｎ手术３９例,手术死亡率１５．４％,全腔静脉与肺动脉连接术１７例,手术死亡率１１．８％。结论提高治疗效果的基本经验是严格常握手术适应证。加强术后监护。     

Clinical Results of the Staged Fontan Procedure in High-Risk Patients

Background. For high-risk Fontan candidates, the introduction of a bidirectional Glenn shunt before total cavopulmonary connection (a two-staged strategy) may extend the indications for the Fontan procedure. The clinical results of the two-staged and one-staged Fontan procedure were thus reviewed and compared.

Methods. Between November 1991 and July 1996, the two-staged strategy was performed in 40 high-risk Fontan candidates with a mean interval of 17.2 months after introducing the bidirectional Glenn shunt (staged group). We considered a young age (<2 years), high mean pulmonary arterial pressure (≥20 mm Hg), high pulmonary vascular resistance (≥3 Wood units), small pulmonary artery (Nakata index <200 mm²/m²), atrioventricular valve incompetence (≥ moderate), distortion of pulmonary artery, anomalous pulmonary venous return, and poor ventricular function as risk factors for the successful completion of Fontan circulation. During the same pe-riod, 68 patients underwent the modified Fontan procedure in a one-step fashion (primary group).

Results. In the staged group after the bidirectional Glenn shunt, the mean pulmonary arterial pressure and ventricular end-diastolic pressure were both found to have decreased significantly to the same level as those in the primary group, whereas the pulmonary artery demonstrated a significantly smaller size than that in the primary group. Operative morbidity was similar in both groups. Operative mortality was also similar and low in both groups (1.5% in the primary group and 0% in the staged group).

Conclusions. A bidirectional Glenn shunt was found to be a useful interim palliation in high-risk Fontan candidates. This two-staged strategy may extend the operative indications for the Fontan procedure.     

Long-term results of atriopulmonary fontan connection for complex cardiac anomalies

To evaluate the long-term results of atriopulmonary Fontan connection, we reviewed the outcome of 20 consecutive patients (single ventricle 8, double outlet right ventricle 8, tricuspid atresia 3, mitral atresia 1; mean age, 13 +/- 11) who underwent this operation between 1981 and 1997 at our institution. Glenn shunt preceded Fontan operation in 1 patient. Four patients had a concomitant bidirectional Glenn anastomosis at the time of the Fontan operation. Follow-up was 100% complete, and total cumulative follow-up of all patients was 241 patient-years (maximum, 20 years). Three hospital deaths and 5 late deaths were observed, resulting in 58 +/- 11% of cumulative survival at 20 years after the operation. Sudden death probably due to arrhythmias was the major cause of the late death, and occurred in 3 patients. Reoperation was required in 5 patients (2%/patient-year) at 1 to 17 years postoperatively. Re-closure of an atrioventricular valve was performed for failure of the valve closure in 3 patients at 1 to 6 years postoperatively. Reconstruction of the stenotic atriopulmonary connection (APC) was concomitantly required in 2 of the 3 patients. Pacemaker implantation was required for bradycardia with atrial fibrillation in 1 patient at 17 years. One patient with atrial flutter developed intraright atrial thrombosis at 12 years after the initial Fontan operation, and this patient underwent conversion to extracardiac conduit cavopulmonary connection with right atrial maze procedure. Atrial tachyarrhythmias are most common complications, and occurred in 12 patients (5.0%/patient-year), resulting in 40 +/- 13% of the cumulative free rate at 15 years after the operation. Thus, failure of the atrioventricular valve closure and stenosis of APC were important early to intermediate complications after the atriopulmonary Fontan connection. On the other hand, atrial tachyarrhythmias were inevitable, and were most common late complications leading to intra-right atrial thrombosis, which was successfully resolved by conversion of the conventional atriopulmonary Fontan anastomosis to extracardiac conduit cavopulmonary connection.