Midterm follow-up of the status of Gore-Tex graft after extracardiac conduit Fontan procedure

Objective: Extracardiac conduit Fontan procedure has some theoretical advantages over other types of Fontan procedures, such as optimized flow dynamics, a lower frequency of arrhythmias, and technical ease of procedure. However, lack of growth potential and thrombogenicity of the artificial conduit is the main concern and can possibly lead to reoperation for the conduit stenosis. In this study, we investigated the change and the status of the Gore-Tex graft used in extracardiac conduit Fontan procedure. Methods: Between 1996 and 2005, 154 patients underwent extracardiac conduit Fontan procedure using Gore-Tex graft. Among these, 46 patients underwent cardiac catheterization during follow-up period. We measured the internal diameter of the conduit and inferior vena cava angiographically. Results: Mean follow-up duration was 36.1 ± 19.7 months. The conduit diameter used was 16 mm in 10 patients, 18 mm in 16, 20 mm in 14, 22 mm in 4, and 24 mm in 2 patients. The mean conduit-to-inferior vena cava cross-sectional area ratio was 1.25 ± 0.33. According to the conduit size used, this ratio was 1.03 ± 0.17 for 16 mm conduits, 1.33 ± 0.37 for 18 mm, 1.33 ± 0.36 for 20 mm, 1.28 ± 0.26 for 22 mm, and 1.05 ± 0.06 for 24 mm conduits (p < 0.05, 16 mm vs 18 mm and 20 mm). The mean percent decrease of the conduit cross-sectional area was 14.3 ± 8.5%, and this did not differ significantly according to the conduit size (p = 0.82). Follow-up duration and the percent decrease of the conduit cross-sectional area did not show significant correlation (r = 0.22, p = 0.14). There was no reoperation due to conduit stenosis. Conclusions: During midterm follow-up of about 3 years, the conduit cross-sectional area decreased by 14%, and this did not differ according to the conduit size used. The extent of decrease of the conduit cross-sectional area remained stable irrespective of the follow-up duration. Sixteen millimeters conduit showed no evidence of clinically significant stenosis, but careful follow-up is warranted because of the possible conduit stenosis relative to the patients’ somatic growth.     

体外循环心脏不停跳下心外管道Fontan手术

目的评估体外循环心脏不停跳下心外管道Fontan手术的方法及其效果。方法本组42例中,男性31例、女性11例,年龄3～19岁,其中心室双人口19例,三尖瓣闭锁10例,二尖瓣闭锁3例,其他复杂先天性心脏病10例。均在常温体外循环心脏不停跳下施行心外管道Fontan手术,其中8例加行开窗术;1例先做了双向腔肺动脉分流术,术后2年行心外管道Fontan手术。结果术后早期和晚期各死亡1例,分别死于急性肝功能衰竭和反复肺部感染,随访1．0～4．5年,40例心功能为Ⅰ级和Ⅱ级,动脉血氧饱合度92％～96％。结论体外循环心脏不停跳下心外管道Fontan手术的近、中期效果满意．可在单一心室修复中选用。     

Optimal conduit size for extracardiac Fontan operation

Background: Lack of conduit growth potential and thrombogenicity are the main drawbacks of the extracardiac Fontan operation (ECFO). Optimal size of the conduit according to the patients age and inferior vena cava diameter has not been established. Objectives: We set out to ascertain whether the optimal dimensions of the conduit could be determined before an ECFO. Methods: Actual and expected age-related inferior vena cava diameters were compared with the extracardiac conduit diameter in 20 patients after ECFO. In 50 other pediatric and adult patients, the distance between intrapericardial part of the inferior vena cava and the undersurface of the right pulmonary artery (IVC–RPA) was measured. Cases of conduit thrombosis were analyzed. Results: The actual diameter of the inferior vena cava was variable and has a weak correlation with anthropmetric data and expected diameter (R=0.07–0.23, P=0.32–0.76). The IVC–RPA distance correlated with height (R=0.87, P=0.0001), but was also variable. At the age of 2–4 years and body weight 12–15 kg IVC diameter and IVC–RPA distance are equal to 60–80% of adult values. Conduit thrombosis developed in two patients with unfavorable Fontan hemodynamics and oversized conduits. Conclusions: Considering the inferior vena cava size, ECFO may be performed at the age of 2–3 years and at a body weight 12–15 kg, when a hemodynamically optimal almost adult sized conduit can be implanted. Optimization of the conduit is necessary on the basis of the actual inferior vena cava diameter and IVC–RPA distance. Anticoagulation postoperatively should be considered to prevent conduit thrombosis in patients with suboptimal Fontan circulation     

Immunohistological findings for an extracardiac conduit in Fontan pathway constructed with pedicled autologous pericardium

Although autologous pericardium has been used in pedicled fashion for various reconstructive procedures in congenital cardiovascular surgery with the expectation that it will be able to grow and remain viable after implantation, no clinical study has evaluated the histological characteristics of implanted pedicled pericardium long after previous implantation. We describe herein histological findings of pedicled pericardium, which had been used for three years as an extracardiac conduit in Fontan pathway. A four-year-old boy with a history of Fontan operation using pedicled autologous pericardial roll required conduit replacement three years after the previous operation, and resected pericardial tissue was examined immunohistologically. Staining for CD34 revealed abundant microvasculature, suggesting preservation of viability. Staining for factor VIII demonstrated the presence of endothelium on the luminal surface of the conduit. Elastica-van Gieson staining revealed a band of elastic tissue, which is generally found not in the native pericardium but in the vascular wall structures. These findings suggested that the pedicled pericardium thus appeared to have remained viable and might have differentiated to resemble tissue of the vascular wall after having been used in Fontan pathway.     

改良外管道Fontan术治疗心脾综合征型复杂先天性心脏病

目的　回顾总结改良外管道Fontan术治疗无脾或多脾综合征伴发的复杂型先天性心脏病(先心病)经验。方法　青紫型复杂先心病病儿11例,无脾9例(右房异构,单心室合并共同房室瓣6例,房室连接不一致的右室双出口2例,纠正性大动脉错位1例) ;多脾2例(左房异构,右室双出口合并共同房室瓣)。平均年龄(6 3±3 7)岁,平均体重(2 1 0±5 5 )kg。直接行改良外管道Fontan术3例,双向Glenn术后行二期外管道Fontan术8例。结果　术后早期发生低心排出量综合征2例,肾衰1例,室上性心动过速1例,胸腔引流(12 0ml d) >10d 2例。超声提示上腔吻合口通畅(流速0 6～0 8m s) ,下腔血流速度0 3～0 5m s ;动脉血氧饱和度9例在0 92～0 95 ,2例为0 86 ;活动能力明显改善,无早期死亡。随访中超声显示上腔静脉血流速度0 8m s,下腔静脉血流速度0 4～0 7m s ,随呼吸而变化。外管道通畅,无血栓形成,管道窗口直径0 34cm ,血流右向左分流。肺静脉回流无梗阻,心室射血分数>0 6 0。心电图未见明显心律失常。生长发育正常,活动自如,经皮氧饱和度>0 90 ,无慢性渗出、蛋白丢失肠病等并发症,无中期死亡。结论　改良外管道Fontan术适用于治疗无脾或多脾综合征型复杂先心病,术后心律失常发生率低。     

心外管道全腔静脉-肺动脉连接术的疗效

目的总结心外管道全腔静脉-肺动脉连接术(total cavopulmonary connection,TCPC)的经验,探讨手术适应证、手术设计、手术方法以及治疗效果.方法回顾分析29例心外管道TCPC患者的临床资料,平均年龄10岁,病种包括三尖瓣闭锁9例,左室型心室双入口9例,二尖瓣闭锁3例,右心室双出口合并左心室发育不良5例,矫正型大动脉转位解剖右心室发育不良3例.全部在体外循环下手术,12例采用心脏停搏,17例在并行转流心脏不停跳下手术.结果早期22例中死亡5例,后7例无死亡;随访3个月～10年,无晚期死亡.发生低心排血量综合征12例,乳糜胸11例.结论心外管道TCPC用于较大儿童或成人患者与其他Fontan类手术比较,临床长期效果较好.但应严格掌握手术适应证,合理的手术设计和术中细致操作,有危险因素时增用开窗术以及围术期严密观察和处理是提高手术近、远期治疗效果的关键.     

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。     

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。     

心外管道全腔静脉肺动脉吻合术治疗复杂性先天性心脏病

目的 评价心外管道全腔静脉肺动脉吻合术(TCPA)治疗复杂先天性心脏病的临床应用价值。方法 1998年6月～2002年7月，26例先天性心脏病复杂畸形的患者接受了心外管道TCPA，包括单心室伴完全型大动脉转位16例，三尖瓣下移畸形2例，右心室双出口伴大动脉转位3例，三尖瓣闭锁伴右心室发育不良5例。19例在全身麻醉低温体外循环下手术，7例在非体外循环下手术。结果 无手术死亡，全部患者治愈出院。术后随访1～47个月，无晚期死亡。所有患者症状消失，无静脉压明显升高现象，超声心动图检查示心外管道血流通畅，无血栓形成，心电图检查示无严重的心律失常，血氧饱和度0．93～0．96，心功能均达Ⅰ～Ⅱ级。结论 心外管道TCPA是一种较为简单的手术方式，易于掌握；术后疗效满意，优于其他术式。     

Comparison of lateral tunnel and extracardiac conduit Fontan procedure

The purpose of this study was to compare the outcomes of lateral tunnel (LT) and extracardiac conduit (ECC) Fontan procedures at a single institution. From April 1995 to December 2006, 165 Fontan procedures were performed (67 LT, 98 ECC). Pre-, intra- and postoperative variable values were compared between two different techniques. Operative mortality was 5 (3 LT, 2 ECC). Immediate postoperative transpulmonary gradient (LT 8.5+/-ECC 2.5 vs. 6.6+/-2.4 mmHg) and central venous pressure (LT 18.3+/-3.8 vs. ECC 15.6+/-2.4 mmHg) showed significant difference (P<0.001). The LT patients had a higher incidence of sinus node dysfunction in the postoperative period (22.4% vs. ECC 11.2%; P=0.05). Mean follow-up was 74.1+/-31.5 months in LT, and 31.7+/-28.1 months in ECC patients. There was one late death. Actuarial survival at 10 years is 92% for LT, and 89% for ECC patients (P=0.796). The LT and ECC, both, showed comparable early and mid-term outcomes in operative morbidity and mortality, postoperative hemodynamics, survival. Use of ECC for modified Fontan operation reduces the risk of sinus node dysfunction and shows better outcome of immediate postoperative hemodynamics.     

Clinical outcome of patients 20 years after Fontan operation — effect of fenestration on late morbidity

Objective: The Fontan operation has been proposed as definitive palliation for an increasing variety of hearts with complex univentricular anatomy, but late morbidity after Fontan operation is still a matter of concern. This retrospective study evaluates the late outcome in patients with Fontan circulation. Methods: We included 121 consecutive patients that underwent Fontan operation between 1984 and 2004. Modifications of the Fontan operation included atriopulmonary anastomosis (APA; n = 28), total cavopulmonary connection (TCPC; n = 63), and fenestrated TCPC (f-TCPC; n = 30). Mean age was 5.8 ± 5.5 years. Post operative mortality, morbidity, hemodynamics, and somatic development were analyzed. Results: Actuarial survival was 87% at 20 years after Fontan operation. There were 10 early deaths, 5 late deaths, and 2 takedowns followed by successful conversion and heart transplantation. Among 108 early-survivors with Fontan circulation, 19 underwent reoperation, including 3 conversions of APA to TCPC. Freedom from reoperation was 76% at 20 years. Freedom from intervention was 34% at 20 years. Freedom from tachyarrhythmia or pacemaker implantation was 23% and 77%, respectively at 20 years. Heterotaxy and atrioventricular valve anomaly were risk factors for late failure and tachyarrhythmias. Patients with fenestrated TCPC had reduced incidence of late tachyarrhythmias, and patients with APA who developed collaterals showed low incidence of late tachyarrythmia. Postoperative sinus node dysfunction or tachyarrhythmias was associated with significantly lower cardiac index. Somatic development was gradually compensated after Fontan operation. Weight normalized completely 15 years postoperatively. Conclusions: Long-term survival after Fontan procedure is encouraging, but late morbidity remains suboptimal. During follow-up, emerging complications should be managed by surgical and interventional procedures. Fenestration in Fontan circulation provided better cardiac output and lower incidence of late tachyarrhythmias, suggesting a benefit of fenestration for late outcome.     

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。     

134例Fontan手术后早期处理经验

目的 总结Fontan手术后早期处理的经验。方法 134例病人，年龄2-34岁，体重12-66kg。施行右房与肺动脉连接72例，右房与右室连接4例。全腔静脉与肺动脉连接58例，术后及时补充胶体溶液，维持适当的呼吸性碱中毒，应用正性肌力药物及降低肺血管阻力药物，早期应用华法林抗凝。结果 术后合并低心输出量综合征40例（29.9%)。房性心动过速33例（24.6%)，非阵发性交界性心动过速19例（14.2%)。低氧血症26例（19.4%)，脑部并发症20例（14.9%)。早期死亡24例，死亡率17.9%。结论 术前严格掌握手术适应证，术中保证吻合口足够大，必要时行开窗术，术后维持足够前负荷，积极采取有效降低肺循环阻力及降低左心房压力的治疗措施，及时纠治低心输出量综合征，心律失常，低氧血症等并发症，早期应用抗凝治疗，可有效提高手术成功率。     

The modified Fontan operation for double inlet left ventricle : Surgical results, early haemodynamic and functional assessment

Twenty-one patients with a double inlet left ventricle underwent a modified Fontan between May 1979 and August 1987. All but the first patient in this series were operated upon after August 1984. Their age at operation ranged from 4 to 23 years with a median age of 8 years. Initial palliation was required in 12 patients and the mean interval to repair was 77.1 months, ranging from 38 to 112. Only one of the criteria of Choussat was exceeded in 12 patients. The pulmonary vascular resistance was always less than 4 Um². A direct atriopulmonary anastomosis was performed in 19 patients and an aortic homograft interposition was used in 2. There was one hospital death due to acute cardiac failure and no late deaths. The average of the mean right and left atrial pressures measured early postoperatively was 12.9 and 8 mmHg respectively. A prolonged hospitalization with a mean of 23 days, ranging from 9 to 69, was required because of recurrent pleural and pericardial effusions which usually resolved after the first 6 postoperative months. The need for early anticoagulation is recommended to prevent the risk of pulmonary thromboembolism observed in 2 of our patients. Cardiac catheterization performed in 19 patients within 1 year after surgery showed that the average of the mean right atrial pressure had decreased to 11.3 mmHg without a gradient across the anastomosis in any case. Two patients required reoperation: one for enlargement of a restrictive ventricular septal defect and the other for closure of a recurrent dehiscence of the patch used for closure of the right a-v valve. Three patients developed a significant subaortic obstruction and have been scheduled for reoperation. Ventricular function was assessed at a mean interval of 11.9 months (17 patients) and 25.5 months (13 patients) from surgery. Gated equilibrium radionuclide ventriculography showed that the ejection fraction was normal (≥50%) and it had increased with time in 10. Yet, the response to exercise was normal in only 5 of the 13 patients re-evaluated. Treadmill exercise testing showed an increase in exercise tolerance from a mean of 7.4 min to a mean of 8.7 min. At the last follow-up, as of August 1987, 17 out of 20 survivors were in NYHA functional class I and only 2 of them remained on diuretic therapy. Two patients with protein-losing enteropathy were in class II, while the last patient who suffered cerebral haemorrhage was in class IV. We conclude that the Fontan operation can be performed in patients with double inlet left ventricle with overall results similar to those reported in the literature for repair of tricuspid atresia.     

New onset arrhythmias after the extracardiac conduit Fontan operation compared with the intraatrial lateral tunnel procedure: early and midterm results

Background

Arrhythmias are one of the main causes of postoperative morbidity superseding Fontan operations. Comparative data on the incidence of sinus node dysfunction after the extracardiac Fontan operation (ECFO) and the intraatrial lateral tunnel Fontan operation (LTFO) are very limited and controversial. The aim of this study was to evaluate whether ECFO decreases the risk of postoperative arrhythmias compared with LTFO.

Methods

Seventy-four consecutive patients received either an LTFO (n = 29, 5 recordings in 1992 to 9 recordings in 1997) or an ECFO (n = 45, 11 recordings in 1995 to 5 recordings in 2001). The rhythm was documented preoperatively and postoperatively with standard electrocardiogram (ECG) recording and ECG monitoring. During follow-up all patients had 2-8 (median 3) standard ECG recordings per year. Additionally 45 patients (65%) had a Holter ECG at least once a year.

Results

Median follow-up post-ECFO was 4.4 years (1.6-7.2) and post-LTFO it was 7.9 years (5.4-11.1). There were 5 early deaths (3 LTFO, 2 ECFO) and 1 late death (LTFO) (total mortality 8%). Sinus rhythm persisted in 37 ECFO patients (86%) as compared with 13 LTFO patients (50%) (p < 0.001). The incidence of new onset supraventricular tachyarrhythmias (SVTs) post-ECFO compared with LTFO was lower: 5 patients (11%) versus 11 patients (38%) early postoperatively (p < 0.001) and none versus 7 patients (27%) during follow-up (p < 0.001), respectively. Early postoperatively 10 LTFO patients (34%) and another 3 patients during follow-up required permanent pacemaker implantation due to bradyarrhythmias, but none of the ECFO patients required this.

Conclusions

Our data suggest that ECFO decreases the incidence of postoperative new onset arrhythmias during early and midterm follow-up compared with LTFO.     

小儿复杂先天性心脏病侧通道和外管道Fontan术比较

目的 总结侧通道和外管道Fontan术纠治小儿危重复杂型先天性心脏病(先心病)的经验.方法 2000年1月至2006年12月128例复杂型心内畸形病儿行改良Fontan术治疗.男76例,女52例.年龄1.9～16.5岁,平均(6.2±3.8)岁;体重8～62 kg,平均(19.0±5.5)kg.病种主要有单心室(SV)59例;右室双出口(DORY)Z5例等.一期Fontan术87例;二期Fontan术41例.两次手术间隔0.8～7.3年,平均(3.9±2.8)年.首次手术为单侧Glenn术(12例)、双侧双向Glenn术(8例)等.行心内侧通道(LT)的Fontan组(LT组)89例,行外管道(ECC)的Fontan组(ECC组)39例.术前均行二维多普勒超声,11例加做核磁共振,30例行心导管和心血管造影.结果 死亡17例(13.3%).LT组病儿年龄明显低于ECC组.32例发生低心输出量综合征,其中11例.肾功能受损导致无尿而行腹膜透析术(2～5 d后尿量恢复).LT组术后心律紊乱的发生率明显高于ECC组.门诊随访3～24个月,均未出现严重并发症,无死亡病例.结论 LT组术后心律紊乱的发生率明显高于ECC组,ECC的Fontan术具有可避免主动脉阻断和心肌缺血、缩短CPB时间和保持更为流线型的下腔静脉回流血的优点,开窗术的远期优越性未能明确.     

心房内侧隧道Fontan术纠治小儿复杂先天性心脏病47例

目的 总结应用心房内侧隧道Fontan术治疗47例小儿复杂先天性心脏病（先心病）的经验。方法 47例病儿年龄1岁5个月－14岁;体重9－42kg。病种包括三尖闭锁10例,单心室17例,右室双出口14例,完全性大动脉转位2例和纠正性大动脉转位4例。结果 术后早期死亡9例,手术死亡率19.1％。1995年前手术死亡率26.3％（5／19例）,1995年后（含1995年）14.3%(含1995年）14.3%(4/28例）。晚期死亡1例。结论 改良Fontan术适应证已扩大至许多解剖上不能纠治、功能上仅有一个单心室腔的复杂性紫绀型先心病,为提高手术疗效须严格掌握手术适应证;板障上开窗可有效降腔静脉和右房压力,减少胸腔引流量,缩短住院,是高体循环心排出量;少数不能耐受Fontan术后循环生理改变 的难治性低心输出量病儿,应早期果断再手术改建为双向腔肺分流术（半Fontan术）