肺动脉高压靶向药物应用于儿童Fontan术围术期1例报道并文献复习

正Fontan术是目前功能性单心室患者治疗中的首选手术方案。术前肺动脉压力和肺血管阻力对于手术的成功与否至关重要。Hosein等~([1])的一项研究表明,术前肺动脉压力过高(肺动脉平均压 15 mmHg;1 mmHg=0.133 kPa)对Fontan术早期及远期预后均存在不良影响。此外,术前较低的肺动脉阻力和良好的心功能储备被认为是Fontan术后患者循环维持稳定的最重要的前提~([2])。由于一氧化氮通路失调及血管内皮功能障碍,Fontan     

一期Fontan手术治疗复杂紫绀型先天性心脏病21例

目的：总结一期房坦（ Fontan）手术治疗复杂紫绀型先天性心脏病的临床经验和疗效。方法2007-06～2012-12该院心血管外科对21例复杂紫绀型先天性心脏病患者施行了一期Fontan单心室手术,同期行房室瓣整形术8例。12例采取心内管道或自体心包内隧道连接,8例采取心外管道连接,1例采取不用管道材料肺动脉直接下拉吻合法。5例保留房间隔缺损或行心房板障开窗。结果20例手术获得成功,1例术后当天因低心排出量综合征死亡。1例房室瓣成形术后随访至25个月出现房室瓣重度反流,行机械瓣置换。术前经皮血氧饱和度为67％～91％,术后经皮血氧饱和度为88％～95％。术后随访（7～52个月）所有患者心功能Ⅰ～Ⅱ级。结论一期Fontan手术治疗复杂紫绀型先天性心脏病安全有效,可以避免多次手术创伤,节约医疗资源,但应注意把握手术适应证。     

Fontan循环

Fontan手术为全腔静脉一肺动脉连接术,是Glenn术后的二期手术方式.常用方法为使用心内隧道或心外管道将腔静脉血流完全导入肺动脉,使功能性单心室患儿获得生存机会.目前Fontan手术的手术指征较"经典"十大标准大为扩大,对于尚难直接Fontan手术时推荐分期手术,手术方式的改进提高了Fontan技术.手术的一些并发症影响了术后的转归,同时,对Fontan手术尚存一些争议.     

2岁以上合并重度肺动脉高压先天性心脏病行单心室修复的初步研究

目的：探讨2岁以上合并重度肺动脉高压先天性心脏病（先心病）行单心室修复的手术指征及效果。方法：回顾性分析年龄〉2岁合并重度肺动脉高压仅能行单心室修复的先心病患儿15例,男性9例,女性6例。年龄2.0～10岁,平均（7.8±3.8）岁。体质量11～24kg,平均（15.32±4.24）kg,术前经皮血氧饱和度（SPO2）88%～95%,平均（91.75±2.29）%。肺动脉压（mPAP）52～91mmHg（（1 mm-Hg=0.133 kPa））,平均（54.61±16.8）mmHg。心脏畸形：单心室7例,三尖瓣闭锁3例,室缺远离两大动脉伴左心室发育不良型右心室双出口2例,心室不均衡型完全型房室通道2例,十字交叉心1例。均在静吸复合麻醉下先期行肺动脉环缩术,后期逐步完成双向格林或全腔肺动脉吻合术。结果：全组无住院死亡;PAB术前后mPAP、SPO2明显下降;PAB术后3～6年内完成双向格林（Glenn）4例,全腔肺动脉吻合术（TCPC）2例。结论：PAB术能有效降低肺动脉压力,≥2岁PAB术后可进一步完成Glenn或TCPC术。     

留孔Fontan手术后延迟经导管关闭房间隔缺损——增进高危病例疗效

高危病例施行Fontan术后并发症较多和死亡率较高,原因多为低心排伴高中心静脉压。作者施行Fontan术时在体静脉与肺静脉回流腔间留置房间隔缺损或开窗,取得满意结果。方法 1988年9月～1991年4月间对10例有两项以上危险因素者作此手术。年龄9个月～33岁。其中三尖瓣闭锁与功能性单心室各5例。危险因素:肺血管阻力>3wood单位,左室舒张末压>1.33kPa(10mmHg),左室射血分数<40％,肺动脉发育不良或严重变形,肺动脉压>2.39kPa(18mmHg),中度房室瓣返流和年龄太小(18个月)或太大(33岁)。手术方法为常规体外循环     

肺动脉血栓内膜剥脱术的远期疗效

目的　观察肺动脉血栓内膜剥脱术 (PTE)对慢性栓塞性肺动脉高压 (CTEPH )患者的远期疗效。方法　 1997年 3月～ 2 0 0 2年 6月 ,共对 15例CTEPH患者行PTE ,14例存活出院。其中 13例随访时间平均 4 0 (17～ 77)个月 ,1例失访 ;随访项目包括心功能状态、血气分析和通过超声心动图测量肺动脉收缩压。结果　随访期内 13例患者均存活 ,其术前心功能NYHA分级均为Ⅲ、Ⅳ级 ;术后 12例患者心功能NYHA分级Ⅰ级、1例Ⅲ级 ;动脉血氧分压 (PaO2 )由术前的 (5 8 1± 8 6 )mmHg上升至(90 8± 6 0 )mmHg (P <0 0 1) ;动脉血氧饱和度由术前的 (90± 5 ) %上升至 (96± 1) % (P <0 0 5 ) ;肺动脉收缩压由术前的 (97 1± 2 2 4 )mmHg下降至 (4 2 6± 10 7)mmHg (P <0 0 1)。结论　肺动脉血栓内膜剥脱术对慢性栓塞性肺动脉高压患者具有很好的远期疗效 ,它能显著改善患者的心肺功能 ,提高其生活质量     

双心室起搏再同步治疗10例慢性充血性心力衰竭患者近期疗效

目的:观察双心室起搏再同步化治疗（CRT）慢性充血性心力衰竭（CHF）患者的临床疗效。方法: 选取经药物治疗但逐步加重的CHF患者10（男6,女4 ）例,年龄（62±10）岁,心功能（NYHA）Ⅲ～Ⅳ级,全部置入双心室三腔起搏器（9例InSync Ⅲ 8042型,1例FRONTIER Ⅱ 5596型）,左室电极置于冠状静脉侧支或后侧支分支内。观察CRT前后左心室功能及体表心电图的变化。结果: 10例患者均顺利完成起搏器置入。起搏方式均为心房感知心室起搏,其中总心室起搏数>90%。所有患者的临床指标明显改善,心功能(NYHA)分级、6分钟步行试验、左室射血分数、左室舒末内径、生活质量评分等较术前显著提高(P<0.05,P<0.01)。结论: CRT治疗CHF患者可改善患者心功能,提高生活质量。     

30例肺动脉血栓内膜剥脱术临床结果

目的:报告肺动脉血栓内膜剥脱术(PTE)治疗慢性栓塞性肺动脉高压的结果。方法:回顾性总结连续30例肺动脉血栓内膜剥脱术的手术要点、围术期处理以及近中期结果。结果:共行PTE术30例,男24例,女6例,平均年龄45．7岁,平均病史48．0个月。术前心功能为纽约心功能协会分级标准(NYHA)Ⅳ级者12例,Ⅲ级者18例。手术均在深低温间断停循环下完成肺动脉血栓及机化内膜剥脱术。围术期死亡1例(死亡率为3．3％)。死亡原因为感染性休克。术后出现神经系统并发症4例(13．3％),再灌注肺水肿7例(23．3％),肺内出血1例(3．3％)。平均体外循环时间191．1 min,平均主动脉阻断时间95．1 min,平均停体外循环时间47．7 min。术后平均拔除气管插管时间85．1 h。肺动脉收缩压由术前的91．4 mmHg(1 mmHg=0．133 kPa)降至术后的48．3 mmHg、动脉血氧分压由56．2 mmHg升至88．9 mmHg、动脉血氧饱和度由0．90升至0．97(P均<0．01-0．05),均有显著改善。随访26例,平均随访时间36．8个月,心功能NYHA分级Ⅰ级22例,Ⅱ级3例,Ⅲ级1例,生活质量明显改善。结论:PTE术是治疗慢性栓塞性肺动脉高压的有效手段,其关键在于选择合适病例,彻底剥离肺动脉血栓和机化内膜,正确预防和治疗各种并发症。     

Fontan循环

Fontan手术为全腔静脉一肺动脉连接术,是Glenn术后的二期手术方式。常用方法为使用心内隧道或心外管道将腔静脉血流完全导入肺动脉,使功能性单心室患儿获得生存机会。目前Fontan手术的手术指征较“经典”十大标准大为扩大,对于尚难直接Fontan手术时推荐分期手术,手术方式的改进提高了Fontan技术。手术的一些并发症影响了术后的转归,同时,对Fontan手术尚存一些争议。     

肺动脉高压患者压力-容积关系分析与纽约心脏病协会心功能分级的相关性

目的探讨肺动脉高压患者的压力-容积相关参数与不同纽约心脏病协会(NYHA)心功能分级间的相关性。方法纳入2015年4月至2016年6月于武汉亚洲心脏病医院就诊的肺动脉高压患者36例,通过股静脉入径右心导管记录右心房压力曲线、右心室压力曲线、肺动脉压力曲线及不同部位血氧饱和度,同期行心脏磁共振成像(MRI)检查获得单个心动周期下的右心室容积,并使用Mass软件测量连续的右心室容积,再进一步获得右心室压力-容积环参数的计算结果。根据NYHA心功能分级进行分组,即NYHA心功能Ⅰ级(Ⅰ组,5例),NYHA心功能Ⅱ级(Ⅱ组,23例),NYHA心功能Ⅲ级(Ⅲ组,8例)。分析各组患者各参数与NYHA心功能分级之间的相关性及组间差异。结果Ⅱ组患者右心室舒张末期压力(RVEDP)[(9.61±4.31)mmHg比(7.40±3.36)mmHg,P=0.03]和右心室收缩末期压力(RVESP)[(91.37±29.62)mmHg比(45.38±28.01)mmHg,P=0.006]、Ⅲ组患者RVEDP[(13.75±7.44)mmHg比(7.40±3.36)mmHg,P=0.03]和RVESP[(93.45±30.56)mmHg比(45.38±28.01)mmHg,P=0.006]均显著高于Ⅰ组,差异均有统计学意义。Spearman相关性分析显示,NYHA心功能Ⅰ级、Ⅱ级、Ⅲ级患者间右心室收缩末期容积(RVESV)(R=0.375,P=0.02)、RVEDP(R=0.377,P=0.03)和RVESP(R=0.378,P=0.02)呈正相关,而右心室舒张末期容积(RVEDV)(R=–0358,P=0.02)呈负相关,差异均具有统计学意义。将患者分为NYHA心功能Ⅰ级比Ⅱ级+Ⅲ级进行分析显示,RVESP(R=0.493,P=0.008)和右心室动脉有效弹性(RVEa)(R=0.345,P=0.02)呈正相关;分为Ⅰ级+Ⅱ级比Ⅲ级进行分析时,RVEDV(R=0.383,P=0.02)、RVESV(R=0.398,P=0.02)和RVEDP(R=0.360,P=0.03)呈正相关,差异均有统计学意义。ROC曲线分析发现,当患者分为NYHAⅠ级和Ⅱ+Ⅲ级两组、预测结局为NYHAⅡ/Ⅲ级时,RVEa灵敏度为0.871,特异度为0.800时,ROC曲线下面积为0.836,P=0.02;RVESP灵敏度为0.903,特异度为0.800时,ROC曲线下面积为0.892,P=0.006,。结论右心室压力-容积参数可能是判断肺动脉高压患者临床心功能状态的更为客观的指标,可作为量化心功能分级的参考选择,对于慢性进展性但又症状偏轻患者的心功能评价具有潜在价值,值得临床医师更多的关注和研究。     

Clinical outcome of 193 extracardiac Fontan patients: the first 15 years.

OBJECTIVES: We sought to evaluate the mid-term outcome of hospital survivors with extracardiac Fontan circulation. BACKGROUND: Few data exist about the mid-term and long-term results of the extracardiac Fontan operation. METHODS: From November 1988 to November 2003, 221 patients underwent an extracardiac Fontan procedure as primary (9 patients) or secondary (212 patients) palliation, at a mean age of 72.2 months (range 13.1 to 131.3 months). A total of 165 of 193 early survivors underwent programmed noninvasive follow-up evaluations and at least one cardiac catheterization. RESULTS: The overall survival, including operative deaths, was 85% at 15 years. Freedom from late failure among hospital survivors is 92% at 15 years. A total of 127 of 165 survivors (77%) were in New York Heart Association functional class I. The incidence of late major problems was 24% (42 major problems in 36 of 165 patients): 19 patients had arrhythmias (11%), 5 patients had obstruction of the extracardiac conduit (3%) and 6 of the left pulmonary artery (3.5%), and 5 patients experienced ventricular failure (3%), leading to heart transplantation in 3 patients. Protein-losing enteropathy was found in two patients (1%). The incidence of late re-interventions was 12.7% (21 of 165 patients, including 15 epicardial pacemaker implantations). Four patients died (2.3%), two after heart transplantation. CONCLUSIONS: After 15 years of follow-up, the overall survival, the functional status, and the cardiopulmonary performance of survivors of the extracardiac Fontan procedure compare favorably with other series of patients who underwent the lateral tunnel approach. The incidence of late deaths, obstructions of the cavopulmonary pathway, re-interventions, and arrhythmias is lower than that reported late after other Fontan-type operations.     

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.     

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.     

Is it necessary to routinely fenestrate an extracardiac fontan?

OBJECTIVES: This study was conducted to assess the need for, and use of, fenestration of an extracardiac conduit Fontan. BACKGROUND: Fenestration of a Fontan connection has been proposed as a means of improving outcomes of single ventricle palliation. The benefit of fenestration is likely to be greatest in the early postoperative period when patients may experience increased pulmonary vascular resistance and decreased ventricular function due to the effects of cardiopulmonary bypass, aortic cross-clamping and positive pressure ventilation. However, there are potential drawbacks to fenestration. The utility of fenestration with extracardiac Fontan operation has not been determined. METHODS: Since 1992, 81 patients have undergone a modification of the Fontan procedure in which an extracardiac inferior cavopulmonary conduit is used in combination with a previously staged bidirectional Glenn anastomosis. We conducted a retrospective review of these patients. RESULTS: Fenestration was performed selectively in 32 patients (39%), including only 2 of the last 38 (5%). In seven patients, a fenestration was placed or clipped in the early postoperative period without cardiopulmonary bypass. There were two operative deaths. Prolonged (>2 weeks) pleural drainage occurred in 13 patients, 8 with fenestration and 5 without. In addition to undergoing earlier Fontan in our experience, patients who had a fenestration placed had significantly higher preoperative pulmonary vascular resistance, significantly higher common atrial pressure after Fontan and significantly lower post-Fontan systemic arterial oxygen saturation. Fontan pressure did not differ between nonfenestrated and fenestrated patients. At follow-up ranging to five years, there were two late deaths and no patients developed protein losing enteropathy. CONCLUSIONS: Fenestration is not necessary in most Fontan patients when an extracardiac conduit technique is performed as described in this article, and therefore, should not be performed routinely with the extracardiac conduit Fontan. The need for fenestration should be assessed after cardiopulmonary bypass when hemodynamics can be evaluated accurately. Fenestration can be placed and revised easily without bypass and with minimal intervention in patients with an extracardiac conduit Fontan.     

Hypoplastic left heart syndrome: hemodynamic and angiographic assessment after initial reconstructive surgery and relevance to modified Fontan procedure

After undergoing initial reconstructive surgery for hypoplastic left heart syndrome performed between August 1985 and March 1989, 59 patients (age range 3 to 27 months, mean 13.8 +/- 4.5) underwent elective cardiac catheterization in anticipation of a modified Fontan procedure. Five important hemodynamic and anatomic features considered to be components of successful reconstructive surgery were specifically addressed. 1) Interatrial communication: Only two patients had a measured pressure difference of greater than 4 mm Hg across the atrial septum. 2) Tricuspid valve function: Angiography demonstrated significant tricuspid valve regurgitation in only five patients (moderate in two and severe in three). 3) Aortic arch: Pressure tracings from the right ventricle to the descending aorta revealed a gradient greater than 25 mm Hg in only two patients. 4) Pulmonary vasculature: Ten patients had a calculated pulmonary vascular resistance greater than 4 U.m2; 51 (86%) of the 59 patients had no evidence of distortion (stenosis or hypoplasia) of either the left or the right pulmonary artery. 5) Right ventricular function: Five patients had an end-diastolic pressure in the right ventricle greater than 12 mm Hg and two patients had qualitative assessment of decreased ventricular function. Comparison of catheterization data between survivors and nonsurvivors of the subsequent modified Fontan procedure showed that only significant tricuspid regurgitation is a possible predictor of poor outcome. After first stage reconstructive surgery for hypoplastic left heart syndrome, most survivors have favorable anatomy and hemodynamics at follow-up cardiac catheterization for a subsequent Fontan procedure.     

Ventricular function in the single ventricle before and after Fontan surgery

To better delineate the importance of ventricular function in patients with a single ventricle and assess its relation to outcome after the Fontan procedure, 47 patients with a single ventricle were studied. Ventricular ejection fraction was estimated by radionuclide angiocardiography. Before Fontan surgery, ejection fraction was 0.57 ± 0.10 (mean ± standard deviation). This differed significantly from the normal mean left ventricular ejection fraction of 0.68 ± 0.09 (p < 0.001) derived in our laboratory by radionuclide angiocardiographic methods. Age, ventricular morphology and the presence of pulmonary artery band or systemic to pulmonary artery shunts had no statistical relation to ventricular ejection fraction in patients with a single ventricle. Serial preoperative evaluation in 15 patients over 3.8 ±1.3 years revealed no significant change in ventricular ejection fraction; however, increased atrioventricular valve regurgitation was documented in 4 of these 15. Modified Fontan procedure was performed in 24 of the 47 study patients; 7 have died, 1 has undergone cardiac transplantation and 1 faces possible transplantation. No difference was noted in preoperative ejection fraction between survivors and nonsurvivors. Ventricular morphology, age at Fontan surgery and operative factors such as bypass and cross-clamp time were not related to functional outcome. Preoperative ejection fraction of 0.52 ± 0.08 decreased to 0.39 ± 0.11 (p < 0.001) when evaluated 1.16 ± 0.44 years after Fontan surgery. In patients with a single ventricle (1) ventricular ejection fraction is less than that of the normal systemic ventricle; (2) during childhood, ejection fraction is not related to age or ventricular morphology; and (3) ventricular ejection fraction frequently decreases after a Fontan repair. Thus, long-term studies of clinical course and ventricular function are essential before altering recommendations for pursuing the Fontan repair.     

Current management of late failure after classic Fontan modifications: Fontan conversion

BACKGROUND: Re-establishment of Fontan circulation by eliminating the drawbacks of classic Fontan modifications has been proposed recently to improve the functional class and quality of life of patients with failed Fontan circulation. METHODS: Five patients underwent extracardiac and lateral tunnel Fontan conversion due to failure of the Fontan circulation, after classic Fontan modifications. All of the patients were female and mean age was 10.2 +/- 2.2 years (range: 8-14 years). Previous Fontan modifications were atrio-pulmonary anastomosis in 3 and Kawashima operation in 2 patients. Time to re-operation for Fontan conversion was 6 +/- 1.5 years (range: 4-8 years). Indications for re-operation were right heart failure, right atrial dilatation, and intractable atrial arrhythmias in patients with previous atrio-pulmonary anastomosis, progressive hypoxia and exercise intolerance in patients with previous Kawashima operation due to pulmonary arteriovenous fistulas. Previous atrio-pulmonary anastomoses were converted to intra-atrial or lateral tunnel Fontan operation with modified right atrial Maze procedure. Extracardiac Fontan completion was carried out after previous Kawashima operations by redirection of hepatic veins to the lungs. RESULTS: There was no mortality and no major morbidity. All patients were discharged from the hospital in good condition and in sinus rhythm. No prolonged or recurrent effusions were observed. On follow-up, all patients were in sinus rhythm and had NYHA class I functional capacity. In two Kawashima patients, SpO(2) gradually increased from 60 % to 90 % six months after the operation. DISCUSSION: We suggest that Fontan conversion should be considered in patients with previous atrio-pulmonary anastomosis, when right atrial dilatation or intractable atrial arrhythmias with deteriorating functional status develops. Redirection of hepatic venous flow to lungs induces regression of pulmonary arteriovenous fistulas and improves arterial saturation in patients with previous Kawashima operation.     

Early results of extracardiac fontan procedure with autologous pericardial tube conduit

BACKGROUND: Different types of conduits are used for extracardiac Fontan procedure. The use of autologous pericardial tube as an alternative conduit for connecting the inferior vena cava to the pulmonary artery is investigated. METHODS: We performed 25 extracardiac Fontan procedures by using pericardial tube between June 2000 and October 2003. Fifteen patients were male. Mean age and weight were 7.6 +/- 4.9 years (range 3 to 24 years) and 22.8 +/- 10.4 kg (range 12 - 50 kg), respectively. RESULTS: All patients survived after extracardiac Fontan procedure. Prolonged chest tube drainage (> 7 days) was required in 9 (36 %) patients. Follow-up was complete and mean follow-up was 24.6 +/- 11.5 months (1 - 39 months). One patient died 3 months postoperatively. Routine serial postoperative echocardiographic examinations in all patients and magnetic resonance angiography in 6 patients with suspect flow patterns in echocardiography did not reveal any problems with the Fontan circuit. CONCLUSIONS: Fresh autologous pericardial tube conduit is a suitable and safe alternative for extracardiac Fontan procedures. Major advantages are availability, no cost, easy handling and hemostasis, low risk of thrombosis and emboli, and growth potential.     

Fontan's operation: results, immediate and long-term postoperative complications

The Fontan operation and its modifications can be relatively safely performed in the treatment of many complex congenital heart diseases, such as univentricular heart and tricuspid atresia. The main postoperative complications following the Fontan procedure and the incremental risk factors involved in their development are investigated. Between January 1984 and January 1988 eleven patients (6 females and 5 males), ranging in age from 2 to 15 years (mean age: 7.3 +/- 3.7) and in weight from 10.8 to 50 Kg (mean weight 22.3 +/- 12.7) underwent the Fontan operation in our Department. No hospital death occurred. The mean postoperative stay in the Intensive Care Unit was 6.3 +/- 3.9 days. There were two surgical re-entries for the same patient: cardiac tamponade (the day of operation) and residual atrial septal defect (2 days following the operation). Eight patients had significant signs of venous stasis, with severe hepatomegaly and pleural effusion. The 11 patients discharged were followed-up for a period of between 3 and 48 months (mean follow-up: 26.4 +/- 18). There were 2 cases of recurrent pleural effusion, 10 to 15 days after discharge. One late death occurred 2 years after the Fontan procedure (massive pulmonary embolism after re-operation). Short and medium-term rate of complications is related to increased post-operative values of mean right atrial pressure (greater than 15 mmHg). Mean right atrial pressures greater than 15 mmHg appear to be correlated with Nakata index values less than 250 mm2/m2. The experience reviewed confirms the excellent results of the Fontan operation, as regards survival and functional recovery.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.