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.     

The first successful implantation of an intravenous AAIR pacemaker into autologous extracardiac lateral tunnel Fontan in the child

Patients with a single ventricle have complex anatomy that requires staged palliation which is usually the Fontan procedure. This procedure has undergone a lot of modifications to improve hemodynamics. Despite these efforts, sinus node dysfunction (SND) and bradyarrythmias are still common complications after Fontan operation, therefore there is a need of pacemakers implantation. Unfortunately, the most frequent technique of creating Fontan cannale – the extracardiac lateral tunnel makes the transvenous access to the atrium difficult or impossible to achieve. We report a case of successful implantation of an endocardial atrial lead for SND in patient with an extracardiac autologous Fontan tunnel.     

Use of covered stents to occlude extracardiac Fontan fenestration

INTRODUCTION: A fenestration is usually surgically created to improve the post-operative course of patients undergoing total cavopulmonary connection. It, however, has a potentially deleterious effect on the long-term period. Closure of these fenestrations is usually performed by interventional catheterization. No device has been specially designed and closure of extracardiac fenestration, in particular, can be challenging. We report our experience in occlusion of such fenestrations using covered stents (Numed Inc). METHODS: From July 2005 to October 2005, we attempted to occlude extracardiac Fontan fenestration using CP covered stents in 4 consecutive patients. RESULTS: All patients had a successful occlusion of the fenestration. The procedure was performed from femoral or jugular vein respectively in three and one patient. Mean central venous pressure did not increase significantly (from 11.25 to 12.75 mmHg) whereas mean oxygen saturation increased significantly from 92% to 99% (p=0.0047). Abolition of shunt was obtained immediately after insertion of the covered stent in all patients. No early complications were observed. All patients were ambulatory the day after the procedure. CONCLUSION: The insertion of a covered stent inside the extracardiac Fontan conduit allowed the exclusion of the fenestration in all patients without the need of crossing the fenestration and with no early mortality or morbidity. Long-term follow-up are needed before considering the use of such device as the device of choice in that application.     

Arrhythmias after Fontan operation: comparison of lateral tunnel and extracardiac conduit

Background

Arrhythmias are frequent causes of morbidity and mortality in patients with single ventricle physiology after Fontan operation. The aim of this study was to evaluate which type of Fontan procedure—lateral tunnel (LT) or extracardiac conduit (EC)—provides superior outcomes related to the problem of early postoperative and 1-year follow-up arrhythmias.

Methods

We retrospectively analyzed the incidence, types, and duration of rhythm disorders in 101 consecutive patients who received either LT (n = 60) or EC (n = 41) between April 1997 and March 2006 in Slovak Children's Cardiac Center, Bratislava (Slovakia). Weight, age, sex, and the type of heart morphology did not differ significantly between the 2 groups. The rhythm was monitored and documented perioperatively and postoperatively with standard electrocardiogram (ECG) recording and continual ECG monitoring. Duration of extracorporeal circulation, duration of aortic crossclamp and hemodynamic variables were analyzed with respect to the development of early arrhythmias in both groups. Twenty-four-hour ECG Holter monitoring (DMS 300-7, Holterreader, Producer DMS, Nevada, USA) was used to detect arrhythmias at the 1-year follow-up.

Results

Early postoperative rhythm abnormalities were identified in 31 patients (52%) who underwent LT and in 22 patients (54%) who underwent EC. The most frequent type of rhythm disturbance was junctional rhythm in both groups. The bivariate analysis revealed that there was no significant difference in the incidence, type, or duration of early onset arrhythmias between the 2 groups. Although, there was no significant difference in the duration of arrhythmia since the admission form the operating room. The need of aortic crossclamp was significantly lower in EC group (P < .001). However, this did not correlate with lower incidence of early onset arrhythmias with EC modification. At the 1-year follow-up, the prevalence of arrhythmias was similar in both groups.

Conclusions

Extracardiac conduit as compared with LT does not provide superior outcomes related to the problem of early and 1-year onset arrhythmias. Other factors than the risk of early postoperative and early follow-up arrhythmias should be considered in surgical preference of modification strategy.     

Transcatheter fenestration of autologous pericardial extracardiac Fontan via the transhepatic approach

We describe a case of a patient with hypoplastic left heart syndrome (HLHS) who developed protein-losing enteropathy (PLE) following autologous pericardial extracardiac Fontan (APEF) operation with successful resolution of PLE following transcatheter fenestration via the transhepatic approach.     

Catheter closure of tube graft fenestration in extracardiac Fontan operation

As the extracardiac Fontan operation evolves, a reliable method for creating and subsequently closing communications between the systemic and pulmonary venous chambers would become useful. We describe a simple and safe technique of fenestration amenable to coil occlusion, which can be carried out in most cardiac catheterization laboratories.     

Single-stage Fontan procedure: early and late outcome in 124 patients

INTRODUCTION AND OBJECTIVES: The Fontan procedure was designed to palliate complex congenital heart disease with univentricular physiology. A retrospective study was made to document the determinants of early (/= 31 days) mortality with the modified Fontan procedure performed in one-stage over a 22-year period. MATERIAL AND METHODS: Between 1978 and 2000, 102 atriopulmonary, 16 cavopulmonary, and 6 Kawashima type anastomoses were performed to palliate complex congenital heart defects in 124 patients with a mean age of 7.3 4.7 years. Forty-five patient and procedure-related variables were analyzed in relation to mortality. All events were verified. RESULTS: There were 29 early (23%) and 20 late (16%) deaths. Estimated survival at 30 days, 2 years, 5 years, and 20 years was 78, 75, 66, and 50%, respectively. Subaortic stenosis, protein-losing enteropathy, and arrythmia were observed in 8, 5 and 33 patients, respectively, after surgery. Univariate and multivariable analysis indicated that left ventricular end-diastolic pressure (>/= 13 mmHg), mean pulmonary pressure (>/= 19 mmHg), mitral stenosis/atresia, atrioventricular valve regurgitation, visceral heterotaxia, absence of fenestration, risk factors criteria, duration of extracorporeal circulation, and operative technique were associated with early mortality. Reoperation, arrhythmia, and pacemaker implantation were predictors of late death. Forty percent remained free from surgical or catheter reintervention after Fontan operation at 20 years. CONCLUSIONS: The outcome of Fontan procedure is profoundly affected by patient-related variables (ventricular function and pulmonary circulation). Postoperative arrhythmia and reoperation shortened the lifespan of the Fontan circulation model in patients with atriopulmonary connections. Total cavopulmonary anastomosis improves the physiology of univentricular circulation. In the light of our findings, the modified Fontan procedure (one or two stages) should be performed early in life to better preserve ventricular and pulmonary vascular function.     

Use of a bifurcated ePTFE graft for off-pump extracardiac Fontan completion

A 6-year-old girl underwent off-pump extracardiac Fontan completion. Intrapericardial inferior caval vein was found to be accompanied by the hepatic vein on the left side; a 20 x 10 x 10-mm bifurcated ePTFE graft was used without external shunting. This approach is practical for the surgeons and may result in lower perioperative Fontan pressures/transpulmonary gradients as well as a shorter intubation period and hospital stay.     

Closure of extracardiac Fontan fenestration by using the covered Cheatham Platinum stent.

OBJECTIVE: The purpose of this study was to describe our experience concerning the use of covered Cheatham Platinum (CP) stent in patients with fenestrated total cavopulmonary connection (TCPC). BACKGROUND: Closure of TCPC fenestrations has been achieved by utilizing different devices designed to close intra-cardiac or aorto-pulmonary communications. METHODS: We used the covered CP stent in 6 patients with fenestrated TCPC. Median age and weight were 11 years and 38 Kg, respectively. Femoral approach was used in all but 1 patient having bilateral thrombosis of femoral veins. The CP stent was crimped on a BiB balloon in 5 patients and on a simple balloon in 1 patient. The balloon's diameter was the same size or 1-2 mm larger than the TCPT conduit, according to angiographic diameter. RESULTS: Mean procedural and fluoroscopy time were 41 +/- 8 and 8 +/- 2 min, respectively. Immediate full occlusion of fenestration was obtained in all patients. Mean central venous pressure was not significantly increased from 10.8 +/- 2.5 to 11.8 +/- 2.8 mm Hg and oxygen saturation significantly increased from (91.5 +/- 4.4)% to (98.5 +/- 1.9)% (P = 0.003). No procedural or intra-hospital complications occurred. In particular, no arrhythmias, systemic embolism, or acute venous thrombosis were observed. At a median follow-up of 2.8 months all patients have normal oxygen saturation and are symptom-free. CONCLUSIONS: The covered CP stent can be easily and effectively used for closure of TCPC fenestrations. This method has the advantage to avoid protrusion of prosthetic material into the left atrium and to prevent early or late embolism.     

经导管封堵全腔静脉肺动脉连接术外管道开窗的初步临床应用

目的 探讨经导管封堵全腔静脉肺动脉连接术外管道开窗的方法及疗效.方法 2007年3至7月共行3例外管道开窗封堵术,男2例,女1例,年龄分别为7、14及8岁,术前诊断均为发绀,属复杂先天性心脏病,不能行双心室矫治,而行全腔静脉肺动脉连接术,术中均加行外管道开窗.术后患者持续存在口唇发绀,血氧饱和度降低.3例患者行心导管及造影检查后证实上、下腔静脉与肺动脉吻合口通畅,无狭窄改变,肺动脉平均压均小于15 mm Hg(1 mm Hg=0.133 kPa),分别采用AGA公司Amplatzer动脉导管未闭封堵器及房间隔缺损封堵器对外管道与右房之间的交通(开窗)进行封堵.结果 所有患者均封堵成功,封堵后即刻、15 min、30 min及1 h分别测肺动脉及腔静脉压,与封堵前比较无明显升高,且患者无明确不适症状,血氧饱和度较封堵术前均有提高,发绀减轻,超声心动图及造影证实封堵器形态及位置良好.无术中及术后并发症,术后随访3至6个月,经超声心动图证实封堵器形态及位置良好,无残余分流.结论 经导管外管道开窗封堵术技术成功率高、疗效确切,但远期疗效尚需密切随访.     

193例老年肾移植的临床分析

目的：分析高龄患者术前合并疾病、术后并发症特点,观察对肾移植效果的影响。方法：对我院1990年1月至2004年12月间的193例60岁以上肾移植受者的临床资料进行回顾性分析,总结术前合并疾病、术后并发症的情况及对肾移植预后的影响。结果：术前合并疾病以消化系统（30例,15.5%）和心血管系统（25例,13.0%）为主。术后发生急性排斥33例（17.1%）、感染56例（29.0%）。随访4～95个月,患者1年、3年存活率分别为88%、84%,移植肾1年、3年存活率分别为86%、80%。主要死亡原因为肺部感染和心衰。结论：高龄终末期肾病患者术前合并疾病较多,以消化系统和心血管疾病为主。预防心血管疾病和重症感染,以减少带肾死亡,有助于提高肾移植效果。     

Closure of the fenestration in the extracardiac Fontan with the Amplatzer duct occluder device.

We report the successful use of the Amplatzer duct occluder for the delayed closure of the fenestration in three patients who underwent an extracardiac modified Fontan procedure. At the moment of closure, the patients were 5.5, 2.7, and 3 years old (29 months, 3 months, and 14 months after the Fontan procedure, respectively). Immediate full occlusion was achieved in all cases. In addition, arterial saturation increased significantly (> 5%) with no hemodynamic deterioration. There were no complications during or after the procedure, and the patients were discharged in good conditions the day after and with uneventful follow-up. In conclusion, the Amplatzer duct is safe and effective for the closure of the fenestration in the extracardiac Fontan. Cathet Cardiovasc Intervent 2001;54:88-92.     

Clinical spectrum of infective endocarditis: 15 years experience

Four hundred and sixty-six patients (277 males, 189 females; mean age 23.2 years) diagnosed as cases of infective endocarditis during the past 15 years were retrospectively analysed. Two-thirds of patients belonged to the 15 to 35 years age group. The most common predisposing cardiac lesion was rheumatic heart disease seen in 73.4 percent patients. Mitral valve prolapse and right-sided endocarditis were infrequent, seen in four patients each. Blood culture positivity was 28.7 percent in adults and 61 percent in children. Commonest organism isolated was staphylococcus aureus in adults (39.3%) and streptococcus viridans in children (48%). Salmonella typhi was detected in 17 patients and showed excellent response to ciprofloxacin and gentamycin. Overall mortality was 13.9 percent and resistant heart failure was the leading cause of death. Our study presents the clinical spectrum of infective endocarditis and highlights the comparison with western studies.