Comparison of somatic development and status of conduit after extracardiac Fontan operation in young and older children

Objective: We set out to examine whether the extracardiac Fontan operation (ECFO) in young children is beneficial for the early postoperative course and whether it has a negative impact on the mid-term hemodynamics and growth of the children due to absent growth potential of the prosthetic conduit. Therefore we compared our medium-term experience with ECFO in children under 4 years of age to that in older children regarding the incidence of postoperative complications, somatomotoric development and conduit status. Methods and results: Between 11/95 and 12/02 ECFO was performed in 30 children under 4 years of age and 21 older children aged 4–13 years. There were no deaths in children under 4 years of age and two older children died. No prolonged support (>72 h) of suprarenin was required in small children compared to 4 older children. In twenty-seven children, who underwent postoperative heart catheterization no pulmonary artery or systemic vein distortion occurred. One re-operation and one transcatheter intervention were performed because of the partial conduit stenosis. During the median follow-up of 4.8 years a manifestly accelerated postoperative weight gain was observed in children operated on under 4 years of age, compared to that in older children (up to the 50 vs. 10th percentile, P<0.05). Conclusions: The ECFO could be performed in young children with low morbidity and mortality rates. In the majority of patients, implanted prosthetic grafts maintain stable form without the development of stenosis. Accelerated somatic development, especially in small children, is to be observed after completion of the Fontan circulation.     

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。     

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.     

Thrombosis of intracardiac or extracardiac conduits after modified Fontan operation in patients with azygous continuation of the inferior vena cava.

BACKGROUND: The Fontan procedure in patients with azygous continuation of the inferior vena cava, requires a cavo-pulmonary anastomosis, and deviation of the hepatic venous drainage to the pulmonary arteries using an intra- or extracardiac conduit. METHODS: We report thrombosis of two pericardial conduits and one Gore-Tex (W. L. Gore & Assoc, Flagstaff, AZ) graft used for deviation of hepatic venous blood to the pulmonary arterial tree in 3 patients aged 11, 24, and 28 years. Two of the conduits (pericardial) were intraatrial. The Gore-Tex graft was placed in an extracardiac position. The two pericardial conduits obstructed completely. RESULTS: One patient died at reoperation. In the 2nd patient, the conduit was excised and the hepatic veins were allowed to drain into the atrium. In the 3rd patient, partial thrombosis of the Gore-Tex conduit was noted 30 months after operation. The thrombus resolved with oral anticoagulation. CONCLUSIONS: Conduits carrying only hepatic venous blood flow may have a higher risk of thrombosis. Anticoagulation or alternative methods of directing hepatic blood flow to the pulmonary circulation must be considered in these patients.     

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.     

Computational fluid dynamic study of flow optimization in realistic models of the total cavopulmonary connections

OBJECTIVES AND BACKGROUND: In the Fontan circulation, pulmonary and systemic vascular resistances are in series. The influence of various inferior vena cava to pulmonary artery connections in this unique circulatory arrangement was evaluated using computation fluid dynamics methods. METHODS: Realistic three-dimensional models of total cavopulmonary connections were created from angiographic measurements to include the hepatic vein, superior vena cava, and branches of the pulmonary arteries. Steady-state finite volume analyses were performed using identical in vivo boundary conditions. Computational solutions calculated the percent hydraulic power dissipation and left-to-right pulmonary arterial flow distribution. RESULTS: Simulations of the lateral tunnel, intra-atrial tube, extracardiac conduit with left and right pulmonary artery anastomosis demonstrated extracardiac conduit with left pulmonary artery anastomosis having the lowest energy loss. Varying the extracardiac conduit from 10 to 30 mm resulted in the least energy dissipation at 20 mm. Serial dilation of the lateral tunnel pathway showed a small incremental worsening of energy loss. CONCLUSIONS: Maximizing energy conservation in a low-energy flow domain, such as the Fontan circulation, can be significant to its fluid dynamic performance. Although computational modeling cannot predict postoperative failure or functional outcome, this study confirms the importance of local geometry of the surgically created pathway in the total cavopulmonary connection.     

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.     

Extracardiac conduit Fontan procedure: early and intermediate results

Objective: The extracardiac Fontan procedure, as compared with classic atriopulmonary connections, may have the potential for optimizing ventricular and pulmonary vascular function by maximizing the laminar flow principle, by the avoidance of intra-atrial suture lines and cardiac manipulation, and by minimizing cardiopulmonary bypass time. In this study the clinical results of this procedure are assessed. Methods: From January 1990 until January 1997, 45 patients (33 males and 12 females) with a median age of 4.0 years (range 2.7–38 years) underwent an extracardiac Fontan procedure for univentricular physiology. The underlying diagnoses included tricuspid atresia (n=19), double-inlet left ventricle (n=11), and complex anomalies (n=15). Forty patients (89%) were in sinus rhythm. The median ventricular ejection fraction was 60%. In 37 patients (82%) the procedure was staged. Results: Median cardiopulmonary bypass time was 72 min, with a decrease to a median time of 24 min in the last ten patients. Aortic cross-clamping was avoided in 33 patients (73%). The intraoperative Fontan pressure and transpulmonary gradient were low: 13.6±3.2 and 8.5±3.9 mmHg, respectively. Transient supraventricular tachyarrhythmias were observed in six patients (13%). There was no early or late mortality. At a median follow-up of 64 months (range 26–105 months), 39 patients (87%) were in NYHA class I, four (9%) were in NYHA class II, and two (4%) were in class III. Forty patients (89%) remained in sinus rhythm. The median ventricular ejection fraction was 59%. The median arterial oxygen saturation raised from 82% preoperatively to 97%. Functional class (P=0.02), maintenance of sinus rhythm (P=0.04), and preservation of ventricular function (P=0.05) was superior in patients who were appropriately staged. None of the patients had atrial thrombus, chronic pleural effusions, or protein losing enteropathy. Conclusions: In the majority of patients, the extracardiac Fontan procedure, when performed as a staged procedure, provides excellent early and midterm results in terms of quality of life, maintenance of sinus rhythm, and preservation of ventricular function.     

Fluid dynamic comparison of intra-atrial and extracardiac total cavopulmonary connections

OBJECTIVE: Extracardiac total cavopulmonary connection has recently been introduced as an alternative to intra-atrial procedures. The purpose of this study was to compare the hydrodynamic efficiency of extracardiac and intra-atrial lateral tunnel procedures in total cavopulmonary connections. METHODS: Intra-atrial lateral tunnel, extracardiac tunnel, and extracardiac conduit with and without caval vein offset were performed on explanted sheep heart preparations and studied in an in vitro flow loop. A rate of fluid-energy dissipation analysis was performed for each model using simultaneous measurement of pressure and flow at each inlet and outlet of the right side of the heart. Preparations were perfused by using a steady flow blood pump at 4 flow indices (1-6 L/min/m 2) with the inferior vena cava carrying 65% of the total venous return. RESULTS: Fluid-power losses were consistently lower for the extracardiac conduit procedure compared with the two tunnel configurations (P <.01). A further reduction in energy dissipation of up to 36% was noted in the extracardiac procedure, with 5 mm offset of the extracardiac conduit toward the distal right pulmonary. The intra-atrial and extracardiac tunnel procedures were least efficient, with losses 73% greater than the optimal extracardiac conduit procedure. CONCLUSIONS: The extracardiac conduit procedure provides superior hemodynamics compared with the intra-atrial lateral tunnel and extracardiac tunnel techniques. This hydrodynamic advantage is markedly enhanced by the use of conduit-superior vena cava offset, particularly at high physiologic flows that are representative of exercise. These data suggest additional rationale for the use of extracardiac conduit procedures for final-stage completion of the Fontan circulation.     

Depopulated vena caval homograft: a new venous conduit

OBJECTIVE: Completion of the Fontan procedure is frequently performed by using an extracardiac conduit between the inferior vena cava and the pulmonary artery. Most centers use a polytetrafluoroethylene graft for the extracardiac conduit, and because re-endothelialization is unlikely, anticoagulation is used for a variable period. This study explores the use of an alternate large-caliber venous conduit. METHODS: The superior vena cava was replaced in 8 minipigs with either a polytetrafluoroethylene interposition graft (2 pigs) or a depopulated (acellular), cryopreserved superior vena caval homograft (6 pigs). After 6 months, the animals were killed, and the grafts were examined for patency and histology, including immunostaining. No anticoagulation was used. RESULTS: Polytetrafluoroethylene grafts have a cross-sectional luminal narrowing, ranging from 16% to 40%. Histology showed only partial intimal ingrowth, with excessive subendothelial fibrosis and early calcification. In contrast, the depopulated venous homografts showed minimal luminal narrowing, ranging from 2% to 9%. These grafts were completely repopulated by the recipient with an endothelial lining, which stained positively for factor VIII, and a subendothelial region appropriately recellularized by myofibroblasts, which stained positively for smooth muscle actin and procollagen. There was no evidence of an immune response to the venous homografts, as judged by staining for T-cell surface antigen, CD4, and CD8. Thrombus was not seen in any of the grafts. CONCLUSION: Depopulated, cryopreserved vena caval homografts might be superior conduits for cavopulmonary connection during completion of the Fontan operation by using the extracardiac conduit technique.     

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.     

Reconstruction of the RVOT with valved biological conduits: 25 years experience with allografts and xenografts

Objective: The reconstruction of the RVOT in congenital heart disease often requires the implantation of a valved conduit. Although allografts are considered the conduit of choice their availability is limited and therefore xenografts are implanted as well. We compared the long-term durability of both grafts in the RVOT over a 25-year period. Methods: Between January 1974 and August 1999, 505 patients (median age 4.0 years, range 2 days–31 years; median weight 14.5 kg, range 2.2–76.6 kg; median body length 103 cm, range 48–183 cm) with congenital malformations (PA 25.3%, TOF 14.5%, TOF+PA 2.4%, DORV 4.2%, TGA+PS 8.7%, TAC 24.8%, and other 20.2%) received their first valved conduit (174 xenografts: median diameter 14 mm, range 8–27 mm; 331 allografts: median diameter 19 mm, range 8–30 mm). Results: Follow-up is 3017 patient-years. The 10-year survival-probability for all patients. was 66% with a mean reoperation-free interval for conduit-exchange of 13.3 years (mean reoperation-free interval for allografts, 16.0 years; mean reoperation-free interval for xenograft, 10.3 years). One hundred and thirteen patients underwent a conduit-exchange, mostly due to conduit stenosis. Fourteen patients had a second exchange and three patients a third exchange. For patients with conduit diameters <18 mm (n=235: allograft n=116, xenograft n=119; median age 9 months, range 0–27.3 years), the mean reoperation-free interval was 11.2 years (mean interval allograft, 13.1 years; mean interval xenograft, 8.6 years, P=0.03). For conduit diameters ≥18 mm (n=270: allograft n=215, xenograft n=55, median age 7.4 years, range 0–34.3 years) the mean interval from freedom of conduit exchange was 15.1 years (for allografts 14.1 years, for xenografts 12.5 years, P<0.01). Comparing xenografts to allografts, we found no difference in patient survival probability (P=0.62). There was no significant difference between antibiotic (n=198) preserved vs. cryopreserved (n=133) allografts (P=0.06). Blood group compatibility of allografts to recipients had no significant influence on allograft function (P=0.42). The donors allograft origin, whether aortic or pulmonary valve, had also no significant influence on allograft long-term function (P=0.15). Conclusion: For the reconstruction of the right ventricular outflow tract (RVOT) allografts show significantly better long-term durability than xenografts regardless of the age at implantation and the diameter.     

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.     

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 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.     

Model of complete separation of the hepatic veins from the systemic venous system

Background. In patients undergoing a Fontan operation, partial diversion of the hepatic veins to the pulmonary venous atrium has been tried with various techniques. They failed because of the development of intrahepatic collaterals leading to an unacceptable right-to-left shunting. We postulate that to avoid the formation of intrahepatic collaterals, the totality of the liver has to be drained into the same pressure compartment. We have designed a model of cavopulmonary anastomosis in which a prosthetic conduit reproduces an azygos continuation, associated with the diversion of the totality of the hepatic venous return. This article reports on the early hemodynamics and the fate of the separation of the two venous compartments in long-term survivors.

Methods. Eighteen goats were operated on; the pulmonary artery and hepatic vein pressures were recorded. During month 2, an opacification of the inferior vena cava and the cavopulmonary connection was performed. Between months 6 and 14, another opacification was performed, together with pressure recording at both ends of the conduit.

Results. Postoperatively the pulmonary artery pressure was pulsatile with a mean of 10 mm Hg and the hepatic vein pressure was 0 mm Hg. The first angiogram showed patent tubes with fast progression of the contrast. Throughout the inferior vena cava injection, there was no opacification of the portal or hepatic veins. The late study showed a narrowed conduit in all animals. During the injection, a collateral was injected, feeding into the inferior mesenteric vein. No collateral circulation could be seen draining directly into the liver. The median gradient between the two ends of the conduit was 11 mm Hg.

Conclusions. The isolation of the entire hepatic venous drainage is feasible and efficient for the separation of two pressure compartments. No intrahepatic collaterals are observed with this model at short- or long-term follow-up. The separation of the hepatic venous drainage should persist without collateral circulation as long as the inferior vena cava pressure stays at the levels observed in Fontan circulation.     

Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass

From November 1988 to May 1989, four patients underwent total right heart bypass by means of bidirectional cavopulmonary anastomosis and interposition of an extracardiac conduit from the inferior vena cava to the pulmonary artery. All of them had an uneventful postoperative course, and there have been no early or late deaths. We propose this technique as an alternative surgical option in candidates for a Fontan procedure with (1) hypoplasia or atresia of the left atrioventricular valve, (2) common atrioventricular valve, (3) anomalies of systemic and pulmonary venous return, or (4) auricular juxtaposition.     

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.     

Venous cannulation for extracorporeal circulation after total cavopulmonary connection with extracardiac conduit

We report two cases of venous cannulation after a total cavopulmonary connection (TCPC) with extracardiac conduit. Venous cannulation was performed via side graft sutured to the extracardiac conduit in an end-to-side fashion. The first case was a 3-year-old girl, who suffered from an atypical inferior vena cava obstruction after TCPC. The obstruction region was distal to the anastomosis site of extracardiac conduit and inferior vena cava. She underwent a surgical release of obstruction under cardiopulmonary bypass without circulatory arrest. The second case was a 2-year-old girl, who needed an extracorporeal membrane oxygenation support after TCPC due to severe low cardiac output syndrome. She was decannulated successfully after thirty-nine-hour support.     

Results of an extracardiac pericardial-flap lateral tunnel Fontan operation.

Objective: Extracardiac pericardial-flap lateral tunnel Fontan operation has the theoretical advantage of growth potentiality of the extracardiac tunnels. The mid-term results of this technique and morphologic change of the lateral tunnel were studied. Methods: Clinical data were reviewed in 42 patients who underwent extracardiac pericardial-flap lateral tunnel Fontan operation between November 1993 and December 2004. The age was 2.8+/-1.5 years and the body weight was 12.3+/-3.2kg. Extracardiac tunnel was constructed using the pedicled pericardium with the base undetached. By reviewing the follow-up cardiac angiograms (2.3+/-1.4 years postoperatively), ratios of diameter and cross-sectional area of the lateral tunnel to those of inferior vena cava were obtained. Results: There were 4 surgical mortalities (10%). Postoperative morbidity included prolonged pleural effusion in 5 patients and heart block in 1 patient. Follow-up was possible in 37 patients and the follow up duration was 3.8+/-2.2 years. There were two late deaths due to ventricular dysfunction and sudden death of unknown causes. Two patients required reoperation due to subaortic stenosis and stenosis between inferior vena cava and lateral tunnel. In one patient, bradyarrhythmia was observed but there was no thromboembolic complication. Follow-up anteroposterior and lateral diameter ratio were 1.1+/-0.5 and 1.2+/-0.5. The cross-sectional area ratio was 2.6+/-2.3. In 5 patients, fusiform dilatation of the lateral tunnel was observed, but in the remaining patients, the lateral tunnel preserved tubular morphology with good hemodynamics. Conclusions: Extracardiac pericardial-flap lateral tunnel Fontan operation is relatively simple and feasible even in patients with previous median sternotomies. The mid-term results were acceptable, and the lateral tunnel demonstrated a tendency to preserve its tubular shape. However in some patients, dilatation of the pericardial-flap tunnel was observed during follow up. Longer follow-up is required to determine the morphologic changes of the lateral tunnel and the value of this technique.