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Objectives: Our goals were to compare the outcome of the intra-atrial lateral tunnel (ILT) and the extracardiac conduit (ECC) techniques for staged total cavopulmonary connection (TCPC) and to compare the current modifications of the TCPC technique, i.e. the prosthetic ILT technique with the current ECC technique with a ≥18-mm conduit., Methods: We included patients who had undergone a staged TCPC between 1988 and 2008. Records were reviewed for patient demographics, operative details and events during follow-up (death, surgical and catheter-based reinterventions and arrhythmias)., Results: Of the 208 patients included, 103 had the ILT (51 baffle, 52 prosthetic) technique and 105 had the ECC technique. Median follow-up duration was 13.2 years (interquartile range 9.5-16.3). At 15 years after the TCPC, the overall survival rate was comparable (81% ILT vs 89% ECC; P = 0.12). Freedom from late surgical and catheter-based reintervention was higher for patients who had ILT than for those who had ECC (63% vs 44%; P = 0.016). However, freedom from late arrhythmia was lower for patients who had ILT than for those who had ECC (71% vs 85%, P = 0.034). In a subgroup of patients who had the current TCPC technique, when we compared the use of a prosthetic ILT with ≥18-mm ECC, we found no differences in freedom from late arrhythmias (82% vs 86%, P = 0.64) or in freedom from late reinterventions (70% vs 52%, P = 0.14)., Conclusions: A comparison between the updated prosthetic ILT and current ≥18-mm ECC techniques revealed no differences in late arrhythmia-free survival or late reintervention-free survival. Overall, outcomes after the staged TCPC were relatively good and reinterventions occurred more frequently in the ECC group, whereas late arrhythmias were more common in the ILT group., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: This study aims to compare the outcome of the two co-existing modifications of staged total cavopulmonary connection (TCPC) - the intra-atrial lateral tunnel (ILT) and the extracardiac conduit (ECC)., Methods: We included 209 patients after staged TCPC (102 ILT and 107 ECC), operated on between 1988 and 2008. Medical and surgical records were reviewed for (1) patient demographics and cardiac anatomy; (2) pre-Fontan procedures; (3) pre-Fontan haemodynamics and cardiac functional status; (4) operative details; (5) postoperative hospital course; (6) follow-up information on arrhythmias and thrombo-embolic events; (7) post-Fontan interventions; and (8) clinical status at last follow-up until June 2008., Results: Median follow-up duration was 4.3 years (interquartile range 1.5-7.4 years). At 6-year follow-up, freedom from Fontan failure (i.e., mortality or re-operations for Fontan failure) was 83% for the ILT and 79% for the ECC groups (p=0.6); freedom from late re-operations (other than re-operations for Fontan failure) was 79% for the ILT and the ECC groups and freedom from arrhythmias was 83% for the ILT, and 92% for the ECC groups (p=0.022). Multivariable Cox regression analysis identified intensive care unit stay and cardiopulmonary bypass time as risk factors for Fontan failure, but they were not strong predictors. Right ventricular morphology was identified as a risk factor for arrhythmias. The occurrence of thrombo-embolic events was low with no difference between the ILT and the ECC groups, and irrespective of the postoperative use of anticoagulant or anti-platelet aggregation therapy. At most recent follow-up, sinus rhythm was present in 70% of patients; in 23% of the patients, ventricular function was found to be moderately or severely impaired at echocardiography., Conclusions: Outcome after staged ILT- and ECC-type Fontan operations is good, with comparable freedom from late re-operations and freedom from Fontan failure at 6-year follow-up. The incidence of arrhythmias was significantly lower in the ECC group. Right ventricular morphology was identified as a risk factor for arrhythmias., (Copyright (c) 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.)

Objective: This study assessed clinical condition at midterm follow-up after total cavopulmonary connection for a functionally univentricular heart performed on children younger than 5 years., Methods: Thirty-four Fontan patients (median age 10.4 years, range 6.8-20.7 years, 22 boys, median follow-up 7.8 years, 5.0-17.8 years) underwent electrocardiography, Holter monitoring, bicycle exercise testing, cardiac magnetic resonance imaging, and N-terminal prohormone brain natriuretic peptide (NT-pro-BNP) analysis., Results: Twenty-three patients (68%) were in sinus rhythm. Holter monitoring demonstrated normal mean heart rate, low maximal heart rate, and no clinically significant arrhythmias or sinus node dysfunction. With maximal bicycle ergometry (n = 19), maximum workload (60% of normal), maximum heart rate (90% of normal), and maximal oxygen uptake (69% of normal) were all significantly lower in the Fontan group than in a control group (P < .001). Variables of submaximal exercise indicated less efficient oxygen uptake during exercise in all Fontan patients. Ejection fraction was lower than in control subjects (59% +/- 13% vs 69% +/- 5%, P < .001). Mean end-diastolic and end-systolic volumes and ventricular mass were higher than in control subjects (P < .001). Mean NT-pro-BNP levels were increased relative to reference values, but only 8 patients had levels above the upper reference limit., Conclusion: At midterm follow-up, Fontan patients were in acceptable clinical condition, with preserved global ventricular function, moderately decreased exercise capacity, and NT-pro-BNP levels within reference range. Systemic ventricular mass was elevated, however, suggesting contractility-afterload mismatch. Long-term consequences for ventricular function merit further investigation.

Purpose: To assess pulmonary artery (PA) size, flow variables, and wall shear stress (WSS) in patients after Fontan operation at a young age., Materials and Methods: Flow in the branch PA was obtained with phase contrast velocity-encoded cardiovascular magnetic resonance imaging in 14 patients before and after low-dose dobutamine stress (7.5 microg/kg/min) and in 17 healthy controls at rest., Results: At rest, stroke index, total flow, average, and peak flow rate were all statistically significantly lower in patients than in controls (P<0.001). With stress-testing, all variables increased in patients (P<0.001), apart from stroke index, which did not change. At rest, branch PA area did not differ between patients and controls. Distensibility was lower in patients than in controls (P<0.001). With stress-testing, area and distensibility did not change. At rest, WSS was lower in patients than in controls (P<0.001). WSS increased with stress-testing (P<0.001), but not to the same levels as during resting conditions of the control group., Conclusion: PA size is normal long-term after Fontan operation at a young age. Flow variables, distensibility, and WSS are significantly lower compared to healthy controls, and do not show adequate reactions with stress-testing, which is suggestive of pulmonary artery endothelial and/or vascular dysfunction., (Copyright (c) 2008 Wiley-Liss, Inc.)

After Fontan operation, patients are limited in increasing cardiac output and in exercise capacity. This has been related to impaired preload or other factors leading to decreased global ventricular performance with stress. To study these factors, the stress responses of functionally univentricular hearts were assessed at rest and during low-dose dobutamine stress using cardiovascular magnetic resonance imaging. Thirty-two patients after Fontan completion at young age were included (27 with total cavopulmonary connection, 5 with atriopulmonary connection; mean age 13.3 years, range 7.5 to 22.2; 23 male patients; median follow-up after Fontan operation 8.1 years, range 5.2 to 17.8). A multiphase short-axis stack of 10 to 12 contiguous slices of the systemic ventricle was obtained at rest and during low-dose dobutamine stress cardiovascular magnetic resonance imaging (maximum 7.5 microg/kg/min). With stress-testing, heart rate, ejection fraction, and cardiac index increased adequately (p <0.001). There was an abnormal decrease in end-diastolic volume and an adequate decrease in end-systolic volume (p <0.001). Stroke volume did not change with stress testing (p = 0.15). At rest, dominant left ventricles had higher ejection fractions than dominant right ventricles (p = 0.01), but this difference disappeared with stress testing. In conclusion, a functionally univentricular heart after Fontan completion at young age has an adequate increase in ejection fraction with beta-adrenergic stimulation. However, as a result of impaired preload with stress, cardiac output can be increased only by increasing heart rate.