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The Single Ventricle Reconstruction trial randomised neonates with hypoplastic left heart syndrome to a systemic-to-pulmonary-artery shunt strategy. Patients received care according to usual institutional practice. We analysed practice variation at the Stage II surgery to attempt to identify areas for decreased variation and process control improvement. Prospectively collected data were available in the Single Ventricle Reconstruction public-use database. Practice variation across 14 centres was described for 397 patients who underwent Stage II surgery. Data are centre-level specific and reported as interquartile ranges across all centres, unless otherwise specified. Preoperative Stage II median age and weight across centres were 5.4 months (interquartile range 4.9-5.7) and 5.7 kg (5.5-6.1), with 70% performed electively. Most patients had pre-Stage-II cardiac catheterisation (98.5-100%). Digoxin was used by 11/14 centres in 25% of patients (23-31%), and 81% had some oral feeds (68-84%). The majority of the centres (86%) performed a bidirectional Glenn versus hemi-Fontan. Median cardiopulmonary bypass time was 96 minutes (75-113). In aggregate, 26% of patients had deep hypothermic circulatory arrest >10 minutes. In 13/14 centres using deep hypothermic circulatory arrest, 12.5% of patients exceeded 10 minutes (8-32%). Seven centres extubated 5% of patients (2-40) in the operating room. Postoperatively, ICU length of stay was 4.8 days (4.0-5.3) and total length of stay was 7.5 days (6-10). In the Single Ventricle Reconstruction Trial, practice varied widely among centres for nearly all perioperative factors surrounding Stage II. Further analysis may facilitate establishing best practices by identifying the impact of practice variation.

BackgroundSystemic to pulmonary collaterals (SPCs) represent an additional and unpredictable source of pulmonary blood flow in patients with single ventricle physiology following bidirectional superior cavopulmonary connection (BCPC). Understanding their influence on patient outcomes has been hampered by uncertainty about the optimal method of quantifying SPC flow.ObjectiveTo quantify SPC flow by cardiac magnetic resonance (CMR) prior to total cavopulmonary connection (TCPC) in order to identify preoperative risk factors and determine influence on postoperative outcomes.DesignSingle centre prospective cohort study.SettingTertiary referral centre.Patients65 patients with single ventricle physiology undergoing CMR for preoperative assessment of TCPC completion underwent quantification of SPC flow. Clinical outcomes of 41 patients in whom TCPC was completed were obtained.Main outcome measuresEarly post-TCPC clinical outcomes associated with SPC flow were assessed, including postoperative chest drainage volume, postoperative chest drainage duration and length of intensive care and hospital stays. Additionally preoperative covariates associated with SPC flow were assessed including age at BCPC and CMR, SpO2 at BCPC and CMR, ventricle type, pulmonary artery (PA) cross-sectional area and PA pulsatility. Different methods of CMR SPC flow quantification were compared.ResultsHigher SPC flow was associated with increased postoperative chest drain volume (r=0.51, p=0.001), chest drain duration (r=0.43, p=0.005), and intensive care unit (r=0.32, p=0.04) and log-transformed hospital stays (r=0.31, p=0.048). The effect of SPC flow on outcome was independent of fenestration, ventricle type and function. Preoperative covariates associated with SPC flow included age at BCPC (β=−0.34, p=0.008), SpO2 at time of CMR (β=0.34, p=0.004) and branch PA cross-sectional area (β=−0.26, p=0.036), model R2=0.34. Moreover, patients with pulsatile pulmonary blood flow had lower SPC flow than those without (0.8 vs 1.3 l/min/m2 p=0.012). SPC flow calculated by the difference between pulmonary venous return and pulmonary artery flow (l/min/m2) showed greatest association with preoperative covariates and strongest correlation with postoperative outcomes compared with other methods of quantification.ConclusionsCMR can provide an effective measurement of SPC flow prior to TCPC. Young age at BCPC, high preoperative oxygen saturation and smaller PAs are associated with increased SPC flow, which may promote increased postoperative pleural drainage and lengthen recovery.

Abnormal height and adiposity are observed after the Fontan operation. These abnormalities may be associated with worse functional outcome. We analyzed data from the National Heart, Lung, and Blood Institute Pediatric Heart Network cross-sectional study of Fontan patients. Groups were defined by height ( z-score <−1.5 or ≥−1.5) and body mass index (body mass index [BMI] z-score <−1.5 or −1.5 to 1.5 or ≥1.5). Associations of anthropometric measures with measurements from clinical testing (exercise, echocardiography, magnetic resonance imaging) were determined adjusting for demographics, anatomy, and pre-Fontan status. Relationships between anthropometric measures and functional health status (FHS) were assessed using the Child Health Questionnaire. Mean age of the cohort (n = 544) was 11.9 ± 3.4 years. Lower height- z patients (n = 124, 23%) were more likely to have pre-Fontan atrioventricular valve regurgitation ( P = .029), as well as orthopedic and developmental problems (both P < .001). Lower height- z patients also had lower physical and psychosocial FHS summary scores (both P < .01). Higher BMI- z patients (n = 45, 8%) and lower BMI- z patients (n = 53, 10%) did not have worse FHS compared to midrange BMI- z patients (n = 446, 82%). However, higher BMI- z patients had higher ventricular mass-to-volume ratio ( P = .03) and lower % predicted maximum work ( P = .004) compared to midrange and lower BMI- z patients. Abnormal anthropometry is common in Fontan patients. Shorter stature is associated with poorer FHS and non-cardiac problems. Increased adiposity is associated with more ventricular hypertrophy and poorer exercise performance, which may have significant long-term implications in this at-risk population.

Objective: The aim of the study was to compare the effect of two different regimens of milrinone on hemodynamics and oxygen saturation in pediatric patients undergoing Fontan procedure. Design: This was a randomized study. Setting: Cardiac centers. Patients: This study included 116 patients undergoing Fontan procedure. Material and Methods: Group E: Milrinone was started as infusion 0.5 μg/kg/min without a loading dose at the beginning of cardiopulmonary bypass (CPB) followed by infusion 0.5-0.75 μg/kg/min in the pediatric cardiac surgical intensive care unit (PSICU). Group L: Milrinone was started as a loading dose 50 μg/kg over 10 min before weaning from CPB followed by infusion 0.5-0.75 μg/kg/min in the PSICU. Measurements: Heart rate, mean arterial blood pressure, central venous pressure, transpulmonary pressure, cardiac index, pharmacological support, lactate level, urine output, oxygen saturation, ICU, and hospital length of stay. Main Results: There were no changes in the heart rate and mean arterial blood pressure (P > 0.05). The increase in the postoperative central venous pressure, transpulmonary pressure and lactate level was lower in Group E than Group L (P < 0.05). The increase in the postoperative cardiac index, oxygen saturation, and urine output was higher in Group E than Group L (P < 0.05). The requirement for pharmacological support was lower in the Group E (P < 0.05). The ICU and hospital length of stay were shorter in the Group E than Group L (P < 0.05). Conclusion: Early use of milrinone during Fontan procedure facilitated the weaning from CPB, decreased the elevation in the central venous pressure, transpulmonary gradient pressure, and the requirement for pharmacological support. Furthermore, it increased the cardiac index and arterial oxygen saturation.

Objective: To compare the efficacy of aspirin and warfarin for prophylaxis of thrombosis in patients undergoing total cavopulmonary anastomosis. Evaluate whether coagulation factors (VII, VIII and protein C), clinical data, fenestration or hemodynamic factors, interfere with postoperative thrombosis. Methods: A prospective, randomized study of 30 patients, randomized into Group I (Warfarin) and Group II (AAS), underwent total cavopulmonary shunt with extracardiac conduit, between 2008 and 2011, with follow-up by clinical visits to evaluate side effects and adhesion. Performed transesophageal echocardiography in post operatory time, 3, 6,12 and 24 months; angiotomography at 6, 12 and 24 months to evaluate changes in the internal tube wall or thrombi and pulmonary scintigraphy to evaluate possible PTE. Results: Two deaths in group I; 33.3% of patients had thrombus (46.7% in Group II). The previous occurrence of thrombus and low levels of coagulation protein C were the only factors that influenced the time free of thrombus (P=0.035 and 0.047). Angiotomographic evaluation: 35.7% in group II presented material accumulation greater than 2 mm (P=0.082). Scintigraphy: two patients had PTE in group II. Five patients had difficulty to comply with the treatment, 4 in group I with INR ranging from 1 to 6.4. Conclusion: The previous occurrence of thrombus is a risk factor for thrombosis in the postoperative period. Patients using AAS tend to deposit material in the tube wall. The small sample size did not allow to conclude which is the most effective drug in the prevention of thrombosis in this population.

Objective The preferred surgical approach for systemic-pulmonary shunts has changed from thoracotomy to sternotomy in our institution, to concomitantly manage the ductus arteriosus during surgery. The purpose of this study was to compare the outcomes of systemic-pulmonary shunts for neonates with functionally univentricular hearts based on surgical approach. Methods Fifty-two neonates with functionally univentricular hearts underwent systemic-pulmonary shunt via sternotomy ( n  = 28) or thoracotomy ( n  = 24). Patient characteristics, achievement rates of right heart bypass, and survival rates were compared for the different approaches. Results Prenatal diagnosis was made more common in the sternotomy group ( p  = 0.006). The shunt was placed more centrally in the sternotomy group. The ductus arteriosus was ligated or banded in most patients in the sternotomy group (26/28) and in a few patients in the thoracotomy group (6/24). Frequency of ductal management in the early postoperative phase was not different between the groups (21 vs 25 %), but three new incisions had to be made in the thoracotomy group. No differences were seen in the achievement rates of bidirectional cavopulmonary shunts (86 vs 87 % at 10 months of age) and total cavopulmonary connection (81 vs 81 % at 2 years of age), or in the survival rates (92 vs 96 % at 8 years). Conclusions There were no differences in short- and long-term outcomes between the groups. The sternotomy approach might be preferable in the current era of prenatal diagnosis, to allow simultaneous duct management during systemic-pulmonary shunt surgery, particularly in patients with large ducts associated with functionally univentricular hearts.

ObjectivePatients undergoing single-ventricle palliation have experienced significant improvement in survival in the recent era. However, a substantial proportion of these patients undergo reoperations. We performed a review of the Australia and New Zealand (ANZ) Fontan Registry to determine the overall reintervention and reoperative burden in these patients.MethodsA retrospective longitudinal cohort study was performed using data from patients who underwent a Fontan operation between 1975 and 2016 from the ANZ Fontan Registry. The data obtained included Fontan operation, reinterventions and most recent follow-up status. We examined the type and timing of reinterventions and survival.ResultsOf the 1428 patients identified, 435 (30%) underwent at least one reintervention after the Fontan operation: 110 patients underwent early reintervention and 413 underwent late reinterventions. Excluding Fontan conversion and transplantation, 220 patients underwent at least one interventional procedure and 209 patients underwent at least one reoperation. Fenestration closure and pacemaker-related procedures were the most common catheter and surgical interventions, respectively. The cumulative incidence of reintervention following Fontan was 23%, 37% and 55% at 10, 20 and 30 years, respectively. Survival and freedom from failure were worse in patients requiring later reintervention after Fontan surgery (51% vs 83% and 42% vs 69%, respectively at 30 years, p<0.001). This difference persisted after excluding pacemaker-related procedures (p<0.001). Operative mortality for non-pacemaker late reoperations after Fontan was 6%.ConclusionsA substantial proportion of Fontan patients require further intervention to maintain effective single-ventricle circulation. Patients undergoing reoperation after Fontan have higher rates of mortality and failure, despite intervention.

Objective Elevated energy loss in the total cavopulmonary connection (TCPC) is hypothesised to have a detrimental effect on clinical outcomes in single-ventricle physiology, which may be magnified with exercise. This study investigates the relationship between TCPC haemodynamic energy dissipation and exercise performance in single-ventricle patients. Methods Thirty consecutive Fontan patients with TCPC and standard metabolic exercise testing were included. Specific anatomies and flow rates at rest and exercise were obtained from cardiac MR (CMR) and phase-encoded velocity mapping. Exercise CMR images were acquired immediately following supine lower limb exercise using a CMR-compatible cycle ergometer. Computational fluid dynamics simulations were performed to determine power loss of the TCPC anatomies using in vivo anatomies and measured flows. Results A significant negative linear correlation was observed between indexed power loss at exercise and (a) minute oxygen consumption (r=−0.60, p<0.0005) and (b) work (r=−0.62, p<0.0005) at anaerobic threshold. As cardiac output increased during exercise, indexed power loss increased in an exponential fashion (y=0.9671x3.0263, p<0.0001). Conclusions This is the first study to demonstrate the relationship between power loss and exercise performance with the TCPC being one of the few modifiable factors to allow for improved quality of life. These results suggest that aerobic exercise tolerance in Fontan patients may, in part, be a consequence of TCPC power loss.

ObjectiveMore patients with Fontan physiology are reaching adulthood. The purpose of this meta-analysis was to evaluate the late outcomes of patients palliated with Fontan procedure and to assess the risk factors for mortality.MethodsPubMed, Embase and Web of Science were queried to retrieve observational studies of survival in patients following the Fontan procedure with ≥5 years of follow-up. A random-effects model was used to determine pooled survival estimates at 5, 10 and 15 years. Meta-regression was used to assess potential moderators for death.ResultsNineteen articles with a total of 5859 patients were included. The weighted mean follow-up time was 8.94±2.64 years with overall 8.3% deaths and 1.5% transplants. Pooled survival estimates at 5, 10 and 15 years were 90.7%, 87.2% and 87.5%, respectively; and 88.4%, 85.7% and 84.1%, respectively, for studies that included all three time intervals (n=4). Earliest surgical year included in the study, proportion of atriopulmonary connections versus extracardiac conduit or lateral tunnel, and older age at Fontan were associated with higher rates of death, but ventricular morphology was not. Protein-losing enteropathy, reoperation and pacemaker insertion were reported in 2.1%, 5.6% and 6.8% patients, respectively.ConclusionsSurvival following the Fontan procedure has improved with time and is influenced by Fontan type and age at the time of Fontan. At a mean follow-up of 8.9 years, there was no significant association between survival and ventricular morphology, not taking into account the mortality prior to Fontan.

BackgroundThe Fontan circulation is associated with an increased risk of thromboembolic events (TEs). As many as 25% of these thrombotic events result in fatality. More subtle adverse effects on the pulmonary circulation from embolic thrombi may further impair adequate functioning of the circuit. Despite these well-documented phenomena, the most optimal approaches to thromboprophylaxis are still not clearly defined.ObjectiveA meta-analysis of published trials in English on PubMed and Cochrane libraries that evaluated the role of using TE prophylaxis in patients who underwent the Fontan procedure was conducted.Methods10 studies with a total number of 1200 patients with an average follow-up time of 7.1 years were identified. A random effect model was used.ResultsThe incidence of TE was significantly less in patients who received TE prophylaxis (using either aspirin or warfarin) compared with patients who did not receive TE prophylaxis (OR 0.425, 95% CI 0.194 to 0.929, p<0.01, I2=37%). The incidence of TE was significantly lower in patients who received aspirin compared with no TE prophylaxis (OR 0.363, 95% CI 0.177 to 0.744, p<0.01, I2=0%) and who received warfarin compared with no TE prophylaxis (OR 0.327, 95% CI 0.168 to 0.634, p<0.01, I2=2.5%). There was no significant difference in incidence of TE between warfarin and aspirin (OR 0.936, 95% CI 0.609 to 1.438, p=0.54, I2=0%). Furthermore, there was no significant difference in incidence of early TE (within 6 months of the operation) or late TE (>6 months) between patients receiving warfarin and aspirin (OR 0.784, 95% CI 0.310 to 1.982, p=0.37, I2=8%) and (OR 0.776, 95% CI 0.249 to 2.42, p=0.3, I2=45%), respectively. When only total cavopulmonary connection patients were included, there was again no difference between warfarin and aspirin in the incidence of TE (OR 0.813, 95% CI 0.471 to 1.401, p=0.34, I2=11%).ConclusionsThis study shows a significantly lower incidence of TE after Fontan procedure if either aspirin or warfarin is used. This meta-analysis suggests no significant difference in incidence of early or late TE in patients receiving aspirin compared with warfarin.