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Background: Fontan procedure, the standard surgical palliation to treat children with single ventricular defects, causes systemic complications over years due to lack of pumping at cavopulmonary junction. A device developed specifically for cavopulmonary support is thus considered, while current commercial ventricular assist devices (VAD) induce high shear rates to blood, and have issues with paediatric suitability., Aim: To demonstrate the feasibility of a small, valveless, non-invasive to blood and pulsatile rotary pump, which integrates impedance and peristaltic effects., Methods: A prototype pump was designed and fabricated in-house without any effort to optimise its specification. It was then tested in vitro, in terms of effect of pumping frequency, background pressure differences and pump size on output performance., Results: Net flow rate (NFR) and maximum pressure head delivery are both reasonably linearly dependent on pumping frequency within normal physiological range. Positive linearity is also observed between NFR and the extent of asymmetric pumping. The device regulates NFR in favourable pressure head difference and overcomes significant adverse pressure head difference. Additionally, performance is shown to be insensitive to device size., Conclusions: The feasibility of the novel rotary pump integrating impedance and peristaltic effects is demonstrated to perform in normal physiological conditions without any optimisation effort. It provides promising results for possible future paediatric cavopulmonary support and warrants further investigation of miniaturisation and possible haemolysis., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Patients with single ventricle defects undergoing the Fontan procedure eventually face Fontan failure. Long-term cavopulmonary assist devices using rotary pump technologies are currently being developed as a subpulmonary power source to prevent and treat Fontan failure. Low hydraulic resistance is a critical safety requirement in the event of pump failure (0 RPM) as a modest 2 mmHg cavopulmonary pressure drop can compromise patient hemodynamics. The goal of this study is therefore to assess the passive performance of a viscous impeller pump (VIP) we are developing for Fontan patients, and validate flow simulations against in-vitro data. Two different blade heights (1.09 mm vs 1.62 mm) and a blank housing model were tested using a mock circulatory loop (MCL) with cardiac output ranging from 3 to 11 L/min. Three-dimensional flow simulations were performed and compared against MCL data. In-silico and MCL results demonstrated a pressure drop of < 2 mmHg at a cardiac output of 7 L/min for both blade heights. There was good agreement between simulation and MCL results for pressure loss (mean difference - 0.23 mmHg 95% CI [0.24-0.71]). Compared to the blank housing model, low wall shear stress area and oscillatory shear index on the pump surface were low, and mean washout times were within 2 s. This study demonstrated the low resistance characteristic of current VIP designs in the failed condition that results in clinically acceptable minimal pressure loss without increased washout time as compared to a blank housing model under normal cardiac output in Fontan patients., (© 2023. Springer Nature Limited.)

Objective(s): The Fontan procedure is a common palliative intervention for sufferers of single ventricle congenital heart defects that results in an anastomosis of the venous return to the pulmonary arteries called the total cavopulmonary connection (TCPC). In patients with palliated single ventricular heart defects, the Fontan circulation passively directs systemic venous return to the pulmonary circulation in the absence of a functional sub-pulmonary ventricle. Therefore, the Fontan circulation is highly dependent on favourable flow and energetics, and minimal energy loss is of great importance. The majority of in vitro studies, to date, employ a rigid TCPC model. Recently, few studies have incorporated flexible TCPC models, without the inclusion of commercially available conduits used in these surgical scenarios., Method: The methodology set out in this study successfully utilizes patient-specific phantoms along with the corresponding flowrate waveforms to characterise the flow haemodynamic performance of extracardiac Gore-Tex conduits. This was achieved by comparing a rigid and flexible TCPC models against a flexible model with an integrated Gore-Tex conduit., Results: The flexible model with the integrated Gore-Tex graft exhibited greater levels of energy losses when compared to the rigid walled model. With this, the flow fields showed greater levels of turbulence in the complaint and Gore-Tex models compared to the rigid model under ultrasound analysis., Conclusion: This study shows that vessel compliance along with the incorporation of Gore-Tex extracardiac conduits have significant impact on the flow haemodynamics in a patient-specific surgical scenario.

Limited donor organs and alternative therapies have led to a growing interest in the use of blood pumps as a treatment strategy for patients with single functional ventricle. The present study examines the use of collapsible and flexible impeller, cage, and diffuser designs of an axial blood pump for Fontan patients. Using one-way fluid-structure interaction (FSI) studies, the impact of blade deformation on blood damage and pump performance was investigated for flexible impellers. We evaluated biocompatible materials, including Nitinol, Bionate 80A polyurethane, and silicone for flow rates between 2.0-4.0 L/min and rotational speeds of 3000-9000 rpm. The level of deformation experienced by a cage and diffuser made of surgical stainless steel (control), Nitinol, and Bionate 80A polyurethane was also predicted using one-way FSI. The fluid pressure on the surface of the impeller, cage, and diffuser was determined using computational fluid dynamics (CFD), and then, the surface pressure was exported and used to investigate the impeller, cage, and diffuser deformation using finite element analysis. Finally, deformed impeller geometries were imported into the CFD software to determine the implication of deformation on pressure generation, blood damage index, and fluid streamlines. It was found that rotational speed, and not flow rate, is the largest determinant of impeller deformation, occurring at the blade trailing edges. The models predicted the maximum impeller deformation for Nitinol to be 40 nm, Bionate 80A polyurethane to be 106 μm, and silicone to be 2.8 mm, all occurring at 9000 rpm. The effects of silicone deformation on performance were significant, particularly at speeds above 5000 rpm where a decrease in pressure generation of more than 10% was observed. Despite this loss, the pressure generation at 5000 rpm exceeded the level required to alleviate Fontan complications. A blood damage estimation was performed and levels remained low. The effect of significant impeller deformation on blood damage was inconsistent and requires additional investigation. Cage and diffuser geometries made of steel and Nitinol deformed minimally but Bionate 80A experienced unacceptable levels of deformation, particularly in the free-flow case without a spinning impeller. These results support the continued evaluation of a flexible, pitch-adjusting, axial-flow, mechanical assist device as a clinical therapeutic option for patients with dysfunctional Fontan physiology., (© 2020 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

Objective: Customized Fontan designs, generated by computer-aided design (CAD) and optimized by computational fluid dynamics simulations, can lead to novel, patient-specific Fontan conduits unconstrained by off-the-shelf grafts. The relative contributions of both surgical expertise and CAD to Fontan optimization have not been addressed. In this study, we assessed hemodynamic performance of Fontans designed by both surgeon's unconstrained modeling (SUM) and by CAD., Methods: Ten cardiac magnetic resonance imaging datasets were used to create 3-dimensional (3D) models of Fontans. Baseline computational fluid dynamics simulations assessed Fontan indexed power loss (iPL), hepatic flow distribution, and percentage of conduit surface area with abnormally low wall shear stress for venous flow (<1 dyne/cm 2 ). Fontans not meeting thresholds were redesigned using 2 methods: SUM (ie, original venous anatomy without the Fontan was 3D printed and sent to surgeon for Fontan redesign with clay modeling) and CAD (ie, the same 3D geometry was sent to engineers for iterative Fontan redesign guided by computational fluid dynamics). Both groups were blinded to each other's results., Results: Eight Fontans were redesigned by SUM and CAD methods. Both SUM and CAD redesigns met iPL thresholds. SUM had lower iPL, whereas CAD demonstrated balanced hepatic flow distribution and lower wall shear stress percentage. Wall shear stress percentage shared an inverse relationship with iPL, preventing oversized Fontan designs., Conclusions: Customized Fontan conduits with low iPL can be created by either a surgeon or CAD. CAD can also improve hepatic flow distribution and prevent oversized Fontan designs. Future studies should investigate workflows that combine SUM and CAD to optimize Fontan conduits., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: The use of Y-grafts for Fontan completion is hypothesized to offer more balanced hepatic flow distribution (HFD) and decreased energy losses. The purpose of this study was to evaluate the hemodynamic performance of Y-grafts over time using serial cardiac magnetic resonance data and to compare their performance with extracardiac Fontan connections., Methods: Ten Fontan patients with commercially available Y-graft connections and serial postoperative cardiac magnetic resonance data were included in this study. Patient-specific computational fluid dynamics simulations were used to estimate HFD and energy losses. Y-graft performance was compared with 3 extracardiac conduit Fontan groups (n = 10 for each) whose follow-up times straddle the Y-graft time points., Results: Y-graft HFD became significantly more balanced over time (deviation from 50% decreased from 18% ± 14% to 8% ± 8%; P = .015). Total cavopulmonary connection resistance did not significantly change. Y-grafts at 3-year follow-up showed more balanced HFD than the extracardiac conduit groups at both the earlier and later follow-up times. Total cavopulmonary connection resistance was not significantly different between any Y-graft or extracardiac conduit group., Conclusions: Y-grafts showed significantly more balanced HFD over a 3-year follow-up without an increase in total cavopulmonary connection resistance, and therefore may be a valuable option for Fontan completion. Additional follow-up data at longer follow-up times are still needed to thoroughly characterize the potential advantages of Y-graft use., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

The need to simulate the normal operating conditions of the human body is the key factor in every study and engineering process of bioelectronic devices designed for implantation. The Fontan procedure is an example of such a process aimed to support the human body function. It is a standard treatment method for patients with a functionally univentricular heart. However, it has significant drawbacks such as overload of the only functional heart ventricle that often leads to the necessity of the heart transplantation. In this study, we analyze the total cavopulmonary connection (TCPC) influence on the blood with and without connected auxiliary blood circulation pump. We investigate four different types of TCPC configurations, analyze blood pressure and different flow rate, study the turbulent kinetic energy distribution, and evaluate hydraulic and power losses for various cases. Finally, we calculate volumetric scalar shear stresses distribution and demonstrate the high potential of TCPC configuration with connected rotary pump as a tool for the load redistribution in the functional heart ventricle. This work is particularly relevant for improving existing TCPCs' quality that can extend the life of Fontan patients. Moreover, it also applies to the reduction of morbidity and mortality of the patients waiting for the heart transplantation., (© 2019 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

Various congenital heart defects (CHDs) are characterized by the existence of a single functional ventricle, which perfuses both the systemic and pulmonary circulation. A three-stage palliation procedure, including the final Fontan completion, is often adopted by surgeons to treat patients with such CHDs. The completion Fontan involves the creation of a total cavopulmonary connection (TCPC), commonly accomplished with an extracardiac conduit. This TCPC results in nonphysiologic flow conditions that can lead to systemic venous hypertension, reduced cardiac output, and ultimately the need for heart transplantation. A modest pressure rise of 5-6 mm Hg could correct the abnormal flow dynamics in these patients. To achieve this, we propose a novel conceptual design of a dual-propeller pump inside a flared TCPC. The TCPC dual-propeller conjunction was examined for hydraulic performance, blood flow pattern, and potential for hemolysis inside the TCPC using computational fluid dynamics (CFD). The effect of axial distance between the two propellers on the blood flow interference and energy loss was studied to determine the optimal separation distance. Both the inferior vena cava (IVC) and superior vena cava (SVC) propellers provided a pressure rise of 1-20 mm Hg at flow rates ranging from 0.4 to 7 lpm while rotating at speeds of 6,000-12,000 rpm. Larger separation distance provided favorable performance in terms of flow interference, energy loss, and blood damage potential. The ability of a dual-propeller micropump to provide the required pressure rise would help to augment the cavopulmonary flow and mimic flows seen in normal biventricular circulation.

An implantable rotary blood pump was developed to provide long-term mechanical right heart support for patients who have failing Fontan circulation. The objective of this study was to evaluate the pump in vivo in a 30 day sheep study. Pump speed was set at 3,900 rpm for the duration of the study, and pump power was between 4.3 and 4.6 W. The pump inlet pressures for the superior vena cava (SVC) and inferior vena cava (IVC) were 14 ± 15 and 11 ± 15 mm Hg, respectively, over the duration of the study. Hematocrit remained stable at 30% ± 4%. Partial thromboplastin time (PTT) steadily increased from 30 s preoperatively to a high of 59 s on postoperative day 20, while prothrombin time (PT) remained at 20 ± 2 s for the duration of the study. The implantation and postoperative recovery were successful, and the animal demonstrated normal physiologic pulmonary and venous pressures and cardiac output. On pump inspection, the IVC and SVC inlets were completely clear of any deposits, but there were small thrombi (approximately 0.5 mm diameter) between each of the three rotor blades and along 20% of the parting line of the two volute halves. A complete right heart bypass was performed, postoperative recovery was successful, and the pump demonstrated adequate circulatory support and normal physiologic pulmonary and venous pressures. This study was the first successful test of a right heart replacement device in a chronic animal study.

In patients with a single ventricle, failure of the cardiovascular system may be prevented by substituting the missing sub-pulmonary ventricle with a pump. The aim of this study was to design and evaluate a device for long-term cavopulmonary support. A radial pump with two inlets and two outlets, a single impeller, mechanical bearings, and dual motor configuration was developed. Motor and fluid dynamic components were designed and simulated using computational methods including thermal effects. Hydraulic properties were determined in-vitro with 3D-printed prototypes. The pump design was virtually implanted in an MRI-derived total cavopulmonary connection (TCPC). Computational fluid dynamics (CFD) showed flow fields without regions of flow stagnation (velocity < 0.1 m/s) and only minor recirculations within the pump between 2-10 L/min against pressure heads of 0-50 mmHg at 2500-5000 rpm. The computed maximum temperature increase of blood due to motor heat was 1.3 K. Virtual implantation studies showed that the pump would introduce an additional volume of approximately 4 mL. Experimentally determined hydraulic performance results agreed well with CFD (deviation of <1.3 mmHg) and indicated pressure-sensitive characteristics (∼-2.6 mmHg/(L/min)) while balancing the two inlet pressures (∆P < 2.5 mmHg) under imbalanced inflow conditions. Through in-silico and in-vitro investigations, we demonstrated a promising pump design, which fulfills the basic requirements for long-term cavopulmonary support., (Copyright © 2019 IPEM. Published by Elsevier Ltd. All rights reserved.)

The current methodology of Fontan palliation results in a one "pump" circulatory system with passive flow to the lungs. Inherent hemodynamic differences exist between a biventricular circulatory system and this modified physiology, leading to a host of long-term complications. Mechanical circulatory support (MCS) is a potential option to combat these pathophysiological conditions. In this study, we examine the VentriFlo True Pulse Pump as a MCS option to support a failing Fontan patient. An in vitro circulatory loop was used to model a failing Fontan patient, reproducing pathophysiological pressures and flow rates. The VentriFlo True Pulse Pump was positioned as a right sided support, testing multiple cannulation and baffle restriction strategies, as well as various pumping parameters including flow rate, frequency, stroke volume and the ejection to filling time ratio. A 10 mm Hg decrease in IVC pressure and 0.75 L/min increase in cardiac output were achieved using a complete baffle restriction strategy. Additional cannulation and banding strategies were not as successful. Pump flow rate and frequency significantly impacted hemodynamics, while the ejection to filling time ratio did not. Though not ideal, complete baffle restriction was necessary to achieve successful support. The ability to tune individual pumping parameters for a given MCS device will have a substantial impact on the pressures and flow augmentation seen in a Fontan circulation. Both future pump design and off-label VADs for Fontan use should consider the pump configuration and parameter combinations presented here, which offered successful support., (© 2018 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

Mechanical circulatory support and heart transplantation are both becoming increasingly common among Fontan patients as this growing cohort of patients is reaching young adulthood. Although device placement may present challenges, the new focus is on selecting the correct cannulation sites and devices at the correct time to successfully bridge these challenging patients to heart transplant. Herein, we report the use of a number of mechanical circulatory support strategies tailored to the clinical setting that culminated in 130 days of support before cardiac transplantation of a young adult with a failing Fontan circulation., (Copyright © 2018. Published by Elsevier Inc.)

Tissue engineering holds great promise for the advancement of cardiovascular surgery as well as other medical fields. Tissue-engineered vascular grafts have the ability to grow and remodel and could therefore make great advances for pediatric cardiovascular surgery. In 2001, we began a human clinical trial evaluating these grafts in patients with a univentricular physiology. Herein, we report the long-term results of patients who underwent implantation of tissue-engineered vascular grafts as extracardiac total cavopulmonary conduits. Tissue-engineered vascular grafts seeded with autologous bone marrow mononuclear cells were implanted in 25 patients with univentricular physiology. The graft is composed of a woven fabric of poly-l-lactide acid or polyglycolic acid and a 50:50 poly (l-lactic-co-ε-caprolactone) copolymer. Patients were followed up with postoperatively in a multidisciplinary clinic. Median patient age at operation was 5.5 years and the mean follow-up period was 11.1 years. There was no graft-related mortality during the follow-up period. There was also no evidence of aneurysmal formation, graft rupture, graft infection, or calcification. Seven (28%) patients had asymptomatic graft stenosis and underwent successful balloon angioplasty. Stenosis is the primary complication of the tissue-engineered vascular graft. Avoidance of anticoagulation therapy would improve patients' quality of life. Tissue-engineered vascular grafts have feasibility in pediatric cardiovascular surgery., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Transcatheter stent-secured completion of total cavopulmonary connection (TCPC) after surgical preparations during the Glenn anastomosis procedure has been reported, but complications from this approach have precluded its clinical acceptance., Aims: To analyse cardiovascular morphology and dimensions in children with bidirectional Glenn anastomosis, regarding the optimal device design for transcatheter Fontan completion without special surgical "preconditionings"., Methods: We retrospectively analysed 60 thoracic computed tomography and magnetic resonance angiograms performed in patients with a median age of 4.1 years (range: 1.8-17.1 years). Additionally, we simulated TCPC completion using different intra-atrial stent-grafts in a three-dimensional model of the representative anatomy, and performed calculations to determine the optimal stent-graft dimensions, using measured distances., Results: Two types of cardiovascular arrangement were identified: left atrium interposing between the right pulmonary artery (RPA) and inferior vena cava, with the right upper pulmonary vein (RUPV) orifice close to the intercaval axis (65%); and intercaval axis traversing only the right(-sided) atrial cavity, with the RUPV located posterior to the atrial wall (35%). In the total population, the shortest median RPA-to-atrial wall distance was 1.9mm (range: 0.6-13.8mm), while the mean intra-atrial distance along the intercaval axis was 50.1±11.2mm. Regardless of the arrangement, 83% of all patients required a deviation of at least 5.9±2.4mm (range: 1.2-12.7mm) of the stent-graft centre at the RUPV level anteriorly to the intercaval axis to avoid covering or compressing this vein. Fixing the anterior deviation of the curved stent-graft centre at 10mm significantly decreased the range of bend angle per every given RUPV-RPA distance., Conclusions: For both types of cardiovascular arrangement, after conventional bidirectional Glenn anastomosis, the intra-atrial curved stent-graft seemed most suitable for achieving uncomplicated TCPC completion percutaneously without previous surgical "preconditionings" in the majority of children. Experimental study is necessary to validate this conclusion., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Background: Despite advances in the Fontan procedure, there is an unmet clinical need for patient-specific graft designs that are optimized for variations in patient anatomy. The objective of this study is to design and produce patient-specific Fontan geometries, with the goal of improving hepatic flow distribution (HFD) and reducing power loss (P loss ), and manufacturing these designs by electrospinning., Methods: Cardiac magnetic resonance imaging data from patients who previously underwent a Fontan procedure (n = 2) was used to create 3-dimensional models of their native Fontan geometry using standard image segmentation and geometry reconstruction software. For each patient, alternative designs were explored in silico, including tube-shaped and bifurcated conduits, and their performance in terms of P loss and HFD probed by computational fluid dynamic (CFD) simulations. The best-performing options were then fabricated using electrospinning., Results: CFD simulations showed that the bifurcated conduit improved HFD between the left and right pulmonary arteries, whereas both types of conduits reduced P loss . In vitro testing with a flow-loop chamber supported the CFD results. The proposed designs were then successfully electrospun into tissue-engineered vascular grafts., Conclusions: Our unique virtual cardiac surgery approach has the potential to improve the quality of surgery by manufacturing patient-specific designs before surgery, that are also optimized with balanced HFD and minimal P loss , based on refinement of commercially available options for image segmentation, computer-aided design, and flow simulations., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: We evaluated the outcomes of patients who underwent extracardiac Fontan circulation procedures (ECFP) and received 16-mm polytetrafluoroethylene conduits., Methods: From June 1997 to May 2015, among the 408 patients who underwent ECFP, 66 patients (Group S) also received 16-mm polytetrafluoroethylene conduits. To compare this patient cohort with similarly sized patients who received larger conduits, a matched cohort (66 patients, Group L) was selected according to age and body weight., Results: The mean age, body weight and Nakata index at ECFP in Groups S and L were 2.9 ± 1.2 and 3.1 ± 1.2 years (P = 0.243), 13.0 ± 2.4 and 13.0 ± 2.3 kg (P = 0.101), 175.3 ± 59.0 and 236.1 ± 75.1 mm2/m2 (P = 0.005), respectively. The mean follow-up periods for Groups S and L were 7.8 ± 6.0 and 9.1 ± 4.9 years (P = 0.150), respectively. The conduit size of Group L was 19.2 ± 1.4 mm (P < 0.001). There was no significant difference in mortality between the groups (P = 0.109). The freedom from reoperation was 94.0 ± 3.4% in Group S and 79.3 ± 5.7% in Group L after 10 years (P = 0.070). Late-occurring morbidities included protein-losing enteropathy (2 in Group S, 4 in Group L; P = 0.491) and thromboembolism (0 in Group S, 4 in Group L; P = 0.206). There was a significant difference in conduit-related events between the groups (1 in Group S, 5 in Group L; P = 0.031). In a paired cohort from the 2 groups including patients who were followed-up for more than 10 years (15 patients from each group), the body mass index was 51.0 ± 33.2% in Group S and 30.3 ± 34.2% in Group L. The decreases in the conduit cross-sectional areas for the paired patients in Group S (n = 20) and Group L (n = 20) were 14.9 ± 19.7% and 24.5 ± 15.5% (P = 0.076), respectively. Coarse liver parenchyma was detected in 9 of 23 (39.1%) patients from Group S and in 7 of 18 (38.8%) patients from Group L upon ultrasonography., Conclusions: The 16-mm polytetrafluoroethylene conduit for ECFP showed acceptable outcomes and good haemodynamic status in small-sized patients. According to our results, small patients do not require large conduits to accommodate their growth potential., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Fontan completion, resulting in a total cavopulmonary connection (TCPC), is accomplished using a lateral tunnel (LT), extracardiac conduit (ECC), or recently a bifurcated Y-graft. The local energetic differences between these graft types have not been substantially analyzed under exercise conditions. The present study evaluates the energetic performance of Y-grafts under simulated exercise conditions, compares their performance to the previous LT/ECC Fontan options, and discusses implications for exercise tolerance and hemodynamic predictability. Twenty Y-graft and 20 LT/ECC patients were analyzed. TCPC anatomies and flow waveforms were reconstructed using patient-specific cardiac magnetic resonance (CMR) images and phase-contrast CMR. Computational fluid dynamics simulations were performed to quantify indexed power loss (iPL) under both resting and simulated exercise conditions. Comparisons between graft types were investigated. iPL was significantly higher (p < 0.01) for Y-grafts at all activity levels. No significant interaction effects were observed between graft type and activity level. iPL at rest was strongly correlated (r 2  = 0.97, p < 0.001) with iPL at moderate exercise for Y-grafts, but less so for the LT/ECC cohort (r 2  = 0.66, p < 0.001). Similar results were seen for intense exercise, with a strong correlation for Y-grafts (r 2  = 0.94, p < 0.001) and a moderate correlation for LT/ECC (r 2  = 0.52, p < 0.001). Commercially available Y-grafts were found to have significantly higher iPL at all activity levels, suggesting worse exercise tolerance than the LT/ECC alternatives. Y-grafts offered impressive hemodynamic predictability which was not seen in the LT/ECC cohort. Our results encourage the further evaluation of an area-preserving Y-graft design to offer both improved energetic performance and hemodynamic predictability. Commercial Y-grafts show worse energetics, but more predictable responses than traditional Fontan connections under simulated exercise conditions. During simulated exercise conditions, commercially available Y-grafts show predictable but inferior energetic performance compared to lateral tunnel and extracardiac conduit Fontan connections, suggesting poorer exercise capacity. If Y-graft use is continued, these results encourage further evaluation of a cross sectional area-preserving Y-graft design as a additional alternative for Fontan completion.

The failing Fontan physiology may benefit from ventricular assist device (VAD) mechanical circulatory support, although a subpulmonary VAD placed at the Fontan connection has never successfully supported the Fontan circulation long term. The HeartWare CircuLite continuous flow VAD was examined for Fontan circulatory support in an in vitro mock circulation. The VAD was tested in three different scenarios: VAD in parallel, baffle restricted VAD in parallel, and VAD in series. Successful support was defined as simultaneous decrease in inferior vena cava (IVC) pressure of 5 mm Hg or more and an increase in cardiac output (CO) to 4.25 L/min or greater. The VAD in parallel scenario resulted in a CO decrease to 3.46 L/min and 2.22 mm Hg decrease in IVC pressure. The baffle restricted VAD in parallel scenario resulted in a CO increase to 3.9 L/min increase in CO and 20.5 mm Hg decrease in IVC pressure (at 90% restriction). The VAD in series scenario resulted in a CO of 1.75 L/min and 5.9 mm Hg decrease in IVC pressure. We successfully modeled chronic failing Fontan physiology using patient-specific hemodynamic and anatomic data. Although unsuccessful in supporting Fontan patients as defined here, the HeartWare CircuLite VAD demonstrates the possibility to reduce Fontan pressure and increase CO with a VAD in the Fontan connection. This study provides insight into pump performance and design issues when attempting to support Fontan circulation. Refinements in VAD design with specific parameters to help support this patient population is the subject of our future work.

Background: Transcatheter stent-secured Glenn anastomosis, aiming to reduce the invasiveness of palliation in patients with univentricular heart defects, has been reported in large experimental animals. The advent of biodegradable stents and tissue-engineered vascular grafts will make this procedure a reality in human patients. However, the relationship between the superior vena cava (SVC) and the right pulmonary artery (RPA) is different in humans., Aim: To characterise vascular anatomy in children with univentricular hearts, regarding technical aspects and device design for this procedure., Methods: Retrospective analysis of 35 thoracic computed tomography angiograms at a mean age of 18.1±22.4 months., Results: Two types of arrangement between the SVC and the RPA were identified: anatomy convenient for immediate wire passage and stent deployment between the two vessels (60%); and pattern of early RPA branching, requiring the perforation wire to traverse the intervascular space to avoid entrance into the upper RPA branch (40%). In patients with the convenient vascular arrangement, the vessels were nearly perpendicular, having immediate contact, with the posterior SVC aspect partially "wrapping" the adjacent RPA in most patients. In patients with early RPA branching, the mean shortest SVC-to-central RPA distance was 4.3±2.7mm. For the total population, the mean length of proximal SVC that allowed stent deployment without covering the brachiocephalic vein was 15.6±5.1mm., Conclusions: A trumpet-shaped covered stent in a craniocaudal orientation reaching from the SVC into the prebranching RPA seems most suitable for achieving bidirectional Glenn anastomosis percutaneously in humans. However, the short length of the proximal SVC and the presence of early RPA branching pose challenges for optimal design of the dedicated device., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

The aim of this work is to develop and test a lumped parameter model of the cardiovascular system to simulate the concurrent use of pulsatile (PVAD) and continuous flow (CVAD) ventricular assist device (VAD) on Fontan patients. Echocardiographic and hemodynamic data of five Fontan patients were retrospectively collected and used to simulate the patients' baseline hemodynamics. Then, for each patient, the following assistance modality was simulated for the cavopulmonary and the single ventricle (SV): (a) CVAD for cavopulmonary assistance (RCF) and PVAD assisting the SV (LCF) (RPF + LCF), (b) CVAD assisting SV and PVAD for cavopulmonary assistance (LPF + RCF). The numerical model can well reproduce patients' baseline. The cardiac output increases more importantly in the LCF + RPF configuration (35 vs. 8%). Ventricular volume decreases more evidently in the configuration LCF + RPF (28 vs. 6%), atrial pressure decreases in the LCF + RPF modality (10%), while it slightly increases in the RCF + LPF modality. The pulmonary arterial pressure slightly decreases (increases) in the configuration RCF + LPF (LCF + RPF). Ventricular external work increases in both configurations because of the total increment of the cardiac output. However, artero-ventricular coupling improves in both configurations: RCF + LPF-14%, LCF + RPF-41%. The pulsatility index decreases (increases) by 8% (13.8%) in the configuration LCF + RPF (RCF + LPF). A model could permit us to simulate extreme physiological conditions of the implantation of both CF and PF VAD on the Fontan patient and could permit to choose the proper VAD on the base of patients' condition. The configuration LCF + RPF seems to maximize the hemodynamic benefits., (© 2016 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

Objective: Fontan completion, resulting in a total cavopulmonary connection, is accomplished using a lateral tunnel, extracardiac conduit, or bifurcated Y-graft. The use of Y-grafts is hypothesized to provide symmetric hepatic blood flow distribution to the lungs, a factor related to pulmonary arteriovenous malformations. The present study evaluates the hemodynamic performance of the largest commercially available Y-graft cohort to date, highlights 6 representative cases, and compares commercially available Y-graft performance with lateral tunnel/extracardiac conduit connections., Methods: A total of 30 patients with commercially available Y-grafts and 30 patients with lateral tunnel/extracardiac conduits were analyzed. Total cavopulmonary connection anatomies and flow waveforms were reconstructed using cardiac magnetic resonance images and phase-contrast cardiac magnetic resonance. Computational fluid dynamic simulations were performed to quantify total cavopulmonary connection power loss, resistance, and hepatic flow distribution. Comparisons between graft types were investigated., Results: Total cavopulmonary connection resistance was significantly higher for Y-grafts. Hepatic flow distribution was similar overall but showed discrepancies at extreme values with more unbalanced flow in the Y-graft cohort. Power loss was more sensitive to left pulmonary artery stenosis in the Y-graft cohort. Prediction of Y-graft hepatic flow distribution is multifactorial., Conclusions: Commercially available Y-grafts do not inherently provide more balanced hepatic flow distribution than lateral tunnel/extracardiac conduit connections, which are more energetically favorable and less sensitive to pulmonary artery stenosis. Graft type should be considered on an individual basis because hemodynamic performance is based on a combination of factors, including pulmonary flow distribution, pulmonary artery stenosis, and superior vena cava positioning., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

An intravascular axial flow pump is being developed as a mechanical cavopulmonary assist device for adolescent and adult patients with dysfunctional Fontan physiology. Coupling computational modeling with experimental evaluation of prototypic designs, this study examined the hydraulic performance of 11 impeller prototypes with blade stagger or twist angles varying from 100 to 600 degrees. A refined range of twisted blade angles between 300 and 400 degrees with 20-degree increments was then selected, and four additional geometries were constructed and hydraulically evaluated. The prototypes met performance expectations and produced 3-31 mm Hg for flow rates of 1-5 L/min for 6000-8000 rpm. A regression analysis was completed with all characteristic coefficients contributing significantly (P < 0.0001). This analysis revealed that the impeller with 400 degrees of blade twist outperformed the other designs. The findings of the numerical model for 300-degree twisted case and the experimental results deviated within approximately 20%. In an effort to simplify the impeller geometry, this work advanced the design of this intravascular cavopulmonary assist device closer to preclinical animal testing., (Copyright © 2015 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

Atrioventricular valve regurgitation is one of the predictors of adverse outcomes after the Fontan procedure. We describe our surgical technique of GoreTex (W. L. Gore & Associates, Inc., Flagstaff, AZ, USA) bridge to repair a common atrioventricular valve in single-ventricular circulation. The repair includes a GoreTex strip that is secured to the mid-line of both superior and inferior bridging leaflets and annulus to obtain a better coaptation of the leaflets and prevent further dilatation of the annulus. We have applied this technique for 7 consecutive patients with excellent outcomes., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

The Fontan procedure is one of the common surgical treatments for circulatory reconstruction in pediatric patients with congenital heart disease. In Fontan circulation, low pulsatility may induce localized lung ischemia and may impair the development of pulmonary peripheral endothelial cells. To promote pulmonary circulation in Fontan circulation, we have been developing a pediatric pulmonary circulatory pulsation device using shape memory alloy fibers attached from the outside of total cavopulmonary connection. In this study, we developed a new thermal control system for the device and examined its functions. We mounted on the device 16 fibers connected in parallel around an ePTFE graft circumferentially. To provide optimized contraction, we designed the new thermal control system. The system consisted of a thermistor, a pressure sensor, and a regulator that was controlled by the adaptive thermodynamic transfer functions. We monitored the parameters and calculated heat transfer function as well as pressure distribution on the graft surface. Then we examined and compared the dynamic contractile pressure and changes in surface temperature. As a result, by the application of the control based on the new feedback system analysis, the circumferential contractile pressure increased by 35%. The adaptive thermodynamic regulation was useful for the selection of alternative thresholds of the surface temperature of the graft. The system could achieve effective contraction for the pulsatile flow generation by the device.

Objectives: To evaluate Fontan fenestration closure using the Amplatzer Duct Occluder II device (ADO2)., Background: Fontan fenestration closure is a well established catheter intervention. A range of occlusion devices developed for other interventions have been utilized. Most of these devices, and especially the most commonly used Amplatzer Septal Occluder, are relatively bulky. The ADO2 is a low profile, flexible, and inexpensive device which seems well suited to the Fontan fenestration., Methods: This study details retrospective review of patients undergoing Fontan fenestration closure with an ADO2 device. Outcome measures included procedural success, pre- and post-procedural differences in oxygen saturation and mean pulmonary artery pressure and complications., Results: Thirteen patients were eligible for inclusion during the 34 month study period. All procedures were technically successful. There was a significant increase in oxygen saturations (Mean=+12%, P<0.01) after fenestration closure but no significant change in mean pulmonary artery pressure (Mean=+0.5 mm Hg, P=0.08). There were no procedural complications., Conclusion: Fontan fenestration closure with the ADO2 device is a simple, short, and cost-effective procedure. The ADO2 characteristics recommend it to occlusion of varying sizes and morphologies of Fontan fenestration., (© 2014 Wiley Periodicals, Inc.)

Single ventricle anomalies are a challenging set of congenital heart defects that require lifelong clinical management due to progressive decline of cardiovascular function. Few therapeutic devices are available for these patients, and conventional blood pumps are not designed for the unique anatomy of the single ventricle physiology. To address this unmet need, we are developing an axial flow blood pump with a protective cage or stent for Fontan patients. This study investigates the 3-D particle image velocimetry measurements of two cage designs being deployed in a patient-specific Fontan anatomy. We considered a control case without a pump, impeller placed in the inferior vena cava, and two cases where the impeller has two protective stents with unique geometric characteristics. The experiments were evaluated at a cardiac output of 3 L/min, a fixed vena caval flow split of 40%/60%, a fixed pulmonary arterial flow split of 50%/50%, and for operating speeds of 1000-4000 rpm. The introduction of the cardiovascular stents had a substantial impact on the flow conditions leaving the pump and entering the cavopulmonary circulation. The findings indicated that rotational speeds above 4000 rpm for this pump could result in irregular flows in this specific circulatory condition. Although retrograde flow into the superior vena cava was not measured, the risk of this occurrence increases with higher pump speeds. The against-with stent geometry outperformed the other configurations by generating higher pressures and more energetic flows. These results provide further support for the viability of mechanical cavopulmonary assistance as a therapeutic treatment strategy for Fontan patients., (Copyright © 2015 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

Despite the significant contribution of the Fontan procedure to the therapy of complex congenital heart diseases, many patients progress to failure of their Fontan circulation. The use of ventricular assist devices to provide circulatory support to these patients remains challenging. In the current study, a continuous axial-flow pump was used to support a univentricular Fontan circulation. A modified Fontan circulation (atrio-pulmonary connection) was constructed in six Yorkshire piglets (8-14 kg). A Dacron conduit (12 mm) with two branches was constructed to serve as a complete atrio-pulmonary connection without the use of cardiopulmonary bypass. The Impella pump was inserted into the conduit through an additional Polytetrafluoroethylene (PTFE) graft in five animals. Hemodynamic data were collected for 6 hours under the supported Fontan circulation. The control animal died after initiating the Fontan circulation independent of resuscitation. Four pump supported animals remained hemodynamically stable for 6 hours with pump speeds between 18,000 rpm and 22,000 rpm (P1-P3). Oxygen saturation was maintained between 95% and 100%. Normal organ perfusion was illustrated by blood gas analysis and biochemical assays. A continuous axial-flow pump can be used for temporal circulatory support to the failing Fontan circulation as "bridge" to heart transplantation or recovery.

Objectives: In this study, with the use of computational fluid dynamics, we evaluate the postoperative hemodynamic performance of the first cohort of patients undergoing a handcrafted Y-graft Fontan procedure and validate simulation predictions of hepatic blood flow distribution against in vivo clinical data., Methods: An 18-12 × 2-mm handcrafted Y-graft modification of the Fontan procedure was performed in 6 patients. Early (at the time of discharge) and 6-month postoperative 3-dimensional magnetic resonance imaging data were collected. Patient-specific models were constructed for flow simulations., Results: Hepatic blood flow distribution varied among patients. Lung perfusion data (n = 3) showed good agreement with simulations. Postoperative asymmetry in hepatic blood flow distribution was reduced 6 months postoperatively. In 1 patient, low wall shear stress was found in the left limb of the Y-graft, corresponding to the location of subsequent thrombosis in the patient., Conclusions: The credibility and accuracy of simulation-based predictions of postoperative hepatic flow distribution for the Fontan surgery have been validated by in vivo lung perfusion data. The performance of the Y-graft design is highly patient-specific. The anastomosis location is likely the most important factor influencing hepatic blood flow distribution. Although the development of thrombosis is multifactorial, the occurrence in 1 patient suggests that simulations should not solely consider the hepatic blood flow distribution but also aim to avoid low wall shear stress in the limbs., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: To demonstrate the technical feasibility and describe intermediate outcomes for the initial patients undergoing handcrafted, area-preserving, Y-graft modification of the Fontan procedure., Methods: A retrospective review of a pilot study was undertaken to describe preoperative, intraoperative, and postoperative results., Results: Six patients underwent successful procedures and remain alive 3 to 4 years later. The median age at operation was 3.3 years, and median weight was 13.2 kg. Five operations were done without cardiopulmonary bypass and no intraoperative pressure gradients were found. Five patients were extubated by postoperative day 1, Fontan pressures were 12 to 14 mm Hg, transpulmonary gradients were 6 to 8 mm Hg, and no renal or hepatic function abnormalities were found. Length of stay was 10 to 64 days. One patient required venovenous extracorporeal membrane oxygenation for previously undiagnosed plastic bronchitis (64-day stay); another required reoperation for an incidentally diagnosed aortic thrombus (44-day stay). One patient had occlusion of a Y-graft limb noted on magnetic resonance imaging follow-up at 3 months. Catheterization showed excellent hemodynamic parameters and no Fontan obstruction. Occlusion was believed to be due to right-sided pulmonary arteriovenous malformations and widely discrepant flow (80%) to the right lung leading to low flow in the left limb., Conclusions: The area-preserving, bifurcated Y-graft Fontan modification is technically feasible and shows excellent intermediate outcomes. Additional study is required to determine whether the advantages seen in the computational models will be realized in patients over the long-term, and to optimize patient selection for each of the various Fontan options now available., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Dating back to the first published report of the Fontan circulation in 1971, multiple studies have examined the long-term results of this standard procedure for palliation of single-ventricle heart disease in children. Although the technique has evolved over the last 4 decades to include a polytetrafluorethylene (PTFE) conduit for a large percentage of patients, the long-term outcome has not yet been established. The aim of the current study was to investigate the possibility of a late increasing risk for death after 15 years among patients with a modern Fontan operation and to evaluate late morbidity., Methods: Between January 1, 1988, and December 31, 2011, 207 patients underwent the Fontan procedure using an internal or external PTFE conduit plus a bidirectional cavopulmonary connection. Survival and late adverse events were analyzed. Risk factors for early and late mortality were examined using hazard function methodology., Results: At 1, 10, and 20 years, survival for the entire cohort was 95%, 88%, and 76%, respectively, with no deaths in the last 6 years of the study. Hazard modeling showed a 1.3% risk of death per year 24 years after the Fontan procedure, with no late increasing hazard phase. Freedom from reoperations was greater than 90% at 20 years and freedom from thrombotic complications was 98% at 20 years (with greater than 80% of patients on aspirin alone). Survival curves were superimposable for 16- to 20-mm conduits, and the freedom from any reoperation including transplantation was greater than 90% after 20 years. Multivariable risk factor analysis identified only earlier date of operation as a predictor of early and late mortality. By era of surgery, the 10-year predicated survival is 89% for patients undergoing surgery in 2000 and 94% for patients in 2010., Conclusions: Early and late survival after a Fontan operation with a PTFE conduit is excellent, with no late phase of increasing death risk after 20 years. Late functional status is good, the need for late reoperation is rare, and thrombotic complications are uncommon on a standard medical regimen including aspirin as the only anticoagulation medication., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Transseptal puncture (TSP) is a frequently performed procedure for gaining access to the left atrium for catheter ablation, hemodynamic assessment of the left heart, left ventricular assist device implantation, percutaneous left atrial appendage closure or mitral valvuloplasty during childhood and adulthood. The standard technique for transseptal puncture applies mechanical pressure on the fossa ovalis with a Brockenbrough needle. However, this method is not feasible when the fossa ovalis is thick and aneurysmatic. In such patients, the radiofrequency ablation energy systems can be offered as a better alternative for TSP. Here, we aimed to demonstrate the outcome of transseptal puncture performed with an NRG™ RF powered transseptal needle in three patients.

Objectives: To provide comprehensive illustrations of anatomy of the relevant vessels in large experimental animals in an interactive format as preparation for developing an effective and safe transcatheter technique of aortopulmonary and bidirectional cavopulmonary intervascular anastomoses., Methods: Computed tomographic angiographic studies in two calves and two sheep were used to prepare 3D reconstructions of the aorta, pulmonary arteries, and caval and pulmonary veins. Based on these reconstructions, computer simulations of the creation of stent-enhanced aortopulmonary and bidirectional cavopulmonary anastomoses were made., Results: We observed the following major anatomical features: (i) caudal course of the main pulmonary artery and its branches with the proximal right pulmonary artery located immediately caudal to the aortic arch, and with the central left pulmonary artery lying at a substantial distance from the descending aorta; and (ii) the distal right pulmonary artery is located dorsal to the right atrium and inferior caval vein at a substantial distance from the superior caval vein. Animations showed creation of transcatheter analogues of Waterston's and Potts' aortopulmonary shunts through placement of a covered spool-shaped stent, and the transcatheter creation of bidirectional Glenn's cavopulmonary anastomosis, by placement of a long covered trumpet-shaped stent., Conclusions: There are considerable differences in vascular anatomy between large experimental animals and humans. Given the need to elaborate new transcatheter techniques for intervascular anastomoses in suitable animal models before application to human, it is crucial to take these anatomical differences into account during testing and optimization of the proposed procedures., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: The Avalon Elite (Macquet, Rastatt, Germany) double-lumen cannula can provide effective cavopulmonary assistance in a Fontan (total cavopulmonary connection) sheep model, but it requires strict alignment. The objective was to fabricate and test a newly designed paired umbrella double-lumen cannula without alignment requirement., Methods: The paired membrane umbrellas were designed on the double-lumen cannula to bracket infusion blood flow toward the pulmonary artery. Two umbrellas were attached, one 4 cm above and one 4 cm below the infusion opening. Umbrellas were temporarily wrapped and glued to the double-lumen cannula body to facilitate insertion. A total cavopulmonary connection mock loop was used to test cavopulmonary assistance performance and reliability with double-lumen cannula rotation and displacement. The paired umbrella double-lumen cannula also was tested in a total cavopulmonary connection adult sheep model (n = 6)., Results: The bench test showed up to 4.5 L/min pumping flow and approximately 90% pumping flow efficiency at 360° rotation and 8-cm displacement of double-lumen cannula. The total cavopulmonary connection model with compromised hemodynamics was successfully created in all 6 sheep. The cavopulmonary assistance double-lumen cannula with paired umbrellas was smoothly inserted into the superior vena cava and extracardiac conduit in all sheep. At 3.5 to 4.0 L/min pump flow, the systolic arterial blood pressure and central venous pressure returned to normal baseline and remained stable throughout the 90-minute experiment, demonstrating effective cavopulmonary assistance support. Double-lumen cannula rotation and displacement did not affect performance. Autopsy revealed well-opened and positioned paired umbrellas, and double-lumen cannulas were easily removed from the right jugular vein., Conclusions: Our double-lumen cannula with paired umbrellas is easy to insert and remove. The paired umbrellas eliminated the strict alignment requirement and ensured consistent cavopulmonary assistance performance., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

A mechanical blood pump specifically designed to increase pressure in the great veins would improve hemodynamic stability in adolescent and adult Fontan patients having dysfunctional cavopulmonary circulation. This study investigates the impact of axial-flow blood pumps on pressure, flow rate, and energy augmentation in the total cavopulmonary circulation (TCPC) using a patient-specific Fontan model. The experiments were conducted for three mechanical support configurations, which included an axial-flow impeller alone in the inferior vena cava (IVC) and an impeller with one of two different protective stent designs. All of the pump configurations led to an increase in pressure generation and flow in the Fontan circuit. The increase in IVC flow was found to augment pulmonary arterial flow, having only a small impact on the pressure and flow in the superior vena cava (SVC). Retrograde flow was neither observed nor measured from the TCPC junction into the SVC. All of the pump configurations enhanced the rate of power gain of the cavopulmonary circulation by adding energy and rotational force to the fluid flow. We measured an enhancement of forward flow into the TCPC junction, reduction in IVC pressure, and only minimally increased pulmonary arterial pressure under conditions of pump support., (Copyright © 2014 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

While Fontan palliation in the form of the total cavopulmonary connection has improved the management of congenital single ventricle physiology, long-term outcomes for patients with this disease are suboptimal due to the lack of two functional ventricles. Researchers have shown that ventricular assist devices (VADs) can normalize Fontan hemodynamics. To minimize blood contacting surfaces of the VAD, we evaluated the use of an external compression device (C-Pulse Heart Assist System, Sunshine Heart Inc.) as a Fontan assist device. A mock circulation was developed to mimic the hemodynamics of a hypertensive Fontan circulation in a pediatric patient. The Sunshine C-Pulse compression cuff was coupled with polymeric valves and a compressible tube to provide nonblood-contacting pulsatile flow through the Fontan circulation. The effect of the number, one or two, and placement of valves, before or after the compression cuff, on inferior vena cava pressure (IVCP) was studied. In addition, the effect of device inflation volume and compression rate on maintaining low IVCP was investigated. With one valve located before the cuff, the device was unable to maintain an IVCP below 15.5 mm Hg. With two valves, the C-Pulse was able to maintain IVCP as low as 8.5 mm Hg. The C-Pulse provided pulsatile flow and pressure through the pulmonary branch of the mock circulation with a pulse pressure of 16 mm Hg and 180 mL/min additional flow above unassisted flow. C-Pulse compression reduced IVCP below 12 mm Hg with 13 cc inflation volume and compression rates above 105 bpm. This application of an external compression device combined with two valves has potential for use as an artificial right ventricle by maintaining low IVCP and providing pulsatile flow through the lungs., (© 2013 Wiley Periodicals, Inc. and International Center for Artificial Organs and Transplantation.)

De novo fenestration of Goretex conduit of extra-cardiac total cavopulmonary connection in the postoperative period is challenging, and is rarely reported. We report a 17-year-old boy with failing Fontan circuit in whom fenestration was created, aided by an Inoue balloon., (Copyright © 2012 Wiley Periodicals, Inc.)

Objective: The objective of this study was to create a novel animal model to foster the future development of interventional techniques for a cavocaval connection that mimics a Fontan completion., Methods: Ten sheep were studied. All had the superior vena cava-right atrium junction closed using a polytetrafluoroethylene membrane. A valveless Gore-Tex conduit connecting the terminal portion of the superior vena cava to the right atrium was used to bypass the polytetrafluoroethylene occlusion and to allow normal venous drainage through the right atrium. Radio-opaque nitinol rings were placed around the inferior vena cava near its entry in the right atrium to allow better fluoroscopic visualization and to enhance stent stability during transcatheter cavocaval connection., Results: The first 3 animals died during the learning curve as a result of technical issues. The subsequent 7 surviving sheep showed good flow dynamics on cardiac catheterization. Transcatheter cavocaval connection was performed successfully performed in 6 sheep at 0 to 9 months after the initial surgery. Sacrifice was done electively in all animals at 1 to 9 months per protocol. One animal was euthanized early because of an untreatable infection. One sheep was observed as a control without a transcatheter cavocaval connection and was sacrificed at 12 months., Conclusions: A novel, chronic ovine model to foster development of transcatheter techniques for cavocaval connection to mimic a Fontan completion was created. The successful model is easily reproducible after a short learning curve and shows good survival., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

A blood pump specifically designed to augment flow from the great veins through the lungs would ameliorate the poor physiology of the failing univentricular circulation and result in a paradigm shift in the treatment strategy for Fontan patients. This study is the first to examine mechanical cavopulmonary assistance with a blood pump in the inferior vena cava (IVC) and hepatic blood flow. Five numerical models of mechanical cavopulmonary assistance were investigated using a three-dimensional, reconstructed, patient-specific Fontan circulation from magnetic resonance imaging data. Pressure flow characteristics of the axial blood pump, energy augmentation calculations for the cavopulmonary circulation with and without pump support, and hemolysis estimations were determined. In all of the pump-supported scenarios, a pressure increase of 7-9.5 mm Hg was achieved. The fluid power of the cavopulmonary circulation was also positive over the range of flow rates. No retrograde flow from the IVC into the hepatic circulation was evident during support cases. Vessel suction risk, however, was found for greater operating rotational speeds. Fluid shear stresses and hemolysis predictions remained at acceptable levels with normalized index of hemolysis estimations at 0.0001 g/100 L. The findings of this study support the continued design and development of this blood pump technology for Fontan patients with progressive cardiovascular insufficiency. Validation of these flow and performance predictions will be completed in the next round of experimental testing with blood bag evaluation.

Objectives: The total cavopulmonary connection (TCPC), the current palliation of choice for single-ventricle heart defects, is typically created with a single cylindrical tunnel or conduit routing inferior vena caval (IVC) flow to the pulmonary arteries. Previous studies have shown the haemodynamic efficiency of the TCPC to be sub-optimal due to the collision of vena caval flow, thus placing an extra energy burden on the single ventricle. The use of a bifurcated graft as the Fontan baffle (i.e. the 'Optiflo') has previously been proposed on the basis of theoretically improved flow efficiency; however, anatomical constraints may limit its effectiveness in some patients., Methods: In this study, an alternative approach to flow bifurcation is proposed, where a triangular insert is placed at the distal end of the IVC graft. The proof of concept for this design is demonstrated in two steps: first, determining the optimal insert size at a fixed Fontan graft size through a parametric study; then, characterizing the efficiency as a function of graft size when compared with a TCPC control. TCPC power loss and IVC flow distribution were the primary metrics of interest and were evaluated under both resting and simulated exercise conditions using an in-house computational fluid dynamics solver., Results: Results demonstrated that there was an optimal insert size that improved efficiency compared with the TCPC. For an 18-mm Fontan baffle, TCPC power loss was 4.1 vs 3.7 mW with the optimal flow-divider. The optimal insert was then scaled up for a 20-mm graft, with a similar reduction in power loss observed. Flow distribution results were inconsistent, based on sensitivity to the placement of the insert within the baffle., Conclusion: This study demonstrated proof of concept that the flow-divider has the potential to reduce power loss and streamline IVC flow through the TCPC. An appropriate size for the insert in proportion to the Fontan baffle size was identified that reduced losses compared with a TCPC control under both resting and simulated exercise flow conditions.

The Fontan circulation was originally described as palliation for tricuspid atresia but was subsequently used for a wide variety of single-ventricle physiologies, with the common thread being inability to separate the ventricles for independent use in the systemic and pulmonary circulations. Fontan baffle fenestrations have been created since the late 1980s to assist in maintaining the cardiac output in the presence of increased resistance to flow through the pulmonary circuit. Many institutions continue to use an approach to Fontan circulation that utilizes routine fenestration followed by percutaneous device closure at a later time, whereas other institutions use fenestrations sparingly. We present the case of a five-year-old female with hypoplastic left heart syndrome with a fenestrated Fontan, who underwent three-dimensional rotational angiography at the time of catheterization for percutaneous device closure of the fenestration.

Objectives: Single ventricle with apicocaval juxtaposition (ACJ) is a rare, complex anomaly, in which the optimal position of the conduit for completion of total cavopulmonary connection (TCPC) is still controversial. The purpose of this study was to identify a preoperative method for optimal conduit position using the IVC anatomy and computational fluid dynamics (CFD)., Methods: Twenty-four patients with ACJ (5.3 ± 5.7 years) who underwent TCPC were enrolled. A conduit was placed ipsilateral to the cardiac apex in each of 11 patients, of which 9 were intra-atrial and 2 extracardiac (group A) and, in a further 13 patients, extracardiac on the contralateral side (group B). As control, 10 patients with tricuspid atresia were also enrolled (group C). The location of the IVC in relation to the spine was evaluated from the frontal view of preoperative angiogram, using the following index: IVC-index = IVC width overlapping the vertebra/width of the vertebra × 100%. Energy loss was calculated by CFD simulation., Results: IVC-index of group B was larger than groups A and C (45 ± 26 vs. 20 ± 21 and 28 ± 19%, P = 0.03). Postoperative catheterizations showed that, due to its curvature, conduit length in group B was significantly longer than the others (65 ± 12 vs. 36 ± 14 and 44 ± 10 mm, P < 0.001), although there was no statistical difference in central venous pressure or cardiac output. CFD studies revealed less energy loss in group A conduits compared with group B (1.6 ± 0.3 vs. 3.6 ± 0.6 mW, P = 0.05), although this did not appear to be clinically significant. Moreover, CFD simulation showed significant energy loss within the Fontan circulation when the conduit was either compressed or kinked: 4.9 and 18.2 mW respectively., Conclusions: In patients with ACJ, placement of a straighter and shorter conduit on the ventricular apical side provides better laminar blood flow with less energy loss. However, conduit compression and kinking are far more detrimental to the Fontan circulation. A preoperative IVC-index is pivotal for avoiding these factors and deciding the optimal conduit route.

The implementation of simultaneous mechanical cavopulmonary assistance having blood pumps located in both of the vena cavae is investigated as an approach to treating patients with an ailing Fontan physiology. Identical intravascular blood pumps are employed to model the hemodynamic support of a patient-specific Fontan. Pressure flow characteristics, energy gain calculations, and blood damage analyses are assessed for each model. The performance of the dual-support scenario is compared to conditions of mechanical support in the inferior vena cava only and to a nonsupported cavopulmonary circuit. The blood pump in the superior vena cava generates pressures ranging from 1 to 22 mm Hg for flow rates of 1-4 L/min at operating speeds of 1250-2500 rpm. The blood pump in the inferior vena cava produces pressures at levels approximately 20% lower. The blood pumps positively augment the hydraulic energy in the total cavopulmonary connection circuit as a function of flow rate and rotational speed. Scalar stress levels and fluid residence times are at acceptable levels. Damage indices for the dual-support case, however, are elevated slightly above 3.5%. These results suggest that concurrent, mechanical assistance of the inferior vena cava and superior vena cava in Fontan patients has the potential to be beneficial, but additional studies are needed to further explore this approach., (© 2013, Copyright the Authors. Artificial Organs © 2013, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.)

Background: Using a bifurcated Y-graft as the Fontan baffle is hypothesized to streamline and improve flow dynamics through the total cavopulmonary connection (TCPC). This study conducted numerical simulations to evaluate this hypothesis using postoperative data from 5 patients., Methods: Patients were imaged with cardiac magnetic resonance or computed tomography after receiving a bifurcated aorto-iliac Y-graft as their Fontan conduit. Numerical simulations were performed using in vivo flow rates, as well as 2 levels of simulated exercise. Two TCPC models were virtually created for each patient to serve as the basis for hemodynamic comparison. Comparative metrics included connection flow resistance and inferior vena caval flow distribution., Results: Results demonstrate good hemodynamic outcomes for the Y-graft options. The consistency of inferior vena caval flow distribution was improved over TCPC controls, whereas the connection resistances were generally no different from the TCPC values, except for 1 case in which there was a marked improvement under both resting and exercise conditions. Examination of the connection hemodynamics as they relate to surgical Y-graft implementation identified critical strategies and modifications that are needed to potentially realize the theoretical efficiency of such bifurcated connection designs., Conclusions: Five consecutive patients received a Y-graft connection to complete their Fontan procedure with positive hemodynamic results. Refining the surgical technique for implementation should result in further energetic improvements that may help improve long-term outcomes., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Children born with univentricular heart disease typically must undergo three open heart surgeries within the first 2-3 years of life to eventually establish the Fontan circulation. In that case the single working ventricle pumps oxygenated blood to the body and blood returns to the lungs flowing passively through the Total Cavopulmonary Connection (TCPC) rather than being actively pumped by a subpulmonary ventricle. The TCPC is a direct surgical connection between the superior and inferior vena cava and the left and right pulmonary arteries. We have postulated that a mechanical pump inserted into this circulation providing a 3-5 mmHg pressure augmentation will reestablish bi-ventricular physiology serving as a bridge-to-recovery, bridge-to-transplant or destination therapy as a "biventricular Fontan" circulation. The Viscous Impeller Pump (VIP) has been proposed by our group as such an assist device. It is situated in the center of the 4-way TCPC intersection and spins pulling blood from the vena cavae and pushing it into the pulmonary arteries. We hypothesized that Large Eddy Simulation (LES) using high-order numerical methods are needed to capture unsteady powered and unpowered Fontan hemodynamics. Inclusion of a mechanical pump into the CFD further complicates matters due to the need to account for rotating machinery. In this study, we focus on predictions from an in-house high-order LES code (WenoHemo(TM)) for unpowered and VIP-powered idealized TCPC hemodynamics with quantitative comparisons to Stereoscopic Particle Imaging Velocimetry (SPIV) measurements. Results are presented for both instantaneous flow structures and statistical data. Simulations show good qualitative and quantitative agreement with measured data., (Copyright © 2012 Elsevier Ltd. All rights reserved.)