Transcatheter Repair for Patients with Tricuspid Regurgitation.

BACKGROUND: Severe tricuspid regurgitation is a debilitating condition that is associated with substantial morbidity and often with poor quality of life. Decreasing tricuspid regurgitation may reduce symptoms and improve clinical outcomes in patients with this disease. METHODS: We conducted a prospective randomized trial of percutaneous tricuspid transcatheter edge-to-edge repair (TEER) for severe tricuspid regurgitation. Patients with symptomatic severe tricuspid regurgitation were enrolled at 65 centers in the United States, Canada, and Europe and were randomly assigned in a 1:1 ratio to receive either TEER or medical therapy (control). The primary end point was a hierarchical composite that included death from any cause or tricuspid-valve surgery; hospitalization for heart failure; and an improvement in quality of life as measured with the Kansas City Cardiomyopathy Questionnaire (KCCQ), with an improvement defined as an increase of at least 15 points in the KCCQ score (range, 0 to 100, with higher scores indicating better quality of life) at the 1-year follow-up. The severity of tricuspid regurgitation and safety were also assessed. RESULTS: A total of 350 patients were enrolled; 175 were assigned to each group. The mean age of the patients was 78 years, and 54.9% were women. The results for the primary end point favored the TEER group (win ratio, 1.48; 95% confidence interval, 1.06 to 2.13; P = 0.02). The incidence of death or tricuspid-valve surgery and the rate of hospitalization for heart failure did not appear to differ between the groups. The KCCQ quality-of-life score changed by a mean (±SD) of 12.3±1.8 points in the TEER group, as compared with 0.6±1.8 points in the control group (P<0.001). At 30 days, 87.0% of the patients in the TEER group and 4.8% of those in the control group had tricuspid regurgitation of no greater than moderate severity (P<0.001). TEER was found to be safe; 98.3% of the patients who underwent the procedure were free from major adverse events at 30 days. CONCLUSIONS: Tricuspid TEER was safe for patients with severe tricuspid regurgitation, reduced the severity of tricuspid regurgitation, and was associated with an improvement in quality of life. (Funded by Abbott; TRILUMINATE Pivotal ClinicalTrials.gov number, NCT03904147.).

Comparison of Fontan Surgical Options for Patients with Apicocaval Juxtaposition.

Apicocaval juxtaposition (ACJ) is a rare form of viscerocardiac malpositions in association with single-ventricle congenital heart defects. The Fontan surgery is the common palliation, and possible surgical options include ipsilateral, contralateral, and intra-atrial conduits. Concerns include lower hemodynamic performances or risks of conduit compression by the cardiac mass. This study investigates the hemodynamics and clinical outcomes of ACJ patients and potential surgical improvements. Ten consecutive ACJ patients were included, along with a reference cohort of ten non-ACJ patients. Magnetic resonance images were acquired at 6 ± 0.6 year follow-up for anatomical analysis and hemodynamic assessments using computational fluid dynamics. Metrics of interest are deformation index (DI), indexed power loss (iPL), and hepatic flow distribution (HFDoff). A "virtual" surgery was performed to explore potential hemodynamic improvements using a straightened conduit. DI for ACJ patients fell within the DI range of non-ACJ patients. Contralateral conduits had insignificantly higher iPL (0.070 [0.032,0.137]) than ipsilateral conduits (0.041 [0.013,0.095]) and non-ACJ conduits (0.034 [0.011,0.061]). HFDoff was similar for the ipsilateral (21 [12,35]), contralateral (26 [7,41]), and non-ACJ Fontan conduits (17 [0,48]). Virtual surgery demonstrated that a straightened conduit reduced HFDoff and iPL for the contralateral and ipsilateral conduits, potentially leading to improved clinical outcomes. In this limited sample, the hemodynamic performance of ACJ patients was not significantly different from their non-ACJ counterparts. The use of a straightened conduit option could potentially improve patient outcomes. Additionally, the fear of significant compression of conduits for ACJ patients was unsupported.

In Vitro Examination of the VentriFlo True Pulse Pump for Failing Fontan Support.

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.

Characterization of Tricuspid Valve Anatomy and Coaptation Gap in Subjects Receiving Tricuspid Transcatheter Edge-To-Edge Repair: Observations From the bRIGHT TriClip Study.

BACKGROUND: Transcatheter edge-to-edge repair (TEER) for the treatment of tricuspid regurgitation (TR) has experienced fast adoption following commercial approval. Defining the appropriate target population for TEER therapy is important to guide patient selection. The aim of this study was to characterize tricuspid valve anatomy and coaptation gap in subjects receiving TEER for the treatment of TR in a contemporary postmarket setting. METHODS: The bRIGHT study is a prospective, multicenter, single-arm, postmarket study evaluating the safety and effectiveness of the TriClip device. Procedural outcomes included implant success, acute procedural success, TR severity, major adverse events, single-leaflet device attachment, and embolization through 30 postprocedure days. Tricuspid valve characteristics, including morphology, annulus size, and leaflet mobility, were assessed via two-dimensional transesophageal echocardiography from the screening visit by an independent echo core lab to characterize subject variability. Coaptation gap measurements were taken in both the transgastric short-axis (TG SAX) and RV inflow/outflow views. RESULTS: The independent echo core lab performed a detailed assessment of the tricuspid valve on 135 consecutive subjects with available TG SAX views from 24 sites. Tricuspid valve morphologies included 2 to 5 leaflets, with a non-trileaflet valve in 28% of subjects and ≥4 leaflets in 21% of subjects. The etiology of TR was functional in 91% (96/105), mixed in 7% (7/105), and lead induced in 2% (2/105) of subjects. Leaflet mobility was mildly restricted in 69% (78/113) and moderately restricted in 7% (8/113) of subjects. Annulus diameter averaged 4.7 ± 0.7 cm with a range of 2.5 to 6.2 cm. From the TG SAX view, the coaptation gap measured 8.1 ± 3.1 and 5.2 ± 2.3 mm in the central and mid regions of the anterior-septal coaptation line and 6.6 ± 3.2 and 3.8 ± 2.1 mm in the central and mid regions of the septal-posterior coaptation line, respectively. From the right ventricular inflow/outflow view, the coaptation gap measured 4.7 ± 2.4, 5.2 ± 2.4, and 4.6 ± 3.0 mm in the anterior, mid, and posterior regions of the tricuspid valve, respectively. Thirty-day TR reduction (by number of grades) was similar among subjects with coaptation gaps of <7 mm, 7 to 10 mm, and >10 mm. CONCLUSION: A broad range of anatomies was observed in this postmarket population. Characterization of the tricuspid valve and coaptation gap will help to better understand and better define the target patient population for tricuspid TEER therapy.

Long-term durability of a new surgical aortic valve: A 1 billion cycle in vitro study.

Background: This study assessed the long-term hemodynamic functional performance of the new Inspiris Resilia aortic valve after accelerated wear testing (AWT). Methods: Three 21-mm and 23-mm Inspiris valves were used for the AWT procedure. After 1 billion cycles (equivalent to 25 years), the valves' hemodynamic performance was compared with that of the corresponding zero-cycled condition. Next, 1 AWT cycled valve of each valve size was selected at random for particle image velocimetry (PIV) and leaflet kinematic tests, and the data were compared with data for an uncycled Inspiris Resilia aortic valve of the same size. PIV was used to quantitatively evaluate flow fields downstream of the valve. Valves were tested according to International Standards Organization 5840-2:2015 protocols. Results: The 21-mm and 23-mm valves met the International Organization for Standardization (ISO) durability performance requirements to 1 billion cycles. The mean effective orifice areas for the 21-mm and 23-mm zero-cycled and 1 billion-cycled valves were 1.89 ± 0.02 cm2 and 1.94 ± 0.01 cm2, respectively (P < .05) and 2.3 ± 0.13 cm2 and 2.40 ± 0.11 cm2, respectively (P < .05). Flow characterization of the control valves and the study valves demonstrated similar flow characteristics. The velocity and shear stress fields were also similar in the control and study valves. Conclusions: The Inspiris Resilia aortic valve demonstrated very good durability and hemodynamic performance after an equivalent of 25 years of simulated in vitro accelerated wear. The study valves exceeded 1 billion cycles of simulated wear, 5 times longer than the standard requirement for a tissue valve as stipulated in ISO 5840-2:2015.

Fontan Geometry and Hemodynamics Are Associated With Quality of Life in Adolescents and Young Adults.

BACKGROUND: Despite favorable short-term outcomes, Fontan palliation is associated with comorbidities and diminished quality of life (QOL) in the years after completion. We hypothesized that poor Fontan hemodynamics and ventricular function are associated with worse QOL. METHODS: This was a single-center study of Fontan survivors aged more than 12 years. Subjects completed a cardiac magnetic resonance scan and QOL questionnaire. Cardiac magnetic resonance-derived variables included Fontan geometry, and hemodynamics. Computational fluid dynamics simulations quantified power loss, pressure drop, and total cavopulmonary connection resistance across the Fontan. Quality of life was assessed by completion of the Pediatric Quality of Life Inventory. Longitudinal and cross-sectional comparisons were made between cardiac magnetic resonance and computational fluid dynamics parameters with patient-reported QOL. RESULTS: We studied 77 Fontan patients, median age 19.7 years (interquartile range, 17.1 to 23.6), median time from Fontan completion 16 years (interquartile range, 13 to 20). Longitudinal data were available for 48 patients; median time between cardiac magnetic resonance and QOL was 8.1 years (interquartile range, 7 to 9.4). Median patient-reported Pediatric Quality of Life Inventory total score was 80 (interquartile range, 67.4 to 88). Greater power loss and smaller left pulmonary artery diameter at baseline were associated with worse QOL at follow-up. Greater pressure drop was associated with worse QOL at the same time point. CONCLUSIONS: For Fontan survivors, measures of computational fluid dynamics hemodynamics and geometry are associated with worse QOL. Interventional strategies targeted at optimizing the Fontan may improve QOL.

The role of flow stasis in transcatheter aortic valve leaflet thrombosis.

OBJECTIVE: With the recent expanded indication for transcatheter aortic valve replacement to low-risk surgical patients, thrombus formation in the neosinus is of particular interest due to concerns of reduced leaflet motion and long-term transcatheter heart valve durability. Although flow stasis likely plays a role, a direct connection between neosinus flow stasis and thrombus severity is yet to be established. METHODS: Patients (n = 23) were selected to minimize potential confounding factors related to thrombus formation. Patient-specific 3-dimensional reconstructed in vitro models were created to replicate in vivo anatomy and valve deployment using the patient-specific cardiac output and idealized coronary flows. Dye was injected into each neosinus to quantify washout time as a measure of flow stasis. RESULTS: Flow stasis (washout time) showed a significant, positive correlation with thrombus volume in the neosinus (rho = 0.621, P < .0001). Neither thrombus volume nor washout time was significantly different in the left, right, and noncoronary neosinuses (P ≥ .54). CONCLUSIONS: This is the first patient-specific study correlating flow stasis with thrombus volume in the neosinus post-transcatheter aortic valve replacement across multiple valve types and sizes. Neosinus-specific factors create hemodynamic and thrombotic variability within individual patients. Measurement of neosinus flow stasis may guide strategies to improve outcomes in transcatheter aortic valve replacement.

Transcatheter Edge-to-Edge Repair for Treatment of Tricuspid Regurgitation.

BACKGROUND: Tricuspid regurgitation (TR) is a frequent disease with a progressive increase in mortality as disease severity increases. Transcatheter therapies for treatment of TR may offer a safe and effective alternative to surgery in this high-risk population. OBJECTIVES: The purpose of this report was to study the 1-year outcomes with the TriClip transcatheter tricuspid valve repair system, including repair durability, clinical benefit and safety. METHODS: The TRILUMINATE trial (n = 85) is an international, prospective, single arm, multicenter study investigating safety and performance of the TriClip Tricuspid Valve Repair System in patients with moderate or greater TR. Echocardiographic assessment was performed by a core laboratory. RESULTS: At 1 year, TR was reduced to moderate or less in 71% of subjects compared with 8% at baseline (p < 0.0001). Patients experienced significant clinical improvements in New York Heart Association (NYHA) functional class I/II (31% to 83%, p < 0.0001), 6-minute walk test (272.3 ± 15.6 to 303.2 ± 15.6 meters, p = 0.0023) and Kansas City Cardiomyopathy Questionnaire (KCCQ) score (improvement of 20 ± 2.61 points, p < 0.0001). Significant reverse right ventricular remodeling was observed in terms of size and function. The overall major adverse event rate and all-cause mortality were both 7.1% at 1 year. CONCLUSION: Transcatheter tricuspid valve repair using the TriClip device was found to be safe and effective in patients with moderate or greater TR. The repair itself was durable at reducing TR at 1 year and was associated with a sustained and marked clinical benefit with low mortality after 1 year in a fragile population that was at high surgical risk. (TRILUMINATE Study With Abbott Transcatheter Clip Repair System in Patients With Moderate or Greater TR; NCT03227757).

Target Flow-Pressure Operating Range for Designing a Failing Fontan Cavopulmonary Support Device.

Fontan operation as the current standard of care for the palliation of single ventricle defects results in significant late complications. Using a mechanical circulatory device for the right circulation to serve the function of the missing subpulmonary ventricle could potentially stabilize the failing Fontan circulation. This study aims to elucidate the hydraulic operating regions that should be targeted for designing cavopulmonary blood pumps. By integrating numerical analysis and available clinical information, the interaction of the cavopulmonary support via the IVC and full assist configurations with a wide range of simulated adult failing scenarios was investigated; with IVC and full assist corresponding to the inferior venous return or the entire venous return, respectively, being routed through the device. We identified the desired hydraulic operating regions for a cavopulmonary assist device by clustering all head pressures and corresponding pump flows that result in hemodynamic improvement for each simulated failing Fontan physiology. Results show that IVC support can produce beneficial hemodynamics in only a small fraction of failing Fontan scenarios. Cavopulmonary assist device could increase cardiac index by 35% and decrease the inferior vena cava pressure by 45% depending on the patient's pre-support hemodynamic state and surgical configuration of the cavopulmonary assist device (IVC or full support). The desired flow-pressure operating regions we identified can serve as the performance criteria for designing cavopulmonary assist devices as well as evaluating off-label use of commercially available left-side blood pumps for failing Fontan cavopulmonary support.

Computational modeling of a right-sided Fontan assist device: Effectiveness across patient anatomies and cannulations.

The use of mechanical circulatory support for failing Fontan patients is an area of growing interest, as the increased life expectancy of these patients continues to be accompanied by numerous end-organ complications. In vitro work has shown positive results using the CentriMag device for right-sided Fontan support, however the generalizability across various patient anatomies and cannulations is unknown. Computational simulations are first validated against in vitro modeling, then used to assess generalizability and further explore hemodynamic metrics including relative pressure changes, hepatic flow distribution, wall shear stress and power added. Computational modeling matched previous in vitro work very well, with vessel flow rates and relative average pressure change each within 1%. Positive results were seen across all patient anatomies and cannulations. On average, pressure from the vena cava to pulmonary arteries increased by 5.4 mmHg corresponding to 32 mW of power added. Hepatic flow distribution and wall shear stress were within acceptable ranges, with an average hepatic flow distribution of 47% and all patients showing ≤ 1% of the total Fontan connection surface area at a wall shear stress above 150 Pa. The positive results previously seen using CentriMag as a right-sided Fontan support device were found to be repeatable across multiple patient anatomies and cannulations. While animal models and eventual patient studies will provide further insight into the efficacy of this support strategy, our findings here suggest this method may reproduce right heart function.

Non-Newtonian Effects on Patient-Specific Modeling of Fontan Hemodynamics.

The Fontan procedure is a common palliative surgery for congenital single ventricle patients. In silico and in vitro patient-specific modeling approaches are widely utilized to investigate potential improvements of Fontan hemodynamics that are related to long-term complications. However, there is a lack of consensus regarding the use of non-Newtonian rheology, warranting a systematic investigation. This study conducted in silico patient-specific modeling for twelve Fontan patients, using a Newtonian and a non-Newtonian model for each patient. Differences were quantified by examining clinically relevant metrics: indexed power loss (iPL), indexed viscous dissipation rate (iVDR), hepatic flow distribution (HFD), and regions of low wall shear stress (AWSS). Four sets of "non-Newtonian importance factors" were calculated to explore their effectiveness in identifying the non-Newtonian effect. No statistical differences were observed in iPL, iVDR, and HFD between the two models at the population-level, but large inter-patient variations exist. Significant differences were detected regarding AWSS, and its correlations with non-Newtonian importance factors were discussed. Additionally, simulations using the non-Newtonian model were computationally faster than those using the Newtonian model. These findings distinguish good importance factors for identifying non-Newtonian rheology and encourage the use of a non-Newtonian model to assess Fontan hemodynamics.

Y-graft modification to the Fontan procedure: Increasingly balanced flow over time.

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.

Cross-Sectional Magnetic Resonance and Modeling Comparison From Just After Fontan to the Teen Years.

BACKGROUND: Little is known of baseline anatomic, hemodynamic, and fluid dynamic cardiac magnetic resonance data in single-ventricle patients immediately after Fontan. A comparison from that time point to the teen years can demonstrate clinical course, potentially predict future events, and may shed some light regarding how to optimize outcome. This cross-sectional study is meant to characterize these variables from just after Fontan to the teenage years. METHODS: The anatomy, flows, and computational fluid dynamic modeling of 22 patients 3 to 9 months after Fontan (age 3 ± 1.1 years) and 25 teens (age 16 ± 1.8 years) were compared. Significance was defined as P less than .05. RESULTS: The percentage of Fontan pathway stenosis was greater with cardiac index and fenestration flow while caval return was lower in teens than in younger patients (for Fontan pathway stenosis, 43% vs 21%, P = .009); however, hepatic flow distribution was more evenly distributed in older patients. Pulmonary artery size kept up with somatic growth. In the teen group, indexed power loss (R = .39), percentage of Fontan pathway stenosis (R = .62), and particle resident time (R = .42) deteriorated as time from Fontan increased (P < .04 for all). CONCLUSIONS: There are mostly aspects of deterioration with a few bright spots of stability in anatomy, blood flow, and fluid dynamic variables in Fontan patients from the postoperative period to the teenage years. Most notably, Fontan pathway stenosis increases with decreasing flows while pulmonary artery size and hepatic flow distribution remain stable or improved. These data may be aid in designing improved Fontan reconstruction to optimize clinical outcome and to understand future complications.

Impact of Free-Breathing Phase-Contrast MRI on Decision-Making in Fontan Surgical Planning.

Fontan surgical planning ranks proposed surgical options according to their hemodynamics assessed by computational fluid dynamic (CFD) modeling. CFD commonly utilizes blood flow acquired under breath-holding (BH) conditions. Ignoring the free-breathing (FB) effect on blood flow waveforms may impact the ranking of surgical options. This study investigates such a potential impact by including ten Fontan patients who had blood flow acquisitions under both BH and FB conditions. A virtual surgery platform was used to generate two surgical options for each patient: (1) a traditional Fontan conduit and (2) a Y-graft. These options were ranked based on clinically relevant hemodynamic metrics: power loss (PL) and hepatic flow distribution (HFD). No difference was found in the ranking of options between using FB and BH flow acquisitions. The findings indicated that decision-making is not affected by the types of flow acquisition for Fontan surgical planning.

Cardiac Magnetic Resonance-Derived Metrics Are Predictive of Liver Fibrosis in Fontan Patients.

BACKGROUND: Liver fibrosis is a serious complication of single ventricle Fontan survivors. Its causes are of great interest, and potential solutions to halt or delay progression are needed. The purpose of this study is to investigate if prior hemodynamics and anatomy can predict liver fibrosis severity in these patients. METHODS: Twenty-one Fontan patients with cardiac magnetic resonance (CMR) data obtained greater than 1 year before liver biopsy data were included. Computational fluid dynamic simulations were performed to quantify total cavopulmonary connection (TCPC) flow dynamics using patient-specific anatomies and blood flow waveforms reconstructed from CMR data. Collagen deposition (a measure of liver fibrosis) was quantified by digital image analysis of Sirius red-stained slides. Statistical analyses were performed to investigate potential relationships between Fontan hemodynamics and liver fibrosis. RESULTS: With an average time of 6.7 ± 2.9 years (range, 2-11 years) between CMR and biopsy, TCPC resistance and left pulmonary artery stenosis showed significant, positive correlations with magnitude of liver fibrosis (r = 0.54, P = .026; and r = 0.55, P = .028, respectively). The change in inferior vena cava flow rate over time also showed a significant positive correlation with magnitude of liver fibrosis (r = 0.91, P = .001). CONCLUSIONS: TCPC resistance, left pulmonary artery stenosis, and increased inferior vena cava flow are positively associated with liver fibrosis after Fontan operation and hold promise as important predictors of hepatic decline. These findings encourage preprocedural planning and interventional strategies to improve TCPC performance and reduce vessel stenosis. Further investigation is warranted to design the ideal Fontan circulation and optimize flow dynamics to reduce the risk of liver fibrosis.

Analysis of Inlet Velocity Profiles in Numerical Assessment of Fontan Hemodynamics.

Computational fluid dynamic (CFD) simulations are widely utilized to assess Fontan hemodynamics that are related to long-term complications. No previous studies have systemically investigated the effects of using different inlet velocity profiles in Fontan simulations. This study implements real, patient-specific velocity profiles for numerical assessment of Fontan hemodynamics using CFD simulations. Four additional, artificial velocity profiles were used for comparison: (1) flat, (2) parabolic, (3) Womersley, and (4) parabolic with inlet extensions [to develop flow before entering the total cavopulmonary connection (TCPC)]. The differences arising from the five velocity profiles, as well as discrepancies between the real and each of the artificial velocity profiles, were quantified by examining clinically important metrics in TCPC hemodynamics: power loss (PL), viscous dissipation rate (VDR), hepatic flow distribution, and regions of low wall shear stress. Statistically significant differences were observed in PL and VDR between simulations using real and flat velocity profiles, but differences between those using real velocity profiles and the other three artificial profiles did not reach statistical significance. These conclusions suggest that the artificial velocity profiles (2)-(4) are acceptable surrogates for real velocity profiles in Fontan simulations, but parabolic profiles are recommended because of their low computational demands and prevalent applicability.

An in vitro analysis of the PediMag and CentriMag for right-sided failing Fontan support.

OBJECTIVE: Right-sided mechanical circulatory support for failing Fontan physiology has been largely unsuccessful due to inherent hemodynamic differences between these patients and the target populations for most assist devices. This study uses advanced benchtop modeling of Fontan physiology to examine the use of PediMag and CentriMag to improve failing Fontan hemodynamics. METHODS: Each device was attached to a compliance-matched, patient-specific total cavopulmonary connection in vitro model that used resistances, compliances, and programmable waveforms to establish "failing Fontan" baseline hemodynamics (cardiac output [CO] = 3.5 L/min and central venous pressure ∼17 mm Hg). The ability of the assist devices to improve failing Fontan hemodynamics (reduce inferior vena cava pressure and augment CO) was investigated. RESULTS: Requiring complete Fontan pathway restriction, PediMag reduced inferior vena cava pressure by ∼10 mm Hg and supported CO augmentation up to 5 L/min. This was accompanied by an increase in superior vena cava pressure of ∼6 mm Hg. CentriMag produced similar hemodynamic changes without the need for pathway restriction or an increase in superior vena cava pressure. CONCLUSIONS: PediMag and CentriMag right-sided support led to a decrease in inferior vena cava pressure and augmentation of cardiac output. In the case of CentriMag, this is accomplished without an increase in superior vena cava pressure or the need for restrictive banding. This work provides further data to help with the optimal design of a Fontan assist device to ameliorate the growing need.

The effect of respiration-driven flow waveforms on hemodynamic metrics used in Fontan surgical planning.

OBJECTIVE: Poor total cavopulmonary connection (TCPC) hemodynamics have been hypothesized to be associated with long-term complications in Fontan patients. Image-based Fontan surgical planning has shown great potential as a clinical tool because it can pre-operatively evaluate patient-specific hemodynamics. Current surgical planning paradigms commonly utilize cardiac-gated phase contrast magnetic resonance (MR) imaging to acquire vessel flows. These acquisitions are often taken under breath-held (BH) conditions and ignore the effect of respiration on blood flow waveforms. This study investigates the effect of respiration-driven flow waveforms on patient-specific hemodynamics using real-time MR acquisitions. METHODS: Patient-specific TCPCs were reconstructed from cardiovascular MR images. Real-time phase contrast MR images were acquired under both free-breathing (FB) and breath-held conditions for 9 patients. Numerical simulations were employed to assess flow structures and hemodynamics used in Fontan surgical planning including hepatic flow distribution (HFD) and indexed power loss (iPL), which were then compared between FB and BH conditions. RESULTS: Differences in TCPC flow structures between FB and BH conditions were observed throughout the respiratory cycle. However, the average differences (BH - FB values for each patient, which are then averaged) in iPL and HFD between these conditions were 0.002 ±â€¯0.011 (p = 0.40) and 1 ±â€¯3% (p = 0.28), respectively, indicating no significant difference in clinically important hemodynamic metrics. CONCLUSIONS: Respiration affects blood flow waveforms and flow structures, but might not significantly influence the values of iPL or HFD. Therefore, breath-held MR acquisition can be adequate for Fontan surgical planning when focusing on iPL and HFD.

The first cohort of prospective Fontan surgical planning patients with follow-up data: How accurate is surgical planning?

OBJECTIVE: Fontan surgical planning is an image-based, collaborative effort, which is hypothesized to result in improved patient outcomes. A common motivation for Fontan surgical planning is the progression (or concern for progression) of pulmonary arteriovenous malformations. The purpose of this study was to evaluate the accuracy of surgical planning predictions, specifically hepatic flow distribution (HFD), a known factor in pulmonary arteriovenous malformation progression, and identify methodological improvements needed to increase prediction accuracy. METHODS: Twelve single-ventricle patients who were enrolled in a surgical planning protocol for Fontan surgery with pre- and postoperative cardiac imaging were included in this study. Computational fluid dynamics were used to compare HFD in the surgical planning prediction and actual postoperative conditions. RESULTS: Overall, HFD prediction error was 17 ± 13%. This error was similar between surgery types (15 ± 18% and 18 ± 10% for revisions vs Fontan completions respectively; P = .73), but was significantly lower (6 ± 7%; P = .05) for hepatic to azygous shunts. Y-grafts and extracardiac conduits showed a strong correlation between prediction error and discrepancies in graft insertion points (r = 0.99; P < .001). Improving postoperative anatomy prediction significantly reduced overall HFD prediction error to 9 ± 6% (P = .03). CONCLUSIONS: Although Fontan surgical planning can offer accurate HFD predictions for specific graft types, methodological improvements are needed to increase overall accuracy. Specifically, improving postoperative anatomy prediction was shown to be an important target for future work. Future efforts and refinements to the surgical planning process will benefit from an improved understanding of the current state and will rely heavily on increased follow-up data.

Relationship of Aortic Stiffness to Exercise and Ventricular Volumes in Single Ventricles.

BACKGROUND: Patients with single ventricle (SV) may often undergo aortic reconstruction that creates a stiff large vessel, increasing afterload and affecting exercise performance. The objective of this study was to determine the relationship of pulse wave velocity (PWV) and distensibility in reconstructed and normal aortic arches after Fontan with exercise variables. METHODS: PWV and distensibility of the descending aorta at the level of the diaphragm (DAo) were calculated with real-time exercise cardiac magnetic resonance in 48 patients with SV after Fontan (18 after aortic reconstruction; 30 without aortic reconstruction) and compared with metabolic exercise stress test variables. RESULTS: PWV was greater in the reconstructed group than in the non-reconstructed group (median 4.4 m/s [range: 2.3 to 9.8 m/s] versus 3.6 [range: 2.6 to 6.3 m/s], respectively, p = 0.003). Statistically significant inverse correlations were found between PWV and end-diastolic, end-systolic, and stroke volumes at rest and at exercise in the reconstructed group. In addition, inverse correlations also existed in the reconstructed group between distensibility of the DAo and the exercise variables such as peak oxygen pulse (R = 0.56, p = 0.02), peak oxygen consumption (R = 0.63, p = 0.008), oxygen consumption at ventilatory anaerobic threshold (R = 0.48, p = 0.04), and peak work (R = 0.54, p = 0.02). Similar correlations were not seen in patients with non-reconstructed aortas. CONCLUSIONS: Patients with SV with reconstructed aortas have increased aortic stiffness, increasing afterload on the ventricle. Native DAo stiffness distal to the reconstruction is inversely correlated with exercise performance, presumably to decrease impedance mismatch to maintain homogeneity of the aortic wall. This information suggests a possible mechanism for decreased exercise performance in patients with SV with aortic reconstructions.