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Introduction. Computational modelling has been used widely in biological and clinical applications, but relatively less in surgical design and optimization. Magnetic resonance image (MRI)-based right ventricle (RV) models were introduced for patients with repaired Tetralogy of Fallot (rTOF) to assess ventricle cardiac function, and to identify morphological and mechanical parameters which can be used to predict and optimize post-surgery cardiac outcome. Tetralogy of Fallot is a common congenital heart defect which includes a ventricular septal defect and severe right ventricular outflow obstruction, account for the majority of cases with late onset RV failure. The current surgical approach for the patients with repaired ToF including pulmonary valve replacement/insertion (PVR) has yielded mixed results. It is of great interest to identify parameters which may be used to predict surgical cardiac function outcome after PVR. Data, Model, and Methods. Cardiac Magnetic Resonance (CMR) data from 20 healthy volunteers (11 males, mean year : 22.8) and 56 TOF patients (37 males, mean year : 25.3) were provided by Children's Hospital - Boston, Harvard Medical School from our NIH-funded project (R01 HL089269). RV wall thickness (WT), circumferential and longitudinal curvature (C-cur and L-cur), surface area (SA) and surface to volume ratio (SVR) were obtained based on CMR data for morphological analysis. 6 healthy volunteers and 16 TOF patients were chosen to construct 3D computational models for mechanical analysis. The 3D CMR-based RV/LV/Patch combination models included a) isotropic and anisotropic material properties, b) myocardial fiber orientation, c) active contraction with two zero-load geometries, and d) fluid-structure interactions. The models were used to obtain the assessment for RV mechanical conditions, which might be helpful for PVR surgical outcome prediction. All the computational models were built and solved in a commercial finite element software ADINA. Statistical methods including Linear Mixed- effort Method and Logistical regression were used in the morphological and mechanical analysis to find out potential indicators for predicting PVR outcome from the morphological and mechanical parameters. Results. In morphological analysis, statistically significant differences were found in RV SA and SVR between better-outcome patient group (BPG) and worse-outcome patient group (WPG). At begin of ejection, mean RV SA of BPG was 13.6% lower than that from WPG (241.1 cm2 v.s. 279.0 cm2, p =0.0161). Mean RV SVR of BPG was 13.1% lower than that from WPG (1.26 cm2/ml v.s. 1.45 cm2/ml, p =0.0271). Similar results were also found in RV SA and SVR at begin of filling. Furthermore, RV EF change from pre- to post-PVR were found negatively correlated with RV SA and SVR. In mechanical analysis, 22 structure-only models with one zero-load geometry (1G) were constructed to obtain stress/strain distributions. Stress-P1 from BPG was found to be closer to that from HG, compared to Stress- P1 of WPG. At the beginning of ejection, mean Stress-P1 of BPG was only 6.8% higher than that from healthy group (p =0.6889), while average Stress-P1 of WPG was 84.1% higher than that of healthy group (p =0.0418). Similar results were also found at begin of filling. The results suggested that comparing patients' RV stress values with healthy RV stress values may help identify patients with possible better outcome. The models with two zero-load geometries (2G models) and FSI models were also constructed. Their numerical results indicated that 2G models can provide end-ejection and end-filling results which were not available in 1G models, and FSI models can provide flow velocity, pressure and shear stress information which lacked in structure-only models (1G and 2G models). Conclusion. In vivo image-based 3D patient- specific computational models could lead to considerable potential gain not only in surgical design and outcome prediction, but also in understanding the mechanisms of RV failure for patients with repaired TOF.

Background: Children with complex congenital heart disease (CHD) are exposed to cyanosis from birth until their surgical repair and are often not expected to participate in physical activities to the same extent as healthy peers because of a limited maximal exercise capacity (V̇O2max). Despite limitations in V̇O2max, these children may still have the capacity to perform most daily physical activity because it requires only a submaximal effort. The purpose of this research was to examine the relationships between submaximal exercise capacity, daily physical activity and cyanosis exposure, in children with complex CHD. Methods: Children with a single functioning ventricle (Fontan), tetralogy of Fallot or transposition of the great arteries, 10 to 17 years old were deemed eligible. The Bruce treadmill protocol with breath-by-breath analysis of oxygen consumption was used to assess submaximal exercise capacity. Five measures of submaximal exercise capacity were evaluated: energy consumption (V̇O2) at the ventilatory threshold, V̇O2 at a heart rate of 130 beats per minute (bpm), metabolic equivalents (METs) at ventilatory threshold, METs at 130 bpm and heart rate at stage 1 of the Bruce protocol. Moderate-to vigorous physical activity (MVPA) was measured (Actical accelerometer with 15 second epochs) for 7 consecutive days. Exposure to cyanosis was calculated by subtracting the child’s date of birth from the date of surgical repair. Results: Participants were children with a Fontan single ventricle (n=5), tetralogy of Fallot (n=4) or transposition of the great arteries (n=7). Daily physical activity was positively associated with V̇O2 at ventilatory threshold (r = 0.78, n = 16, p = < 0.01) and V̇O2 at a heart rate of 130 bpm (r = 0.61, n = 16, p = 0.01). Children who did more than 60 minutes of physical activity per day (n=4) achieved significantly higher energy expenditure before reaching ventilatory threshold, (95% CI of the difference [8.23, 24.85], t(14) = 4.27, p = < 0.01) and at a heart rate of 130 bpm (95% CI of the difference [1.61, 14.33], t(14) = 2.69, p = 0.02). Lastly, V̇O2 at ventilatory threshold was negatively associated with days spent in cyanosis (r = .55, n = 16, p = 0.03), Conclusion: Higher V̇O2 at ventilatory threshold and V̇O2 at a heart rate of 130 bpm was associated with more daily minutes spent in moderate-to-vigorous physical activity. These results suggest that children who meet the recommended 60 minutes of MVPA would have a higher submaximal exercise capacity (V̇O2 at ventilatory threshold or a heart rate of 130 bpm), than children who did not meet the MVPA guidelines. Lastly, children who were exposed to cyanosis for a longer period of time had a lower submaximal V̇O2 at ventilatory threshold, than children who were exposed to cyanosis for a shorter period of time.

Magnetic resonance imaging nowadays supplies a noninvasive method in clinical applications. For tetralogy of Fallot (TOF) patients, after undergoing clinical operation, their cardiac anatomy still cannot supply sufficient blood flow in the pulmonary arteries with respect to the normal. In this study, we use phase contrast MR imaging to evaluate of regurgitation and turbulence of flow in pulmonary arteries after repair of TOF. We use parameters such as coefficient of variance (CV), regurgitant fraction (RF), and normalized area variation (NAV) to analyze the difference between repaired patients and normal controls. Our result also shows that CV and regurgitant fraction have loose relation. This study may provide more information to help doctors in clinical diagnosis. In the meanwhile, another three parameters were used to evaluate patients and normal persons. We use windkessel volume to see the difference of flow volume between inlet and outlet in the pulmonary arteries. We use pulse wave velocity (PWV) to discuss the propagating velocity of pressure wave on the vascular wall. We use pulmonary vascular resistance (PVR) to analyze the resistance of blood wall. PWV and PVR may lose information by means of insufficient points in a cardiac cycle, but the result may still be a kind of reference.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease (CHD). The understanding of the genetics and inheritance of TOF is limited. Although about 15% of cases are associated with a 22q11.2 deletion, the majority have no known aetiology. Even in 22q11.2 Deletion Syndrome (22q11DS), factors that increase the likelihood of CHD expression are poorly understood. We aimed to determine the prevalence and phenotypes of CHD in relatives of adults with TOF. We also investigated the prevalence of CHD in relatives without a 22q11.2 deletion of individuals with 22q11DS. Offspring of patients with TOF had the greatest prevalence of CHD. Diverse cardiac phenotypes, including left heart obstructive lesions, were found in families. We also found that unaffected relatives of individuals with 22q11DS had a greater prevalence of complex CHD over population expectations, suggesting that modifier genetic factors may be involved in expression of CHD in 22q11DS.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease (CHD). The understanding of the genetics and inheritance of TOF is limited. Although about 15% of cases are associated with a 22q11.2 deletion, the majority have no known aetiology. Even in 22q11.2 Deletion Syndrome (22q11DS), factors that increase the likelihood of CHD expression are poorly understood. We aimed to determine the prevalence and phenotypes of CHD in relatives of adults with TOF. We also investigated the prevalence of CHD in relatives without a 22q11.2 deletion of individuals with 22q11DS. Offspring of patients with TOF had the greatest prevalence of CHD. Diverse cardiac phenotypes, including left heart obstructive lesions, were found in families. We also found that unaffected relatives of individuals with 22q11DS had a greater prevalence of complex CHD over population expectations, suggesting that modifier genetic factors may be involved in expression of CHD in 22q11DS.

Magnetic Resonance Imaging (MRI) provides noninvasive method in clinical application. For the patients of Tetralogy of Fallot underwent surgical correction,regurgitation and turbulence in blood flow may still present in pulmonary arteries.In this study,Phase Contrast MR Imaging will be used to quantitate,and to observe blood flow in after repair Tetralogy of Fallot (TOF) patients.We use 3 parameters,which are Coefficient of Variance (CV),Regurgitant Fraction (RF) and Net Flow analysis to analyze two situations of blood flow in patients¡¦ left pulmonary artery ¡Bright pulmonary artery and main pulmonary artery.We also compare normal subjects to patients in this experiment. The pulmonary circulation is the action of blood flow from right ventricular to main pulmonary artery then to left pulmonary artery and right pulmonary artery. We use three additional parameters: rPA / lPA flow ratio¡BPeak Velocities and Total Flow to observe the pulmonary blood flow. We use rPA / lPA flow ratio to identify the tendency of blood flow, with Peak Velocities to find out Peak Velocity in normal difference between patient.Meanwhile, the measurement of Total Flow is applied to observe the flow from main pulmonary artery to left and right pulmonary artery. We hope to use these parameters that can help doctors on clinical diagnosis.

Recently magnetic resonance imaging has become more and more popular in clinical applications. In clinical studies, the heart of the TOF patient has some congenital defects. These defects lead to insufficient blood flowing into the pulmonary arteries, rendering the necessary of repair for TOF patients. However, even after repair, the blood in pulmonary arteries still cannot flow in the same way with those of normal people. For this reason, studies on the flow behavior of pulmonary arteries in TOF patients would be valuable in clinical applications. In this study, we focus on the quantitative analysis of in-plane flow in branch pulmonary arteries (left and right pulmonary arteries) after repair of tetralogy of Fallot (TOF) by means of phase-contrast MR imaging. The regurgitation and turbulence were evaluated by coefficient of variance (CV) and regurgitant fraction. Vector map of in-plane flow was also included in order to facilitate the observation of flow patterns. Our result shows a positive correlation of CV and regurgitant fraction in terms of turbulence and regurgitation. Therefore, we conclude that CV and regurgitant fraction as well as vector maps may be helpful to quantitate in-plane flow for after repair of TOF patients, providing a more accurate analysis in clinical diagnosis.

Magnetic resonance imaging (MRI) is an useful technique that provides a noninvasive method in clinical applications. For the patient of tetralogy of Fallot (TOF) after repaired, turbulence and regurgitation in blood flow may appear in pulmonary arteries. In this study, phase contrast MR imaging was applied and vortical flow patterns in the pulmonary arteries of patients after repair of TOF has been investigated. There are two major part of this study. Firstly we simulated vortical flow patterns of star, focus, and saddle which are most frequently appeared in blood flow. Quadrant index has been proposed for pattern analysis. In the second part we applied these parameters to in vivo data of repaired TOF patients, and compared with other parameters such as vorticity, coefficient of variance (CV), and regurgitant fraction (RF). Our result shows that the linear correlation between the mean of CV of velocity and mean of CV of vorticity in right pulmonary artery (RPA) as well as pulmonary trunk (PT) is larger than that in left pulmonary artery (LPA). This study shows that vorticity may provide some useful information of flow patterns and therefore helps doctors in clinical diagnosis