The Fontan procedure, although an imperfect solution for children born with a single functional ventricle, is the only reconstruction at present short of transplantation. The haemodynamics associated with the total cavopulmonary connection, the modern approach to Fontan, are severely altered from the normal biventricular circulation and may contribute to the long-term complications that are frequently noted. Through recent technological advances, spear-headed by advances in medical imaging, it is now possible to virtually model these surgical procedures and evaluate the patient-specific haemodynamics as part of the pre-operative planning process. This is a novel paradigm with the potential to revolutionise the approach to Fontan surgery, help to optimise the haemodynamic results, and improve patient outcomes. This review provides a brief overview of these methods, presents preliminary results of their clinical usage, and offers insights into its potential future directions.
BACKGROUND: Open surgery effectively treats mesial temporal lobe epilepsy, but carries the risk of neurocognitive deficits, which may be reduced with minimally invasive alternatives.
OBJECTIVE: To describe technical and clinical outcomes of stereotactic laser amygdalohippocampotomy with real-time magnetic resonance thermal imaging guidance.
METHODS: With patients under general anesthesia and using standard stereotactic methods, 13 adult patients with intractable mesial temporal lobe epilepsy (with and without mesial temporal sclerosis [MTS]) prospectively underwent insertion of a saline-cooled fiberoptic laser applicator in amygdalohippocampal structures from an occipital trajectory. Computer-controlled laser ablation was performed during continuous magnetic resonance thermal imaging followed by confirmatory contrast-enhanced anatomic imaging and volumetric reconstruction. Clinical outcomes were determined from seizure diaries.
RESULTS: A mean 60% volume of the amygdalohippocampal complex was ablated in 13 patients (9 with MTS) undergoing 15 procedures. Median hospitalization was 1 day. With follow-up ranging from 5 to 26 months (median, 14 months), 77% (10/13) of patients achieved meaningful seizure reduction, of whom 54% (7/13) were free of disabling seizures. Of patients with preoperative MTS, 67% (6/9) achieved seizure freedom. All recurrences were observed before 6 months. Variances in ablation volume and length did not account for individual clinical outcomes. Although no complications of laser therapy itself were observed, 1 significant complication, a visual field defect, resulted from deviated insertion of a stereotactic aligning rod, which was corrected before ablation.
CONCLUSION: Real-time magnetic resonance-guided stereotactic laser amygdalohippocampotomy is a technically novel, safe, and effective alternative to open surgery. Further evaluation with larger cohorts over time is warranted.
Background: Aortic valve (AV) calcification preferentially occurs on the fibrosa side while the ventricularis side remains relatively unaffected. Here, we tested the hypothesis that side-dependent activation of bone morphogenic protein (BMP) pathway in the endothelium of the ventricularis and fibrosa is associated with human AV calcification.
Methods and Results: Human calcified AVs obtained from AV replacement surgeries and non-calcified AVs from heart transplantations were used for immunohistochemical studies. We found SMAD-1/5/8 phosphorylation (a canonical BMP pathway) was higher in the calcified fibrosa than the non-calcified fibrosa while SMAD-2/3 phosphorylation (a canonical TGFβ pathway) did not show any difference. Interestingly, we found that BMP-2/4/6 expression was significantly higher on the ventricularis endothelium compared to the fibrosa in both calcified and non-calcified AV cusps; however, BMP antagonists (crossvienless-2/BMPER and noggin) expression was significantly higher on the ventricularis endothelium compared to the fibrosa in both disease states. Moreover, significant expression of inhibitory SMAD-6 expression was found only in the non-calcified ventricularis endothelium.
Conclusions: SMAD-1/5/8 is preferentially activated in the calcified fibrosa endothelium of human AVs and it correlates with low expression of BMP antagonists and inhibitory SMAD6. These results suggest a dominant role of BMP antagonists in the side-dependent calcification of human AVs.
Total cavopulmonary connection (TCPC) geometries have great variability. Geometric features, such as diameter, connection angle, and distance between vessels, are hypothesized to affect the energetics and flow dynamics within the connection. This study aimed to identify important geometric characteristics that can influence TCPC hemodynamics. Anatomies from 108 consecutive patients were reconstructed from cardiac magnetic resonance (CMR) images and analyzed for their geometric features. Vessel flow rates were computed from phase contrast CMR. Computational fluid dynamics simulations were carried out to quantify the indexed power loss and hepatic flow distribution. TCPC indexed power loss correlated inversely with minimum Fontan pathway (FP), left pulmonary artery, and right pulmonary artery diameters. Cardiac index correlated with minimum FP diameter and superior vena cava (SVC) minimum/maximum diameter ratio. Hepatic flow distribution correlated with caval offset, pulmonary flow distribution, and the angle between FP and SVC. These correlations can have important implications for future connection design and patient follow-up.
Total cavopulmonary connection is the result of a series of palliative surgical repairs performed on patients with single ventricle heart defects. The resulting anatomy has complex and unsteady hemodynamics characterized by flow mixing and flow separation. Although varying degrees of flow pulsatility have been observed in vivo, non-pulsatile (time-averaged) boundary conditions have traditionally been assumed in hemodynamic modeling, and only recently have pulsatile conditions been incorporated without completely characterizing their effect or importance. In this study, 3D numerical simulations with both pulsatile and non-pulsatile boundary conditions were performed for 24 patients with different anatomies and flow boundary conditions from Georgia Tech database. Flow structures, energy dissipation rates and pressure drops were compared under rest and simulated exercise conditions. It was found that flow pulsatility is the primary factor in determining the appropriate choice of boundary conditions, whereas the anatomic configuration and cardiac output had secondary effects. Results show that the hemodynamics can be strongly influenced by the presence of pulsatile flow. However, there was a minimum pulsatility threshold, identified by defining a weighted pulsatility index (wPI), above which the influence was significant. It was shown that when wPI < 30%, the relative error in hemodynamic predictions using time-averaged boundary conditions was less than 10% compared to pulsatile simulations. In addition, when wPI < 50, the relative error was less than 20%. A correlation was introduced to relate wPI to the relative error in predicting the flow metrics with non-pulsatile flow conditions.
Computational fluid dynamics (CFD) tools have been extensively applied to study the hemodynamics in the total cavopulmonary connection (TCPC) in patients with only a single functioning ventricle. Without the contraction of a sub-pulmonary ventricle, pulsatility of flow through this connection is low and variable across patients, which is usually neglected in most numerical modeling studies. Recent studies suggest that such pulsatility can be non-negligible and can be important in hemodynamic predictions. The goal of this work is to compare the results of an in-house numerical methodology for simulating pulsatile TCPC flow with experimental results. Digital particle image velocimetry (DPIV) was acquired on TCPC in vitro models to evaluate the capability of the CFD tool in predicting pulsatile TCPC flow fields. In vitro hemodynamic measurements were used to compare the numerical prediction of power loss across the connection. The results demonstrated the complexity of the pulsatile TCPC flow fields and the validity of the numerical approach in simulating pulsatile TCPC flow dynamics in both idealized and complex patient specific models.