Shiva Sharma

Trends and outcomes after prenatal diagnosis of congenital cardiac malformations by fetal echocardiography in a well defined birth population, Atlanta, Georgia, 1990-1994.

OBJECTIVES In this study we used a population-based approach to assess the impact of fetal echocardiography on a well defined birth population with nearly complete ascertainment of cardiac defects. BACKGROUND Although fetal echocardiography is being used more frequently in the prenatal diagnosis of congenital cardiac malformations, its impact on the diagnosis and surveillance of cardiac defects has not been described in defined populations. METHODS All stillborn and live-born infants with diagnosed cardiac defects and whose mothers resided in the metropolitan Atlanta area from January 1990 through December 1994 were ascertained through an established birth defects surveillance system. All fetuses with cardiac defects diagnosed prenatally by a pediatric of cardiac defects, diagnostic trends and adverse fetal outcomes were described. RESULTS We identified 1,589 infants with congenital cardiac malformations, for a live-birth prevalence rate of 8.1/1,000 (95% confidence interval [CI] 7.8 to 8.6). Overall, 97 (6.1%) of these cases of cardiac malformations were diagnosed prenatally. The proportion of cardiac defects diagnosed prenatally rose from 2.6% in 1990 to 12.7% in 1994, a nearly fivefold increase. The proportion of cardiac defects diagnosed prenatally during the study varied by the type of defect, from a low of 4.7% for atrial septal defects to a high of 28% for hypoplastic left heart syndrome. Prenatally diagnosed cardiac malformations were associated with a high incidence of infant mortality (30.9%, 95% CI 2.4 to 5.4) and fetal wastage (17.5%, 95% CI 6.2 to 11.3). CONCLUSIONS These data show that fetal echocardiography is being used increasingly in the prenatal diagnosis of congenital cardiac malformations in metropolitan Atlanta. Few pregnancy terminations were reported as a result of such diagnoses. However, the study had limited power (10%) to detect a meaningful decrease in birth prevalence rates for congenital heart disease. In addition, survival of infants was not improved after prenatal diagnosis with fetal echocardiography.

One hundred pulmonary valve replacements in children after relief of right ventricular outflow tract obstruction

BACKGROUND Surgical repair of obstructive lesions of the right ventricular outflow tract (RVOT) in children commonly creates pulmonary valve incompetence that may eventually require pulmonary valve replacement (PVR). We reviewed our experience with PVR late after RVOT reconstruction. METHODS We performed 100 PVRs in 93 children 1.1 months to 22.4 years (median 8) after RVOT reconstruction. Children with right ventricular to pulmonary artery conduits and primary PVRs were excluded. Age at PVR was 4.5 months to 27.9 years (median 9.5 years). Initial diagnosis was tetralogy of Fallot and variants, 62; critical pulmonary stenosis, 15; pulmonary atresia with intact ventricular septum, 7; and others, 9. Eleven patients had a redo PVR. A total of 62 PVRs were homografts; 38 were porcine valves. RESULTS There was one early death. On follow-up of 5 months to 12.4 years (mean 4.9 years) there were no late deaths although 1 child underwent cardiac transplantation. Actuarial freedom from redo PVR at 8 years was 100% for porcine valves but 70% for homograft valves (p = 0.17). For children younger than 3 years at PVR, freedom from reoperation was 76% at 1 year and 39% at 8 years compared with freedom from redo PVR at 8 years of 100% for children older than 3 years. On latest echocardiogram 97% of porcine valves had mild or no pulmonary regurgitation compared with 72% of homograft valves. CONCLUSIONS PVR after RVOT reconstruction can be performed with low risk. Porcine valves may be superior to homograft valves although this advantage may be due to older age at time of PVR.

In vitro flow experiments for determination of optimal geometry of total cavopulmonary connection for surgical repair of children with functional single ventricle

OBJECTIVES This study sought to evaluate the effect of offsetting cavopulmonary connections at varying pulmonary flow ratios to determine the optimal geometry of the connection. BACKGROUND Previous investigators have demonstrated energy conservation within the streamlined contours of the total cavopulmonary connection compared with that of the atriopulmonary connection. However, their surgical design of connecting the two cavae directly opposite each other may result in high energy losses. Others have introduced a unidirectional connection with some advantages but with concerns about the formation of arteriovenous malformation in the lung excluded from hepatic venous return. Thus, an optimal surgical design has not been determined. METHODS In the present models, the caval connections were offset through a range of 0.0 to 2.0 diameters by 0.5 superior cava diameter increments. Flow ratios were fixed for superior and inferior cavae and varied for right and left pulmonary arteries as 70:30, 60:40, 50:50, 40:60 and 30:70 to stimulate varying lung resistance. Pressure measurements and flow visualization were done at steady flows of 2, 4 and 6 liters/min to stimulate rest and exercise. RESULTS Our data show that the energy losses at the 0.0-diameter offset were double the losses of the 1.0 and 1.5 diameters, which had minimal energy losses. This result was attributable to chaotic patterns seen on flow visualization in the 0.0-diameters offset. Energy savings were more evident at the 50:50 right/left pulmonary artery ratio. Energy losses increased with increased total flow rates. CONCLUSIONS The results strongly suggest the incorporation of caval offsets in future total cavopulmonary connections.

Toward designing the optimal total cavopulmonary connection: an in vitro study

BACKGROUND Understanding the total cavopulmonary connection (TCPC) hemodynamics may lead to improved surgical procedures which result in a more efficient modified circulation. Reduced energy loss will translate to less work for the single ventricle and although univentricular physiology is complex, this improvement could contribute to improved postoperative outcomes. Therefore to conserve energy, one surgical goal is optimization of the TCPC geometry. In line with this goal, this study investigated whether addition of caval curvature or flaring at the connection conserves energy. METHODS TCPC models were made varying the curvature of the caval inlet or by flaring the anastomosis. Steady flow pressure measurements were made to calculate the power loss attributed to each connection design over a range of pulmonary flow splits (70:30 to 30:70). Particle flow visualization was performed for each design and was qualitatively compared to the power losses. RESULTS Results indicate that curving the cavae toward one pulmonary artery is advantageous only when the flow rate from that cavae matches the flow to the pulmonary artery. Under other pulmonary flow split conditions, the losses in the curved models are significant. In contrast, fully flaring the anastomosis reduced losses over the range of pulmonary flow splits. Power losses were 56% greater for the curving as compared to flaring. Fully flaring without caval offset reduced losses 45% when compared to previous models without flaring. If flaring on all sides was implemented with caval offset, power losses reduced 68% compared to the same nonflared model. CONCLUSIONS The results indicate that preferentially curving the cavae is only optimal under specific pulmonary flow conditions and may not be efficient in all clinical cases. Flaring of the anastomosis has great potential to conserve energy and should be considered in future TCPC procedures.

The total cavopulmonary connection resistance: a significant impact on single ventricle hemodynamics at rest and exercise

Little is known about the impact of the total cavopulmonary connection (TCPC) on resting and exercise hemodynamics in a single ventricle (SV) circulation. The aim of this study was to elucidate this mechanism using a lumped parameter model of the SV circulation. Pulmonary vascular resistance (1.96+/-0.80 WU) and systemic vascular resistances (18.4+/-7.2 WU) were obtained from catheterization data on 40 patients with a TCPC. TCPC resistances (0.39+/-0.26 WU) were established using computational fluid dynamic simulations conducted on anatomically accurate three-dimensional models reconstructed from MRI (n=16). These parameters were used in a lumped parameter model of the SV circulation to investigate the impact of TCPC resistance on SV hemodynamics under resting and exercise conditions. A biventricular model was used for comparison. For a biventricular circulation, the cardiac output (CO) dependence on TCPC resistance was negligible (sensitivity=-0.064 l.min(-1).WU(-1)) but not for the SV circulation (sensitivity=-0.88 l.min(-1).WU(-1)). The capacity to increase CO with heart rate was also severely reduced for the SV. At a simulated heart rate of 150 beats/min, the SV patient with the highest resistance (1.08 WU) had a significantly lower increase in CO (20.5%) compared with the SV patient with the lowest resistance (50%) and normal circulation (119%). This was due to the increased afterload (+35%) and decreased preload (-12%) associated with the SV circulation. In conclusion, TCPC resistance has a significant impact on resting hemodynamics and the exercise capacity of patients with a SV physiology.

Patient-specific surgical planning and hemodynamic computational fluid dynamics optimization through free-form haptic anatomy editing tool (SURGEM)

The first version of an anatomy editing/surgical planning tool (SURGEM) targeting anatomical complexity and patient-specific computational fluid dynamics (CFD) analysis is presented. Novel three-dimensional (3D) shape editing concepts and human–shape interaction technologies have been integrated to facilitate interactive surgical morphology alterations, grid generation and CFD analysis. In order to implement “manual hemodynamic optimization” at the surgery planning phase for patients with congenital heart defects, these tools are applied to design and evaluate possible modifications of patient-specific anatomies. In this context, anatomies involve complex geometric topologies and tortuous 3D blood flow pathways with multiple inlets and outlets. These tools make it possible to freely deform the lumen surface and to bend and position baffles through real-time, direct manipulation of the 3D models with both hands, thus eliminating the tedious and time-consuming phase of entering the desired geometry using traditional computer-aided design (CAD) systems. The 3D models of the modified anatomies are seamlessly exported and meshed for patient-specific CFD analysis. Free-formed anatomical modifications are quantified using an in-house skeletization based cross-sectional geometry analysis tool. Hemodynamic performance of the systematically modified anatomies is compared with the original anatomy using CFD. CFD results showed the relative importance of the various surgically created features such as pouch size, vena cave to pulmonary artery (PA) flare and PA stenosis. An interactive surgical-patch size estimator is also introduced. The combined design/analysis cycle time is used for comparing and optimizing surgical plans and improvements are tabulated. The reduced cost of patient-specific shape design and analysis process, made it possible to envision large clinical studies to assess the validity of predictive patient-specific CFD simulations. In this paper, model anatomical design studies are performed on a total of eight different complex patient specific anatomies. Using SURGEM, more than 30 new anatomical designs (or candidate configurations) are created, and the corresponding user times presented. CFD performances for eight of these candidate configurations are also presented.

Application of an adaptive control grid interpolation technique to morphological vascular reconstruction

The problem of interslice magnetic resonance (MR) image reconstruction arises in a broad range of medical applications. In such cases, there is a need to approximate information present in the original subject that is not reflected in contiguously acquired MR images because of hardware sampling limitations. In the context of vascular morphology reconstruction, this information is required in order for subsequent visualization and computational analysis of blood vessels to be most effective. Toward that end we have developed a method of vascular morphology reconstruction based on adaptive control grid interpolation (ACGI) to function as a precursor to visualization and computational analysis. ACGI has previously been implemented in addressing various problems including video coding and tracking. This paper focuses on the novel application of the technique to medical image processing. ACGI combines features of optical flow-based and block-based motion estimation algorithms to enhance insufficiently dense MR data sets accurately with a minimal degree of computational complexity. The resulting enhanced data sets describe vascular geometries. These reconstructions can then be used as visualization tools and in conjunction with computational fluid dynamics (CFD) simulations to offer the pressure and velocity information necessary to quantify power loss. The proposed ACGI methodology is envisioned ultimately to play a role in surgical planning aimed at producing optimal vascular configurations for successful surgical outcomes.

Importance of accurate geometry in the study of the total cavopulmonary connection: computational simulations and in vitro experiments.

AbstractPrevious in vitro studies have shown that total cavopulmonary connection (TCPC) models incorporating offset between the vena cavae are energetically more efficient than those without offsets. In this study, the impact of reducing simplifying assumptions, thereby producing more physiologic models, was investigated by computational fluid dynamics (CFD) and particle flow visualization experiments. Two models were constructed based on angiography measurements. The first model retained planar arrangement of all vessels involved in the TCPC but incorporated physiologic vessel diameters. The second model consisted of constant-diameter vessels with nonplanar vascular features. CFD and in vitro experiments were used to study flow patterns and energy losses within each model. Energy losses were determined using three methods: theoretical control volume, simplified control volume, and velocity gradient based dissipation. Results were compared to a simplified model control. Energy loss in the model with physiologically more accurate vessel diameters was 150% greater than the simplified model. The model with nonplanar features produced an asymmetric flow field with energy losses approximately 10% higher than simplified model losses. With the velocity gradient based dissipation technique, the map of energy dissipation was plotted revealing that most of the energy was dissipated near the pulmonary artery walls.

Fontan hemodynamics: Importance of pulmonary artery diameter

OBJECTIVE We quantify the geometric and hemodynamic characteristics of extracardiac and lateral tunnel Fontan surgical options and correlate certain anatomic characteristics with their hemodynamic efficiency and patient cardiac index. METHODS AND RESULTS The study was conducted retrospectively on 22 patients undergoing Fontan operations (11 extracardiac and 11 lateral tunnel operations). Total cavopulmonary connection geometric parameters such as vessel areas, curvature, and offsets were quantified using a skeletonization method. Energy loss at the total cavopulmonary connection junction was available from previous in vitro experiments and computational fluid dynamic simulations for 5 and 9 patients, respectively. Cardiac index data were available for all patients. There was no significant difference in the mean and minimum cross-sectional vessel areas of the pulmonary artery between the extracardiac and lateral tunnel groups. The indexed energy dissipation within the total cavopulmonary connection was strongly correlated to minimum cross-sectional area of the pulmonary arteries (R(2) value of 0.90 and P < .0002), whereas all other geometric features, including shape characteristics, had no significant correlation. Finally, cardiac index significantly correlated with the minimum pulmonary artery area (P = .006), suggesting that total cavopulmonary connection energy losses significantly affect resting cardiac output. CONCLUSIONS The minimum outlet size of the total cavopulmonary connection (ie, minimum cross section of pulmonary artery) governs the energy loss characteristics of the total cavopulmonary connection more strongly than variations in the shapes corresponding to extracardiac and lateral tunnel configurations. Differences in pulmonary artery sizes must be accounted for when comparing energy losses between extracardiac and lateral tunnel geometries.

Total Cavopulmonary Connection Flow With Functional Left Pulmonary Artery Stenosis Angioplasty and Fenestration In Vitro

Background— In our multicenter study of the total cavopulmonary connection (TCPC), a cohort of patients with long-segment left pulmonary artery (LPA) stenosis was observed (35%). The clinically recognized detrimental effects of LPA stenosis motivated a computational fluid dynamic simulation study within 3-dimensional patient-specific and idealized TCPC pathways. The goal of this study was to quantify and evaluate the hemodynamic impact of LPA stenosis and to judge interventional strategies aimed at treating it. Methods and Results— Simulations were conducted at equal vascular lung resistance, modeling both discrete stenosis (DS) and diffuse long-segment hypoplasia with varying degrees of obstruction (0% to 80%). Models having fenestrations of 2 to 6 mm and atrium pressures of 4 to 14 mm Hg were explored. A patient-specific, extracardiac TCPC with 85% DS was studied in its original configuration and after virtual surgery that dilated the LPA to 0% stenosis in the computer medium. Performance indices improved exponentially (R2>0.99) with decreasing obstruction. Diffuse long-segment hypoplasia was ≈50% more severe with regard to lung perfusion and cardiac energy loss than DS. Virtual angioplasty performed on the 3-dimensional Fontan anatomy exhibiting an 85% DS stenosis produced a 61% increase in left lung perfusion and a 50% decrease in cardiac energy dissipation. After 4-mm fenestration, TCPC baffle pressure dropped by ≈10% and left lung perfusion decreased by ≈8% compared with the 80% DS case. Conclusions— DS <60% and diffuse long-segment hypoplasia <40% could be considered tolerable because both resulted in only a 12% decrease in left lung perfusion. In contrast to angioplasty, a fenestration (right-to-left shunt) reduced TCPC pressure at the cost of decreased left and right lung perfusion. These results suggest that pre-Fontan computational fluid dynamic simulation may be valuable for determining both the hemodynamic significance of LPA stenosis and the potential benefits of intervention.