Advanced Imaging Guidance for Ductus Venosus Stenting in Obstructed Total Anomalous Pulmonary Venous Return.

Obstructed infracardiac total anomalous pulmonary venous return is nearly always a surgical emergency in which infants present in severe cardiopulmonary distress. Ductal venosus stenting can provide a temporizing option for premature, low birth weight infants with high risk for surgical complications. In challenging anatomic cases, virtual reality, 3D-printed models, and fusion image guidance can aid in procedural planning and provide support for successful intervention.

Computational Fluid Dynamic Assessment of Patients with Congenital Heart Disease from 3D Rotational Angiography.

For congenital heart disease patients, multiple imaging modalities are needed to discern anatomy and functional information such as differential blood flow. During cardiac catheterization, 3D rotational angiography (3DRA) can provide CTA-like images, enabling anatomical information and intraprocedural guidance. We seek to establish whether unique aspects of this technique can also generate quantitative functional blood flow information. We propose that systematic integration of 3DRA imaging, catheter hemodynamic information, and computational fluid dynamics (CFD), can provide quantitative information regarding blood flow dynamics and energetics, without additional imaging or procedures. We report a single center retrospective feasibility study comprising four patients with 3DRA imaging and a complete set of hemodynamic data. 3DRA was processed and segmented to reconstruct vascular regions of interest (ROI), and a computational grid for CFD modeling of blood flow through the ROI was generated. Blood flow was simulated by integrating catheter hemodynamic data to devise boundary conditions at vascular ROI inlets and outlets. The 3DRA-based workflow successfully generated key computational outputs commonly used for cardiovascular applications, including flow patterns, distribution fractions, wall shear stress. Computational outputs obtained were as detailed and resolved as those obtained from more commonly used CT or MR angiography. Accuracy was confirmed by comparing computed flow distributions with measurements for 2 cases, showing less than 2.0% error from the measured data. Systematic integration of catheter hemodynamic information, 3DRA imaging, and CFD modeling, provides an effective and feasible alternative to obtain important quantitative blood flow information and visualization, without additional imaging.

Computed Tomography-Derived Normative Values of Right Ventricular Outflow Tract Structures in the Pediatric Population.

Recent advances in available percutaneous device technology require accurate measurements and quantification of relationships between right ventricular outflow tract (RVOT) structures in children with and without congenital heart disease to determine device suitability. To date, no population study has described normal reference ranges of these measurements by computed tomography (CT). We aimed to establish normative values for four CT-derived measurements between RVOT structures from a heterogeneous population without heart disease and develop z scores useful for clinical practice. Patients without heart disease who underwent cardiac CT between April 2014 and February 2021 at Children's Hospital Colorado were included. Distance between the right ventricular (RV) apex to pulmonary valve (PV), PV to pulmonary trunk bifurcation, and bifurcation to the right and left pulmonary artery was measured. Previously validated models were used to normalize the measurements and calculate Z scores. Each measurement was plotted against BSA and Z scores distributions were used as reference lines. Three-hundred and sixty-four healthy patients with a mean age of 8.8 years (range 1-21), 58% male, and BSA of 1 m2 (range 0.4-2.1) were analyzed. The Haycock formula was used to present data as predicted values for a given BSA and within equations relating each measurement to BSA. Predicted values and Z-score boundaries for all measurements are presented.We report CT-derived normative data for four measurements between RVOT structures from a heterogeneous cohort of healthy children. Knowledge of this normative data will be useful in both determining device fit and customizing future devices to accommodate the diverse pediatric size range.

Advancing Wearable Biosensors for Congenital Heart Disease: Patient and Clinician Perspectives: A Science Advisory From the American Heart Association.

Wearable biosensors (wearables) enable continual, noninvasive physiologic and behavioral monitoring at home for those with pediatric or congenital heart disease. Wearables allow patients to access their personal data and monitor their health. Despite substantial technologic advances in recent years, issues with hardware design, data analysis, and integration into the clinical workflow prevent wearables from reaching their potential in high-risk congenital heart disease populations. This science advisory reviews the use of wearables in patients with congenital heart disease, how to improve these technologies for clinicians and patients, and ethical and regulatory considerations. Challenges related to the use of wearables are common to every clinical setting, but specific topics for consideration in congenital heart disease are highlighted.

Patient Screening for Self-Expanding Percutaneous Pulmonary Valves Using Virtual Reality.

BACKGROUND: In recent years, self-expanding technology to treat pulmonary regurgitation in the native right ventricular outflow tract became Food and Drug Administration approved in the United States and is now routinely used. The current practice for selection of patients who are candidates for these devices includes screening for "anatomic fit," performed by each of the manufacturing companies. Our study aims to validate the use of virtual reality (VR) as a tool for local physician-led screening of patients. METHODS AND RESULTS: This retrospective study from Children's Hospital Colorado included patients who underwent pulmonary valve replacement and had screening for a Harmony TPV or Alterra Prestent performed between September 2020 and January 2022. The data from the commercial companies' dedicated analysis for self-expanding transcatheter pulmonary valve frames evaluation with perimeter analysis were collected. VR simulation was performed blinded by 2 congenital interventional cardiologists using Elucis VR software and an Oculus Quest 2 headset. Among the 27 evaluated cases, the use of a self-expandable valve was recommended by companies' dedicated analysis in 23 cases (85.2%), by VR assessment in 26 cases (96.3), and finally implanted in 25 cases (92.6%). Regarding the level of agreement, both modalities (manufacturer and VR) were good at screening-in patients who received a self-expanding valve (100% versus 96.1%). When it came to screening-out the patients, VR presented good capacity to accurately classify nonsuitable patients (50% versus 100%). CONCLUSIONS: Our institutional experience with VR transcatheter pulmonary valve implantation planning accurately predicted clinical outcomes. This paves the way for routine use of VR in patient selection for self-expanding valve technologies.

Fusion imaging for guidance of pulmonary arteriovenous malformation embolisation with minimal radiation and contrast exposure.

Hereditary haemorrhagic telangiectasia is an inherited disorder characterised by vascular dysplasia that leads to the development of arteriovenous malformations. Pulmonary arteriovenous malformations occur in approximately 30% of patients with haemorrhagic telangiectasia. Given the complex characteristics of haemorrhagic telangiectasia lesions, the application of three-dimensional fusion imaging holds significant promise for procedural guidance and decrease in contrast and radiation dosing. We reviewed all patients who underwent transcatheter approach for pulmonary arteriovenous malformation occlusion with fusion image guidance from June 2018 to September 2023 from a single centre. A total of nine cases with haemorrhagic telangiectasia and transcatheter occlusion of pulmonary arteriovenous malformations using fusion imaging were identified. Five (56%) were male, mean age at procedure was 15.7 years (10-28 years) and mean number of pulmonary arteriovenous malformations intervened was three per patient (1-7). Two of the cases were complex repeat embolisations. The mean fluoroscopy time was 40.6 min (10.7-68.8 min), with mean contrast dose of 28.8 mL (11-60 mL; mean of 0.51 mL/kg) and mean radiation dose of 66.3 mGy (25.6-140 mGy; mean of 40.5 mGy/m2). There were no complications reported during the procedures, with no additional interventions necessary. Fusion imaging in pulmonary arteriovenous malformations embolisation for patients with haemorrhagic telangiectasia is feasible and has the potential to reduce contrast and radiation doses. To our knowledge, we describe the lowest radiation and contrast doses per patient using fusion imaging technology reported in the literature to date.

Use of Three-Dimensional Intracardiac Echocardiography Catheter in the Evaluation of Prosthetic Pulmonary Valves after Transcatheter Replacement.

Transcatheter pulmonary valve replacement (TPVR) is commonly performed in patients with congenital heart disease as a safe alternative to replacement via open heart surgery. Intracardiac echocardiography (ICE) is a useful technique for evaluating multiple structures that are difficult to assess by other echocardiographic techniques, particularly the pulmonary valve. To our knowledge, the use of three-dimensional (3D) ICE catheters to evaluate prosthetic valves after TPVR has not been reported. Three-dimensional ICE catheters offer a comprehensive evaluation of transcatheter-deployed pulmonary valves through 3D, 3D color, xPlane, and multiplane reconstruction. The aim of this study is to demonstrate the feasibility of using 3D ICE catheters, outline their role in evaluating post-TPVR deployment success and complications, consider their additive value to two-dimensional ICE, and present our institutional experience with it in 50 cases of TPVR.

Fontan Fenestration Reduction with an Occlutech Atrial Flow Regulator: Unique Views from Three-Dimensional Intracardiac Echocardiography.

Three-dimensional intracardiac echocardiography (3D ICE) has gained popularity in interventional cardiology given its improved spatial and temporal imaging in assessing intracardiac anatomy pre- and post-intervention. We describe the use of 3D ICE in the reduction of a Fontan fenestration with an Occlutech atrial flow regulator (AFR) device.

Staged Acclimatization in a Failing Fontan by AFR in AFR: Ever-Decreasing Circles.

We describe serial implantation of atrial flow regulator (AFR) devices in the Fontan fenestration of a 4-year-old patient. Initially, the fenestration size was decreased using a 6/5 AFR, resulting in improved saturations and hemodynamics. One year later, further improvement was achieved by placing a 4/10 AFR inside the original device. (Level of Difficulty: Advanced.).

Percutaneous VA-ECMO from Cannulation to Decannulation: Novel Use of a Vascular Closure Device in Pediatrics.

VA-ECMO can be lifesaving in cardiogenic shock in children. While surgical vascular repair is the current standard of care for decannulation, it comes with notable risks. We present a series of eight patients who underwent decannulation with a collagen plug-based vascular closure device (MANTA) for the common femoral artery. Seven of the patients were successfully decannulated without access site-related vascular complications. One required conversion to surgical cut-down with arterial repair due to device failure. This series demonstrates the successful use of the MANTA device in percutaneous VA-ECMO decannulation in the pediatric population, while highlighting potential technical challenges for success.

Computed tomographic parenchymal lung findings in premature infants with pulmonary vein stenosis.

BACKGROUND: Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional method of evaluating neonates for potential structural changes contributing to severe lung dysfunction and pulmonary hypertension but may miss pulmonary venous stenosis due to the absence of contrast and potential overlap in findings between developmental pulmonary vein pulmonary vein stenosis and lung disease of prematurity. OBJECTIVE: To describe the parenchymal changes of pediatric patients with both prematurity and pulmonary vein stenosis, correlate them with venous disease and to describe the phenotypes associated with this disease. MATERIALS AND METHODS: A 5-year retrospective review of chest CT angiography (CTA) imaging in patients with catheterization-confirmed pulmonary vein stenosis was performed to identify pediatric patients (< 18 years) who had a history of prematurity (< 35 weeks gestation). Demographic and clinical data associated with each patient were collected, and the patients' CTAs were re-reviewed to evaluate pulmonary veins and parenchyma. Patients with post-operative pulmonary vein stenosis and those with congenital heart disease were excluded. Data was analyzed and correlated for descriptive purposes. RESULTS: A total of 17 patients met the inclusion criteria (12 female, 5 male). All had pulmonary hypertension. There was no correlation between mild, moderate, and severe grades of bronchopulmonary dysplasia and the degree of pulmonary vein stenosis. There was a median of 2 (range 1-4) diseased pulmonary veins per patient. In total, 41% of the diseased pulmonary veins were atretic. The right upper and left upper lobe pulmonary veins were the most frequently diseased (n = 13/17, 35%, n = 10/17, 27%, respectively). Focal ground glass opacification, interlobular septal thickening, and hilar soft tissue enlargement were always associated with the atresia of an ipsilateral vein. CONCLUSION: Recognition of the focal parenchymal changes that imply pulmonary vein stenosis, rather than chronic lung disease of prematurity changes, may improve the detection of a potentially treatable source of pulmonary hypertension, particularly where nonangiographic studies result in a limited direct venous assessment.

Case Report: Catheter-based mechanical thrombectomy using the Indigo aspiration system in a case of systemic-to-pulmonary shunt thrombosis.

A 53-day-old girl with absent right atrioventricular (AV) connection, malposed great vessels, and pulmonary atresia underwent placement of a central shunt on the sixth day of her life. Her postoperative course was complicated by progressive desaturation, and computed tomographic angiography (CTA) demonstrated near-complete occlusion of her left pulmonary artery (LPA). Angiography demonstrated a nonocclusive thrombus in the distal central shunt and a thrombus with complete occlusion of the LPA. The Indigo aspiration system (Penumbra) was used to remove the thrombus from the central shunt and LPA, allowing placement of a stent in the narrowed LPA. Subsequent angiography showed a wide patient central shunt and LPA. The Indigo aspiration system (Penumbra) provides a viable option for removing thrombus in a patients refractory to other methods.

Management of Complex Pulmonary Vein Stenosis at Altitude Combining Comprehensive Percutaneous Interventional Treatment with Sirolimus, Pulmonary Hypertension Medications and Intraluminal Imaging with Optical Coherence Tomography.

BACKGROUND: Pulmonary vein stenosis (PVS) is a growing problem for the pediatric congenital heart population. Sirolimus has previously been shown to improve survival and slow down the progression of in-stent stenosis in patients with PVS. We evaluated patients before and after initiation of sirolimus to evaluate its effects on re-intervention and vessel patency utilizing Optical Coherence Tomography (OCT). METHODS: We performed a retrospective study, reviewing the charts of patients with PVS, who had been prescribed sirolimus between October 2020 and December 2021. OCT was performed in the pulmonary vein of interest as per our published protocol. Angiographic and OCT imaging was retrospectively reviewed. Statistical analysis was performed using Chi square and Wilcoxon signed-rank test to compare pre-and post-sirolimus data. RESULTS: Ten patients had been started and followed on sirolimus. Median age at sirolimus initiation was 25 months with median weight of 10.6 kg and average follow-up of 1 year. Median total catheterizations were 7 for patients prior to starting sirolimus and 2 after starting treatment (p = 0.014). Comparing pre- and post-sirolimus, patients were catheterized every 3 months vs every 11 months (p = 0.011), median procedure time was 203 min vs 145 min (p = 0.036) and fluoroscopy time, 80 min vs 57.2 min (p = 0.036). 23 veins had severe in-stent tissue ingrowth prior to SST (luminal diameter < 30% of stent diameter). Post-sirolimus, 23 pulmonary veins had moderate to severe in-stent tissue ingrowth that responded to non-compliant balloon inflation only with stent luminal improvement of > 75%. CONCLUSION: Our study suggests that the addition of sirolimus in patients with moderate-severe PVS helps to decrease disease progression with decrease frequency of interventions. Reaching therapeutic levels for sirolimus is critical and medication interactions and side-effects need careful consideration. OCT continues to be important for evaluation and treatment guidance in this patient population.

Two-Stage Biventricular Repair of Complex Aortic Atresia.

We describe the successful 2-stage treatment of an infant with double-outlet right ventricle, aortic valve atresia, normally related great vessels, muscular outlet ventricular septal defect, and ductal arch origin of the cephalic vessels using a hybrid ductal stent and branch pulmonary artery banding followed by a comprehensive Yasui-type biventricular repair.

Computed Tomographic Angiography Provides Reliable Coronary Artery Evaluation in Infants With Pulmonary Atresia Intact Ventricular Septum.

Evaluate the use of coronary CTA as an initial assessment for determining Right Ventricle Dependent Coronary Circulation (RVDCC) in neonates with Pulmonary Atresia with Intact Ventricular Septum (PA IVS). Retrospective review of cases with coronary CTA and compare with available catheter angiography, pathology, surgical reports, and outcomes from Mar 2015 to May 2022. In our cohort of 16 patients, 3 were positive for RVDCC, confirmed by pathologic evaluation, and there was concordance for presence or absence of RVDCC with catheter angiography in 5 patients (4 negatives for RVDCC, 1 positive). Clinical follow up for the 8 patients that underwent RV decompression had no clinical evidence of myocardial ischemia. Our findings suggest that coronary CTA is reliable as first-line imaging for determination of RVDCC in neonates with PA IVS. These findings, if supported by further prospective study, may reserve invasive coronary angiography for cases with diagnostic uncertainty or at the time of necessary transcatheter interventions.

Piggyback mounting for stent and valve deployment during percutaneous pulmonary valve implantation.

OBJECTIVES: We report our experience in simultaneously implanting multiple stents and valves mounted on a single balloon before and during transcatheter pulmonary valve placement. BACKGROUND: Heterogeneity and complexity of the right ventricular outflow tract (RVOT) may complicate stent deployment when preparing a landing zone for transcatheter pulmonary valve implantation. METHODS: Retrospective analysis of patients from Children's Hospital of Colorado, USA; and Oslo University Hospital, Norway, undergoing transcatheter pulmonary valve replacement that had at least two stents mounted on a single balloon, deployed in the RVOT. RESULTS: Over a 42-month period, a total of 50 subjects from the two centers met inclusion criteria for the study. Subjects were predominantly male (58%), and the median age was 17 years (4-78 years). In six subjects (12%), there was need for prestenting with use of the double or triple stent piggyback technique. Forty subjects (80%) had a Melody ™ TPV implanted. In 45 cases (90%), one or more stents were mounted over the pulmonary valve using its delivery system, either the Ensemble for the Melody™ TPV or the Edwards Commander for the SAPIEN 3 THV. Thirty-seven subjects (74%) had one stent mounted and eight subjects (16%) had two stents mounted over the pulmonary valve for simultaneous deployment. No complications related to this technique were reported. CONCLUSIONS: The piggyback technique aims to simplify and facilitate adequate conduit preparation and valve insertion by minimizing manipulation across the outflow tract and decreasing the risk of stent distortion, misalignment, and embolization.

Simultaneous deployment of a covered stent and a Sapien S3 as a bridge to surgical valve replacement in acute infective endocarditis.

A 25-year-old male presented to our institution with acute right ventricular (RV) failure secondary to an infected pulmonary prosthetic valve (Melody® Valve, Medtronic Inc., Minneapolis, MN, USA), 3 months after percutaneous placement. Due to his decompensated state, his surgical risk was determined to be very high during his acute illness. As a bridge to surgical valve replacement, we elected to percutaneously implant a Sapien S3 valve (Edwards Lifesciences, Irvine, CA, USA), mounted within a covered stent and deployed simultaneously to exclude the mass of infected tissue within the RV outflow tract, and improve his hemodynamics. Our patient tolerated the procedure well; recovered fully and returned 2 months later for planned surgical valve replacement. .

Technical Feasibility on the Use of Optical Coherence Tomography in the Evaluation of Pediatric Pulmonary Venous Stenosis.

Pulmonary vein stenosis (PVS) in children is a morbid disease and limited progress has been made in improving outcomes for this heterogenous group of patients. Evaluation is currently limited to imaging techniques that fail to provide an adequate overview of the intraluminal and luminal pathology perpetuating our limited understanding of this condition. Optical coherence tomography (OCT) is an imaging modality which provides intraluminal profiling with microstructural detail through optical reflective technology. We sought to evaluate whether its use was technically feasible in pediatric PVS and whether the imaging data provided potentially useful outputs for clinical utility. Eleven patients were prospectively selected from our cardiac catheterization for OCT evaluation of their pulmonary veins (PV) during elective catheterization for PVS. Measurements were taken both pre and post intervention using both manual and automated tools. Stent morphology was characterized. Eleven patients had evaluation of 34 pulmonary veins, with 7 patients having more than one assessment, for a total of 25 overall catheterizations. Most patients were female (75%). Median age at cardiac catheterization was 35 months (range 5-45 months). Median weight of subjects was 10.6 kg (3.7-14.2) with a median BSA documented at 0.505 m2 (0.21-0.57). Median number of pulmonary veins involved was 3, (range 1-5 veins) and median contrast volume of 2.9 mL/kg (0.7-3.7) given. Median radiation dose (DAP) was 6095 µGy·cm2 (1670-12,400). Median number of previous cardiac catheterizations was 7 (range 1-11). All of the vessels with a diameter < 5 mm were adequately visualized. Of all the OCT images acquired, in 15 vessels (44%) contrast was used to clear the vessels from blood as an angiogram was required at the time, in the other 19 vessels (56%), saline was used with adequate imaging. There were no complications related to OCT. OCT is technically feasible to use in pediatric patients without any directly related complications. It provides intraluminal anatomy in children with both native and treated pulmonary venous stenosis when vessel size is less than 5 mm.

Baseline Values of the Compensatory Reserve Index in a Healthy Pediatric Population.

The objective of this study is to describe the compensatory reserve index (CRI) baseline values in a healthy cohort of healthy pediatric patients and evaluate the existing correlation with other physiological parameters that influence compensatory hemodynamic mechanisms. CRI is a computational algorithm that has been broadly applied to non-invasively estimate hemodynamic vascular adaptations during acute blood loss. So far, there is a lack of baseline values from healthy individuals, which complicates accurately estimating the severity of the hemodynamic compromise. Additionally, the application of this technology in pediatric populations is limited to a few reports, highlighting a marked variability by age, weight, and other physiological parameters. The CRI of 205 healthy subjects from 0 to 60 years of age were prospectively evaluated from January to February 2020 at several public outpatient clinics in El Salvador; vital signs and sociodemographic data were also collected during this period. After data collection, baseline values were classified for each age group. Multiple correlation models were tested between the CRI and the other physiological parameters. CRI value varies significantly for each age group, finding for patients under 18 years old a mean value lower than 0.6, which is currently considered the lower normal limit for adults. CRI presents strong correlations with other physiological variables such as age, weight, estimated blood volume, and heart rate (R > 0.8, R2 > 0.6, p < 0.0001). There is significant variability in the CRI normal values observed in healthy patients based on age, weight, estimated blood volume, and heart rate.