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.

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.

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.

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.

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.

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.

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.

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.

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.

Implementation of virtual reality for patient distraction during diagnostic cardiac catheterisation.

Until now, the application of virtual reality as a distraction model has been widely described in the medical field, showing different benefits offered on patient's perception, particularly related to pain and anxiety. Previous clinical experience of virtual reality applications on surgical intervention has shown how during procedures with local anaesthesia, this modality improves patients' experience without changing times, costs, and clinical outcomes. Herein, we report our experience with three patients during diagnostic cardiac catheterisation, showing the effect of this technology on patients' perception and metrics during the procedure.

Baseline intracardiac echocardiography predicts haemodynamic changes and Doppler velocity patterns during follow-up after percutaneous pulmonary valve implantation.

BACKGROUND: Intracardiac echocardiography Doppler-derived gradients have previously been shown to correlate with post-procedure echocardiographic evaluations when compared with invasive gradients measured during percutaneous pulmonary valve implantation, suggesting that intracardiac echocardiography could offer an accurate and predictable starting point to estimate valve function after percutaneous pulmonary valve implantation. METHODS: We performed a retrospective chart review of 51 patients who underwent percutaneous pulmonary valve implantation between September 2018 and December 2019 in whom intracardiac echocardiography was performed immediately after valve implantation. We evaluated the correlation between intracardiac echocardiography gradients and post-procedural Doppler-derived gradients. Among the parameters assessed, those which demonstrated the strongest correlation were used to create a predictive model of expected echo-derived gradients after percutaneous pulmonary valve implantation. The equation was validated on the same sample data along with a subsequent cohort of 25 consecutive patients collected between January 2020 and July 2020. RESULTS: All the assessed correlation models between intracardiac echocardiography evaluation and post-procedure transthoracic echocardiographic assessments were statistically significant, presenting moderate to strong correlations. The strongest relationship was found between intracardiac echocardiography mean gradients and post-procedural transthoracic echocardiographic mean gradients. Therefore, an equation was created based on the intracardiac echocardiography-derived mean gradient, to allow prediction of the post-procedural and follow-up transthoracic echocardiographic-derived mean gradients within a range of ±5 mmHg from the observed value in more than 80% of cases. CONCLUSIONS: There is a strong correlation between intracardiac echocardiography and post-procedure transthoracic echocardiographic. This allowed us to derive a predictive equation that defines the expected transthoracic echocardiographic Doppler-derived gradient following the procedure and at out-patient follow-up after percutaneous pulmonary valve implantation.

Novel Minimal Radiation Approach for Percutaneous Pulmonary Valve Implantation.

The aim of the study is to evaluate the impact of multimodality imaging technology during percutaneous pulmonary valve implantation (PPVI). Among percutaneous procedures, PPVI traditionally has one of the highest patient radiation exposures. Different protocol modifications have been implemented to address this problem (i.e., improvements in guidance systems, delivery systems, valve design, post-implantation evaluation). Although the effectiveness of individual modifications has been proven, the effect of an approach which combines these changes has not been reported. We performed a retrospective chart review of 76 patients who underwent PPVI between January 2018 and December 2019. Patients were classified in "Traditional protocol," using routine biplane angiography and/or 3D rotational angiography (3DRA); and "Multimodality protocol" that included the use of VesselNavigator for guidance, selective 3DRA for coronary evaluation, Long DrySeal Sheath for valve delivery, and Intracardiac Echocardiography for valve evaluation after implantation. Radiation metrics, procedural time, and clinical outcomes were compared between groups. When the traditional protocol group was compared with the multimodality protocol group, a significant reduction was described for total fluoroscopy time (31.6 min vs. 26.2 min), dose of contrast per kilogram (1.8 mL/Kg vs. 0.9 mL/Kg), DAP/kg (26.6 µGy·m2/kg vs. 19.9 µGy·m2/kg), and Air Kerma (194 mGy vs. 99.9 mGy). A reduction for procedure time was noted (140 min vs. 116.5 min), but this was not statistically significant. There was no difference in clinical outcomes or the presence of complications between groups. The combination of novel technology in PPVI caused a significant reduction in radiation metrics without increasing the complication rate in our population.

Coronary compression testing by balloon interrogation during pulmonary valve implantation: room for doubt?

OBJECTIVE: To evaluate the reliability of balloon coronary compression testing during percutaneous pulmonary valve implantation. BACKGROUND: Despite the widespread use of the 'balloon coronary test' as the preferable method to rule out the risk of coronary compression, this adverse event has been described after pulmonary valve implantation where coronary balloon test suggested no risk or low risk, calling into question the accuracy of the test. METHODS: We performed a retrospective chart review of 84 patients who underwent pulmonary valve implantation between January 2018 and December 2019 and selected 36 patients whose archived imaging was suitable to perform quantitative analysis of the 'balloon coronary test'. We focused on the spatial disparity between the right ventricular outflow tract position defined by the inflated testing balloon and the eventual implanted valve position, to classify the test as inaccurate or accurate. RESULTS: In total, 36.1% of cases were classified as having an inaccurate coronary balloon test. Among the baseline characteristics, right ventricular outflow tract substrate was identified as a significant predictor of test accuracy. Related to this characteristic, the type of testing balloon used and the size of the eventually implanted valve were found to be associated with test accuracy. CONCLUSIONS: Based on our findings, balloon coronary testing is not an accurate method of predicting final valve position with respect to fixed structures in the thorax. This may translate to a high false positive rate for the likelihood of coronary compression in pulmonary valve implantation.

The Compensatory Reserve Index Responds to Acute Hemodynamic Changes in Patients with Congenital Heart Disease: A Proof of Concept Study.

Patients with congenital heart disease (CHD) who undergo cardiac procedures may become hemodynamically unstable. Predictive algorithms that utilize dense physiologic data may be useful. The compensatory reserve index (CRI) trends beat-to-beat progression from normovolemia (CRI = 1) to decompensation (CRI = 0) in hemorrhagic shock by continuously analyzing unique sets of features in the changing pulse photoplethysmogram (PPG) waveform. We sought to understand if the CRI accurately reflects changing hemodynamics during and after a cardiac procedure for patients with CHD. A transcatheter pulmonary valve replacement (TcPVR) model was used because left ventricular stroke volume decreases upon sizing balloon occlusion of the right ventricular outflow tract (RVOT) and increases after successful valve placement. A single-center, prospective cohort study was performed. The CRI was continuously measured to determine the change in CRI before and after RVOT occlusion and successful TcPVR. Twenty-six subjects were enrolled with a median age of 19 (interquartile range (IQR) 13-29) years. The mean (± standard deviation) CRI decreased from 0.66 ± 0.15 1-min before balloon inflation to 0.53 ± 0.16 (p = 0.03) 1-min after balloon deflation. The mean CRI increased from a pre-valve mean CRI of 0.63 [95% confidence interval (CI) 0.56-0.70] to 0.77 (95% CI 0.71-0.83) after successful TcPVR. In this study, the CRI accurately reflected acute hemodynamic changes associated with TcPVR. Further research is justified to determine if the CRI can be useful as an early warning tool in patients with CHD at risk for decompensation during and after cardiac procedures.

Using in vitro model to assess stent-stent interaction in patients with coronary artery compression.

BACKGROUND: Percutaneous stent implantation in the coronary artery is a potential bailout option after coronary compression during Transcatheter pulmonary valve replacement, but the structural integrity of the coronary stent in this environment is uncertain. METHODS: An invitro model was constructed to represent the interaction between coronary and outflow tract stents. One AndraStent, four CP stents, one Edwards SAPIEN XT valve stent and combination of AndraStent with CP stent were incrementally compressed against a coronary stent. The stent diameters were measured with a code developed in MATLAB. The relative decrease in diameter was calculated for each stent over a range of compression. RESULTS: In all combinations of stents, coronary stent lost significantly more relative diameter when compared with larger outflow tract stents. At maximum compressive amplitude, average percent decrease in diameters of AndraStent(r) versus coronary stent: 8.1 ± 0.8% and 36.5 ± 11.1% respectively. CP stent™ versus coronary stent, the decrease was 5.7 ± 0.8% and 45.2 ± 9.2% respectively. Combined CP stent™ mounted within the AndraStent(r), versus coronary stent: 3.9 ± 0.5% and 54.5 ± 4.2% respectively. Edwards Sapien stent versus the coronary stent: 1.7 ± 0.1% and 68.9 ± 1.1% respectively. CONCLUSION: The relative deformation of the coronary stent was significantly higher than deformation of the other stents. Edwards Sapien XT valve stent has greater relative radial force demonstrated by the greater relative decrease in diameter of the coronary stent at final compressive amplitude. These results support the concern that coronary stenting in the presence of coronary compression by an outflow tract stent implantation is likely to result in a significantly compromised coronary lumen.

Comparison of Patients Undergoing Surgical Versus Transcatheter Pulmonary Valve Replacement: Criteria for Referral and Mid-Term Outcome.

Pulmonary regurgitation and/or stenosis (PS) is challenging in patients with congenital heart defects. Our aim was to identify if criteria for referral were different between surgical (SPVR) and transcatheter pulmonary valve replacement (TPVR) populations, and to further assess if any baseline differences influence the resultant ventricular remodeling at medium-term follow-up. Retrospective chart review of patients post-SPVR or TPVR at our center from 2013 to 2015 was conducted. Volumetric data from cardiac magnetic resonance (CMR), 1 year before and 1 year after PVR was obtained. PS was defined as peak-peak gradient ≥35 mmHg by catheterization or peak gradient ≥50 mmHg by echocardiography. Thirty patients underwent PVR: 15 SPVR and 15 TPVR (1 hybrid). The indications for SPVR referral were: 2+ CMR parameters in 80% of patients; decreased left ventricular ejection fraction and hemodynamic findings and/or abnormal exercise stress test in 20%. The indications for TPVR referral were predominantly symptoms ± hemodynamic findings in 66% of patients, 2+ CMR findings in 44% of patients. At referral, SPVR group had significantly larger right ventricular (RV) volumes than TPVR group. Biventricular function was not significantly different. Post-PVR, both groups had significantly decreased RV volumes and increased LV diastolic volumes. The SPVR group improved LV cardiac output and biventricular function whereas TPVR group had no significant improvement. The patients in the SPVR group were mostly referred based on CMR volumetric criteria, whereas the patients in the TPVR group were mostly referred due to exercise intolerance with only occasional abnormalities on CMR. 1 year after PVR, both groups had near-normal biventricular volumes and function irrespective of characteristics at referral.

Subclinical Decrease in Myocardial Function in Asymptomatic Infants of Diabetic Mothers: A Tissue Doppler Study.

Infants of diabetic mothers (IDMs) with hypertrophic cardiomyopathy are recognized to have impaired myocardial performance, but less is known about ventricular function in IDMs without hypertrophy. We hypothesized that in asymptomatic newborns with normal two-dimensional echocardiographic evaluations, pulsed wave tissue Doppler imaging (TDI) would suggest a subclinical decrease in the cardiac function of IDMs compared to infants of non-diabetics (nIDMs). This is a retrospective cohort study of asymptomatic neonates ≥36 weeks gestation, at 0-7 days of life, with normal standard echocardiograms. Systolic (S'), early diastolic (E'), and late diastolic (A') TDI velocities were measured at the mitral valve (MV) annulus, basal interventricular septum (IVS), and tricuspid valve (TV) annulus, and averaged from three consecutive cardiac cycles. Demographic, perinatal, and echocardiographic variables were compared between IDM and nIDM groups. Of 631 subjects, 75 IDMs were identified. The mean gestational age of the entire cohort was 39.33 weeks (±1.26), birth weight 3.44 kg (±0.56), and body surface area (BSA) 0.21 m2 (±0.02). IDMs had significantly greater birth weight and BSA, lower gestational age, older maternal age, and higher incidence of maternal obesity and hypertension than nIDMs (p < 0.001). On multivariable analysis, IDMs had significantly lower S' (p ≤ 0.03) and E' (p < 0.001) velocities, and higher E/E' ratios (p < 0.001) at the MV, IVS, and TV than nIDMs. In asymptomatic newborn IDMs without cardiac hypertrophy, pulsed wave TDI suggests a subclinical decrease in systolic and diastolic myocardial function compared to nIDMs.