The World Society for Pediatric and Congenital Heart Surgery: 2021 Update of the World Database for Pediatric and Congenital Heart Surgery.

BACKGROUND: The World Database for Pediatric and Congenital Heart Surgery (WDPCHS), sponsored by the World Society for Pediatric and Congenital Heart Surgery (WSPCHS), provides complex programmatic outcomes analyses for all members of the WSPCHS. METHODS: The Data center, currently at Kirklin Institute for Research in Surgical Outcomes (KIRSO), University of Alabama, Birmingham (USA), provides biannual reports to all active members of the database. This report presents a descriptive analysis of these procedures submitted from January 1, 2017 to December 31, 2020. RESULTS: A total of 37,386 procedures were submitted with an overall mortality of 4.3%. The majority of submissions were from Asian countries. The majority of cases submitted from these countries were of Society of Thoracic Surgeons (STS)-European Association for Cardio-Thoracic Surgery (STAT) Mortality Categories I and II. CONCLUSIONS: The WSPCHS accomplished one of its missions in 2017 when the WDPCHS began accepting data from pediatric and congenital heart surgery programs across the globe. In doing so, it became one of the first organizations to create a platform for the exchange of knowledge and experience, regardless of the socioeconomic status of the particular program or country.

Partial Anomalous Pulmonary Venous Connection Repair: Customized Approach and Outcomes.

Alternative options for the correction of partial anomalous pulmonary venous connection (PAPVC) have been proposed. Each can be associated with variable risk for dysrhythmias, caval or pulmonary venous (PV) obstruction. A selective customized strategy to address PAPVC taking into account atrial shunt (AS) and growth potential was pursued. Between September 2014 and August 2018 21 PAPVC patients were identified. Two levels of reference determined the chosen repair strategy; azygous vein (AzV) and cavoatrial junction (CAJ). Six (Group-A) with PAPVC entering SVC cephalad to AV underwent a combined in-situ cavoatrial autologous reconstruction with atrial appendage advancement flap (CARAF). PAPVC entering caudally to AzV (Group-B) underwent alternative repair (caval division/Warden-type or intraatrial rerouting) (n = 15). Age was 8.3 (IQR:4.2-18.5) years for Group-A (vs 11.9; IQR:8.8-34.7 in Group-B) (p = 0.07). In Group-A 5(83%) had AS (vs 12[80%] Group-B; p = 0.9). None had left SVC in Group-A (vs 1 in Group-B; p = 0.9). Preoperative advanced imaging and echocardiographic hemodynamic evaluation was undertaken. Follow-up was complete (median 2.9; IQR:1.2-3.7 years). Freedom from atrial dysrhythmias, caval or PV obstruction was assessed. There were no early or late deaths. ICU and hospital length of stay were 1.8 ± 1.1 and 3.2 ± 0.5 days, respectively. No atrial dysrhythmias occurred postoperatively in Group-A (vs 1 in Group-B; p = 0.9). No permanent pacemaker was implanted. All patients remained in normal sinus rhythm. There were no early or late caval/PV obstruction. A customized approach reserves the advantages of each technique tailored to patient's needs. Expanding surgical capacity with favorable outlook for all PAPVC variations, irrespective of association with AS, can maximize efficiency and reproducibility paired with the lowest morbidity.

Central Vascular Thrombosis in Neonates with Congenital Heart Disease Awaiting Cardiac Intervention.

Central vascular thrombosis (CVT) in critically ill neonates carries significant clinical implications. Neonates with congenital heart disease (CHD) awaiting cardiac intervention might be associated with increased risk of thrombosis. Outcome analysis was undertaken. An analysis of 77 neonates with CHD who were admitted to the NICU prior to cardiac intervention between January 2015 and December 2016 was undertaken. Patients requiring extracorporeal life support prior to any cardiac intervention, or receiving prophylactic anticoagulation not related to central vascular catheter (CVC) were excluded. Diagnosis of CVT was provided based on clinical indication and verified with imaging that warranted anticoagulation therapy. Location of CVC and extent of CVT along with treatments, outcomes, and vascular access types and durations were assessed. Logistic regression multivariate analysis was used to assess predictors of outcome. Neonates with CHD were complicated with CVT in 10.4%. Longer duration of CVC was also associated with thrombosis in neonates with CHD (72.7 days vs. 29.3 days, p < 0.001). Independent predictors of outcome included lower gestational age and CHD with single-ventricle (SV) anatomy (p < 0.05). In neonates with CHD awaiting cardiac intervention risk of CVT is substantial. Duration CVC, lower gestational age and SV anatomy are risk determinants of outcome. Standardized development of customized surveillance protocols tailored to this unique subsets of neonates and adherence to quality guidelines can influence outcome.

Right Ventricular Outflow Tract Reintervention in the Transcatheter Era: Outcomes and Cost Analysis.

Surgical pulmonary valve insertion (SPVI) for re-entry right ventricular outflow tract intervention (RVOTI) remains an established and reproducible approach. Fast-track in patients undergoing RVOTI of the comprehensive valve program targets early ICU and hospital discharge (Hd). Feasibility study for outcome and cost analysis was undertaken. Between January 2015 and December 2016, 34 patients underwent re-entry RVOTI. Seventeen had SPVI and 17 transcatheter PVI (TPVI). Surgical perioperative fast-track protocol was used. Echocardiographic evaluation preoperatively (TTE-1), after RVOTI (TTE-2), at hospital discharge (TTE-3), and follow-up (TTE-4) were obtained. Cost Analysis included procedural and hospital costs. Mean follow-up period was 11.3 ± 6.9 months. All patients were extubated prior to ICU arrival. Mean age was 8.5 ± 7.8 for SPVI [vs 28.5 ± 8.6 years for TPVI] (p < 0.05). There was no hospital mortality or 30-day readmission for SPVI (versus 1 for TPVI).Mean hospital length of stay (LOS) was 4.1 ± 1.1 days for SPVI [vs 1.1 ± 0.7 days for TPVI] (p < 0.05). Number of prior sternal re-entry had no influence on outcome. RV systolic pressure referenced to LVSP (rRVSP, %) and diastolic dimension (RVEDDi, z score) showed sustainable improvement (TTE-2, TTE-3, TTE-4) in both groups compared to TTE-1 (p < 0.05). Mean total hospital cost was $5475.86 ± 2503.91 lower after SPVI (p = 0.09), 21.7% procedural cost reduction. Patients undergoing RVOTI can be safely stratified, based on a customized concept, towards SPVI or TPVI. Standardized strategy can advocate a fast-track path. SPVI is associated with comparable mid-term outcomes to TPVI although SPVI is delivered in younger patients. Despite longer LOS SPVI is associated with reduced hospital cost. Multisite studies might help determine suitability for each strategy on cost containment/quality of life basis.

Standardized Approach to Intervention for Intestinal Malrotation in Single Ventricle Patients with Heterotaxy Syndrome: Impact on Interstage Attrition and Time to Superior Cavopulmonary Connection.

Heterotaxy syndrome (HS) is a significant determinant of outcome in single ventricle (SV) physiology. Attrition rate and time-related events associated with intestinal malrotation (IM) are, yet, to be determined. We sought to evaluate hospital and interstage outcomes in relation with operative intervention for IM (IMO). Twelve SV/HS patients, who underwent IMO, from January 2004 to December 2016, were studied. Early shunt failure, time to superior cavopulmonary connection (SCPC) and interstage attrition were assessed. Since September 2014, based on a comprehensive standardized protocol, IMO was performed at the time of hospitalization for stage-I palliation (S1P) irrespective of clinical manifestations. Patients were assigned to Group A (n = 8): expectant /symptoms-driven versus Group B (n = 4): protocol-driven. At S1P 7 had systemic-to-pulmonary shunt (SPS), 1 SPS with anomalous pulmonary venous return (APVR) repair (Group A) compared to 2 SPS, 1 SPS with APVR repair and 1 Norwood operation (Group B). Median duration from S1P to IMO was 82 days (range 57-336; Group A) compared to 14 days (range 11-31; Group B); p < 0.05. Median age at IMO was 87 days (range 8-345) [Group A: 99 days (range 68-345) vs Group B: 25 days (range 8-39)] (p < 0.05). Early SPS failure occurred in 25% (2 of 8) for Group A compared to none in Group B (p < 0.05). Hospital mortality following IMO was 25% [Group A: 37.5% (3 of 8) vs Group B: 0; p < 0.05]. Interstage survival was 67% [Group A: 50% (4 of 8) vs Group B: 100%; p < 0.05]. Time to SCPC following S1P was 186 days (range 169-218) for Group A compared to 118 days (range 97-161) (Group B); p < 0.05. Operative intervention for IM in SV/HS is associated with significant interstage attrition and might impact the time to SCPC. SPS is at risk for early failure after IMO. A comprehensive standardized concept can mitigate detrimental implications.

Long noncoding RNA NEAT1 (nuclear paraspeckle assembly transcript 1) is critical for phenotypic switching of vascular smooth muscle cells.

In response to vascular injury, vascular smooth muscle cells (VSMCs) may switch from a contractile to a proliferative phenotype thereby contributing to neointima formation. Previous studies showed that the long noncoding RNA (lncRNA) NEAT1 is critical for paraspeckle formation and tumorigenesis by promoting cell proliferation and migration. However, the role of NEAT1 in VSMC phenotypic modulation is unknown. Herein we showed that NEAT1 expression was induced in VSMCs during phenotypic switching in vivo and in vitro. Silencing NEAT1 in VSMCs resulted in enhanced expression of SM-specific genes while attenuating VSMC proliferation and migration. Conversely, overexpression of NEAT1 in VSMCs had opposite effects. These in vitro findings were further supported by in vivo studies in which NEAT1 knockout mice exhibited significantly decreased neointima formation following vascular injury, due to attenuated VSMC proliferation. Mechanistic studies demonstrated that NEAT1 sequesters the key chromatin modifier WDR5 (WD Repeat Domain 5) from SM-specific gene loci, thereby initiating an epigenetic "off" state, resulting in down-regulation of SM-specific gene expression. Taken together, we demonstrated an unexpected role of the lncRNA NEAT1 in regulating phenotypic switching by repressing SM-contractile gene expression through an epigenetic regulatory mechanism. Our data suggest that NEAT1 is a therapeutic target for treating occlusive vascular diseases.

Developing a measure to assess identity reconstruction in patients with multiple sclerosis.

PURPOSE/OBJECTIVE: To develop and test initial validation of a theory-driven quantitative measure of identity reconstruction in patients with multiple sclerosis (MS) based upon previous qualitative research. Research Method/Design: This study uses a cross-sectional survey design, in which 137 patients living with MS were recruited from an outpatient MS Center in the Southeastern U.S. Participants completed demographic items, Identity Reconstruction Assessment Scales (IRAS), Patient Determined Disease Steps, Medical Outcomes Study Measures of Patient Adherence, Chronic Disease Self-Efficacy Scale, Hospital Anxiety and Depression Scale, Leeds MS Quality of Life Questionnaire, MS-Related Stigma Scale, and Posttraumatic Growth Inventory-Short Form. RESULTS: The IRAS conformed to a 3-factor solution consisting of 23 items accounting for 42.6% of the variance. The 3 factors, labeled as "sustained identity" (α = .84), "reactionary identity" (α = .74), and "integrated identity" (α = .65), were not significantly correlated with each other, necessitating and allowing for independent scoring of the scales. Higher scores on "sustained identity" scale were associated with less anxiety, depression, perceived disability, and MS-related stigma, as well as with increased self-efficacy, treatment adherence, and quality of life. "Reactionary identity" scale was positively correlated with anxiety and MS-related stigma. "Integrated identity" was significantly associated with age and perceived disability. CONCLUSIONS/IMPLICATIONS: Identity reconstruction provides needed context for understanding adjustment to and living with MS. Examination of the IRAS within a larger sample and in other disease groups can provide additional construct validity evidence. (PsycINFO Database Record