PEDIATRIC CARDIOLOGY

Transitioning from the safety of the womb to the outside world for neonates with life-threatening cardiovascular conditions: The IMmediate Postpartum Access to Cardiac Therapy (IMPACT) Procedure
Padiyath A, Lynch JM, Montenegro LM, Nicolson SC, Nelson O, Szwast AL, Shillingford AJ, Falkensammer CB, Savla JJ, Moldenhauer J, Khalek N and Rychik J
The IMmediate Postpartum Access to Cardiac Therapy (IMPACT) procedure is a multidisciplinary, collaborative, highly coordinated clinical service in which a planned delivery and intensive neonatal care are offered for conditions where there is a high likelihood of postnatal instability. This process includes prenatal consultation with the parent(s), involving each service engaged with the delivery, postnatal resuscitation, and procedural care. A Cesarean section delivery is planned in an operating room with immediate access to a multifunctional procedural suite where the neonate can undergo rapid cardiac evaluation and initiation of interventional treatments which can have a positive, life-saving impact. This review describes the details of this unique procedure and multidisciplinary program at one institution.
Valve Frame Geometry and Arrhythmia Risk Following Self-Expanding Transcatheter Pulmonary Valve Replacement
Soszyn N, Yuen G, Franco SR, von Alvensleben JC, Morgan GJ and Zablah JE
Though major complications during transcutaneous pulmonary valve replacement (TPVR) are rare, clinically-significant ventricular arrythmia (CSVA) has been reported following self-expanding valve placement. We assess whether alterations in valve frame dimensions and geometry within the right ventricular outflow tract (RVOT) post-implantation in patients who underwent TPVR with Harmony TPV25 or Alterra contribute to CSVA risk. A single center review was performed of patients who underwent TPVR with either Harmony TPV25 or Alterra Pre-stent between August 2019 and April 2023. Using post-procedural 3D rotational acquisitions, minimum and maximum diameters were measured at 5 locations along the valve frame and perimeter and cross-sectional area (CSA) were measured closest to the pulmonary artery bifurcation and right ventricular (RV) cavity. To assess the relationship between the RVOT and valve frame, a RVOT-to-valve frame ratio together with percentage expansion, circularity and expansion ratios, and eccentricity indices were calculated. Twenty-eight patients were included (14 Harmony TPV25, 14 Alterra pre-stent). CSVA was seen more often in patients with congenital pulmonary stenosis (p = 0.02). CSVA was associated with a larger mean valve frame perimeter (118.3 vs 108.6 mm, p = 0.03) and CSA (1124.5 vs 926.2mm, p = 0.03) closest to the RV cavity. No associations between difference in diameters, RVOT-to-valve frame ratio, parameters evaluating valve frame geometry, and level of implant and CSVA were demonstrated. No single mechanism was identified that contributed to CSVA in patients following self-expanding valve implantation. Future studies implementing these mathematical constructs and measurements to a larger cohort of self-expanding valve patients may yield more instructive results.
Impact of Acute Energy Drink Consumption on Heart Rate Variability in Children and Adolescents. A Randomized Trial
Mandilaras G, Li P, Dalla-Pozza R, Jakob A, Haas NA and Oberhoffer FS
The EDUCATE study investigated the acute impact of energy drink (ED) consumption on heart rate variability (HRV) in children and adolescents, with a focus on how these stimulant-rich beverages influence cardiac autonomic function. Given the popularity of EDs among young people, this study assessed the immediate cardiovascular response to ED intake. This randomized, double-blind, placebo-controlled crossover trial involved 26 healthy participants aged 10-18 years. Each participant consumed a weight-adjusted ED or placebo in two separate test sessions. HRV was monitored via a 24-h Holter ECG, with analysis centered on time-domain measures, such as the standard deviation of normal RR intervals (SDNN) and root-mean square of successive differences (RMSSD), along with frequency-domain metrics. Statistical analysis included a two-way repeated-measures ANOVA to examine the effects of "beverage" and "time." The study revealed a significant increase in the SDNN in the ED group within the first hour post-consumption, with a mean difference of 17.692 ms compared with that in the placebo group (SDNN: ED = 133.346 ± 50.217 ms vs. placebo = 115.654 ± 47.583 ms, p = 0.023). However, no significant differences in the SDNN were observed in the subsequent time intervals (60-240 min). In addition, frequency-domain parameters, such as total power, RMSSD, LF, HF, and the LF/HF ratio, showed no significant changes across the four-hour observation period, indicating that sympathetic activation was transient. The findings suggest that ED consumption in children and adolescents leads to a temporary increase in autonomic activity, marked by elevated SDNN, without lasting dysregulation. While the cardiovascular effects are brief, acute sympathetic activation underscores the need for regulated ED intake among minors. Further studies are recommended to explore the long-term effects of regular ED consumption on cardiovascular health in youth.
When High-Risk Ductal Stenting Goes Wrong: Use of Percutaneous VV ECMO Support and Acute Left Pulmonary Artery Loss
Dalby S, Ehsan L, Dossey A, Greiten L and Angtuaco M
Patent ductus arteriosus (PDA) stenting is a vital intervention for neonates with ductal-dependent blood flow, offering an attractive alternative to surgical shunt placement. Despite its benefits, the procedure poses risks such as ductal spasm, branch pulmonary artery compromise, and pseudoaneurysm formation. This report presents two complex neonatal cases with distinct outcomes. The first patient experienced severe ductal spasm, pseudoaneurysm formation, and ductal dissection. Innovative use of percutaneous venovenous extracorporeal membrane oxygenation (VV ECMO) provided hemodynamic stability, enabling staged interventions and eventual successful ductal stenting. The second patient experienced acute left pulmonary artery flow loss due to ductal spasm. Transcatheter attempts to restore flow failed, necessitating surgical stent removal and Blalock-Taussig-Thomas shunt placement. These cases highlight the challenges of PDA stenting in complex ductal anatomies and the importance of advanced imaging, careful wire and catheter selection, and multidisciplinary collaboration. Notably, the first reported use of percutaneous VV ECMO during PDA stenting demonstrates its potential as a lifesaving adjunct for these cases.
Screening for Structural Heart Defects: A Single-Center Retrospective Cost Analysis for Fetal Echocardiography in Adults with Congenital Heart Disease
Leone DM, Ittleman B, Virk K, Albright C, Arya B and Deen J
Fetal echocardiography (FE) is recommended for parents with congenital heart disease (pCHD) due to a 3-6% recurrence risk of congenital heart disease (CHD). This study aimed to evaluate the cost of FE for detecting neonatal CHD in pCHD. FE data were collected between 12/2015 and 12/2022. Parents were stratified by CHD complexity: "simple" (class I) and "complex" (class II/III). Cost analysis compared universal FE with selective FE following a positive level II screening anatomical ultrasound (SAU). Primary outcomes included the cost and number needed to screen (NNT) to detect one case of neonatal CHD. Of 419 pCHD cases, 48 were analyzed separately due to additional FE indications. Among the remaining 371 cases (73% maternal, 27% paternal; mean maternal age: 31 years), 14 postnatal CHD cases were detected (3.8%). Recurrence rates were 1.9% for simple pCHD (n = 156) and 5.1% for complex pCHD (n = 215). Universal FE increased the cost of detecting neonatal CHD. The cost per detected case was $267,157 for simple CHD (NNT = 560) and $135,125 for complex CHD (NNT = 288). The lower sensitivity of SAU reduced the cost of universal FE. In this single-center cohort, the recurrence risk of CHD in pCHD is higher than in the general population, particularly in complex cases. Universal screening in simple pCHD is costlier with high-sensitivity SAU. Targeted screening in complex pCHD may offer a better cost-to-risk ratio, highlighting the need for early detection to improve outcomes. The cost effectiveness is dependent on local SAU sensitivity rates.
Epicardial Pacemaker Lead Related Cardiac Strangulation: The Importance of Early Recognition
Kendall S, Agouba R, Murray J, Morrison ML, McCrossan B, Grant B, Sands A, Casey F and Nolke L
Lead strangulation is a dangerous complication of epicardial pacemaker insertion. This complication has been increasingly highlighted lately. Our institution has recently identified four cases over the past five years. This study's aim was to 1) identify risk factors for strangulation and 2) prospectively screen existing epicardial pacemaker patients for unrecognized strangulation or features that would prompt closer review. Patients known to the pacemaker clinic with epicardial pacemakers inserted from 2005 to 2023 were included. Electronic health records were used to locate all subjects and gather data. Risk factors were identified using Firth's penalized method of logistic regression. Forty-five patients were included, of which four (8.8%) had evidence of strangulation. Posterior-anterior (PA) chest radiographs all demonstrated characteristic looping patterns of the pacing leads, with confirmation on CT angiography. All affected patients underwent revision surgery. Implantation at a weight of less than 6.5 kg was associated with a significantly increased incidence of strangulation (OR 25, P 0.044). Other factors including lead length, presence of structural cardiac disease, and dual-chamber insertion were not statistically significant. No patients who were prospectively screened were found to have strangulation. Children undergoing insertion of a pacemaker early in infancy are at particularly high risk of strangulation and should be closely monitored following surgery. Regular chest radiography (every three years) to screen for this complication is advised. Larger multi-center studies to pool data for this relatively rare complication may help identify other risk factors for strangulation.
Pulmonary Atresia with Intact Ventricular Septum, an Evolving Strategy in the Era of PDA Stenting: Single Center Experience
Shibbani K, Nigro J, Rao R, Gordon BM, Justino H, AlShawabkeh L and El-Said H
Repair or palliation of pulmonary atresia with intact ventricular septum (PA/IVS) often falls into one of 4 categories: cardiac transplant, 2-ventricular circulation, 1.5 ventricle circulation, or single ventricle circulation. The optimal management strategy has been an area of much debate. We sought to review the management strategy of patients with PA/IVS at our institution to better understand what metrics can be used to guide management and initial interventions. The study aims to examine the outcomes of a single-center approach to managing patients with PA/IVS. Our cohort included 29 patients; one patient underwent a planned transplant at ten days of life (3.4%), 12 underwent repair via a two-ventricle circulation (41.4%), 7 underwent repair with 1.5 ventricle circulation (24.1%), and 7 underwent repair with single ventricle circulation (24.1%). Survival was achieved in 93.1% with two patients (6.9%) expiring. The TV annulus z-score was significantly different between the three groups, with the 1 V group having the smallest median TV annulus z-score at - 4.04 (IQR - 4.60- - 3.60) and the 2 V group having the largest median TV z-score at - 1.4 (IQR - 2.24- - 0.12). Six patients underwent late right ventricular decompression. We present a post-hoc algorithm to help guide treatment strategies for patients with PA/IVS.
Correction: The Relationship Between Ambulatory Blood Pressure and Atherogenic Index with LV Geometry Pattern in Obese Children: Results from a Cross-Sectional Study in Serbia
Bojko B, Claudia S, Vladimir V, Aleksandra K, Stefan S, Marko J, Ilija B and Maciej B
Prenatal Diagnosis of Congenital Heart Disease in Liveborn Infants in the New England Region
Haxel CS, Wang A, Levine JC, Drucker N, Hart MR, Glatz JA, Ferdman D, Karnik R, Tsirka A, Arya P, Doherty M, Laraja K, Hagenbuch S and Rotondo KM
Prenatal diagnosis of congenital heart disease requiring early cardiac catheterization or surgical intervention enables optimal delivery planning for appropriate postnatal cardiovascular intervention and care. This allows for improved morbidity and mortality. Prior national data reported prenatal diagnosis rates of 32% for congenital heart disease requiring intervention in infants in the first 6 months of life in the New England region. With improved technology, access to care and changes to the obstetrical ultrasound guidelines for mid trimester fetal study, it is expected that diagnostic rates should improve. The New England Congenital Cardiology Association (NECCA) conducted a quality improvement study to determine the rates of prenatal detection in the current era with the hypothesis that there has been improvement in detection rates in this region. Ten of fourteen medical centers delivering pediatric cardiology care in New England contributed prenatal diagnosis data for 286 infants born at the participating centers during a one year period. The overall prenatal detection rate was 68%. Detection rates ranged from 39 to 90%. When fetal echocardiogram was performed at a pediatric cardiology center, the detection rate was 95% with only 7 moderate (7/195; 3.6%) and 3 severe (3/195; 1.5%) diagnostic discrepancies. Prenatal diagnostic rate and accuracy are high among pediatric cardiology centers in the New England region, and much improved over historical data. To improve fetal detection of congenital heart disease further, future work is needed to better determine the etiology of missed prenatal diagnoses and efforts should be focused on increasing appropriate referrals to pediatric cardiology centers for fetal evaluation.
Cardiovascular Performance in Neonates with Hypoxic-Ischemic Encephalopathy Under Therapeutic Hypothermia: Evaluation by Conventional and Advanced Echocardiographic Techniques
Nunes VAC, Assef JE, Le Bihan DCS, Barretto RBM, Magalhães M and Pedra SRFF
This study aimed to evaluate the hemodynamic and ventricular performance of neonates with hypoxic-ischemic encephalopathy (HIE) undergoing therapeutic hypothermia using conventional and advanced echocardiographic techniques. This observational, prospective study included 22 neonates with HIE matched with 22 healthy neonates. Echocardiographic studies were performed 24 h after achieving target temperature during hypothermia and 24 h after rewarming. Evaluated echocardiographic parameters included ejection fraction (EF), shortening fraction (SF), right ventricular fractional area change, biventricular Tei index, right ventricular s' wave velocity, tricuspid annular plane systolic excursion, biventricular stroke volume and cardiac output, left ventricular (LV) and right ventricular (RV) global longitudinal strain (GLS), LV circumferential and radial strain, LV twist, and LV torsion. LV EF and SF did not change significantly between the hypothermia and rewarming periods (EF:73 ± 7% vs. 74 ± 5%, p = 0.21; SF:39 ± 6% vs. 41 ± 5%, p = 0.26); however, both were higher after rewarming compared to the control group (EF:70 ± 5%, p = 0.003; SF:36 ± 4%, p = 0.002). There were no significant differences in LV GLS, circumferential and radial strain, twist, and torsion between the HIE and control groups. Pulmonary artery systolic pressure (PASP) and RV GLS were worse in the study group compared to the control group (PASP: hypothermia 45 ± 24 mmHg, p = 0.01; rewarming 53 ± 34 mmHg, p = 0.01; control group 29 ± 11 mmHg; RV GLS: hypothermia 18 ± 5%, p = 0.02; rewarming: 18 ± 4%, p = 0.01; control group 21 ± 2%). Therapeutic hypothermia appears to have no detrimental impact on LV systolic function. RV GLS was the only parameter that demonstrated impaired RV systolic function during therapeutic hypothermia, likely due to elevated PASP.
Transcatheter Closure of Ventricular Septal Defects after Upfront Transvenous Antegrade Cannulation from the Right Ventricle
Sagar P, Thejaswi P, Garg I and Sivakumar K
Transcatheter closure (TCC) of certain ventricular septal defect (VSD) subtypes typically requires arteriovenous loop (AVL) formation or retrograde transarterial deployment. Upfront transvenous cannulation from the right ventricle avoids arterial access and loop-related complications. We retrospectively reviewed data of patients who underwent TCC for perimembranous, intraconal, and post-surgical residual VSDs at our institution (January 2019-December 2023). Patients receiving upfront transvenous VSD cannulation and closure were compared to those undergoing antegrade closure after AVL formation. Upfront retrograde closure cases were excluded. In a cohort of 163 patients, upfront transvenous cannulation was performed in 116 (71%) and AVL formation in 31 (19%). Upfront transvenous cannulation use increased from 25.6% in 2019 to 93% in 2023. For the upfront group, the median patient age was 55 months (IQR, 17.8-120.8), median weight was 15.8 kg (IQR, 9.6-29.8), indexed VSD size was 9.2 mm/m (IQR, 5.5-14.8), and 68.9% had pulmonary arterial hypertension. Cannulation was successful in 83.6% (97/116) of cases, with a switch to retrograde transarterial cannulation in 16.4% (19/116). No deaths or pacemaker implantations occurred. Compared to AVL group, upfront group had significantly shorter procedural times (p = 0.002) and lower radiation exposure (p < 0.001). Smaller patient weight (OR: 0.97, 95%CI: 0.95-0.99), larger indexed VSD size to patient weight (OR: 36.5, 95%CI: 2.49-533.9) and to BSA (OR: 1.23, 95%CI: 1.06-1.4) were significant independent predictors for successful transvenous cannulation. Upfront transvenous VSD cannulation simplifies transvenous TCC by eliminating the need for arterial access and AVL formation, particularly successful in small patients with large defects.
Rebuttal to Letter to the Editor: Congenital Heart Defects in Patients with Anorectal Malformations: A Retrospective Cohort Study of 281 Patients
de Beaufort CMC, Mackay TM, Stevens MF, Polderman JAW, de Jong JR, van der Hulst AE, Straver B and Gorter RR
Differentiating Kawasaki Disease and Multisystem Inflammatory Syndrome in Children Using Blood Composite Scores: Insights into Clinical Outcomes and Predictive Indices
Ulu K, Çağlayan Ş, Coşkuner T, Vazgeçer EO, Öner T and Sözeri B
The study sought to assess the clinical utility of complete blood count-derived composite scores, suggesting their potential as markers of inflammation and disease severity in Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) with Kawasaki-like features. This retrospective study analyzed data from 71 KD and 73 MIS-C patients and 70 healthy controls. The KD group showed a higher rate of coronary involvement (26.7% vs. 10.9%), while the MIS-C group had a higher intensive care unit (ICU) admission rate (34.2% vs. 2.8%). Platelet counts, lymphocyte counts, mean platelet volume (MPV), MPV/Lymphocyte (MPVLR), and MPV/Platelet (MPVPR) ratios demonstrated the highest specificities in distinguishing MIS-C than KD (84.5%, 83.1%, 91.1%, 88.7%, and 88.7%, respectively). Monocyte counts, MPV, and MPVPR demonstrated the highest specificities to predictive ICU admission in the MIS-C group (83.3%, 89.6%, and 89.6%, respectively). Lymphocyte counts, platelet/lymphocyte ratio (PLR), neutrophil/lymphocyte ratio (NLR), MPVLR, and Systemic Immune-Inflammation Index (SII) parameters were found to have high negative predictive values for predicting KD patients without coronary artery lesions (CALs) (85.7%, 86.1%, 87.1%, 87.1%, and 85.7%, respectively)., Systemic Inflammation Response Index (SIRI), MPVPR, and CRP were independently predictive of ICU admission in the MIS-C group, and lymphocyte count and IVIG resistance were also identified as significant predictors of CALs in the KD group. NLR, MPVLR, MPVPR, and NPR indices effectively differentiate MIS-C from KD and predict ICU admission in MIS-C. NLR, PLR, MPVLR, and SII are valuable in excluding CALs in KD with high negative predictive values. In addition, SIRI and MPVLR were independent predictors of ICU admission in MIS-C, and lymphocyte count was identified as an independent predictor of CALs in KD.
Effect of Balloon-Dilated Restenosis on Exercise Performance and Cardiac Function in Children After Coarctation Repair
Baelen R, Fordel N, Coomans I, De Wolf D, Vandekerckhove K, Martens T, François K and Bové T
Restenosis occurs commonly after aortic coarctation (CoA) repair, usually requiring treatment by balloon dilation. Its effect on physical exercise performance is not documented. A retrospective analysis of exercise testing and echocardiographic assessment was performed in children after CoA repair. A group without restenosis (= group 1) and a group with recoarctation, treated by single balloon dilation (= group 2) were compared by matching for age and gender at a 2:1 ratio. Group 1 included 40 children and group 2 comprised 21 children, with mean age of 12.3 ± 2.3y and 11.9 ± 2.9y (p = 0.536) at evaluation. Group 2 children were younger at CoA repair (group 1: median 32.0d (11.0-188.0)-group 2: 9.0d (5.5-19.0), p = 0.011). Arterial hypertension was frequently noticed (group 1: 23(57.5%)-group 2: 7(33.3%), p = 0.106). Echocardiography revealed comparable cardiac function properties, showing more left ventricular (LV) hypertrophy in all CoA patients compared to their peers (Posterior wall thickness z-value: 1.03 ± 1.65, p < 0.001)). Exercise results were generally comparable, despite small differences in favor of group 1 (predicted % peak VO = group 1: 95.2 ± 21.4%-group 2: 82.3 ± 10.5%, p = 0.024; peak VO/kg = group 1: 38.9 ± 9.5 ml/min/kg-group 2: 33.8 ± 6.9 ml/min/kg, p = 0.034; VO/WR slope = group 1: 12.6 ± 2.0 ml/min/watt-group 2: 11.3 ± 1.7 ml/min/watt, p = 0.018). Compared to uncomplicated coarctation patients, children developing a surgical restenosis after CoA repair, and treated effectively by balloon dilation, had lower-but still acceptable-oxygen uptake during exercise. As intrinsic cardiovascular function characteristics were similar, further study to evaluate the impact of non-disease-specific factors is mandatory.
The Role of Fetal Echocardiography in Predicting Postnatal Pulmonary Venous Obstruction in Neonates with TAPVR: A Single Center Experience
Alqahtani M, Tolani D, Dugulescu M and Keelan J
Total anomalous pulmonary venous return (TAPVR) is a high risk and rare cardiac malformation with a low prenatal detection rate and predicting obstruction in these cases is difficult. We sought to investigate fetal echocardiographic parameters associated with postnatal pulmonary venous obstruction (PPVO). We performed a retrospective review of 26 patients with TAPVR who had a fetal echocardiogram from 2010 to 2021. Blinded readers analyzed the fetal Doppler features of the pulmonary veins (PV) and vertical veins (VV) at final fetal assessment. Of the 26 patients, 17 (65%) were prenatally diagnosed and overall 10 (38.5%) developed PPVO. All patients had additional associated congenital heart disease. Compared to patients without PPVO, patients with PPVO had a lower VV variability index (VI) (0.51 (0.17-0.79) vs 1.43 (0.54-2.58); p = 0.003), a higher likelihood of having an abnormal VV or PV Doppler pattern (p = 0.042, p = 0.015), a higher VV minimum velocity (0.435 m/s (0.17-1.05) vs 0.14 m/s (- 0.16 to 0.35) p = 0.001) and a higher VV mean velocity (0.641 m/s (0.31-1.15) vs 0.321 m/s (0.19-0.5); p = 0.009). A fetal VV VI of ≤ 0.8 was associated with PPVO (100% sensitivity, 86% specificity). Our data show that some fetal PV and VV Doppler indices are significantly different between those that develop PPVO and those who do not, with the most significant difference seen in the VV variability index and VV minimum velocity. Prenatal predictors of PPVO have the potential to influence prenatal counseling, perinatal care, surgical timing, and outcomes associated with this critical diagnosis.
Development and Validation of KCPREDICT: A Deep Learning Model for Early Detection of Coronary Artery Lesions in Kawasaki Disease Patients
Yang L, Shen X, Liu Y, Chen J, Zou Y, Xu L, Ji W, Zhang Y, Liu T and Cao Q
Kawasaki disease (KD) is a febrile vasculitis disorder, with coronary artery lesions (CALs) being the most severe complication. Early detection of CALs is challenging due to limitations in echocardiographic equipment (UCG). This study aimed to develop and validate an artificial intelligence algorithm to distinguish CALs in KD patients and support diagnostic decision-making at admission. A deep learning algorithm named KCPREDICT was developed using 24 features, including basic patient information, five classic KD clinical signs, and 14 laboratory measurements. Data were collected from patients diagnosed with KD between February 2017 and May 2023 at Shanghai Children's Medical Center. Patients were split into training and internal validation cohorts at an 80:20 ratio, and fivefold cross-validation was employed to assess model performance. Among the 1474 KD cases, the decision tree model performed best during the full feature experiment, achieving an accuracy of 95.42%, a precision of 98.83%, a recall of 93.58%, an F1 score of 96.14%, and an area under the receiver operating characteristic curve (AUROC) of 96.00%. The KCPREDICT algorithm can aid frontline clinicians in distinguishing KD patients with and without CALs, facilitating timely treatment and prevention of severe complications. The use of the complete set of 24 diagnostic features is the optimal choice for predicting CALs in children with KD.
Pleural Drainage, Clinical Characteristics, and Management Strategies in the Perioperative Fontan Patient: A Multicenter Report
Duran SR, Winder M, Reeder RW, Plummer ST, Sznycer-Taub N, Savoca M, DiMaria MV, Gordon EE, Bhaskar P, Raymond TT, Das A, Tortorich A, Lyman A, Bertrandt RA, Kozyak BW, Frank DU, Greiten LE, Bailly DK and Lay AS
Prolonged pleural drainage and chylothorax are common in postoperative Fontan patients and are associated with increased morbidity and mortality. Multiple medical and interventional treatment strategies exist and vary between centers. This is a retrospective multicenter observational cohort study of pediatric patients who underwent Fontan operation at 8 pediatric cardiac surgical institutions from 1/1/2019 to 12/31/2021. Data were obtained from institutional records and collected from the Pediatric Cardiac Critical Care Consortium (PC). 185 patients underwent Fontan operation with median age of 3.8 years [IQR 3.2-4.5]. Chest tube drainage for > 14 days occurred in 40 patients (22%). Chylothorax occurred in 33 patients (18%, incidence 9.1-26.2% across centers). Compared to non-chylothorax patients, those with chylothorax had lower preoperative ventricular end diastolic pressures (8 vs. 9 mm Hg, p = 0.019), greater chest tube utilization (13 vs. 7 days, p =  < 0.001), ICU LOS (7 vs. 4 days, p = 0.001), hospital LOS (12 vs. 9 days, p < 0.001), and more weight loss (- 2.7% vs. 0.8%, p = 0.019). Using a receiver-operating characteristic curve, chest tube output > 18.8 mL/kg/day on POD 2 predicted chylothorax with an AUC of 0.73. Common chylothorax treatments were diet modification (n = 15, 45%) and sildenafil (n = 14, 52%). Interventional procedures were used in six chylothorax patients (18%). Postoperative chylothorax in Fontan patients was associated with increased chest tube utilization, postoperative interventions, greater weight loss, and longer ICU and hospital LOS. Center level variation suggests outcomes and resource utilization could be improved with further studies and establishment of best practices.
Racial and Educational Isolation are Associated with Worse Outcomes in Congenital Heart Disease
Sooy-Mossey M, Sethi NJ, Mulder H, Chiswell KE, Hoffman TM, Hartman RJ, Walsh MJ, Welke K, Paolillo JA, Sarno LA, Sang CJ, D'Ottavio A, Osgood C, Bravo MA, Miranda ML and Li JS
Sociodemographic factors influence outcomes in children with congenital heart disease (CHD). We predict an association between measures of social isolation and outcomes in infants with complex CHD. These measures, racial (RI) and educational (EI) isolation range from 0 to 1, with 0 being no isolation and 1 being fully isolated within a specific population. We identified patients less than 1 year old undergoing CHD surgery in North Carolina from 2008 to 2013. We used negative binomial and logistic regression models to assess the case-mix adjusted associations between RI and EI and length of stay, complications, mortality, and resource utilization. We quantified the association of race with these indices and outcomes. We included 1217 infants undergoing CHD surgery. Black infants had increased LOS (p < 0.001), other complications (p = 0.03), and death (p = 0.02). RI up to 0.3 was associated with decreased outpatient encounters (p < 0.001). RI was associated with increased inpatient encounters RI up to 0.3 (p < 0.001) but decreased for RI beyond 0.3 (p = 0.01). There was an association with increased risk of one or more emergency department visits (p = 0.001) at higher levels of EI. Race and RI showed a cumulative effect with children with Black race and greater than median RI having increased LOS (p < 0.001) and fewer outpatient encounters (p = 0.02). RI, EI, and Black race are associated with poorer outcomes. Children with Black race and greater than median levels of RI are at the highest risk of poor outcomes. These differences may be caused by differential access to resources or community support.
Evaluating the Sapien® XT Valve in Native Right Ventricular Outflow Tracts After Tetralogy of Fallot Repair: Mid- and Long-Term Results
Odemis E, Celikyurt A, Kizilkaya MH and Demir İH
Although the long-term outcomes of the surgical grafts are well defined and reported, the data regarding the mid-and long-term results of the balloon-expandable percutaneous valves in the native right ventricular outflow tract (RVOT) is limited. We retrospectively evaluated 42 patients who underwent PPVI (Sapien® XT valve) to native RVOT due to severe pulmonary regurgitation (PR) and/or moderate to severe pulmonary stenosis (PS) between August 2015 and November 2020. The median patient age at the time of PPVI was 13.4 years (6.1-36.5 years). The median body weight of the patients was 42 kg (15-110 kg). The rate of patients who were followed up without the need for percutaneous or surgical intervention was 97.4% at the end of year 1, 89.3% at the end of year 3, and 85.8% at the end of year 5. At the end of year 6, the proportion of patients requiring no procedure remained constant, with year 5 at 85.8%, but decreased to 70.2% at the end of year 7. Although the early results are very encouraging, it is seen that PPVI in patients with RVOT in the long term brings some problems. The most important of these is tricuspid valve problems, which were not considered before the procedure. Patients requiring reintervention due to pulmonary regurgitation show similar characteristics to surgical valves' long-term results.
Patent Ductus Arteriosus Morphology Changes in Preterm Neonates Undergoing Transcatheter Device Closure
Simpson MK, Bhombal S, Hamrick SE, Hash S, Adamson M, He Z, Hogan K, Huang H, Jergel A, Kim DW, LeFevre AS, Mills M, Saini A and Ligon RA
Evaluate patent ductus arteriosus (PDA) morphology changes in the preterm neonate undergoing transcatheter PDA closure (TCPC). We propose the type F ductus is associated with lower corrected gestational age (CGA) and improved TCPC outcomes. Retrospective review of premature neonates undergoing TCPC at a large volume institution from November 2020 to November 2023. Two independent cardiologists blindly reviewed angiograms to classify the PDA as one of the 6 published morphologies. Procedural characteristics and outcomes were compared for two cohorts of TCPC patients constituting the type F and the non-type F ductus; statistical significance set at p-value < 0.05. 104 TCPC neonates met inclusion criteria; these were categorized as type F (n = 44) and non-type F (n = 60) ductal morphology. Patients with type F ductal morphology was associated with African American race (p = 0.007), lower procedural/chronological age (p < 0.001), weight (p = 0.002), and CGA (p < 0.001). Procedural length was significantly shorter in the type F group (41 vs 57 min; p < 0.01). Unsuccessful TCPC (n = 2) and device embolization (n = 2) events all occurred in patients with a non-type F ductal morphology. The CGA cut off < 34 weeks represents a good predictor of having a type F ductus (sensitivity 0.93, specificity 0.67). Younger chronological and CGA are associated with type F ductal morphology. At this institution, a type F ductus was associated with a shorter TCPC procedure time and demonstrated a lower complication rate. This data may prompt consideration of earlier TCPC referral. However, determining ideal TCPC timing in premature neonates is multifactorial and requires a multidisciplinary review team.
Correction: Response to the Letter Titled "Same Entity, Different Hemodynamics: Residual VSD After TOF Repair and Isolated VSD Closure" From Dr. Safak ALPAT
Nakayama Y and Shinkawa T