Outcomes of Neonates Born with Symptomatic Tetralogy of Fallot and Absent Ductus Arteriosus.

OBJECTIVE: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were as follows: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure. RESULTS: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI: 1.07,5.27; P = .034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA). CONCLUSIONS: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population.

Impact of Management Strategy on Feeding and Somatic Growth in Neonates with Symptomatic Tetralogy of Fallot: Results from the Congenital Cardiac Research Collaborative.

OBJECTIVE: To evaluate early growth following primary or staged repair of neonatal symptomatic tetralogy of Fallot (sTOF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of consecutive infants with sTOF who underwent initial intervention at age ≤30 days, from 2005 to 2017. Management strategies were either primary repair or staged repair (ie, initial palliation followed by complete repair). The primary outcome was change in weight-for-age z-score (ΔWAZ) from the initial intervention to age 6 ± 2 months. Secondary outcomes included method and mode of feeding, feeding-related medications, and feeding-related readmissions. Propensity score adjustment was used to account for baseline differences between groups. A secondary analysis was performed comparing patients stratified by the presence of adequate growth (6-month ΔWAZ > -0.5) or inadequate growth (6-month ΔWAZ ≤ -0.5), independent of treatment strategy. RESULTS: The study cohort included 143 primary repair subjects and 240 staged repair subjects. Prematurity was more common in the staged repair group. After adjustment, median ΔWAZ did not differ between treatment groups over the first 6 months of life (primary: -0.43 [IQR, -1.17 to 0.50]; staged: -0.31 [IQR, -1.31 to 0.71]; P = .55). For the entire cohort, ΔWAZ was negative (-0.36; IQR, -1.21 to 0.63). There were no between-group differences in the secondary outcomes. Secondary analysis revealed that the subjects with adequate growth were more likely to be orally fed at initial hospital discharge (P = .04). CONCLUSIONS: In neonates with sTOF, growth trajectory over the first 6 months of life was substandard, irrespective of treatment strategy. Those patients with adequate growth were more likely to be discharged from the index procedure on oral feeds.

Inferior and Superior Vena Cava Saturation Monitoring After Neonatal Cardiac Surgery.

OBJECTIVES: Superior vena cava oxygen saturation (SVC O 2 ) monitoring is well described for early detection of hemodynamic deterioration after neonatal cardiac surgery but inferior vena cava vein oxygen saturation (IVC O 2 ) monitoring data are limited. DESIGN: Retrospective cohort study of 118 neonates with congenital heart disease (52 single ventricle) from February 2008 to January 2014. SETTING: Pediatric cardiac ICU. PATIENTS: Neonates (< 30 d) with concurrent admission IVC O 2 and SVC O 2 measurements after cardiac surgery with cardiopulmonary bypass. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The primary aim was to correlate admission IVC O 2 and SVC O 2 . Secondary aims included: correlate flank or cerebral near-infrared spectroscopy with IVC O 2 and SVC O 2 , respectively, and exploratory analysis to evaluate associations between oximetry data and a composite adverse outcome defined as any of the following: increasing serum lactate or vasoactive support at 2 hours post-admission, cardiac arrest, or mortality. Admission IVC O 2 and SVC O 2 correlated ( r = 0.54; p < 0.001). However, IVC O 2 measurements were significantly lower than paired SVC O 2 (mean difference, -6%; 95% CI, -8% to -4%; p < 0.001) with wide variability in sample agreement. Logistic regression showed that each 12% decrease in IVC O 2 was associated with a 12-fold greater odds of the composite adverse outcome (odds ratio [OR], 12; 95% CI, 3.9-34; p < 0.001). We failed to find an association between SVC O 2 and increased odds of the composite adverse outcome (OR, 1.8; 95% CI, 0.99-3.3; p = 0.053). In an exploratory analysis, the area under the receiver operating curve for IVC O 2 and SVC O 2 , and the composite adverse outcome, was 0.85 (95% CI, 0.77-0.92) and 0.63 (95% CI, 0.52-0.73), respectively. Admission IVC O 2 had strong correlation with concurrent flank near-infrared spectroscopy value ( r = 0.74; p < 0.001). SVC O 2 had a weak association with cerebral near-infrared spectroscopy ( r = 0.22; p = 0.02). CONCLUSIONS: In postoperative neonates, admission IVC O 2 and SVC O 2 correlate. Lower admission IVC O 2 may identify a cohort of postsurgical neonates at risk for low cardiac output and associated morbidity.

Palliation Strategy to Achieve Complete Repair in Symptomatic Neonates with Tetralogy of Fallot.

Neonates with symptomatic tetralogy of Fallot (sTOF) may undergo palliations with varying physiology, namely systemic to pulmonary artery connections (SPC) or right ventricular outflow tract interventions (RVOTI). A comparison of palliative strategies based on the physiology created is lacking. Consecutive sTOF neonates undergoing SPC or RVOTI from 2005-2017 were reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was survival with successful complete repair (CR) by 18 months. A variety of secondary outcomes were assessed including overall survival, hospitalization-related comorbidities, and interstage reinterventions. Propensity score adjustment was utilized to compare treatment strategies. The cohort included 252 SPC (surgical shunt = 226, ductus arteriosus stent = 26) and 68 RVOTI (balloon pulmonary valvuloplasty = 48, RVOT stent = 11, RVOT patch = 9) patients. Genetic syndrome (29 [42.6%] v 75 [29.8%], p = 0.04), weight < 2.5 kg (28 [41.2%] v 68 [27.0%], p = 0.023), bilateral pulmonary artery Z-score < - 2 (19 [28.0%] v 36 [14.3%], p = 0.008), and pre-intervention antegrade flow (48 [70.6%] v 104 [41.3%], p < 0.001) were more common in RVOTI. Significant center differences were noted (p < 0.001). Adjusted survival to CR by 18 months (HR = 0.87, 95% CI = 0.63-1.21, p = 0.41) and overall survival (HR = 2.08, 95% CI = 0.93-4.65, p = 0.074) were similar. RVOTI had increased interstage reintervention (HR = 2.15, 95% CI = 1.36-3.99, p = 0.001). Total anesthesia (243 [213, 277] v 328 [308, 351] minutes, p < 0.001) and cardiopulmonary bypass times (117 [103, 132] v 151 [143, 160] minutes, p < 0.001) favored RVOTI. In this multicenter comparison of physiologic palliation strategies for sTOF, survival to successful CR and overall survival were similar; however, reintervention burden was significantly higher in RVOTI.

The incidence of recurrent laryngeal nerve injury resulting in vocal cord paralysis following interventional congenital catheterisation procedures.

BACKGROUND: Recurrent laryngeal nerve injury leading to vocal cord paralysis is a known complication of cardiothoracic surgery. Its occurrence during interventional catheterisation procedures has been documented in case reports, but there have been no studies to determine an incidence. OBJECTIVE: To establish the incidence of left recurrent laryngeal nerve injury leading to vocal cord paralysis after left pulmonary artery stenting, patent ductus arteriosus device closure and the combination of the procedures either consecutively or simultaneously. METHODS: Members of the Congenital Cardiovascular Interventional Study Consortium were asked to perform a retrospective analysis to identify cases of recurrent laryngeal nerve injury after the aforementioned procedures. Twelve institutions participated in the analysis. They also contributed the total number of each procedure performed at their respective institutions for statistical purposes. RESULTS: Of the 1337 patients who underwent left pulmonary artery stent placement, six patients (0.45%) had confirmed vocal cord paralysis. 4001 patients underwent patent ductus arteriosus device closure, and two patients (0.05%) developed left vocal cord paralysis. Patients who underwent both left pulmonary artery stent placement and patent ductus arteriosus device closure had the highest incidence of vocal cord paralysis which occurred in 4 of the 26 patients (15.4%). Overall, 92% of affected patients in our study population had resolution of symptoms. CONCLUSION: Recurrent laryngeal nerve injury is a rare complication of left pulmonary artery stent placement or patent ductus arteriosus device closure. However, the incidence is highest in patients undergoing both procedures either consecutively or simultaneously. Additional research is necessary to determine contributing factors that might reduce the risk of recurrent laryngeal nerve injury.

Data quality methods through remote source data verification auditing: results from the Congenital Cardiac Research Collaborative.

BACKGROUND: Multicentre research databases can provide insights into healthcare processes to improve outcomes and make practice recommendations for novel approaches. Effective audits can establish a framework for reporting research efforts, ensuring accurate reporting, and spearheading quality improvement. Although a variety of data auditing models and standards exist, barriers to effective auditing including costs, regulatory requirements, travel, and design complexity must be considered. MATERIALS AND METHODS: The Congenital Cardiac Research Collaborative conducted a virtual data training initiative and remote source data verification audit on a retrospective multicentre dataset. CCRC investigators across nine institutions were trained to extract and enter data into a robust dataset on patients with tetralogy of Fallot who required neonatal intervention. Centres provided de-identified source files for a randomised 10% patient sample audit. Key auditing variables, discrepancy types, and severity levels were analysed across two study groups, primary repair and staged repair. RESULTS: Of the total 572 study patients, data from 58 patients (31 staged repairs and 27 primary repairs) were source data verified. Amongst the 1790 variables audited, 45 discrepancies were discovered, resulting in an overall accuracy rate of 97.5%. High accuracy rates were consistent across all CCRC institutions ranging from 94.6% to 99.4% and were reported for both minor (1.5%) and major discrepancies type classifications (1.1%). CONCLUSION: Findings indicate that implementing a virtual multicentre training initiative and remote source data verification audit can identify data quality concerns and produce a reliable, high-quality dataset. Remote auditing capacity is especially important during the current COVID-19 pandemic.

Standardized Perioperative Feeding Protocol Improves Outcomes in Patients With d-Transposition of the Great Arteries Undergoing Arterial Switch Operation.

OBJECTIVE: To determine whether the presence of a standardized feeding protocol improves outcomes in a subset of neonates undergoing cardiac surgery. DESIGN: Retrospective cohort study. SETTING: Cardiovascular ICU at a freestanding academic children's hospital. PATIENTS: Neonates with a diagnosis of d-transposition of the great arteries undergoing arterial switch operation from January 2007 to June 2017. INTERVENTIONS: Initiation of perioperative feeding protocols. MEASUREMENTS AND MAIN RESULTS: Patients were evaluated before and after implementation of standardized perioperative feeding protocols in neonates with d-transposition of the great arteries undergoing arterial switch operation. Low-risk patients born after initiation of nurse-driven protocols were compared with a similar historical group. Data obtained included time to achievement of feeding goals, with primary outcome being weight gain at hospital discharge. Other measures analyzed included duration of mechanical ventilation and postoperative hospital length of stay. Overall, 33 patients in the protocol group were compared with 44 patients in the historical group. No significant baseline differences existed between the two cohorts. The protocol group achieved improved feeding outcomes in nearly all measured categories, including introduction to enteral feeds preoperatively (91% vs 59%; p < 0.01) and earlier attainment of postoperative full enteral feeds of 120 mL/kg/d (2 vs 5 d; p < 0.01). Protocol patients had significantly improved weight gain at the time of discharge (60 vs 1 g; p < 0.01), while achieving shorter postoperative length of stay (10.1 vs 12.6 d; p = 0.04). CONCLUSIONS: An aggressive, but safe, perioperative feeding protocol implemented in a homogenous low-risk neonatal cardiac surgical population improves feeding outcomes, including increased weight gain, as well as decreased postoperative length of stay. Consideration for perioperative feeding protocol implementation and further study should be given.

A large ventricular fibroma requiring surgical resection in a symptomatic 3-month-old infant.

Cardiac Fibromas are primary cardiac tumours more common in children than in adults. Surgical intervention is often not required except in the case of limited cardiac output or significant arrhythmia burden. We present a symptomatic 3-month-old infant who had successful surgical intervention for a giant right ventricle fibroma found on prenatal imaging.

Aspiration After Congenital Heart Surgery.

Dysphagia and vocal cord dysfunction are frequent complications after congenital heart surgery. Both are risk factors for aspiration, which can lead to pneumonia, progressive lung disease, and respiratory arrest. A protocol was implemented to promote early detection of aspiration in a high-risk cohort of patients. Retrospective data were collected on all patients under 120 days old who underwent the Norwood procedure, aortic arch repair, Blalock-Taussig shunt placement, or cervical cannulation for extracorporeal membrane oxygenation from 10/2012 to 05/2016 at a single institution. Patients underwent an assessment of symptoms, fiberoptic endoscopic evaluation of swallowing (FEES), and modified barium swallow (MBS) study in the postoperative period prior to initiating oral feeds. Patients with and without aspiration were compared. Of the 96 patients included in the study, one-third (33%) of patients had evidence of vocal cord dysfunction by FEES and just over half (51%) had evidence of aspiration by FEES or MBS. Most (73%) of the patients with aspiration were asymptomatic and a majority (53%) of patients with aspiration had normal vocal cord function. Aspiration is common after congenital heart surgery, and an assessment of vocal cord or swallow function in isolation may lead to underdiagnosis. A comprehensive protocol including MBS and FEES is necessary for the early detection of vocal cord dysfunction and aspiration and may prevent adverse outcomes in high-risk postoperative patients.

Anchor balloon, buddy wire, and wire and sheath techniques to deploy percutaneous pulmonary valves in tetralogy of fallot patients.

Advances in surgical techniques in tetralogy of Fallot (TOF) patients have improved survival of these patients into adulthood. The procedure requires right ventricular outflow tract or trans-annular patch with resultant pulmonary stenosis and/or regurgitation. As such, adult patients seen with this condition may have increasing right ventricular hypertrophy and/or right ventricular dilation. Recently, the Sapien XT valve (Edwards Lifesciences, CA) was approved by the FDA for pulmonary implantation. In some cases, advancing the valve in right ventricular outflow tract is difficult. This is a case series of delivering Sapien XT valves in TOF patients with severe pulmonary regurgitation and/or stenosis, using the anchor balloon, buddy wire, and the novel, wire and sheath techniques. © 2017 Wiley Periodicals, Inc.

Correction to: Routine Sildenafil Does Not Improve Clinical Outcomes After Fontan Operation.

The original version of this article unfortunately contained a mistake. The presentation of Table 2 was incorrect .The corrected table is given below.

Routine Sildenafil Does Not Improve Clinical Outcomes After Fontan Operation.

Fontan operation can be complicated by persistent chest tube output (CTO) leading to prolonged hospital length of stay (LOS). Postoperative sildenafil administration has been shown to improve clinical outcomes in selected patients after Fontan. We initiated a practice change utilizing intravenous (IV) sildenafil in early postoperative period in all patients undergoing Fontan operation with aim to decrease LOS and CTO. Nineteen patients (February 2014-May 2016) received 0.35 mg/kg sildenafil IV (three doses) followed by enteral, 1 mg/kg every eight hours until hospital discharge. Clinical outcomes were compared to 84 pre-protocol controls. Vital signs were recorded after second sildenafil dose. Demographics were similar between groups. Sildenafil group had longer median LOS [9 (7, 11) vs. 13 (8, 25) days, p = 0.016]. CTO days were longer [6 (5, 8) vs. 8 (6, 13) days, p = 0.011]. Sildenafil group had longer mechanical ventilation [6.9 (3.5, 11.1) vs. 4 (2, 7) h, p = 0.045] and longer oxygen therapy [99 (52, 225) vs. 14.5 (14, 56) h, p = 0.001]. There was a trend towards more albumin 5% resuscitation in first 24 h [17 (1, 30) vs. 21 (10, 40) ml/kg, p = 0.069]. There was no difference in inotrope score at 24 h, maximum lactate, or fluid balance. Readmission rates were similar. There was no mortality. IV sildenafil was well tolerated, and no doses were held. Routine early administration of sildenafil after Fontan operation is not associated with an improvement in any measured clinical outcome, including postoperative CTO, LOS, colloid administration, or duration of mechanical ventilation.

Trans-septal approach for percutaneous closure of infra-diaphragmatic veno-venous collateral in a patient after Fontan palliation.

In this article, we present a case of a desaturated Fontan patient with an infra-diaphragmatic venous collateral to the pulmonary vein, which was too tortuous to attempt closure at the source. A trans-septal approach was successfully used to close the collateral in a retrograde manner.

Novel, Long-axis In-plane Ultrasound-Guided Pericardiocentesis for Postoperative Pericardial Effusion Drainage.

Pericardial effusion can be a life-threatening complication in children after cardiac surgery. Percutaneous pericardiocentesis is associated with rare, but serious complications. This retrospective study describes our experience with a novel, long-axis in-plane real-time ultrasound (US)-guided technique for postoperative pericardial effusion drainage in small children. Ten out of sixteen procedures were performed within 14 days of cardiac surgery at a median postoperative day 12 (IQR 2, 99). Median age was 2.7 months (IQR 0.4124) and weight 4.5 kg (IQR 2.5, 41.6). All but one procedure required a single attempt. Fourteen out of sixteen procedures had subxiphoid approach, and two were apical. Median initial drainage was 9 mL/kg (IQR 4.5, 27). Fifty percent of effusions were serous, 25 % chylous, and the remainder bloody. There were no reported complications. This study demonstrates a novel, long-axis pericardiocentesis technique that allows for an easy and safe needle entry into the pericardial space for small children in the early postoperative period.

Early Postoperative Albumin Administration Contributes to Morbidity After the Fontan Operation.

The Fontan operation has low mortality, but is associated with significant postoperative morbidity, including prolonged chest tube output (PCTO), which is associated with prolonged hospital length of stay (PLOS). We sought to identify variables present early in the clinical course that could predict patients at high risk for PCTO and PLOS. Retrospective data were collected on 84 Fontan (extracardiac conduit) operations from 1/2008 to 12/2013 at a single institution. PCTO was defined as ≥8 days (>75th percentile); PLOS was defined as ≥12 days postoperatively (>75th percentile). Multivariate regression was used to determine covariates associated with PCTO and PLOS. Median age was 3.5 years (IQR 3-5); weight was 14.5 kg (IQR 13-17). There was no mortality. LOS was 9 days (IQR 3-11), and duration of chest tube drainage 6 days (IQR 5-8) at 15 ml/kg/day (IQR 9-20). In univariate analysis, only systemic right ventricle, 24-h 5 % albumin administration, 24-h fluid balance, and 12-h inotrope score were associated with PCTO. In multivariate analysis, only 5 % albumin administration in first 24 h (p < 0.001) and PCTO were independently associated with PLOS. ROC curve analysis showed patients receiving >25 ml/kg of 5 % albumin in first 24-h predicted PLOS (94 % specificity, 93 % sensitivity, AUC = 0.95, p < 0.001). Increased colloid in the first 24-h post-CPB strongly predicts PCTO and PLOS after Fontan operation, potentially providing an early identification of a cohort with unfavorable Fontan physiology. A better understanding of the role of colloid resuscitation after Fontan is necessary, and efforts to reduce perioperative colloid administration could decrease hospital morbidity.

Systemic thrombolysis with recombinant tissue plasminogen activator for acute life-threatening Blalock-Taussig shunt obstruction.

Modified Blalock-Taussig shunt (mBTS) obstruction can be life-threatening, especially when it represents the only source of pulmonary blood flow. Current therapeutic options to reverse obstruction include surgical shunt revision/replacement, interventional endovascular procedures including balloon angioplasty and/or stent placement, and a combination of local and systemic thrombolytic therapy. We report two cases of acute mBTS thrombosis successfully treated with systemic recombinant tissue plasminogen activator in infants convalescing after cardiac surgery when the clinical status and resources precluded traditionally described rescue therapies.

Ultrasound- versus landmark-guided femoral catheterization in the pediatric catheterization laboratory: a randomized-controlled trial.

Ultrasound (US) is the standard of care for vascular access in many clinical scenarios. Limited data exist regarding the benefits of US- versus landmark (LM)-guided femoral vascular access in the pediatric catheterization laboratory. This study aimed to compare US- and LM-guided vascular access in the pediatric catheterization laboratory. A single operator randomized 95 patients (201 vessels) to undergo either LM- or US-guided vascular access. The primary end point was the access success rate. Number of attempts, inadvertent access, time to sheath placement, and complications also were compared between the two groups. No difference was seen in the overall access success rate: 98 % with US versus 93 % with LM (p = 0.17). The success rate for the targeted vessel was higher with US (89 %) than with LM (67 %) (p = 0.012). US facilitated fewer attempts (1.1 ± 0.4 vs 1.4 ± 0.9; p = 0.048) and improved the first-attempt success rate (87 vs 77 %; p = 0.049). The time to access did not differ significantly between the two groups (US 2:55 ± 4:03 vs LM 3:37 ± 2:54; p = 0.28). No differences in complication rates were noted. The benefits of US were accentuated in the subgroup weighing less than 10 kg. In this study, US access in the pediatric catheterization laboratory did not improve overall success. However, US improved accuracy and reduced the number attempts necessary for access without prolonging the access time of the procedure. Small children realized the greatest benefit of US-guided access.

Comparison of PLANE Technique versus Standard Echocardiography Guidance for Pediatric Pericardiocentesis.

Percutaneous pericardiocentesis remains a challenging and potentially dangerous procedure, particularly in small, critically ill patients. We present outcomes of the PLANE (pericardiocentesis using long-axis in-plane real-time echocardiography) technique for pediatric pericardiocentesis compared with a standard echocardiography (ECHO) guidance cohort. This was a retrospective chart review of all children undergoing percutaneous pericardiocentesis from March 2013 to February 2021 at a single center. A total of 78 procedures were performed, 52 utilizing PLANE technique and 26 utilizing standard ECHO-guidance technique. There was 100% technical success rate with only one minor complication for the entire cohort. Procedures were evenly split between the bedside intensive care unit and cardiac catheterization laboratory. PLANE technique was utilized in significantly younger (1.4 vs. 8.4 years, p = 0.008) and smaller (11.1 vs. 31.8 kg, p = 0.007) patients, as well as in most patients deemed high risk (postoperative < 7 days, extracorporeal membrane oxygenation (ECMO) support, and/or weight less than 5 kg; 19/22, p = 0.021). Other patient characteristics were similar between the two groups. There was a trend toward PLANE technique utilization by noncardiology trained operators. The PLANE technique for pediatric pericardiocentesis is safe and effective and can be effectively utilized in small and high-risk patient populations. The technical similarity to other long-axis ultrasound-guided procedures may facilitate adoption and mastery by critical care trained operators.

Epidemiology of Postoperative Junctional Ectopic Tachycardia in Infants Undergoing Cardiac Surgery.

BACKGROUND: Junctional ectopic tachycardia (JET) complicates congenital heart surgery in 2% to 8.3% of cases. JET is associated with postoperative morbidity in single-center studies. We used the Pediatric Cardiac Critical Care Consortium data registry to provide a multicenter epidemiologic description of treated JET. METHODS: This is a retrospective study (February 2019-August 2022) of patients with treated JET. Inclusion criteria were (1) <12 months old at the index operation, and (2) treated for JET <72 hours after surgery. Diagnosis was defined by receiving treatment (pacing, cooling, and medications). A multilevel logistic regression analysis with hospital random effect identified JET risk factors. Impact of JET on outcomes was estimated by margins/attributable risk analysis using previous risk-adjustment models. RESULTS: Among 24,073 patients from 63 centers, 1436 (6.0%) were treated for JET with significant center variability (0% to 17.9%). Median time to onset was 3.4 hours, with 34% present on admission. Median duration was 2 days (interquartile range, 1-4 days). Tetralogy of Fallot, atrioventricular canal, and ventricular septal defect repair represented >50% of JET. Patient characteristics independently associated with JET included neonatal age, Asian race, cardiopulmonary bypass time, open sternum, and early postoperative inotropic agents. JET was associated with increased risk-adjusted durations of mechanical ventilation (incidence rate ratio, 1.6; 95% CI, 1.5-1.7) and intensive care unit length of stay (incidence rate ratio, 1.3; 95% CI, 1.2-1.3), but not mortality. CONCLUSIONS: JET is treated in 6% of patients with substantial center variability. JET contributes to increased use of postoperative resources. High center variability warrants further study to identify potential modifiable factors that could serve as targets for improvement efforts to ameliorate deleterious outcomes.

Early Outcomes From a Multicenter Transcatheter Self-Expanding Pulmonary Valve Replacement Registry.

BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) with the self-expanding Harmony valve (Medtronic) is an emerging treatment for patients with native or surgically repaired right ventricular outflow tract (RVOT) pulmonary regurgitation (PR). Limited data are available since U.S. Food and Drug Administration approval in 2021. OBJECTIVES: In this study, the authors sought to evaluate the safety and short-term effectiveness of self-expanding TPVR in a real-world experience. METHODS: This was a multicenter registry study of consecutive patients with native RVOT PR who underwent TPVR through April 30, 2022, at 11 U.S. CENTERS: The primary outcome was a composite of hemodynamic dysfunction (PR greater than mild and RVOT mean gradient >30 mm Hg) and RVOT reintervention. RESULTS: A total of 243 patients underwent TPVR at a median age of 31 years (Q1-Q3: 19-45 years). Cardiac diagnoses were tetralogy of Fallot (71%), valvular pulmonary stenosis (21%), and other (8%). Acute technical success was achieved in all but 1 case. Procedural serious adverse events occurred in 4% of cases, with no device embolization or death. Hospital length of stay was 1 day in 86% of patients. Ventricular arrhythmia prompting treatment occurred in 19% of cases. At a median follow-up of 13 months (Q1-Q3: 8-19 months), 98% of patients had acceptable hemodynamic function. Estimated freedom from the composite clinical outcome was 99% at 1 year and 96% at 2 years. Freedom from TPVR-related endocarditis was 98% at 1 year. Five patients died from COVID-19 (n = 1), unknown causes (n = 2), and bloodstream infection (n = 2). CONCLUSIONS: In this large multicenter real-world experience, short-term clinical and hemodynamic outcomes of self-expanding TPVR therapy were excellent. Ongoing follow-up of this cohort will provide important insights into long-term outcomes.