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Pulmonary embolism is increasing in prevalence among pediatric patients; although still rare, it can create a significant risk for morbidity and death within the pediatric patient population. Pulmonary embolism presents in various ways depending on the patient, the size of the embolism, and the comorbidities. Treatment decisions are often driven by the severity of the presentation and hemodynamic effects; severe presentations require more invasive and aggressive treatment. We describe the development and implementation of a pediatric pulmonary embolism response team designed to facilitate rapid, multidisciplinary, data-driven treatment decisions and management.

A previously healthy nine-year-old boy with anomalous aortic origin of the left coronary artery (AAOLCA) with high-risk anatomy demonstrated negative stress on magnetic resonance imaging. Invasive cardiac catheterization for intracoronary flow measurement was performed and demonstrated compromised coronary flow during pharmacologic stress and significant stenosis on angiography. The patient underwent surgical intervention with normalization of coronary flow upon postoperative evaluation. Invasive intracoronary flow determination with angiography under provocative stress is emerging as a critical data point for risk stratification and management decision-making in high-risk AAOLCA patients with negative noninvasive perfusion studies.

Heart failure (HF) is common in adult congenital heart disease (ACHD) patients; however, use of continuous-flow ventricular assist devices (CF-VADs) remains rare. We reviewed outcomes of patients with congenital heart disease greater than or equal to 18 years of age at the time of CF-VAD implant at the affiliated pediatric and adult institutions between 2006 and 2020. In total, 18 ACHD patients (15 with great anatomical complexity) received 21 CF-VADs. Six patients (median age 34 years) received seven percutaneous CF-VADs with a median duration of support of 20 days (3–44 days) with all patients survived to hospital discharge and two patients were bridged to durable CF-VADs. Fourteen patients (median age 38 years) received durable CF-VADs. Thirteen patients (93%) survived to hospital discharge and the median duration of support was 25.8 months (6.4–52.1 months). Estimated survival on durable CF-VAD at 1, 3, and 5 years was 84%, 72%, and 36%, respectively. Three patients were successfully bridged to transplantation. Device-related complications include cerebrovascular accident (n = 5), driveline infection (n = 3), device infection requiring chronic antibiotic therapy (n = 4), gastrointestinal bleeding (n = 6), and presumed pump thrombosis (n = 5). These results show percutaneous and durable CF-VADs can support ACHD patients with advanced HF.

AbstractBackground: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.

Understanding optimal ventricular assist device (VAD) parameters for pediatric patients is valuable given the inherent issue of patient-device size mismatch and heterogeneous cardiac anatomy in children. We evaluated our center’s experience of continuous-flow VAD (CF-VAD) optimization using cardiac catheterization. We performed a retrospective analysis of all patients on CF-VAD support who underwent hemodynamic heart catheterization from 2013 to 2018. Fifteen patients had 16 hemodynamic catheterizations performed. The indications for hemodynamic optimization by catheterization included clinical signs of heart failure while on CF-VAD (9 of 16, 56%), pretransplant evaluation of pulmonary hypertension (2 of 16, 13%), or assessment of myocardial recovery (5 of 16, 31%). The median age at catheterization was 12 years (interquartile range: 8–16). Median baseline speed of device was 2333 ± 253 rotations per minute. The goal was to find the speed at which optimal hemodynamics were achieved, defined by low wedge pressure with an acceptable central venous pressure. Of the 16 catheterizations, there were 9 (56%) speed increases to achieve optimal hemodynamics and 5 (33%) speed decreases for hemodynamic optimization or for potential explant. The speed was not changed in 2 (13%) catheterizations as the patients were determined to be at an optimal hemodynamic state. Overall, VAD settings were optimized in 75% (14 of 16) of hemodynamic catheterizations. There were no adverse events related to catheterization. Thus, we conclude that catheterization-based hemodynamic assessment is safe and effective for optimizing VAD speed and provides guidance on medical management in children supported on CF-VAD.

AbstractOstium secundum atrial septal defects are mostly closed in the cardiac catheterisation laboratories using either Amplatzer® (Abbott Laboratories, IL) atrial septal occluder, Gore® Cardioform septal occluder and more recently using the recently approved (US FDA approval June 2019) Gore® Cardioform atrial septal defect occluder (W. L. Gore & Associates, AZ). Similar to any new device in the market, there is a learning curve to the deployment of this device. We therefore aim to report the key features about this new Gore Cardioform atrial septal defect occluder device with special emphasis on technical aspects that can be employed during transcatheter closure of challenging ostium secundum atrial septal defects using this device.

AbstractBackground: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.

Background: Anomalous aortic origin of a coronary artery (AAOCA) is associated with sudden death in the young. We sought to determine quality of life (QOL) in patients/families affected by AAOCA.Methods: Patients with AAOCA (8-18 years) were prospectively included from January 2016 to May 2017. Parent proxy and patient Pediatric Cardiac Quality of Life Inventory (PCQLI) were used to evaluate QOL and Pediatric Quality of Life Inventory (PedsQL) Family Impact Module to assess the impact of AAOCA on families, as primary outcomes. Secondary outcomes included peer relationship, anxiety, and depression assessed using patient-reported outcomes measurement information system. Patients deemed high-risk were offered surgery/exercise restriction. Generalized linear mixed regression models were used to determine significant predictors of outcomes.Results: Fifty-three patients, the majority (n = 31, 59%) unrepaired, and 49 caregivers were included. Using PCQLI, patient and parent proxy QOL scores were similar to published scores for children with long-QT syndrome. Patients’ QOL score was associated with exertional symptoms, perceived chronic disease, and altered parent’s concentration ability. Likewise, parent proxy QOL scores were associated with mother’s living situation, exertional symptoms, parent missing work for ≥1 day, and disturbed parental functioning at work. Family impact scores were associated with lower maternal education, among other measures. Risk categories or surgical status did not impact patient, parent proxy reported, or family impact QOL.Conclusion: Anomalous aortic origin of a coronary artery is associated with decreased QOL as perceived by patients and caregiver and is associated with numerous facets of family functioning. These findings are independent of risk categorization or surgical status.

Anomalous aortic origin of a left coronary artery (L-AAOCA) with an intraseptal course is a rare anomaly and can be associated with myocardial ischemia and sudden cardiac death. No surgical or medical intervention is known to improve patient outcomes. A 7-year-old boy with intraseptal L-AAOCA presented with nonexertional chest pain, syncope, and had reversible myocardial ischemia on provocative testing. The patient was started on β-blockade, following which his symptoms improved and resolved over a period of six years. A follow-up dobutamine stress magnetic resonance imaging no longer showed reversible ischemia, and cardiac catheterization with fractional flow reserve did not show coronary flow compromise.

Background: Atrial septal defect and Impella have been proposed for left ventricular unloading in venoarterial extracorporeal membrane oxygenation patients. This work aims at evaluating the haemodynamic changes in venoarterial extracorporeal membrane oxygenation patients after Impella implantation or atrial septal defect realization by a simulation study.Methods: A lumped parameter model of the cardiovascular system was adapted to this study. Atrial septal defect was modelled as a resistance between the two atria. Venoarterial extracorporeal membrane oxygenation and Impella were modelled starting from their pressure-flow characteristics. The baseline condition of a patient undergoing venoarterial extracorporeal membrane oxygenation was reproduced starting from haemodynamic and echocardiographic data. The effects of different atrial septal defect size, Impella and venoarterial extracorporeal membrane oxygenation support were simulated.Results: Impella caused an increment of mean arterial pressure up to 67%, a decrement in mean pulmonary arterial pressure up to 8%, a decrement in left ventricular end systolic volume up to 11% with a reduction up to 97% of left ventricular cardiac output. Atrial septal defect reduces left atrial pressure (19%), increases right atrial pressure (22%), increases mean arterial pressure (18%), decreases left ventricular end systolic volume (11%), increases right ventricular volume (33%) and decreases left ventricular cardiac output (55%).Conclusion: Impella has a higher capability in left ventricular unloading during venoarterial extracorporeal membrane oxygenation in comparison to atrial septal defect with a lower right ventricular overload.

Iatrogenic aortopulmonary communications (IAPCs) are an uncommon but important complication after percutaneous intervention in postoperative patients. Iatrogenic aortopulmonary communications typically occur after balloon angioplasty or other interventions of the pulmonary outflow tract in certain anatomic configurations in which there is a denuded tissue plane between the pulmonary artery and aorta. They can present with signs and symptoms ranging from subtle clues which are difficult to recognize to near immediate hemodynamic instability. Once recognized, these lesions can become management dilemmas, and intraprocedural interventions can be complicated by complex anatomy and inadequate visualization by standard imaging techniques. We report cases where intracardiac echocardiography (ICE) was integral in the evaluation and management of IAPC as complications of prior transcatheter interventions. We found using ICE safely and effectively identified IAPCs and reduced the technical difficulty of intervention after IAPC discovery.

Predictors of right ventricle (RV) dysfunction after continuous‐flow left ventricular assist device (CF‐LVAD) implantation in children are not well described. We explored the association of preimplantation Pulmonary Artery Pulsatility index (PAPi) and other hemodynamic parameters as predictors of prolonged postoperative inotropes/pulmonary vasodilator use after CF‐LVAD implantation. Retrospective chart review. Single tertiary care pediatric referral center. Patients who underwent CF‐LVAD implantation from January 2012 to October 2017. Preimplantation invasive hemodynamic parameters were analyzed to evaluate the association with post‐CF‐LVAD need for prolonged (>72 hours) use of inotropes/pulmonary vasodilators. Preimplantation cardiac catheterization data was available for 12 of 44 patients who underwent CF‐LVAD implant during the study period. Median (IQR) age and BSA of the cohort were 15.3 years (10.2, 18) and 1.74 m2(0.98, 2.03). Group 1 (n= 6) included patients with need for prolonged inotropes/pulmonary vasodilator use after CF‐LVAD implantation and Group 2 (n= 6) included those without. Baseline demographic parameters, cardiopulmonary bypass time, and markers of RV afterload (pulmonary vascular resistance, PA compliance and elastance) were similar among the two groups. PAPi was significantly lower in group 1 compared to group 2 (0.96 vs 3.6, respectively; P= .004). Post‐LVAD stay in the intensive care unit was longer for patients in group 1 (46 vs 23 days, P= .52). Brain natriuretic peptide was significantly higher at 3 months after implantation in group 1; P= .01. The need for inotropes/pulmonary vasodilators in the postoperative period can be predicted by the preimplantation intrinsic RV contractile reserve as assessed by PAPi rather than the markers of RV afterload. Further investigation and correlation with clinical outcomes is needed.

Background:Pediatric pulmonary vein stenosis (PVS) is often progressive and treatment-refractory, requiring multiple interventions. Hybrid pulmonary vein interventions (HPVIs), involving intraoperative balloon angioplasty or stent placement, leverage surgical access and customization to optimize patency while facilitating future transcatheter procedures. We review our experience with HPVI and explore potential applications of this collaborative approach. Methods:Retrospective chart review of all HPVI cases between 2009 to 2023. Results:Ten patients with primary (n = 5) or post-repair (n = 5) PVS underwent HPVI at median age of 12.7 months (range 6.6 months-9.5 years). Concurrent surgical PVS repair was performed in 7/10 cases. Hybrid pulmonary vein intervention was performed on 17 veins, 13 (76%) with prior surgical or transcatheter intervention(s). One patient underwent intraoperative balloon angioplasty of an existing stent. In total, 18 stents (9 bare metal [5-10 mm diameter], 9 drug eluting [3.5-5 mm diameter]) were placed in 16 veins. At first angiography (median 48 days [range 7 days-2.8 years] postoperatively), 8 of 16 (50%) HPVI-stented veins developed in-stent stenosis. Two patients died from progressive PVS early in the study, one prior to planned reintervention. Median time to first pulmonary vein reintervention was 86 days (10 days-2.8 years; 8/10 patients, 13/17 veins). At median survivor follow-up of 2.2 years (2.3 months-13.1 years), 1 of 11 surviving HPVI veins were completely occluded. Conclusions:Hybrid pulmonary vein intervention represents a viable adjunct to existing PVS therapies, with promising flexibility to address limitations of surgical and transcatheter modalities. Reintervention is anticipated, necessitating evaluation of long-term benefits and durability as utilization increases.

With the improvement in detection of congenital heart disease in fetal life, fetal cardiac interventions (FCI) are pushing the envelope in hopes of either altering the natural history of disease or improving survival in certain high-risk lesions. These interventions include fetal aortic valvuloplasty (FAV) for evolving hypoplastic left heart syndrome (eHLHS), fetal atrial septoplasty with or without atrial septal stenting (FAS) for hypoplastic left heart syndrome (HLHS) and variants with intact or severely restrictive atrial septum (IAS/RAS), and fetal pulmonary valvuloplasty (FPV) for severe pulmonary stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS). This review discusses their indications, technical aspects, and outcomes based on available literature.

Clinical research in the treatment of patients with congenital heart disease (CHD) is limited by the wide variety of CHD manifestations and therapeutic options as well as the generally low incidence of CHD. The availability of comprehensive, contemporary outcomes studies is therefore limited. This inadequacy may result in a lack of data‐driven medical decision making. In 2013, clinician scientists at two centers began a research collaboration, the Congenital Catheterization Research Collaborative (CCRC). Over time, the CCRC has grown to include nine cardiac centers from across the United States, with a common data coordinating center. The CCRC seeks to generate high‐quality, contemporary, statistically robust, and generalizable outcomes research which can help address important clinical questions in the treatment of CHD. To date, the CCRC has reported on multicenter outcomes in: neonates with congenital aortic stenosis, infants undergoing right ventricular decompression for pulmonary atresia and intact ventricular septum, and infants with ductal‐dependent pulmonary blood flow. The CCRC has been successful at leveraging large multicenter cohorts of patients in a contemporary period to perform comparative studies. In the future, the CCRC plans to continue to perform hypothesis‐driven retrospective and prospective observational studies of CHD populations where controversy exists or where novel interventions or therapies have emerged. Quality improvement efforts including lesion‐specific registry development may be an additional potential future target.

Patent ductus arteriosus (PDA) is highly prevalent in extremely low birth weight (ELBW), preterm infants. There are diverse management approaches for the PDA in ELBW infants. The objectives of this research were to identify current PDA management practices among cardiologists and neonatologists in the United States, describe any significant differences in management, and describe areas where practices align. A survey of 10 questions based on the management of PDA in ELBW infants was conducted among 100 prominent neonatologists from 74 centers and 103 prominent cardiologists from 75 centers. Among the cardiologists, approximately 50% were interventionists who perform transcatheter PDA closures (TCPC). Fisher’s exact test was performed to compare practice variations among neonatologists and cardiologists. A potentially biased audience including a combination of health care providers belonging to cardiology, neonatology, and surgery were also surveyed during the International PDA Symposium. The results of this survey were not included for statistical comparison, due to this audience being potentially influenced by the Symposium. Statistically significant differences were identified between neonatologists and cardiologists regarding the impact of PDA closure on morbidity and mortality, with 80% cardiologists responding that it does vs 54% of neonatologists (P < 0.001), the need for PDA closure (P < .001), and the preferred method of PDA closure if indicated (P < .001). There was agreement between neonatologists and cardiologists on symptomatic therapy; however more neonatologists favored watchful waiting over intervention in contrast to more cardiologists favoring intervention over observation (77% vs 95%, P < .001). Survey responses also identified a need for further training and research on TCPC. Neonatologists and cardiologists have notable differences in managing PDA, and continued discussion across cardiology and neonatology has the potential to facilitate more of a consensus on best management practices. Further investigation is needed to identify outcomes in transcatheter PDA closure, particularly in ELBW infants.

The use of prostaglandin‐E1 immediately after birth and subsequent surgical creation of the modified Blalock‐Taussig shunt (BTS) shunt have remarkably improved the prognosis and survival of children with congenital heart disease and ductal‐dependent pulmonary blood flow (PBF). Despite the advancement in surgical techniques, bypass strategies, and postoperative management, significant morbidity and mortality after BTS still remain. Patent ductus arteriosus stenting has been shown to be as an acceptable alternative to BTS placement in select infants with ductal‐dependent PBF. Newer procedural techniques and equipment, along with operator experience have all contributed to procedural refinement associated with improved outcomes over the recent years. In this article, we review the procedural and periprocedural details, with an emphasis on recent advances of this procedure.

Infants with ductal‐dependent pulmonary blood flow (PBF) often undergo a palliative procedure to provide a stable source of PBF prior to definitive palliation or repair. In the current era, a surgical shunt or ductal stent is used to provide PBF. We aimed to review the current literature comparing ductal stents to surgical shunts. Four small, single‐center studies and two larger multicenter studies were identified comparing ductal stent to surgical shunt. Combined, these studies showed ductal stent resulted in similar or improved pulmonary artery growth, fewer complications, shorter length of stay, less diuretic use, and improved survival compared to surgical shunt. Despite inherent minor variability among the studies, ductal stent appears to be associated with more frequent reinterventions. Surgical shunts remain essential to the care of these patients, but ductal stent is a reasonable alternative, and may provide some advantages in select patients with ductal‐dependent PBF.

In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echocardiographic parameters associated with 2V circulation, (2) identify echocardiographic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions. We performed a retrospective analysis of patients who underwent RV decompression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1‐year or prior to Glenn circulation). Eighty‐one patients were included. At last follow‐up, 70 (86%) patients had 2V circulations, 7 (9%) had 1.5 ventricle circulations, and 4 (5%) had single ventricle circulations. Follow‐up echocardiograms were available in 43 (53%) patients. The majority of patients had improved RV systolic function, less tricuspid regurgitation (TR), and more left‐to‐right atrial shunting at a median of 350 days after decompression. Multivariable analysis demonstrated that larger baseline tricuspid valve (TV) z‐score (P= .017), ≥ moderate baseline TR (P= .045) and smaller baseline RV area (P< .001) were associated with larger increases in RV area. Baseline RV area ≥6 cm2/m2had 93% sensitivity and 80% specificity for identifying patients who ultimately achieved 2V circulation. All patients with RV area ≥8 cm2/m2at follow up achieved 2V circulation. This finding was confirmed in a validation cohort from a separate center (N = 25). Factors associated with achieving RV area ≥8 cm2/m2 included larger TV z‐score (P = .004), ≥ moderate baseline TR (P= .031), and ≥ moderate postdecompression pulmonary regurgitation (P= .002). Patients with PA/IVS and smaller TV annuli are at risk for poor RV growth. Volume‐loading conditions signal increased capacity for growth sufficient for 2V circulation.

Supplemental Digital Content is available in the text.There is a growing interest in the use of percutaneously delivered ventricular assist devices (PVAD) in the pediatric patient population. A 16 year old female and 18 year old male both status post heart transplantation presented with evidence of graft rejection and acute severe global systolic and diastolic heart failure necessitating hemodynamic catheterization and inotropic support. Both underwent percutaneous Impella CP LVAD (Abiomed, Danvers, MA) placement with close monitoring of right ventricular function. Although pulmonary artery wedge pressure (PAWP) improved, persistently elevated central venous pressures (CVP) and ongoing renal and liver injury prompted insertion of Impella RP RVAD. Explantation of bilateral devices was achieved within 4 days in the 18 year old patient. He would go on to require total artificial heart support before successful retransplantation. Despite bilateral PVAD support and antirejection medications, our 16 year old patient went on to develop multisystem organ failure and ultimately passed away after withdrawal of life-sustaining therapies. We report the first description of biventricular percutaneous Impella VAD for the treatment of acute decompensated heart failure in a pediatric institution. This innovative VAD option for older children/adolescents represents a less-invasive advancement in mechanical support technology with potential applicability across the pediatric population.

Congenital coronary artery fistula is a rare coronary anomaly. Most commonly, such fistulae drain into the right side of the heart or the pulmonary artery. Children with coronary artery fistulae are generally asymptomatic, although they may have left ventricular enlargement in the setting of a moderate sized left to right shunt. Symptoms of congestive heart failure or ischemia are very rare in neonatal period, and suggest the presence of a very large shunt and/or coronary steal. Single center retrospective review of transcatheter intervention on coronary artery fistulae presenting with symptoms in the neonatal period from January 2000 to March 2018. Patients with associated congenital heart diseases (except atrial septal defect) were excluded. Patient records, catheterization data, angiograms and noninvasive imaging were reviewed. Two patients underwent transcatheter intervention for symptomatic coronary artery fistula in the first few weeks of life. The first patient had multiple right and left coronary artery to right ventricle fistulae and presented with severe biventricular systolic dysfunction. Transcatheter closure of the fistulae was performed using multiple Gianturco coils. The second patient had a large left main coronary artery to left ventricle fistula (with left anterior descending and circumflex coronary artery atresia) presenting with symptoms of ischemia. This large fistula was closed using one Amplatzer Vascular Plug type‐II and two Micro‐Vascular Plugs. Both patients had improvement in symptoms post intervention and are doing well at the last follow up at 12 years and 7 months respectively. We hereby describe the rare presentation of symptomatic coronary artery fistulae in the neonatal period and their successful transcatheter management. This is also the first description of left anterior descending and circumflex coronary artery atresia in the setting of a large left main coronary artery to left ventricle fistula.

AbstractBackgroundAnomalous aortic origin of a coronary artery is the second leading cause of sudden cardiac arrest/death in young athletes in the United States of America. Limited data are available regarding family history in this patient population.MethodsPatients were evaluated prospectively from 12/2012 to 02/2017 in the Coronary Anomalies Program at Texas Children’s Hospital. Relevant family history included the presence of CHD, sudden cardiac arrest/death, arrhythmia/pacemaker use, cardiomyopathy, and atherosclerotic coronary artery disease before the age of 50 years. The presence of one or more of these in 1st- or 2nd-degree relatives was considered significant.ResultsOf 168 unrelated probands (171 patients total) included, 36 (21%) had significant family history involving 19 (53%) 1st-degree and 17 (47%) 2nd-degree relatives. Positive family history led to cardiology referral in nine (5%) patients and the presence of abnormal tests/symptoms in the remaining patients. Coronary anomalies in probands with positive family history were anomalous right (27), anomalous left (five), single right coronary artery (two), myocardial bridge (one), and anomalous circumflex coronary artery (one). Conditions present in their family members included sudden cardiac arrest/death (15, 42%), atherosclerotic coronary artery disease (14, 39%), cardiomyopathy (12, 33%), CHD (11, 31%), coronary anomalies (3, 8%), myocardial bridge (1, 3%), long-QT syndrome (2, 6%), and Wolff–Parkinson–White (1, 3%).ConclusionIn patients with anomalous aortic origin of a coronary artery and/or myocardial bridges, there appears to be familial clustering of cardiac diseases in approximately 20% of patients, half of these with early occurrence of sudden cardiac arrest/death in the family.

AbstractBackgroundTranscatheter right ventricle decompression in neonates with pulmonary atresia and intact ventricular septum is technically challenging, with risk of cardiac perforation and death. Further, despite successful right ventricle decompression, re-intervention on the pulmonary valve is common. The association between technical factors during right ventricle decompression and the risks of complications and re-intervention are not well described.MethodsThis is a multicentre retrospective study among the participating centres of the Congenital Catheterization Research Collaborative. Between 2005 and 2015, all neonates with pulmonary atresia and intact ventricular septum and attempted transcatheter right ventricle decompression were included. Technical factors evaluated included the use and characteristics of radiofrequency energy, maximal balloon-to-pulmonary valve annulus ratio, infundibular diameter, and right ventricle systolic pressure pre- and post-valvuloplasty (BPV). The primary end point was cardiac perforation or death; the secondary end point was re-intervention.ResultsA total of 99 neonates underwent transcatheter right ventricle decompression at a median of 3 days (IQR 2–5) of age, including 63 patients by radiofrequency and 32 by wire perforation of the pulmonary valve. There were 32 complications including 10 (10.5%) cardiac perforations, of which two resulted in death. Cardiac perforation was associated with the use of radiofrequency (p=0.047), longer radiofrequency duration (3.5 versus 2.0 seconds, p=0.02), and higher maximal radiofrequency energy (7.5 versus 5.0 J, p<0.01) but not with patient weight (p=0.09), pulmonary valve diameter (p=0.23), or infundibular diameter (p=0.57). Re-intervention was performed in 36 patients and was associated with higher post-intervention right ventricle pressure (median 60 versus 50 mmHg, p=0.041) and residual valve gradient (median 15 versus 10 mmHg, p=0.046), but not with balloon-to-pulmonary valve annulus ratio, atmospheric pressure used during BPV, or the presence of a residual balloon waist during BPV. Re-intervention was not associated with any right ventricle anatomic characteristics, including pulmonary valve diameter.ConclusionTechnical factors surrounding transcatheter right ventricle decompression in pulmonary atresia and intact ventricular septum influence the risk of procedural complications but not the risk of future re-intervention. Cardiac perforation is associated with the use of radiofrequency energy, as well as radiofrequency application characteristics. Re-intervention after right ventricle decompression for pulmonary atresia and intact ventricular septum is common and relates to haemodynamic measures surrounding initial BPV.

Renovascular hypertension (RVH) can be caused by renal artery stenosis (RAS) and/or middle aortic syndrome (MAS). Patients who received surgical or transcatheter treatment for RVH between 1/1991 and 11/2017 were retrospectively reviewed using age = adjusted blood pressure ratio (BPR). Fifty‐three patients diagnosed with RVH at a median age of 4.5 (0–18) years were included. Vascular involvement ranged from MAS with RAS (20), RAS only (32), and MAS only (1). The first intervention was transcatheter in 47 patients (transcatheter group: angioplasty = 41, stenting = 5, and thrombectomy = 1), and surgical in 6 patients (surgical group), occurring at a median age of 6.2 (0.1–19.6) years. There was a change toward transcatheter interventions as the first procedure over the study period. First reinterventions in the transcatheter group (27 lesions in 18 patients) were repeat transcatheter (in 20 lesions) and surgery (7 lesions) at a median of 92 (2–2555) days; in the surgical group (5 lesions in 4 patients) first reinterventions were transcatheter (4 lesions) and repeat surgery (1) at a median of 2.2 (1.1–12.0) years. A total of 136 transcatheter and 30 surgical discrete interventions were performed. There was a significant decline in antihypertensive medications and BPR at 4–6 months after the first intervention and on last follow‐up in patients initially treated by transcatheter means while the decline was not significant in the surgical group (limited by small sample size). Complications were significantly more common in the surgical group (P< .01), 11/27 (41%) vs 10/136 (7.4%). Four patients died (2 from each group): 2 with congenital renal artery atresia and MAS, 2 with MAS and RAS. The median follow‐up interval was 3.6 (0.1‐35.2) years. Pediatric patients with RVH treated with transcatheter means as the first intervention had significant improvement in BPR, as well as decline in antihypertensive medications and were less likely to suffer major complications.

Supplemental Digital Content is available in the text.

Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden cardiac death in young athletes in the USA. Long‐term outcome data for these patients are lacking to date. There is insufficient knowledge on the best approach to these patients and they are managed in a nonuniform manner. An online survey of 15 questions regarding management of AAOCA was sent out to 198 cardiac healthcare providers. The goal was to define gaps in knowledge to justify a dedicated scientific forum for discussion of AAOCA. Descriptive statistics were performed. A total of 91 providers (46%) completed the survey including pediatric cardiology subspecialists (40%), general pediatric cardiologists (24%), cardiovascular (CV) surgeons (22%), adult cardiologists (10%), nurse practitioners (8%), cardiology fellows (3%) and CV anesthesiologist (1%). Forty‐eight percent had been practicing for over 15 years and 28% were in their first 5 years of practice. Fifty‐two percent of the providers cared for adults and 93% cared for children/adolescents. Eighty‐eight percent were affiliated with an academic institution. All but one provider practiced in the USA, 62% practiced in Texas. Half of participants (50%) were very comfortable managing AAOCA patients and 36% were somewhat comfortable. Providers utilized various imaging tests to confirm the anatomy including computed tomography angiography 88%, cardiac magnetic resonance imaging 70%, cardiac catheterization 60%, echocardiogram 12%, IVUS 2% and myocardial perfusion scan 1%. The majority felt comfortable in counseling the families and felt that depending on the type of lesion these patients should get surgical referral (85%) vs clinical follow up (67%) with exercise restriction (65%). There is heterogeneity in the way AAOCA patients are currently evaluated and managed. A knowledge gap exists even with participants from academic institutions. Long‐term data with a defined approach to management of these patients may help to improve outcomes and prevent unnecessary exercise restriction or surgery.

Most diagnostic testing in patients with anomalous aortic origins of coronary arteries, myocardial bridges, and coronary artery changes after Kawasaki disease are performed with the use of noninvasive techniques. In some cases, however, further diagnostic information is needed to guide the clinician in treating these patients. In such instances, cardiac catheterization with invasive anatomic and functional testing is an invaluable tool. Moreover, interventional treatment in the cardiac catheterization laboratory may be performed in a small subset of these patients. As the diagnosis of these conditions is now becoming more common, it is important for pediatric interventional cardiologists to be familiar with these techniques. In this article, the role of angiography, intravascular ultrasound, fractional flow reserve, and optical coherence tomography in these patients is reviewed.

Introduction:Health-related quality of life (HRQOL) is impaired for patients with chronic illnesses and critical congenital heart disease (cCHD). For symptomatic neonates with tetralogy of Fallot who require neonatal intervention (sTOF), existing studies are limited but suggest early surgical management and ongoing disease burden negatively impact HRQOL. The current study aims to explore HRQOL in sTOF patients after primary repair (PR) vs. staged repair (SR; initial palliation with subsequent complete repair).Methods:The Pediatric Quality of Life Inventory (PedsQL) was used to assess HRQOL. Age-appropriate PedsQL modules and a parent survey were sent to all living sTOF patients with neonatal intervention from 2005-2017 across 9 centers. These were compared to established population data from children who are healthy, have chronic illness, and have cCHD. Differences in patient and clinical factors were compared between those with normal and low (>2 standard deviations below the mean) HRQOL scores.Results:Of 511 surveys sent, 143 were returned (28%). Of the respondents (53% male, age 3.2-15.9 years), PedsQL scores were lower than the healthy population (total score 75 vs 84, p<0.001) but similar to patients with chronic illnesses (74, p=0.64) and cCHD (76, p=0.72). Although only 7% and 2% of parents rated their child’s overall health and QOL as fair or poor, up to 27% of patients had PedsQL scores >2 SD below the healthy population mean. Factors associated with low HRQOL included longer duration of inhalational anesthetic exposure within 18 months of life (p=0.04) and any hospital complications after index procedure (p=0.01). There was no association between HRQOL and initial treatment strategy (PR vs SR, p=0.9).Conclusions:Lower HRQOL in sTOF is consistent with those with chronic illnesses and other forms of severe CHD. Initial treatment strategy was not associated with differences in HRQOL in sTOF patients so future comparison to other CHD groups, including non-cyanotic TOF patients, may help elucidate drivers of lower HRQOL. The discrepancy between parental description of QOL and standardized measurement of QOL is striking, warranting further investigation of parental expectations and potential impact on health resource utilization.

Introduction:Neonates with symptomatic tetralogy of Fallot (sTOF) require early intervention with either staged repair (SR; initial palliation with subsequent complete repair) or primary repair (PR). Implications of these neonatal management strategies on late outcomes are not well understood. Thus, we aimed to assess neurodevelopmental (ND) outcomes and identify risk factors for ND concerns.Methods:From 2005-2017, sTOF patients who underwent neonatal intervention across 9 centers were identified. To assess ND, all living patients were sent age-appropriate parent-rating scales of executive function (Behavior Rating Inventory of Executive Function - BRIEF) and psychosocial symptoms (The Behavior Assessment System for Children, 3rd Edition - BASC-3). The results were compared by primary treatment strategy (SR vs. PR) using multivariable models. Patient and clinical factors were compared between those with normal and clinically significant abnormal results (T-score ≥60).Results:Of 572 patients in the initial cohort, surveys were sent to 511 survivors and returned from 144 (28%, 53% male, age 3.2-15.9 years). Up to 37% of respondents had a clinically concerning score on ≥ 1 scales, including 36% with ≥1 abnormal scale present on the BRIEF and 30% with an abnormal scale on the BASC. Across SR and PR strategies, there was no difference in ND outcomes (BRIEF adjusted odds ratio (AOR) 1.55 (0.57, 4.21, p=0.39); BASC AOR 1.13 (0.43, 2.99, p=0.81)). Factors associated with clinically significant executive dysfunction included genetic syndrome (p=0.001), need for invasive ventilatory support (p=0.003) and inotropic support (p=0.01) prior to the index procedure. Only genetic syndrome (p=0.026) was associated with clinically significant psychosocial problems.Conclusions:Late ND abnormalities, specifically executive dysfunction and psychosocial concerns, are common in sTOF patients. While initial treatment strategy does not appear to have impact, intrinsic patient characteristics and pre-procedure measures of clinical severity may predict late ND outcomes and help target increased surveillance for ND complications. Forthcoming analyses will explore existing ND service utilization and parental concerns in the context of these findings.

In single ventricle physiology, focus on pulmonary vascular resistance neglects the resistance in the conduit supplying the pulmonary inflow.

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Introduction:Neonates with symptomatic tetralogy of Fallot (sTOF) require early intervention with staged repair (SR) or primary repair (PR). As sTOF is commonly associated with genetic syndromes, we examined outcomes of SR vs. PR for syndromic sTOF neonates.Hypothesis:We hypothesized that syndromic neonates with sTOF who underwent SR will have lower early mortality compared to PR.Methods:Syndromic neonates with sTOF who underwent initial intervention (initial palliation followed by later SR, or PR) from 2005-2017 were reviewed. The primary outcome was death. Secondary outcomes included complications, and hospital and intensive care unit (ICU) lengths of stay (LOS). Unadjusted and propensity score adjusted outcomes were compared.Results:The 176 syndromic sTOF neonates consisted mostly of DiGeorge (35%), trisomy 21 (16%), and VACTERL (10%). There were 110 and 66 neonates in the SR and PR groups, respectively. DiGeorge syndrome, pulmonary atresia, prematurity, and pre-procedural ventilation were more common in the SR group. The adjusted overall hazard of death was similar between groups, but early mortality favored SR (Figure). Procedural complications were lower in the SR group at the index procedure, but SR is associated with longer cumulative hospital and ICU LOS (Table).Conclusions:In syndromic neonates with sTOF, there was an early survival benefit to SR compared to PR, although there was no overall survival difference. The cumulative cost of the SR strategy was longer hospital and ICU LOS. These findings do not suggest a single superior management strategy in the syndromic sTOF population.

Introduction:Absent ductus arteriosus (ADA) is a poorly described feature associated with tetralogy of Fallot (TOF). We compare characteristics and outcomes of neonates with symptomatic TOF (sTOF) with ADA to those with a ductus arteriosus (DA).Hypothesis:We hypothesized: neonates with sTOF and ADA would have similar outcomes to those born with a DA, and a small portion of the ADA cohort would have critically-deficient pulmonary blood flow (CDPBF).Methods:Consecutive neonates with sTOF who underwent intervention during the neonatal period from 2005—2017 were identified from the Congenital Cardiac Research Collaborative. Those with DA on echocardiogram performed on the first day of life or on fetal echocardiogram were identified and compared to those with ADA. ADA neonates who required ECMO or intervention to augment PBF on the first day of life were defined as having CDPBF. Patient characteristics and outcomes were compared between DA and ADA groups.Results:Among 519 neonates, 460 (89%) had a DA and 59 (11%) had ADA. ADA neonates were more likely to carry a genetic or syndromic diagnosis, had smaller branch PA sizes, and were more likely to receive a transannular patch compared to those with a DA. Despite this, reintervention rates were similar (data not shown), and there was only a small difference in mortality that did not reach statistical significance. Of ADA neonates, 7/59 (12%) had CDPBF. Compared to ADA neonates without CDPBF, those with CDPBF had lower baseline oxygen saturation (58 vs 87%, p=0.031) and smaller pulmonary valve (PV) annular z-score (-2.9 vs -2.5, p=0.015) with similar reintervention rates and mortality.Conclusion:ADA is relatively common in sTOF. Despite higher rates of comorbidities, mid-term reintervention rates and mortality are similar to those with DA. CDPBF is a rare, high-risk feature of infants with sTOF and ADA. Smaller PV size may be a marker of CDPBF. Future work should focus on prenatal factors to identify fetuses with TOF, ADA and CDPBF.

Background:Anomalous aortic origin of a coronary artery (AAOCA) is the second most common cause of sudden cardiac death in youth during exertion. An optimal method to risk stratify patients with AAOCA and atypical symptoms is yet to be defined.Hypothesis:Invasive intracoronary hemodynamic flow measurement can be helpful in scenarios when there is conflict between clinical presentation and non-invasive studies.Methods:Case series of patients <21 years old with AAOCA who underwent cardiac catheterization with instantaneous wave-free ratio (iFR)/fractional flow reserve (FFR) measurement at baseline and during adenosine and dobutamine infusion. Baseline iFR <0.89 and/or FFR <0.8 were considered hemodynamically significant. All patients underwent dobutamine stress cardiac MRI to evaluate for inducible myocardial ischemia during provocative stress. Post-op assessment was described in patients who underwent surgical intervention.Results:A total of 8 patients, including 3 AAOCA of a left (AAOLCA) and 5 AAOCA of a right coronary artery (AAORCA) underwent cardiac catheterization including iFR and FFR measurement [Table]. iFR and FFR were reassuring in 4 patients, and suggestive of significantly impaired coronary flow in 2 AAOLCA and 2 AAORCA. There were no catheterization complications. Shared decision making was discussed, 2 patients with AAOLCA underwent successful surgical repair (patient 4 had normalized iFR/FFR after surgery and patient 8 is awaiting post-op assessment), and 2 are awaiting surgical scheduling. Patients not intervened upon are exercising with no restrictions, except one with concerns for cardiomyopathy.Conclusions:Invasive assessment using iFR/FFR with adenosine and dobutamine is helpful in shared decision making of AAOCA with conflicting noninvasive data and contribute to return to play after surgery. Long-term follow-up is essential to understand the predictive values of iFR/FFR in patients with AAOCA.

Introduction:Anomalous aortic origin of a coronary artery (AAOCA) confers risk of sudden death in the young. Paucity of data exists in AAOCA with high take-off (ht-AAOCA) from the aorta.Hypothesis:Patients with ht-AAOCA may be at risk of inducible myocardial ischemia and surgery might be indicated in a small subset with good medium-term outcomes.Methods:Patients with ht-AAOCA, defined as origin at the sinotubular junction and above by CTA, <25 years old were prospectively enrolled. Myocardial functional studies were obtained under provocative stress: exercise stress test, stress nuclear perfusion imaging, dobutamine stress cardiac MRI, or cardiac catherization (in select patients). These studies were repeated 3 months post-operatively (post-op) in surgical patients.Results:A total of 71 patients (45 male, 63%) with ht-AAOCA were enrolled, median age 15 [IQR 12; 19] years. ht-AAOCA of the right (ht-AAORCA) was seen in 66/71 patients (93%): 15 (23%) had exertional symptoms, 13 (20%) had nonexertional symptoms, 14 (21%) had both, 24 (37%) were asymptomatic. Ht-AAOCA of the left (ht-AAOLCA) was seen in 5/71 patients (7%), only 1 (20%) had exertional symptoms. In ht-AAORCA patients, 50/66 (76%) had abnormal ostia and 6/66 (9%) juxtacommissural origin, as compared to 3/5 (60%) and 2/5 (40%), respectively, in ht-AAOLCA patients. Average intramural course in ht-AAORCA was 4.46 mm and 2.36 mm in ht-AAOLCA. Abnormal functional testing corresponding to the affected territory was seen in 11/71 (15%) ht-AAOCA patients. Surgery was recommended for 18/71 (25%) patients due to concerning symptoms, coronary anatomy, or abnormal functional testing. Of these, 13/18 (72%) underwent coronary artery unroofing (in 12) or reimplantation (in 1), 3 declined surgery, 2 pending decision. All surgical patients have returned to unrestricted exercise after reassuring post-op studies, but 1/18 (6%) awaits completion. At median follow up of 1.8 years (range 0.1-8.9), all patients are alive and well, 93% are exercising with no restrictions.Conclusion:Although ht-AAOCA appears benign in most patients, 15% shows inducible myocardial ischemia. Surgery may be indicated in a subset of patients, with overall excellent medium-term outcomes and freedom from exercise restrictions.

Background:Stenting for aortic coarctation has been shown to be effective in the short term. The safety and longer term efficacy of transcatheter therapy, however, must be well established if the technique is to be widely accepted as an alternative to surgery. In order to determine the frequency, spectrum, and outcome of injury to the aortic wall caused by angioplasty or stenting of aortic coarctation, the nomenclature of mural injury in these patients must be adapted to the conditions of transcatheter therapy. Methods and Results:Between 1989 and July 2005, we inserted stents in 153 patients with aortic coarctation, their median age being 15.8 years. Prior aortic interventions had been performed in 98 patients, and preexisting aneurysms were observed in 19. Stenting resulted in a significant reduction of the gradient across the site of coarctation, from a median of 30 millimetres of mercury to zero (p less than 0.001), with a residual gradient within the aortic arch of 20 millimetres of mercury or more in 5 of patients. Acute injuries to the aortic wall, other than therapeutic tears, were observed in 3 patients (2), none of whom required surgery. At median follow-up of 2.5 years, this being more than 5 years in 30 patients, 4 patients had died, albeit none from complications relating to stenting or catheterization. Acute injuries to the aortic wall did not progress, and new aneurysms were observed in 6 of patients subsequent to follow-up imaging. Stent fractures, and jailed or partially covered brachiocephalic vessels, were observed in 12, and 49, patients, respectively, but did not result in haemodynamic or embolic complications. Conclusions:Stenting for aortic coarctation results in consistent relief of the gradient, and few serious complications in the short and intermediate term. Serious injuries to the aortic wall are uncommon in our experience, and can be minimized with a focus on technical measures, such as pre-dilation before stenting.

Critically ill children with cardiac disease often require prolonged central venous access. Thrombosis of systemic veins or the need to preserve vessels for future cardiac procedures limits sites for placement of central venous catheters in these patients. This study evaluates the use of Broviac placement via the transhepatic approach for this patient population.

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Introduction:Anomalous aortic origin of a coronary artery (AAOCA) with intraseptal course is considered a benign condition, although there have been case reports of myocardial ischemia, arrhythmia, and sudden cardiac death.Hypothesis:Myocardial ischemia is not uncommon in AAOCA with intraseptal course. The study was thus aimed to determine clinical presentation, myocardial perfusion upon provocative stress testing, and management of children with intraseptal AAOCA in a prospective cohort.Methods:Patients with intraseptal AAOCA were prospectively evaluated and managed using a standardized algorithm from 12/2012-05/2019. Stress nuclear imaging and/or magnetic resonance imaging were performed in patients 8.0 years old and/or symptoms of ischemia. Diastolic fractional flow reserve during dobutamine infusion (dFFRd) and/or instantaneous wave-free ratio (iFR) were performed in patients with perfusion deficits or high-risk features. The risks/benefits of exercise restriction and surgery were discussed.Results:A total of 18 patients (female 6, 33.3%), who presented with no symptoms (10, 55.6%), non-exertional (4, 22.2%), and exertional symptoms (4, 22.2%), were enrolled at a median age of 12.4 years (0.3-15.9). Perfusion imaging was performed in 14/18 (77.8%) and was abnormal in 7/14 (50%); dFFRd/iFR was positive in 5/8 (62.5%) (Figures 1). All 4 patients with exertional symptoms and 3/10 (30%) with no or non-exertional symptoms had inducible hypoperfusion. Coronary artery bypass grafting was performed in a 4-year-old child; beta blocker and exercise restriction were recommended in 4 other patients deemed not suitable for surgery. One patient had non-exertional chest pain and 17 were symptom-free at a median follow-up of 2.5 years (0.2-7.1).Conclusion:Up to 50% of patients with intraseptal AAOCA had inducible myocardial hypoperfusion during provocative testing, suggesting the natural history of intraseptal AAOCA may not be completely benign.

Introduction:Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention. Management strategies include initial palliation (IP) followed by later complete repair (CR) or primary repair (PR). A balanced comparison of these strategies is needed.Methods:Consecutive neonates with sTOF who underwent IP or PR at ?30 days of age from 2005-17 were retrospectively reviewed from the 9 center Congenital Catheterization Research Collaborative. Primary outcome was death or transplantation. Secondary outcomes included component (IP, CR, PR) and cumulative (IP+CR): hospital and ICU length of stay, durations of ventilation, inotrope use, cardiopulmonary bypass and anesthesia, procedural complications and reintervention. Outcomes were compared between treatment strategies using propensity score adjustment (PSA) to account for baseline differences between groups.Results:The cohort consisted of 327 IP (surgical, n=241; transcatheter, n=86) and 223 PR patients. Pre-procedural ventilation (28 vs. 18%, p=0.008), prematurity (24 vs. 15%, p=0.013) and DiGeorge syndrome (14 vs. 7%, p=0.018) were more common in the IP group. The observed risk of the primary outcome was not different between groups (10.1 vs 7.1%, p=0.23). PSA successfully balanced the groups. After PSA, the risk of the primary outcome remained similar between groups overall (9.6 vs. 10.7%, p=0.66), but favored IP during early (?3 months) follow-up (HR 0.45 [95% CI 0.22-0.89], p=0.023) and PR during late (>3 months) follow-up (HR 2.38 [0.87-6.52], p=0.091). Secondary outcomes are shown in Table 1.Conclusions:This is the first multicenter comparison of IP and PR for management of neonates with sTOF, adjusted for patient factors to account for confounding by indication. Early mortality, risk exposure and procedural complications were lower in the IP group, but cumulative exposures and reinterventions favored the PR group, suggesting each strategy has potential benefits.