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Microvascular pulmonary arteriovenous malformations (pAVMs) can lead to profound hypoxemia. “Hepatic factor” is postulated to play a role in their development. Certain patients with congenital heart disease are at particular risk to develop pAVMs, including those with heterotaxy syndromes and complex Fontan palliation. Ideally, an underlying cause is identified and corrected, although pAVMs may persist despite those interventions. We report a patient with heterotaxy syndrome s/p Fontan who had pAVMs that persisted despite Fontan revision with equal hepatic flow to both lungs. We employed a novel method to produce a diabolo configuration of a large covered stent to restrict lung flow while maintaining the potential for future dilation.

Previous studies have used cardiopulmonary exercise test (CPET) data to objectively assess physiological changes in patients undergoing percutaneous pulmonary valve implantation. A retrospective review was performed to assess pre- and post-CPET data among patients undergoing Alterra Adaptive Prestent and SAPIEN 3 transcatheter heart valve (Alterra) placement. Of the 7 patients eligible for the study, 5 (71%) were male. The mean age was 22 years (range: 12-49 years). CPET data showed significant (P = 0.03) improvement in ventilatory efficiency (VE/VCO2) while only 2 (29%) patients had an improvement of percent predicted peak oxygen consumption (VO2). These findings suggest favourable haemodynamic changes though further investigation is needed.

Introduction While frequently performed in the adult population, percutaneous coronary artery stent angioplasty (CSA) in infants is rare. CSA in infants is challenging because of limited options in terms of appropriately sized (length and diameter) stents, concern about stenting vessels with significant growth potential and limited data regarding durability of benefit. We report a multicenter case series of infants who underwent CSA.Methods A multicenter, retrospective case series of infants who underwent percutaneous CSA to treat post-operative coronary artery stenoses was performed.Results Six infants from 3 institutions who underwent post-operative CSA were identified. The anatomic diagnoses were d-transposition of the great arteries in 3 cases, anomalous left coronary artery from the pulmonary artery in 2 and supravalvar aortic stenosis in 1. All infants were critically ill at the time of CSA. Diameters of coronary artery stents used ranged from 2.25 to 2.75 mm. There were no procedural complications. All stents were patent immediately after placement and the clinical condition improved or stabilized in all patients. Follow-up angiography was available for 3 patients at 4 to 16 months post-CSA, at which time 67% (2/3) remained patent.Conclusion CSA is a feasible and effective therapy for critically ill infants with post-surgical coronary obstruction. Treatment appears to allow at least short-term reperfusion to facilitate recovery of ventricular function and potential development of collateral circulation when longer-term stent patency is not achieved. Longer-term stent patency and coronary artery health remain unanswered questions.

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.

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Over the last 2 decades, experience with transcatheter pulmonary valve replacement (TPVR) has grown significantly and has become an effective and reliable way of treating pulmonary valve regurgitation, right ventricular outflow (RVOT) obstruction, and dysfunctional bioprosthetic valves and conduits. With the introduction of self-expanding valves and prestents, dilated native RVOT can be addressed with the transcatheter approach. In this article, the authors review the current practices, technical challenges, and outcomes of TPVR.

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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.

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.

Background:Obstruction of the superior vena cava (SVC) is thought to be mostly iatrogenic. Transcatheter interventions relieve stenosis with good long-term results, but there is a paucity of published data. We aim to describe patient characteristics and outcomes after transcatheter SVC intervention.Methods:Single-center, retrospective study of patients who underwent transcatheter SVC intervention from December 2006 to January 2020. Patient characteristics included age, gender, height, weight, and BSA. Clinical parameters included congenital heart disease (CHD), orthotopic heart transplant (OHT), upper extremity central venous line (CVL), extracorporeal membranous oxygenation (ECMO), mortality, and symptoms. Procedural data included pre- and post-intervention SVC pressure gradient, dimensions, and complications.Results:A total of 42 patients (median age 1.5 years, IQR [0.31-15], 64.2% male) underwent 81 procedures with 1 major complication, and 20 patients (47.6%) had re-interventions. 12 patients (31.5%) had symptoms. Affected patient groups were as follows: history of CVL (92.9%), CHD (76.2%), post-OHT (42.9%), and post-ECMO (30.9%). Those who underwent initial balloon angioplasty were significantly younger (mean ± SD: 3.9 ± 6.8 vs. 13.1 ± 11.3 years, p<0.01) and smaller (15.9 ± 20.4 vs. 39.9 ± 31.2 kg, p<0.01) than those with initial stent. A lower pressure gradient and larger post-intervention dimension was achieved after stenting (p<0.01). Re-intervention rates were similar; 50% after balloon and 43.8% after stent, but nearly half of the angioplasty cohort resolved after repeat angioplasty alone, while most stents (85.7%) required serial re-dilation. Affected patient groups were not associated with re-intervention, and there were no significant differences in patient or procedural characteristics between those who did and did not require re-intervention.Conclusions:We describe at-risk groups who warrant surveillance of SVC stenosis in the setting of a low-symptom rate. Strongly consider balloon angioplasty as initial therapy, which may avoid the need for stent and subsequent re-dilations associated with growth. Re-intervention is likely after balloon angioplasty and stenting, but both are safe and effective.

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.

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The aim of this study was to determine the feasibility of performing complete early fetal echocardiography (FE) at <17 weeks of gestation with comparison with standard FE in the midtrimester (17–23 weeks).

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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.

Numerous studies have detailed outcomes of Transcatheter pulmonary valve placement (TPV) in young patients, but there is a paucity of data in older patients. We sought to evaluate short and mid-term outcomes of TPV in patients older than 40 years of age.

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.

Congenital systemic-to-pulmonary collateral arteries or major aortopulmonary collaterals are associated with cyanotic congenital heart disease with decreased pulmonary blood flow. Though it is usually associated with congenital heart diseases, there is an increased incidence of isolated acquired aortopulmonary collaterals in premature infants with chronic lung disease. Interestingly, isolated congenital aortopulmonary collaterals can occur without any lung disease, which may cause congestive heart failure and require closure. We present a neonate with an echocardiogram that showed only left-sided heart dilation. Further workup with a CT angiogram demonstrated an anomalous systemic artery from the descending thoracic aorta supplying the left lower lobe. He eventually developed heart failure symptoms and was taken to the catheterization laboratory for closure of the collateral. However, with the collateral being the only source of blood flow to the entire left lower lobe, he required surgical unifocalization. Isolated aortopulmonary collaterals without any other congenital heart disease or lung disease are rare. Our patient is the first reported case to have an isolated aortopulmonary collateral being the sole pulmonary blood supply to an entire lung segment. Due to its rarity, there is still much to learn about the origin and development of these collaterals that possibly developed prenatally.