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Background: Coronary artery fistulas (CAFs) presenting in infancy are rare, and data regarding postclosure sequelae and follow-up are limited. Methods: A retrospective review of all the neonates and infants ( Results: Forty-eight patients were included from 20 centers. Of these, 30 were proximal and 18 had distal CAF; 39 were large, 7 medium, and 2 had small CAF. The median age and weight was 0.16 years (0.01–1) and 4.2 kg (1.7–10.6). Heart failure was noted in 28 of 48 (58%) patients. Transcatheter closure was performed in 24, surgical closure in 18, and 6 were observed medically. Procedural success was 92% and 94 % for transcatheter closure and surgical closure, respectively. Follow-up data were obtained in 34 of 48 (70%) at a median of 2.9 (0.1–18) years. Angiography to assess remodeling was available in 20 of 48 (41%). I. Optimal remodeling (n=10, 7 proximal and 3 distal CAF). II. Suboptimal remodeling (n=7) included (A) symptomatic coronary thrombosis (n=2, distal CAF), (B) asymptomatic coronary thrombosis (n=3, 1 proximal and 2 distal CAF), and (C) partial thrombosis with residual cul-de-sac (n=1, proximal CAF) and vessel irregularity with stenosis (n=1, distal CAF). Finally, (III) persistent coronary artery dilation (n=4). Antiplatelets and anticoagulation were used in 31 and 7 patients post-closure, respectively. Overall, 7 of 10 (70%) with proximal CAF had optimal remodeling, but 5 of 11 (45%) with distal CAF had suboptimal remodeling. Only 1 of 7 patients with suboptimal remodeling were on anticoagulation. Conclusions: Neonates/infants with hemodynamically significant CAF can be treated by transcatheter or surgical closure with excellent procedural success. Patients with distal CAF are at higher risk for suboptimal remodeling. Postclosure anticoagulation and follow-up coronary anatomic evaluation are warranted.

Background: Stenting of the patent ductus arteriosus (PDA) is an established palliative option for infants with ductal-dependent pulmonary blood flow. Following initial palliation, reintervention on the PDA stent is common, but risk factors have not been characterized. Methods: Infants with ductal-dependent pulmonary blood flow palliated with PDA stent between 2008 and 2015 were reviewed within the Congenital Cardiac Research Collaborative. Rates and risk factors for reintervention were analyzed. Results: Among 105 infants who underwent successful PDA stenting, 41 patients (39%) underwent a total of 53 reinterventions on the PDA stent, with all but one occurring within 6 months of the initial intervention. Stent redilation constituted the majority of reintervention (n=35; 66%) followed by additional stent placement (n=11; 21%) and surgical shunt placement (n=7; 13%). The majority of reintervention was nonurgent, and there were no deaths during the reintervention procedure. All but one reintervention occurred within 6 months of the initial procedure. On univariate analysis, risk factors for reintervention included anticipated single-ventricle physiology, lack of prior balloon pulmonary valvuloplasty, use of drug-eluting stent, and increased ductal tortuosity. Conclusions: In infants with ductal-dependent pulmonary blood flow palliated with PDA stent implantation, reintervention is common, can be performed safely, and is associated with both anatomic/procedural factors and anticipated final physiology.

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 < 0.89 and/or FFR 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: 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.

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, 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.

To evaluate early growth following primary or staged repair of neonatal symptomatic tetralogy of Fallot (sTOF).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.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).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.

Background: Transcatheter pulmonary valve replacement (TPVR) is now an established modality for pulmonary valve replacement in suitable candidates. We aim to describe our experience with TPVR in adults. Methods: This is a descriptive study of all TPVR performed in adults with congenital heart disease at a single institution from 2010–2020. All adult patients (defined as 18 years old or older at TPVR) were included. Time-to-event outcomes were described using Kaplan-Meier estimates with 95% confidence intervals (CIs). Results: Out of a total of 200 patients that had undergone TPVR, 81 patients (57% male) met the inclusion criteria, with a median age and weight of 26 years (IQR 21–37) and 71.0 kg (IQR 54.6–89.0), respectively. In the cohort, 45 (56%) patients had tetralogy of Fallot. While 53 (65%) patients received a Melody valve, a Sapien valve (S3 in 20, XT in eight) was implanted in the rest. Pre-stenting was performed in 49 (52%) patients. One patient died of severe heart failure a year following TPVR. One patient had a second TPVR performed 2.2 years following initial TPVR for severe pulmonary regurgitation. Valve survival at 2.2 years was 94% (95% CI: 87–100%). Four patients developed endocarditis. Endocarditis-free survival was 89% (95% CI: 80–100%) at three years. Conclusions: Our experience suggests favorable results of TPVR in adults with congenital heart disease. Additional research would be warranted with a focus on total valve longevity and patient reported outcomes, in order to improve the understanding of TPVR in this population and further refine this technology.

BACKGROUND Carotid artery (CA) and axillary artery (AA) access are increasingly used for transcatheter stenting of the patent ductus arteriosus (PDA), although reports are limited. METHODS The Congenital Catheterization Research Collaborative (CCRC) reviewed multicenter data from infants who underwent PDA stenting via the CA or AA approach from 2008 to 2017, and compared outcomes to those of infants undergoing PDA stenting via the femoral artery (FA) approach. Post-procedure ultrasound (US) imaging was reviewed. RESULTS Forty-nine infants underwent PDA stenting from the CA (n = 43) or AA (n = 6) approach, compared with 55 infants who underwent PDA stenting from the FA approach. The PDA was the sole pulmonary blood flow (PBF) source in 61% of infants in the CA/AA cohort, compared with 33% of the FA cohort (p

Pulmonary hypertension (PH), defined as mean pulmonary arterial pressure ≥25 mm Hg, may be a complication of a secundum atrial septal defect (ASD). This study sought to evaluate the impact of PH at time of ASD device closure on patient survival. A prospectively collected database of ASD closures was utilized. Patients were stratified by age above and below the cohort median (48 years). Survival was analyzed by preprocedural PH status, age cohort, and echocardiographic resolution of PH at 3 months postdevice closure. PH was present in 48 of 228 patients (21.1%) and was more common in the older cohort (31.3% vs 10.6%, p0.01). ASD size was unrelated to the presence of PH (p = 0.33). Older patients had more medical co-morbidities including diabetes (p = 0.02), hyperlipidemia (p0.01), and systemic hypertension (p0.01) compared with younger patients. PH did not impact survival in patients ≤48 years, but PH was associated with fivefold increased risk of death in patients48 years (p0.01). Patients with preprocedural PH and RVSP ≥40 mm Hg at 3-month follow-up continued to have an increased risk of mortality (p0.01), whereas those with resolution of PH had similar survival to those without PH at time of closure. In conclusion, PH is common in adults with unrepaired ASDs and appears unrelated to defect size. PH in older adults and its persistence closure are strong predictors of a worsened clinical outcome. These patients may benefit from additional risk assessment and advanced medical therapies to mitigate this risk.

Objective 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. Design Retrospective chart review. Setting Single tertiary care pediatric referral center. Patients Patients who underwent CF-LVAD implantation from January 2012 to October 2017. Interventions Preimplantation invasive hemodynamic parameters were analyzed to evaluate the association with post-CF-LVAD need for prolonged (>72 hours) use of inotropes/pulmonary vasodilators. Measurements and main results 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. Conclusions 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 Anatomic lesions are a common cause of decompensation during the interstage period after Norwood stage 1 palliation (S1P). This study describes the risk factors for and outcomes after unplanned surgical and catheter-based interstage cardiac interventions. Methods Participants in the National Pediatric Cardiology Quality Improvement Collaborative registry discharged from the hospital after S1P between 2008 and 2016 were studied. Variables at S1P, interstage, and at stage 2 palliation (S2P) hospitalizations were examined. Multivariable logistic regression was used to compare those who had an unplanned interstage intervention to those who did not. Results Of 1994 participants from 60 programs, 343 (17.1%) had at least 1 unplanned interstage intervention. Aortic valve dilation before S1P, longer S1P cardiopulmonary bypass time, pulmonary artery stent placement between S1P and discharge, aortic arch obstruction on the S1P discharge echocardiogram, and lower weight at S1P discharge were independently associated with receiving an unplanned interstage intervention. Interstage mortality between groups was similar at 6%, as was interstage duration. Participants undergoing unplanned interstage interventions were more likely to undergo heart transplant before S2P or deemed to be unsuitable for S2P (7.3% vs 2.7%, P Conclusions Unplanned interstage interventions after S1P did not increase interstage mortality, but participants with an unplanned intervention were less likely to progress to S2P. Residual anatomic lesions are risk factors for unplanned interstage interventions. For those with progressive ventricular dysfunction in the presence of arch obstruction by echocardiogram, aortic arch reintervention is warranted.

Children with homozygous familial hypercholesterolemia are at risk for early cardiovascular events secondary to coronary artery disease. Current medical therapy does not ameliorate this risk. Liver transplantation offers the most effective option to reduce circulating levels of low-density lipoprotein cholesterol and thereby reduce risk of cardiovascular events. Angiographic evidence of regression of coronary artery disease is presented.

Objectives To describe our initial experience with pediatric transcatheter aortic valve replacement. Background Transcatheter aortic valve replacement (TAVR) has been approved and used to treat calcific aortic stenosis in adult patients. Select pediatric patients with congenital heart disease (CHD) who are poor candidates for conventional surgical aortic valve replacement can benefit from TAVR. Methods A retrospective review was performed to identify and describe pediatric patients with CHD who underwent transcatheter or hybrid aortic valve replacement using a Melody Valve (Medtronic, Minneapolis, MN), or Sapien S3/XT valve (Edwards Life sciences LLC, Irvine, CA). Patients in whom transcatheter valves were implanted on cardiopulmonary bypass were included. Imaging data, procedural elements, and clinical follow-up data were collected to evaluate acute and short-term results. Results A total of eight pediatric patients underwent treatment of aortic valvular disease using balloon expandable valves and delivery systems. Two patients had Melody valve implantation and six received a Sapien valve (one XT/five S3). In one patient, a Melody valve was placed surgically, failed, and was replaced with a Sapien valve 2 years later. Two patients were treated using a standard transfemoral route, four had the valve delivered on cardiopulmonary bypass via a median sternotomy, one was placed with a transapical approach, and one via a carotid cut down. Patients were followed for an average 16 months (range 1-208 weeks). There were no early or late deaths in this cohort. There were no embolic events, and all valves worked well in the immediate postoperative period. Both Melody implants developed moderate to severe regurgitation at 2 years and 4 years, respectively, and both required replacement at that time. One Sapien 3 valve developed a paravalvular leak that required reintervention within 6 months of implantation. Conclusions Transcatheter valves offer a reasonable alternative to traditional surgical aortic valve replacement in certain pediatric patients who are suboptimal surgical candidates. Hybrid approaches and valve delivery on cardiopulmonary bypass has been used in smaller patients. Long-term performance of these valves in young patients has not been studied.

OBJECTIVES To describe a novel technique for mechanical thrombectomy (MT) in young children. BACKGROUND Acute thrombosis in children may be a cause of significant morbidity or mortality. MT options in children are limited due to patient and vessel size, prompting consideration of innovative approaches. METHODS Two-center review of children with symptomatic non-cerebral thrombotic vessel occlusion. A novel approach to MT was performed using a deployed but unreleased amplatzer vascular plug (AVP) to "scrape" the target vessel in retrograde fashion, thereby harvesting the thrombus via the access sheath. RESULTS AVP-MT was performed in four patients at a median age of 2 months (range: 1 day-11 months) and median weight of 5.6 kg (1.8-10.9). In two cases, AVP-MT was performed in isolation in neonates on cervical extracorporeal membrane oxygenation (ECMO) support with cannula-associated obstructive aortic and brachiocephalic thrombus. AVP-MT was also performed as adjunctive therapy in a patient with single ventricle physiology and pulmonary artery thrombosis and in an infant with mitral valve endocarditis and femoral arterial thromboembolism. In all cases, AVP-MT was used to successfully remove thrombus from the target vessel and restore arterial flow, without procedural complications. Recurrent thrombosis occurred in both neonates, due to persistence of the ECMO cannula, with subsequent mortality. The other children were free of recurrent thrombus at 2 and 34 months. CONCLUSIONS Acute relief of symptomatic thrombosis may be achieved using AVP-MT in small children, although thrombosis frequently recurs, and clinical prognosis may remain poor. This strategy should be considered when alternative MT treatment options are not applicable.

Objectives To define patient and ventricular size parameters to guide Impella device (Abiomed, Inc., Danvers, MA) placement for mechanical circulatory support (MCS) in small pediatric patients (10-30 kg). Background There are few options for MCS in children, and there are no data on minimum patient size requirements for placement of the Impella 2.5 device. Methods This was a multicenter study of echocardiographic and magnetic resonance imaging (MRI) derived dimensions of the left ventricle (LV) length and ascending aorta used to define minimum size parameters that are necessary for the placement of the Impella catheter. Results Data were collected from 44 cardiac MRIs conducted in healthy pediatric patients and 39 echocardiograms performed in pediatric patients with cardiomyopathy prior to surgical ventricular assist device (VAD) placement. The Impella 2.5 catheter is 7.5 cm from the pigtail to the aortic annulus marker, thus requiring an LV apical length of 7.5 cm to allow the device to function in an unconstrained fashion. In the cohort of patients undergoing consideration for VAD placement, a minimum LV length of 7.5 cm corresponded to a height of 122 cm, weight of 23 kg, and body surface area (BSA) of 0.89 m2 . In the MRI cohort, this corresponded to a height of 121 cm, weight of 23.9 kg, and BSA of 0.89 m2 . Conclusion MCS with Impella devices is feasible in pediatric patients. This study defines anthropomorphic and anatomic measurements to guide providers in patient selection for MCS using the Impella devices.

In the era of neoadjuvant and adjuvant chemotherapy, extrapulmonary involvement by high-grade osteosarcoma (HGOS) is rare. Importantly however, HGOS metastasis in the form of cardiovascular tumor thrombus portends a dismal prognosis. Thus, prompt and accurate identification of tumor thrombus is crucial. We report here two unique cases of recurrent HGOS, presenting as thrombotic events, in order to increase awareness and index of suspicion among practitioners regarding this presentation. Additionally, we review 14 previously reported pediatric cases of cardiovascular metastatic HGOS.

Background 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. Methods and results 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. Conclusions 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.

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.

Pediatric cardiac interventions via percutaneous common carotid artery (CCA) access have been shown to be safe and effective. However, the impact of placement of a sheath in the carotid artery for interventions on cerebral perfusion is unknown. In this study we used cerebral near-infrared spectroscopy (NIRS) to analyze the effects of percutaneous CCA access for cardiac interventions on cerebral perfusion. This study is a retrospective chart review carried out at a tertiary care center on all pediatric patients who underwent percutaneous cardiac catheterization via carotid artery access from January 2010 to January 2020. All patients who had ipsilateral NIRS recorded on the side of carotid artery access were included. Patients with only partial or no ipsilateral NIRS data were excluded. The primary outcome measure was the change in NIRS upon CCA access; the mean NIRS for 15 min before obtaining access was compared to the mean NIRS during the procedure and to the mean NIRS 15 min after removal of the CCA sheath. We hypothesized that there would be a significant drop in NIRS values on the side of CCA access. There were 48 catheterizations in the study period where percutaneous CCA was accessed. Of those, 21 catheterizations had complete data and were included in the study. 13 (62%) were of males. The median age was 23 days (IQR 7-79). The indications for CCA access were patent ductus arteriosus stent implantation (n = 13; 62%), aortic valvuloplasty (n = 5; 24%), balloon angioplasty of coarctation of aorta (n = 2; 10%), and renal artery angioplasty (n = 1; 4%). In 16 patients (72%), the left common carotid artery was accessed. The median weight of the patients was 3.3 kg (IQR 2.8-2.9). The most common sheath size used was 4F, in 16 patients (72%). The mean NIRS prior to the procedure was 67 ± 15%, during the procedure was 68 ± 20%, and after removal of sheath was 68 ± 21%. Paired t test of cerebral NIRS before, during, and after the procedure showed no significant change with CCA access (P = 0.08). No patient in the series had a documented neurologic deficit following the procedure. Percutaneous CCA access was not associated with a decrease in NIRS on the side of the access during the procedure, suggesting there was no significant acute change in cerebral perfusion with CCA access.

To describe a deployment technique of the Gore Cardioform atrial septal defect (ASD) occluder (W.L. Gore and Associates) for large secundum ASDs and ASDs with challenging anatomy.The Gore Cardioform ASD occluder has recently been approved for closure of secundum ASDs; however, there are limitations to its delivery system.A retrospective study was conducted on the use of a Mullins sheath (Cook Medical) to facilitate Gore Cardioform ASD occluder delivery for secundum ASD closure in the cardiac catheterization laboratory from June, 2017 to December, 2019 at Texas Children's Hospital/Baylor College of Medicine.Out of 98 patients who underwent an attempt at ASD closure using the Gore Cardioform ASD occluder, a Mullins sheath was used in 52 patients (median age, 8 years [interquartile range, 4-13 years] and weight 27.2 kg [interquartile range, 17.9-51.2 kg]), with a successful implant in 46/52 patients (88%). The Mullins sheath was primarily used to deliver large devices (32 mm) in 38/46 successful implants (83%). There were 2 major adverse events (atrial fibrillation requiring cardioversion). At a median follow-up of 43 days (interquartile range, 1-374 days), no patient had more than a mild residual shunt. The ASD size, maximum sheath size, and device size were larger in patients in whom the Mullins sheath was used as compared with those patients in whom a Mullins sheath was not used.The Mullins sheath-facilitated delivery of the Gore Cardioform ASD occluder device may be a useful adjunct technique for closure of large secundum ASDs and secundum ASDs with challenging anatomy.

Background The modified Blalock-Taussig-Thomas shunt (mBTTs) is a critically important palliation for patients with insufficient pulmonary blood flow associated with congenital heart disease. Following creation of a mBTTs, patients experience high rates of early postoperative morbidity and mortality. Methods This is a single-institution retrospective cohort study. A query of the Society of Thoracic Surgeons database identified relevant patients whose health records were manually queried for echocardiography and operative reports. Patients with ductal-dependent systemic circulation were excluded. Primary outcomes were early serious adverse events and in-hospital mortality. Secondary outcomes were time to primary outcomes and postoperative lengths of stay. We investigated the correlation of demographics, presence of competitive pulmonary blood flow, and surgical and anatomic factors on outcomes. Results After exclusions, our cohort resulted in 155 patients. 33 (21.3%) patients experienced an early serious adverse event, ten (6.5%) early shunt malfunction, and 11 (7.1%) in-hospital mortality. Smaller shunt size, smaller shunted pulmonary artery size, surgical approach, and site of proximal shunt anastomosis were independently associated with morbidity and mortality. Conclusions Anatomical elements imparting increased resistance along the mBTTs predispose to increased morbidity and mortality, particularly in the early postoperative period. Despite the significant heterogeneity of patients receiving such shunts, similar risk profiles are observed regardless of lesion or presence of competitive flow. Surgical approach via thoracotomy with shunt anastomosis to the subclavian artery, where feasible, results in the subclavian artery as the point of natural resistance allowing for placement of larger shunts, yielding lower morbidity and mortality.

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.

Endocarditis has emerged as one of the most impactful adverse events after transcatheter pulmonary valve replacement (TPVR), but there is limited information about risk factors for and outcomes of this complication.The purpose of this study was to evaluate risk factors for and outcomes of endocarditis in a large multicenter cohort.The authors established an international registry focused on characterizing endocarditis after TPVR, including the incidence, risk factors, characteristics, and outcomes.Investigators submitted data for 2,476 patients who underwent TPVR between July 2005 and March 2020 and were followed for 8,475 patient-years. In total, 182 patients were diagnosed with endocarditis a median of 2.7 years after TPVR, for a cumulative incidence of 9.5% (95% CI: 7.9%-11.1%) at 5 years and 16.9% (95% CI: 14.2%-19.8%) at 8 years (accounting for competing risks: death, heart transplant, and explant) and an annualized incidence of 2.2 per 100 patient-years. Staphylococcus aureus and Viridans group Streptococcus species together accounted for 56% of cases. Multivariable analysis confirmed that younger age, a previous history of endocarditis, and a higher residual gradient were risk factors for endocarditis, but transcatheter pulmonary valve type was not. Overall, right ventricular outflow tract (RVOT) reintervention was less often to treat endocarditis than for other reasons, but valve explant was more often caused by endocarditis. Endocarditis was severe in 44% of patients, and 12 patients (6.6%) died, nearly all of whom were infected with Staphylococcus aureus.The incidence of endocarditis in this multicenter registry was constant over time and consistent with prior smaller studies. The findings of this study, along with ongoing efforts to understand and mitigate risk, will be critical to improve the lifetime management of patients with heart disease involving the RVOT. Although endocarditis can be a serious adverse outcome, TPVR remains an important tool in the management of RVOT dysfunction.

Invasive fractional flow reserve (FFR) is considered the gold standard to evaluate coronary artery flow. Stress cardiovascular magnetic resonance (sCMR) is an emerging non-invasive tool to evaluate myocardial perfusion in children. We sought to compare sCMR with FFR to determine impaired intracoronary flow in children with anomalous aortic origin of a coronary artery (AAOCA) and/or myocardial bridge (MB) who presented concern for myocardial ischemia.From December 2012 to May 2019, AAOCA and/or MB patients (20 years old) were prospectively enrolled and underwent sCMR and FFR. Abnormal sCMR included perfusion/regional wall-motion abnormality in the involved coronary distribution. FFR was performed at baseline and with dobutamine/regadenoson and considered abnormal if0.8 in the affected coronary segment.Of 376 patients evaluated, a total of 19 (age range, 0.2-17 years) underwent 24 sets of sCMR and FFR studies, with 5 repeat studies following intervention. Types of anomalies included 6 isolated MB/normal CA origins, 5 single CAs, 5 left AAOCAs, and 3 right AAOCAs. Seventeen patients (89.5%) had MB/intramyocardial course - 14 involving the left anterior descending coronary artery and 3 with multivessel involvement. sCMR correlated with FFR in 19/24 sets (7 sCMR and FFR positive, 12 sCMR and FFR negative) and it did not correlate in 5/24 sets. The positive percent agreement was 77.8%, negative percent agreement was 80.0%, and overall percent agreement was 79.2%.Assessment of myocardial perfusion using non-invasive sCMR concurred with FFR, particularly if performed with close proximity in time, and may contribute to risk stratification and decision making in children with AAOCA and/or MB.

Supplemental Material, sj-docx-1-pch-10.1177_2150135120978766 for Decreased Quality of Life in Children With Anomalous Aortic Origin of a Coronary Artery by Hitesh Agrawal, Carlos M. Mery, Sarah A. Sami, Athar M. Qureshi, Cory V. Noel, Katherine Cutitta, Prakash Masand, S. Kristen Sexson Tejtel, Yunfei Wang and Silvana Molossi in World Journal for Pediatric and Congenital Heart Surgery

Introduction: Neonates with tetralogy of Fallot and pulmonary atresia (TOF/PA) with pulmonary blood flow supplied by the ductus arteriosus require early intervention. This may be accomplished by: initial palliation (IP) followed by complete repair (CR) or initial primary repair (PR). The optimal approach for patients with TOF/PA has not been established. Methods: Neonates with TOF/PA who underwent IP or PR from 2005-17 were retrospectively reviewed from the Congenital Catheterization Research Collaborative. The primary outcome was mortality. Outcomes were compared as IP vs PR and IP+CR vs PR. Secondary outcomes included hospital and procedural complications and are listed in table 1. Propensity scoring was used to adjust for baseline differences between strategies. Results: Of 282 neonates with TOF/PA, 106 underwent PR and 176 underwent IP (144 surgical, 32 transcatheter). Prior to initial intervention, IP patients had higher rates of mechanical ventilation (83.9% vs 72.2%, p=0.023) and DiGeorge syndrome (14.77% vs 4.72%, p=0.009). Mortality was greater in the IP cohort (HR 2.7, 95% CI 1.02 - 7.1, p = 0.046), with no mortality in the PR cohort after 6 months post-repair. After adjustment, differences in survival were no longer significant (HR 1.4, 95% CI 0.7 - 3.1, p=0.39). Both cohorts had similar mechanical ventilation duration and inotrope use as well as procedural and hospital complications. Intensive care and hospital length of stay, cardiac bypass (CPB) and anesthesia time favored PR when compared to IP+CR (p= Conclusions: In neonates with TOF/PA, the IP approach is more often utilized in higher-risk patients. Accounting for this difference, IP and PR strategies have similar adjusted survival rates. Perioperative morbidities and lower risk for reintervention generally favor PR.

Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention.This study sought to perform a balanced multicenter comparison of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) versus primary repair (PR) treatment strategies.Consecutive neonates with sTOF who underwent IP or PR at ≤30 days of age from 2005 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was death. Secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital and intensive care unit lengths of stay; durations of cardiopulmonary bypass, anesthesia, ventilation, and inotrope use; and complication and reintervention rates. Outcomes were compared using propensity score adjustment.The cohort consisted of 342 patients who underwent SR (IP: surgical, n = 256; transcatheter, n = 86) and 230 patients who underwent PR. Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the SR group (p ≤0.01). The observed risk of death was not different between the groups (10.2% vs 7.4%; p = 0.25) at median 4.3 years. After adjustment, the hazard of death remained similar between groups (hazard ratio: 0.82; 95% confidence interval: 0.49 to 1.38; p = 0.456), but it favored SR during early follow-up (4 months; p = 0.041). Secondary outcomes favored the SR group in component analysis, whereas they largely favored PR in cumulative analysis. Reintervention risk was higher in the SR group (p = 0.002).In this multicenter comparison of SR or PR for management of neonates with sTOF, adjusted for patient-related factors, early mortality and neonatal morbidity were lower in the SR group, but cumulative morbidity and reinterventions favored the PR group, findings suggesting potential benefits to each strategy.

Patent ductus arteriosus (PDA) stenting is an accepted method for securing pulmonary blood flow in cyanotic neonates. In neonates with pulmonary atresia and single source ductal-dependent pulmonary blood flow (SSPBF), PDA stenting remains controversial. We sought to evaluate outcomes in neonates with SSPBF, comparing PDA stenting and surgical Blalock-Taussig shunt (BTS). Neonates with SSPBF who underwent PDA stenting or BTS at the four centers of the Congenital Catheterization Research Collaborative from January 2008 to December 2015 were retrospectively reviewed. Reintervention on the BTS or PDA stent prior to planned surgical repair served as the primary endpoint. Additional analyses of peri-procedural complications, interventions, and pulmonary artery growth were performed. A propensity score was utilized to adjust for differences in factors. Thirty-five patients with PDA stents and 156 patients with BTS were included. The cohorts had similar baseline characteristics, procedural complications, and mortality. Interstage reintervention rates were higher in the PDA stent cohort (48.6% vs. 15.4%, p 0.001).

An extremely low birthweight infant (940 grams) with a rare variant of obstructed infracardiac total anomalous pulmonary venous return underwent stenting of the venous duct as bridge to later surgical intervention. While technically challenging, this procedure represents a bridge to surgery for infants who might otherwise not be surgical candidates.

To compare management strategies for neonates2.5 kg with tetralogy of Fallot and symptomatic cyanosis who either undergo staged repair (SR) (initial palliation followed by later complete repair) or primary repair (PR).Consecutive neonates with tetralogy of Fallot and symptomatic cyanosis weighing2.5 kg at initial intervention and between 2005 and 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Primary outcome was mortality and secondary outcomes included component (eg, initial palliation, complete repair, or primary repair) and cumulative (SR: initial palliation followed by later complete repair) hospital and intensive care unit lengths of stay, durations of ventilation, inotrope use, cardiopulmonary bypass time, procedural complications, and reintervention. Outcomes were compared with propensity score adjustments with PR as the reference group.The cohort included 76 SR (initial palliation: 53 surgical and 23 transcatheter) and 44 PR patients. The observed risk of overall mortality was similar between SR and PR groups (15.8% vs 18.2%: P = .735). The adjusted hazard of mortality remained similar between groups overall (hazard ratio, 0.59; 95% confidence interval, 0.26-1.36; P = .214), as well as during short-term (4 months: hazard ratio, 0.37; 95% confidence interval, 0.13-1.09; P = .071) and midterm (4 months: hazard ratio, 1.32; 95% confidence interval, 0.30-5.79; P = .717) follow-up. Reintervention in the first 18 months was common in both groups (53.2% vs 48.4%; hazard ratio, 1.69; 95% confidence interval, 0.96-2.28; P = .072). Adjusted procedural complications and neonatal morbidity burden were overall lower in the SR group. Cumulative secondary outcome burdens largely favored the PR group.In this study comparing SR and PR treatment strategies for neonates with tetralogy of Fallot and symptomatic cyanosis and weight2.5 kg, mortality and reintervention burden was high and independent of treatment strategy. Other potential advantages were observed with each approach.

Background There are limited published data focused on outcomes of transcatheter pulmonary valve replacement (TPVR) with either a Sapien XT or Sapien 3 (S3) valve. Objectives This study sought to report short-term outcomes in a large cohort of patients who underwent TPVR with either a Sapien XT or S3 valve. Methods Data were entered retrospectively into a multicenter registry for patients who underwent attempted TPVR with a Sapien XT or S3 valve. Patient-related, procedural, and short-term outcomes data were characterized overall and according to type of right ventricular outflow tract (RVOT) anatomy. Results Twenty-three centers enrolled a total of 774 patients: 397 (51%) with a native/patched RVOT; 183 (24%) with a conduit; and 194 (25%) with a bioprosthetic valve. The S3 was used in 78% of patients, and the XT was used in 22%, with most patients receiving a 29-mm (39%) or 26-mm (34%) valve. The implant was technically successful in 754 (97.4%) patients. Serious adverse events were reported in 67 patients (10%), with no difference between RVOT anatomy groups. Fourteen patients underwent urgent surgery. Nine patients had a second valve implanted. Among patients with available data, tricuspid valve injury was documented in 11 (1.7%), and 9 others (1.3%) had new moderate or severe regurgitation 2 grades higher than pre-implantation, for 20 (3.0%) total patients with tricuspid valve complications. Valve function at discharge was excellent in most patients, but 58 (8.5%) had moderate or greater pulmonary regurgitation or maximum Doppler gradients >40 mm Hg. During limited follow-up (n = 349; median: 12 months), 9 patients were diagnosed with endocarditis, and 17 additional patients underwent surgical valve replacement or valve-in-valve TPVR. Conclusions Acute outcomes after TPVR with balloon-expandable valves were generally excellent in all types of RVOT. Additional data and longer follow-up will be necessary to gain insight into these issues.

Epic Supra valves have been used off-label in the pulmonary position. We aim to evaluate the durability of Epic valves in the pulmonary position. We performed a retrospective review of all Epic valves placed in the pulmonary position from October 2008 to May 2019. Time-to-event analysis was performed using Kaplan-Meier estimates to evaluate freedom from valve intervention, moderate pulmonary regurgitation, and peak velocity greater than 3.5 m/s. Valve dysfunction was a composite of all 3 end points. A total of 79 patients had Epic valves implanted in the pulmonary position. Median age was 18.5 years (15th–85th percentile 11.2–41.0). In total, 1 (1%) 19 mm valve, 4 (5%) 21 mm valves, 8 (10%) 23 mm valves, 23 (29%) 25 mm valves, and 43 (54%) 27 mm valves were implanted. There were no deaths or transplants. Median follow-up was 3.1 years (interquartile range 1.0–5.5). At 5 years, freedom from valve intervention was 95%, freedom from valve dysfunction was 68%, freedom from moderate pulmonary regurgitation was 73%, and freedom from peak velocity greater than 3.5 m/s was 82%. Epic Supra valves provide an acceptable valve replacement in the pulmonary position for children and adults. Longer follow-up is needed to determine valve durability through the entirety of the valve life expectancy.

Instantaneous wave-free ratio (iFR) has been proven to correlate with coronary flow reserve better than fractional flow reserve (FFR) and is non-inferior to FFR in guiding coronary revascularization in ischemic heart disease. There has been no study validating the utility of iFR in children.We performed a retrospective review of clinically indicated cases in which both FFR and iFR were obtained at Texas Children's Hospital from July, 2016 to March, 2019. FFR and iFR were obtained at baseline. Adenosine FFR (FFRa) was used for assessment of coronary artery (CA) stenoses and diastolic dobutamine FFR (dFFRd) for myocardial bridges (MBs). FFRa or dFFRd ≤0.8 and iFR ≤0.89 indicated significant flow impairment.A total of 22 coronary arteries (9 CA stenoses and 13 MBs) were assessed in 20 patients with median age of 13 years (range, 4-21 years) and median weight of 60 kg (range, 19-110 kg). iFR correlated with FFRa (Spearman's rho, 0.87; P.01) in CA stenoses and with dFFRd (Spearman's rho, 0.74; P.01) in MBs and agreed with FFR in 20/22 cases (90.9%). In 1 patient with CA stenosis and 1 MB with normal FFR, iFR was positive and both patients underwent coronary revascularization.iFR correlated with FFR in the assessment of CA stenoses in children. iFR does not require administration of pharmacological agents; thus, it may reduce procedural time, cost, and complications, and result in more widespread adoption of invasive assessment of CA lesions in young patients.

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.

The novel coronavirus (COVID-19) pandemic has placed severe stress on healthcare systems around the world. There is limited information on current practices in pediatric cardiac catheterization laboratories in the United States (US).To describe current practice patterns and make recommendations regarding potential resource allocation for congenital cardiac catheterization during the COVID-19 pandemic.A web-based survey was distributed regarding case candidacy and catheterization laboratory preparedness. Centers were categorized based on the current degree of disease burden in that community (as of April 1, 2020). Data and consensus opinion were utilized to develop recommendations.Respondents belonged to 56 unique US centers, with 27 (48.2%) located in counties with a high number of COVID-19 cases. All centers have canceled elective procedures. There was relative uniformity (88% agreement) among centers as to which procedures were considered elective. To date, only three centers have performed a catheterization on a confirmed COVID-19 positive patient. Centers located in areas with a higher number of COVID-9 cases have been more involved in a simulation of donning and doffing personal protective equipment (PPE) than low-prevalence centers (46.7% vs 10.3%, respectively; P.001). Currently, only a small fraction of operators has been reassigned to provide clinical services outside their scope of practice.At this stage in the COVID-19 pandemic, pediatric/congenital catheterization laboratories have dramatically reduced case volumes. This document serves to define current patterns and provides guidance and recommendations on the preservation and repurposing of resources to help pediatric cardiac programs develop strategies for patient care during this unprecedented crisis.

Background: Intraseptal anomalous aortic origin of a coronary artery is considered a benign condition. However, there have been case reports of patients with myocardial ischemia, arrhythmia, and sudden cardiac death. The purpose of this study was to determine the clinical presentation, myocardial perfusion on provocative stress testing, and management of children with anomalous aortic origin of a coronary artery with an intraseptal course in a prospective cohort. Methods: Patients with anomalous aortic origin of a coronary artery and intraseptal course were prospectively enrolled from December 2012 to May 2019, evaluated, and managed following a standardized algorithm. Myocardial perfusion was assessed using stress imaging. Fractional flow reserve was performed in patients with myocardial hypoperfusion on noninvasive testing. Exercise restriction, β-blockers, and surgical intervention were discussed with the families. Results: Eighteen patients (female 6, 33.3%), who presented with no symptoms (10, 55.6%), nonexertional (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%); fractional flow reserve was positive in 5/8 (62.5%). All 4 patients with exertional symptoms and 3/10 (30%) with no or nonexertional symptoms had myocardial hypoperfusion. Coronary artery bypass grafting was performed in a 4-year-old patient; β-blocker and exercise restriction were recommended in 4 patients not suitable for surgery. One patient had nonexertional chest pain and 17 were symptom-free at median follow-up of 2.5 years (0.2–7.1). Conclusions: Up to 50% of patients with intraseptal anomalous aortic origin of a coronary artery had inducible myocardial hypoperfusion during noninvasive provocative testing. Long-term follow-up is necessary to understand the natural history of this rare anomaly.

Background: Anomalous aortic origin of a coronary artery (CA) is the second leading cause of sudden cardiac death in young athletes. Management is controversial and longitudinal follow-up data are sparse. We aim to evaluate outcomes in a prospective study of anomalous aortic origin of CA patients following a standardized algorithm. Methods: Patients with anomalous aortic origin of a CA were followed prospectively from December 2012 to April 2017. All patients were evaluated following a standardized algorithm, and data were reviewed by a dedicated multidisciplinary team. Assessment of myocardial perfusion was performed using stress imaging. High-risk patients (high-risk anatomy—anomalous left CA from the opposite sinus, presence of intramurality, abnormal ostium—and symptoms or evidence of myocardial ischemia) were offered surgery or exercise restriction (if deemed high risk for surgical intervention). Univariate and multivariable analyses were used to determine predictors of high risk. Results: Of 201 patients evaluated, 163 met inclusion criteria: 116 anomalous right CA (71%), 25 anomalous left CA (15%), 17 single CA (10%), and 5 anomalous circumflex CA (3%). Patients presented as an incidental finding (n=80, 49%), with exertional (n=31, 21%) and nonexertional (n=32, 20%) symptoms and following sudden cardiac arrest/shock (n=5, 3%). Eighty-two patients (50.3%) were considered high risk. Predictors of high risk were older age at diagnosis, black race, intramural course, and exertional syncope. Most patients (82%) are allowed unrestrictive sports activities. Forty-seven patients had surgery (11 anomalous left CA and 36 anomalous right CA), 3 (6.4%) remained restricted from sports activities. All patients are alive at a median follow-up of 1.6 (interquartile range, 0.7–2.8) years. Conclusions: In this prospective cohort of patients with anomalous aortic origin of a CA, most have remained free of exercise restrictions. Development of a multidisciplinary team has allowed a consistent approach and may have implications in risk stratification and long-term prognosis.

To review the most recent literature on pediatric transcatheter ductal intervention including ductus arteriosus occlusion and stenting. With the development and FDA approval of smaller ductal devices, including most recently the Amplatzer Piccolo Occluder (Abbott, Abbott Park, IL), transcatheter ductus arteriosus device closure is now being safely performed in premature infants and patients

BACKGROUND 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. METHODS 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). RESULTS 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

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.

Background 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. Methods 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. Results 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. Conclusions 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.

Objectives: This study sought to determine the safety and feasibility of transcatheter pulmonary valve replacement (TPVR) using the Melody valve in native (nonconduit) right ventricular out...