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Background: We describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus., Methods: We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair., Results: Median age at centralization was 9 days (minimum-maximum, 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n =20 [91%]; 2 after bilateral ductal stents) or bidirectional cavopulmonary connection (n = 2 [9%]) using pericardial roll (n = 14 [64%]), patch-augmented direct anastomosis (n = 7 [32%]), and interposition graft (n = 1 [5%]) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n = 11 [50%]) was associated with significantly inferior survival (P = .01). Five patients (23%) died at a median of 59 days (minimum-maximum, 6-257 days) after centralization, all with noncardiac TAPVC. At the latest follow-up for 17 survivors (median, 13.5 years; minimum-maximum, 0.5-25.1 years after centralization), 12 completed Fontan, 4 completed second-stage palliation, and 1 received a transplant before second-stage palliation. PA reintervention was required in 14 patients (64%), including 3 with reoperations independent of staged palliation. Echocardiography from baseline to before the second stage demonstrated branch PA growth with significantly increased diameters (left, P = .0006; right, P = .0002); z-scores significantly increased for right (P = .004) but not left (P = .11)., Conclusions: Successful single-ventricle palliation is possible, although high risk, for patients with bilateral discontinuous ductal PAs. Early postcentralization mortality remains substantial, particularly with associated noncardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation., Competing Interests: Disclosures Athar M. Qureshi reports a relationship with W. L. Gore & Associates, Inc. that includes: consulting or advisory; with Medtronic PLC that includes: consulting or advisory; and with B. Braun Medical Inc that includes: consulting or advisory. The other authors have no conflicts of interest to disclose., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Unilateral proximal pulmonary artery stenosis is often seen in the setting of postoperative congenital heart disease. Accurate assessment of the hemodynamic significance of such a lesion is important so as to determine "When to intervene?" A thorough evaluation should include symptom assessment, anatomical assessment through detailed imaging, functional assessment using differential pulmonary blood flow measurement and cardiopulmonary exercise testing. Symptoms of exertional dyspnea or intolerance, decreased pulmonary blood flow to stenosed lung, and abnormal exertional performance would be factors to pursue therapy in the setting of significant anatomical narrowing. Safe and effective therapy can be offered through transcatheter or surgical techniques and has been shown to improve exertional performance., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Isolated pulmonary artery (PA) of ductal origin (IPADO) is a rare cardiac defect which requires surgical repair, with or without preceding palliation. We sought to determine the impact of treatment strategy on outcomes. Retrospective study of consecutive patients with IPADO that underwent staged or primary repair from 1/05 to 9/16 at 6 Congenital Cardiac Research Collaborative centers. Patients with single ventricle physiology, major aortopulmonary collaterals, or bilateral IPADO were excluded. Primary outcome was isolated PA z-score at late follow-up. Secondary outcomes included PA symmetry index (isolated:confluent PA diameter) and reintervention burden. Propensity score adjustment was used to account for baseline differences. Of 60 patients in the study cohort, 26 (43%) underwent staged and 34 (57%) primary repair. The staged and primary repair groups differed in weight at diagnosis and presence of other heart disease but not in baseline PA dimensions. Staged patients underwent ductal stent (n = 16) or surgical shunt (n = 10) placement followed by repair at 210 vs. 21 days in the primary repair group (p < 0.001). At median follow-up of 4.5 years post-repair, after adjustment, isolated PA z-score (- 0.74 [- 1.75, - 0.26] vs. - 1.95 [- 2.91, - 1.59], p = 0.012) and PA symmetry index (0.81 [0.49, 1.0] vs. 0.55 [0.48, 0.69], p = 0.042) significantly favored the staged repair group. Freedom from PA reintervention was not different between groups (adjusted HR 0.78 [0.41, 1.48]; p = 0.445). A staged approach to repair of IPADO is associated with superior isolated PA size and symmetry at late follow-up. Consideration should be given to initial palliation in IPADO patients, when feasible.

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.

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 m 2 (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., (© 2019 Wiley Periodicals, Inc.)

Background: Patients with single ventricle anatomy and ductal-dependent pulmonary blood flow may be initially palliated with either modified Blalock-Taussig shunt (BTS) or ductus arteriosus stent (DAS). Comparisons of outcomes during the interstage period and at the time of superior cavopulmonary connection (SCPC) are lacking and may differ between palliation strategies., Methods: Infants with single ventricle anatomy and ductal-dependent pulmonary blood flow palliated with either DAS or BTS from 2008 to 2015 were reviewed across 4 centers. Interstage outcomes, and for those who had SCPC, anatomy, hemodynamics, and perioperative clinical outcomes were compared. Thirty-five patients with DAS and 136 patients with BTS were included., Results: At initial palliation, demographic, clinical variables, and pulmonary artery size were similar. Interstage death, transplant, or unplanned reintervention to treat cyanosis occurred in 25.7% of DAS and 35.8% of BTS, P =0.27. Reintervention was more common with DAS (48.6% versus 2.2%; P <0.001). Twenty-three DAS patients and 111 BTS patients underwent SCPC. Preoperative hemodynamics and overall pulmonary atresia growth were similar, although right pulmonary artery growth was better with DAS (change in z -score: 1.57 versus 0.65, P =0.026). SCPC intraoperative and postoperative courses were similar., Conclusions: In patients with single-ventricle anatomy and ductal-dependent pulmonary blood flow, interstage outcomes, hemodynamics before SCPC, and acute postoperative outcomes were similar. Overall reintervention was more common in the DAS group, driven by more frequent planned reintervention. Unplanned reintervention, death, and transplant were similar. Both groups demonstrated good pulmonary atresia growth. DAS is a reasonable initial palliative alternative to BTS in select patients.

There have been no clinical studies evaluating the use of drug-eluting stents (DES) versus bare metal stents (BMS) for infants who underwent ductus arteriosus (DA) stent placement for ductal-dependent pulmonary blood flow (PBF). We aimed to compare the use of second-generation (fluoropolymer-coated everolimus) DES to BMS in infants who underwent DA stenting for ductal-dependent PBF. A retrospective study of infants who underwent DA stenting for ductal-dependent PBF from January 2004 to March 2018 at a single tertiary care pediatric hospital was performed. Of 94 infants identified, 71 (46 BMS and 25 DES) met inclusion criteria. Baseline characteristics of the DES and BMS cohorts were comparable. The patent lumen to stent diameter on subsequent angiographic evaluation was 81% in DES as compared with 50% in BMS group; p = 0.01. There were 2 deaths early in our experience, both in the BMS group. Unplanned reinterventions were less in the DES group (3, 12% patients) compared with the BMS group (13, 28%), p = 0.03. Pulmonary artery size as assessed using Nakata and pulmonary artery symmetry index was comparable in both the groups. There was no difference in infection rates between the groups. On multivariate analysis, prematurity, BMS, and lower oxygen saturations at discharge were associated with subsequent unplanned reintervention (p = 0.01, 0.03 and 0.03, respectively). In conclusion, our clinical experience suggests that in infants who underwent DA stenting for ductal-dependent PBF, (fluoropolymer-coated everolimus eluting) DES results in less luminal loss and lower unplanned reintervention for cyanosis as compared with BMS implantation., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: In infants with ductal-dependent pulmonary blood flow, initial palliation with patent ductus arteriosus (PDA) stent or modified Blalock-Taussig (BT) shunt have comparable mortality but discrepant length of stay, procedural complication rates and reintervention burdens, which may influence cost. The relative economic impact of these palliation strategies is unknown., Methods and Results: Retrospective study of infants with ductal-dependent pulmonary blood flow palliated with PDA stent (n=104) or BT shunt (n=251) from 2008 to 2015 at 4 centers of the Congenital Catheterization Research Collaborative. Inflation-adjusted inpatient hospital costs were calculated for first year of life using Pediatric Health Information System data. Costs derived from outpatient catheterizations not in Pediatric Health Information System were imputed. Costs were compared using propensity score-adjusted multivariable models, to account for baseline differences between groups. After propensity score adjustment, first year of life costs were significantly lower in PDA stent ($215 825 [190 644-244 333]) than BT shunt ($249 855 [230 693-270 609]) patients ( P=0.05). After addition of imputed costs, first year of life costs were not significantly different between PDA stent ($226 403 [200 274-255 941]) and BT shunt ($252 072 [232 955-272 759]) groups ( P=0.15). Patient characteristics associated with higher costs included: younger gestational age, genetic syndrome, noncardiac diagnoses, procedural complications, extracorporeal membrane oxygenation, duration of ventilation, intensive care unit and hospital length of stay and reintervention ( P≤0.02 for all)., Conclusions: In this first multicenter comparative cost study of PDA stent or BT shunt as palliation for infants with ductal-dependent pulmonary blood flow, adjusted for baseline differences, PDA stent was associated with lower to equivalent costs over the first year of life. Combined with previous evidence suggesting clinical noninferiority, these findings suggest that PDA stent provides competitive health care value.

Objectives: This study sought to determine whether pulmonary artery intervention in patients with unilateral proximal pulmonary artery stenosis (PAS) improves exercise capacity, abnormal ventilatory response to exercise, and symptoms., Background: Stenosis of the branch pulmonary arteries results in pulmonary blood flow maldistribution (PBFM). The resulting ventilation-perfusion mismatch is associated with an increased ventilatory response to exercise and decreased exercise capacity. It is unclear if technical success in relieving branch PAS translates to clinical improvement in exercise capacity and ventilatory response., Methods: Twenty patients with biventricular circulation and a minimum 10% PBFM who underwent transcatheter relief of PAS were enrolled in a multi-institutional prospective cohort study. Pre- and post-procedure assessment of the degree of PBFM, exercise capacity, ventilatory response to exercise, and subjective assessment of breathlessness were collected and analyzed., Results: Technical success was achieved in all patients with significant angiographic improvement in minimal lumen diameter (p = 0.001) and peak gradient (p = 0.001). Median PBFM improved (19.5% [range 12.0% to 31.0%] before vs. 7.0% [range 0% to 33.0%] after; p = 0.003). Exercise capacity was low at baseline and improved significantly post-intervention; percent predicted peak oxygen consumption improved from 70% (range 45% to 96%) to 83% (range 47% to 121%) (p = 0.02). Percent predicted oxygen pulse improved (p = 0.02). Ventilatory response to exercise improved; ventilatory equivalent of carbon dioxide slope post-intervention decreased to 29.3 versus 32.5 pre-intervention (p = 0.01). Subjective assessment of dyspnea improved. Five patients with minimal improvement in PBFM also showed minimal improvement in exercise parameters., Conclusions: Successful relief of unilateral branch PAS results in significant improvements in exercise capacity, ventilatory efficiency, and symptoms., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

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., (© 2019 Wiley Periodicals, Inc.)

Ductal origin of pulmonary artery is a rare anomaly that is frequently misdiagnosed. Patients may present with exertional dyspnoea, recurrent respiratory infections and pulmonary hypertension. The presence of pulmonary hypertension can adversely affect clinical outcome in these patients; hence, early identification and intervention is the key to improve survival. A case of a 3-year-old child presenting with exertional dyspnoea is presented in this report. Chest radiograph revealed right-sided pulmonary hypoplasia and mediastinal shift to the right. Pulmonary artery agenesis was suspected when CT of the chest demonstrated right-sided pulmonary artery agenesis. Cardiac catheterisation revealed the correct diagnosis of ductal origin of right pulmonary artery. The most striking feature of this case is that the clinical presentation is mild compared with the findings on imaging., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: Follow-up of transcatheter pulmonary valve replacement (TPVR) with the Melody valve has demonstrated good short-term and long-term outcomes, but there are no published studies focused on valve performance in the Contegra bovine jugular vein conduit., Methods and Results: This is a retrospective, multicenter study of the short- and intermediate-term outcomes of Melody TPVR within the Contegra conduit in the right ventricle to pulmonary artery position. Data from 13 centers were included in the analysis. During the study period, 136 patients underwent 139 catheterizations for attempted Melody TPVR with a median follow-up of 3 years (1 day to 9.1 years). Of the 136 patients, 117 underwent successful Melody TPVR. Two patients underwent a second Melody TPVR. The majority of patients underwent placement of ≥1 stents before transcatheter pulmonary valve implantation. There was a significant reduction in peak conduit pressure gradient acutely after transcatheter pulmonary valve implantation (39 versus 10 mm Hg; P <0.001). At most recent follow-up, the maximum pulmonary valve gradient by echocardiogram remained significantly reduced relative to prevalve implant measurements (65.9 versus 27.3 mm Hg; P <0.001). The incidence of Melody transcatheter pulmonary valve stent fracture (3.4%) and infectious endocarditis (4.3%) were both low. Serious adverse events occurred in 3 patients., Conclusions: Melody TPVR in Contegra conduits is safe and effective and can be performed in a wide range of conduit sizes with preserved valve function and low incidence of stent fracture and endocarditis., (© 2017 American Heart Association, Inc.)

The purpose of the present study was to assess the results and technical aspects of attempted transcatheter recanalization of occluded pulmonary arteries or major systemic veins from our center. Occluded pulmonary arteries or major systemic veins are often not considered amenable to transcatheter treatment and can be a cause of significant hemodynamic compromise for patients. The records of patients with occluded pulmonary arteries or major systemic veins who underwent cardiac catheterization from April 1997 to February 2011 were reviewed. We identified 18 patients, of whom, 9 had occluded pulmonary arteries and 9 occluded systemic veins. Recanalization was achieved in 17 of the 18 patients (94%). At a median follow-up of 35 months (range 6 months to 12 years), all vessels remained patent. The freedom from reintervention rate in the entire cohort was 61% at 6 months, 35% at 1 year, and 35% at 5 years. Freedom from an unplanned reintervention was 67% at 6 months. Procedural adverse events occurred in 2 patients. One death occurred within 24 hours of the catheterization procedure and 2 late deaths occurred, all unrelated to the procedure. In conclusion, both acute and chronic success at recanalizing the occluded pulmonary arteries and major systemic veins can be achieved using percutaneous techniques. Careful follow-up is needed, because reintervention can be necessary to maintain long-term vessel patency without stenosis., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Percutaneous pulmonary valve technology has had a great impact on patients with congenital and acquired heart disease. In some patients, implantation of a percutaneous pulmonary valve may not be possible due to the morphology of the existing right ventricular outflow tract. In this report, we describe implantation of a Melody transcatheter pulmonary valve in the left pulmonary artery in a patient with acquired right pulmonary artery occlusion and a large right ventricular outflow tract.

The objective of this study was to evaluate the incidence of pre-existing catheterization left pulmonary artery (LPA) gradients and correlation of these gradients with later LPA stenosis after successful patent ductus arteriosus (PDA) occlusion. We performed a single-center review of 130 patients with PDA closure from October 1993 to February 2005. We analyzed the pre-PDA closure LPA pressure gradients at catheterization to determine if these were predictive of late LPA stenosis. On follow-up, a V (max) >2 m/s by echocardiogram (transthoracic echocardiography; TTE) was considered indicative of possible LPA stenosis. Left lung perfusion of <35% was considered diagnostic of significant LPA stenosis. Post PDA closure, possible LPA stenosis by TTE was seen in 8 of 128 patients (6.25%). Seven of these eight had precatheter LPA gradients >7 mm Hg. Five of these had perfusion scans, three of the five had significant LPA stenosis, and two underwent LPA angioplasty. Patients with LPA catheter gradients >7 mm Hg were more likely to have possible LPA stenosis by TTE, significant LPA stenosis by lung scan, and intervention with LPA angioplasty. In conclusion, a preclosure main pulmonary artery-to-LPA pressure gradient >7 mm Hg was found in all patients who developed significant LPA stenosis on follow-up after transcatheter PDA closure. It appears likely that these patients have LPA abnormality rather than stenosis caused by the PDA occlusion device. Patients with preclosure LPA gradients >7 mm Hg should undergo follow-up evaluations for detection of significant stenosis and may require treatment if an important flow abnormality is documented.

BACKGROUND: Anatomic selection for Harmony valve implant is determined with the aid of a screening report and perimeter plot (PP) that depicts the perimeter-derived radius along the right ventricular outflow tract (RVOT) and projects device oversizing. The PP provides an estimation of suitability for implant, but its sensitivity as a screening method is unknown. This study was performed to describe anatomic features and outcomes in patients who underwent Harmony TPV25 implant despite a PP that predicted inadequate oversizing. METHODS: We reviewed RVOT anatomic features and measurements in patients who underwent transcatheter pulmonary valve replacement with the Harmony TPV25 device despite a PP that predicted inadequate oversizing. RESULTS: This study included 22 patients. There were no unsuccessful implants or adverse valve-related events. Anatomic features varied, but all patients fit into 1 of 3 anatomic types characterized by differences in RVOT dimensions. Type 1 patients (n=9) had a long RVOT with a choke point and a wide main pulmonary artery. Type 2 patients (n=6) had a short RVOT that was pyramidal in shape, with no choke point, and extensive main pulmonary artery lengthening/expansion during systole. Type 3 patients (n=7) had a short, bulbous main pulmonary artery with a choke point and an open pulmonary artery bifurcation. CONCLUSIONS: Transcatheter pulmonary valve replacement with the Harmony valve is feasible in some patients whose PP fit analysis predicts inadequate oversizing. All cases in this series fit into 1 of 3 anatomic patterns, which are not identified in the screening report. Implanters must review cases individually to assess the feasibility of the implant. [ABSTRACT FROM AUTHOR]

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

Introduction: The optimal management of scimitar syndrome remains incompletely defined. We (1) evaluated the impact of aortopulmonary collateral (APC) occlusion, (2) compared outcomes according to surgical approach for patients who underwent surgery, and (3) identified anatomic factors associated with longer survival time without scimitar vein repair.Methods: We conducted a single center, retrospective study of 61 patients diagnosed with scimitar syndrome between 1995 and 2019. Right pulmonary artery to total pulmonary artery cross-sectional area (RPA:PA CSA) quantitatively assessed right pulmonary artery size. Anatomical features were analyzed for association with longer survival time without scimitar vein repair.Results: Median follow-up time was 6 years (Q1-Q3, 2-12), with 96% 5-year survival. Twenty-three patients underwent APC occlusion, which significantly decreased symptoms of overcirculation (100%-46%; p = .001) and systolic pulmonary artery pressure (median, 34-29 mmHg; p = .004). Twenty-three patients underwent scimitar vein repair; 5-year freedom from scimitar vein stenosis was 90% among patients who underwent a reimplantation compared with 42% in patients with baffle repair (p = .1). Three patients underwent surgery before the first year of age, with lower 5-year freedom from scimitar vein stenosis (0% vs. 84%; p < .001). On multivariate analysis, a lower RPA:PA CSA was associated with longer survival time without scimitar vein repair (p = .003).Conclusions: APC occlusion improves the clinical status of young and hemodynamically unstable patients. Repair at an early age is associated with an increased risk of scimitar vein stenosis. Scimitar vein repair might be avoided in patients with a smaller right pulmonary artery. [ABSTRACT FROM AUTHOR]

Introduction: Pulmonary atresia with the ventricular septal defect is a rare congenital heart defect with high anatomic variability. The most important management question relates to the sources of pulmonary blood flow. The ability to differentiate between ductal dependence and major aortopulmonary collateral arteries is critical to achieving good outcomes and avoiding life-threatening hypoxia in the postneonatal period. Having accurate information about pulmonary arteries, major aortopulmonary collateral arteries, and sources of blood supply to each pulmonary segment is crucial for choosing the optimal surgical strategy. The purpose of this study is to compare computed tomography angiography (CTA) with cardiac catheterization for anatomic delineation of surgically relevant anatomy in pulmonary atresia with ventricular septal defect with major aortopulmonary collateral arteries. Materials and Methods: Retrospective review of all children with pulmonary atresia with ventricular septal defect with major faortopulmonary collateral arteries cared for at a large tertiary children's hospital who underwent cardiac catheterization with angiography and CTA close to each other without interval therapy. All studies were performed between 2007 and 2011. Results: There were 9 patients who met the inclusion criteria. Pulmonary artery anatomy (confluent vs. nonconfluent) was correctly identified in 9 patients by CTA and 8 patients by catheterization. There were no significant differences between CTA and catheterization in the identification of major aortopulmonary collateral arteries (mean = 3.4 collaterals/study via catheterization; mean = 3.1 collaterals/study via CTA; P = 0.67). CTA was superior to catheterization in the delineation of segmental pulmonary blood flow (P = 0.006). Conclusion: CTA and catheterization are equivalent in their ability to delineate pulmonary artery anatomy and major aortopulmonary collateral arteries. [ABSTRACT FROM AUTHOR]

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. * In infants with ductal-dependent pulmonary blood flow, stenting of the patent ductus arteriosus results in equivalent to superior outcomes compared with surgical shunt placement. * One unique concern about this procedure is increased rates of reintervention. * In this large, multicenter cohort study, [almost equal to]40% of all infants having undergone patent ductus arteriosus stenting for ductal-dependent pulmonary blood flow undergo a subsequent reintervention. * The majority of the reinterventions on the patent ductus arteriosus stent were performed during scheduled surveillance catheterizations in a nonurgent setting. * Stenting of the arterial duct in infants with ductal-dependent pulmonary blood flow is a unique clinical pathway with rates of reintervention that are driven by both anatomic/procedural factors and anticipated final physiology. [ABSTRACT FROM AUTHOR]

Introduction: Ductus arteriosus stenting (DAS) is an accepted method for securing pulmonary blood flow in cyanotic neonates. However, in neonates with pulmonary atresia and single source ductal-dependent pulmonary blood flow (SSPBF), DAS remains controversial. Published outcomes in this population are limited. We sought to evaluate outcomes in neonates with SSPBF pts, comparing DAS and surgical Blalock-Taussig shunt (BTS). Methods: Neonates with SSPBF who underwent DAS or BTS from four centers of the Congenital Catheterization Research Collaborative from January 2008 to December 2015 were retrospectively reviewed. Peri-procedural complications, reintervention prior to ultimate surgical repair, pulmonary artery (PA) diameter and interval growth were compared using available imaging studies. A propensity score was utilized to compare cohorts adjusted for differences in patient factors. Results: Forty patients with DAS and 157 patients with BTS were included. At palliation, the DAS and BTS cohorts had similar age, weight, and expected ultimate physiology. Rates of procedural complications following BTS and DAS were also similar. Following initial palliation, DAS patients were less likely to be treated with inotropes (p <0.01) or diuretics (p = 0.046). Both groups had similar median lengths of hospital stay DAS 19 days (9-38), BTS 13 (10-23), p=0.19 and rates of unplanned reinterventions (17.5% of DAS and 21% in BTS, p= 0.62). Pre-palliation PA diameter was smaller (p=0.02), and PA symmetry was lower (p=0.02) in the DAS cohort. However, by time of definitive repair there was no difference in PA symmetry (0.80 in DAS cohort vs 0.82 in BTS cohort, p=0.64), in PA diameter (Nakata 161mm2/m2 (137-166) in DAS vs 152mm2/m2 (94-227) in BTS, p=0.62) or in PA growth (change in Nakata 51mm2/m2 (-21-61) in DAS vs 24mm2/m2 (-28-131) in BTS, p=0.79). Rates of PA plasty and post-repair PA interventions did not differ between the cohorts. Conclusions: In neonates with SSPBF, DAS and BTS appear to have equivalent outcomes post-palliation and at time of subsequent surgical repair. Despite concerns about DAS in SSPBF, it appears to be a durable approach with unchanged PA growth compared to BTS. [ABSTRACT FROM AUTHOR]

Introduction: Patients with single ventricle (SV) anatomy and ductal-dependent pulmonary blood flow may be palliated in the neonatal period with either modified Blalock-Taussig shunt (BTS) or ductus arteriosus stent (DAS). Comparisons of interstage outcomes, hemodynamics, anatomy, and clinical outcomes at the time of superior cavopulmonary connection (SCPC) have not been performed and may differ based upon initial palliation. Methods: Infants with SV anatomy and ductal-dependent pulmonary blood flow palliated with either DAS or BTS from January 2008 to November 2015 were reviewed from the 4 member centers of the Congenital Catheterization Research Collaborative. Interstage outcomes were compared. For those who had SCPC during the follow-up period, pulmonary artery anatomy, hemodynamics, and peri-operative clinical outcomes were compared, adjusted for center and anatomic diagnosis. Results: Thirty-five patients with DAS and 134 patients with BTS were included. At initial palliation, demographic and clinical variables, and pulmonary artery (PA) z-scores were not different. During the interstage period, death, transplant or unplanned reintervention to treat cyanosis occurred in 25.7% of DAS and 35.8% of BTS (adjusted HR = 0.63 (95% CI 0.27 – 1.44), p = 0.27). 23 patients with DAS and 111 patients with BTS underwent SCPC, at which time there were no differences in pre-operative catheterization-derived hemodynamics, including PA pressure, pulmonary vascular resistance and ventricular end-diastolic pressures. However, PA growth was better in the DAS group (median change in Nakata index: 88 mm2/m2 v. 65 mm2/m2, adjusted p =0.04), particularly of the RPA (median change in z-score: 1.57 v. 0.65, adjusted p = 0.042) while LPA growth was similar (median change in z score: 0.75 v. 0.86, adjusted p = 0.4. SCPC intraoperative and post-operative courses were similar. Conclusions: In patients with SV anatomy and ductal-dependent pulmonary blood flow, interstage outcomes, hemodynamics prior to superior cavopulmonary connection, and acute post-operative outcomes were not different based on initial palliative strategy. However, PA growth was better in the DAS group. The longer-term implications of this difference require ongoing study. [ABSTRACT FROM AUTHOR]

Neonates with symptomatic tetralogy of Fallot (sTOF) with hypoplastic pulmonary arteries (hPA) are considered high risk. Data are needed to inform the impact of hPA on outcomes, and the ideal management strategy. The objectives of this study were to quantify the impact of hPA on outcomes in neonates with sTOF and measure the impact of strategy on pulmonary artery (PA) growth in this population. Neonates with sTOF from 2005 to 2017 were reviewed from the Congenital Cardiac Research Collaborative. Criteria for hPA included a unilateral PA z score <−2.0 and contralateral PA z score <0. Primary outcome was mortality. Secondary outcomes included reintervention and PA growth. We included 542 neonates with sTOF, including 188 (35%) with hPA and 354 (65%) with normal PA, with median follow-up of 4.1 years. Median right and left hPA z scores were −2.19 (25th-75th percentile: −2.55 to −1.94) and −2.23 (25th-75th percentile: −2.64 to −1.91), respectively. Staged repair (vs primary TOF repair) was less common in the hPA cohort (36 vs 44%; P = 0.07). Survival was similar between groups (unadjusted P = 0.16; adjusted P = 0.25). Reintervention was more common in the hPA group (HR: 1.28; 95% CI: 1.01-1.63; P = 0.044); there was no difference after definitive repair (HR: 1.21; 95% CI: 0.93-1.58; P = 0.16). PA growth at 1 year was greater in the hPA cohort, particularly for the right PA (P < 0.001). Despite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort. These findings suggest that the presence of hPA does not adversely impact outcomes in sTOF. [Display omitted] [ABSTRACT FROM AUTHOR]