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We sought to characterize short- and long-term outcomes after superior cavopulmonary connection (SCPC) in children eligible for inclusion of antegrade pulmonary blood flow (APBF) in the SCPC circuit, exploring whether maintaining APBF was associated with outcomes.This was a retrospective cohort study of patients with single-ventricle heart disease and APBF who underwent SCPC at our center between January 1, 2000, and September 30, 2017. Patients were divided into 2 groups: APBF eliminated (APBF-), and APBF maintained (APBF+) at the time of SCPC.Of 149 patients, 108 (72.5%) were in APBF- and 41 (27.5%) were in APBF+. Of those in APBF+, 5 (12.2%) subsequently had APBF eliminated after SCPC. Patients in APBF+ had a higher prevalence of chest tube duration10 days and underwent more interventions during the post-SCPC hospitalization (1.9% vs 12%; P = .008 for both) but had shorter surgical support times at SCPC (P.0001). There were no differences in post-SCPC intensive care unit or hospital length of stay. During the study period, 82 patients (76%) in APBF- and 22 patients (54%) in APBF+ underwent Fontan completion. Patients in APBF+ had a greater weight gain from SCPC to Fontan (6.7 [1.8-22] kg vs 8.15 [4.4-20.6] kg; P = .012) and a shorter hospital length of stay after Fontan (9 [4-107] days vs 7.5 [4-14] days; P = .044).Short-term morbidity associated with maintaining APBF at the time of SCPC is modest, but longer term outcomes suggest potential benefits in those in whom APBF can be successfully maintained.

Background The Fontan circulation challenges the lymphatic system. Increasing production of lymphatic fluid and impeding lymphatic return, increased venous pressure may cause lymphatic dilatation and decrease lymphatic contractility. In-vitro studies have reported a lymphatic diameter-tension curve, with increasing passive stretch affecting the intrinsic contractile properties of each thoracic duct segment. We aimed to describe thoracic duct occlusion pressure and asses if thoracic duct dilation impairs contractility in individuals with a Fontan circulation and lymphatic failure. Methods Central venous pressure and thoracic duct measurements were retrospectively collected from 31 individuals with a Fontan circulation. Thoracic duct occlusion pressure was assessed during a period of external manual compression and used as an indicator of lymphatic vessel contractility. Measurements of pressure were correlated with measurements of the thoracic duct diameter in images obtained by dynamic contrast-enhanced MR lymphangiography. Results The average central venous pressure and average pressure of the thoracic duct were 17 mm Hg. During manual occlusion, the thoracic duct pressure significantly increased to 32 mm Hg. The average thoracic duct diameter was 3.3 mm. Thoracic duct diameter correlated closely with the central venous pressure. The rise in pressure following manual occlusion showed an inverse correlation with the diameter of the thoracic duct. Conclusion Higher central venous pressures are associated with increasing diameters of the thoracic duct. When challenged by manual occlusion, dilated thoracic ducts display a decreased ability to increase pressure. Dilatation and a resulting decreased contractility may partly explain the challenged lymphatic system in individuals with a Fontan circulation.

Plastic bronchitis (PB) and protein-losing enteropathy (PLE) are rare but potentially devastating complications of the Fontan circulation. PB occurs in ∼4% of Fontan patients, typically presents within 2 to 3 years of Fontan completion with chronic cough, wheezing, fever, or acute asphyxiation, and is characterised by proteinaceous airway casts that are expectorated or found on bronchoscopy. PLE develops in 4% to 13% of patients, usually within 5 to 10 years post Fontan, and manifests with edema, ascites, hypoalbuminemia, lymphopenia, hypogammaglobulinemia, and elevated fecal alpha-1 antitrypsin 1. These disorders have similar pathophysiology involving disruption of the lymphatic system resulting from elevated central venous pressure combined with elevated lymphatic production and inflammation, resulting in lymphatic drainage into low-pressure circuits such as the airways (PB) and duodenum (PLE). Our understanding of these disorders has greatly improved over the past decade as a result of advances in imaging of the lymphatic system through magnetic resonance lymphangiography and early success with lymphatic interventions including lymphatic embolisation, thoracic duct embolisation, and percutaneous thoracic duct decompression. Both PB and PLE require a multidisciplinary approach that addresses and optimises residual hemodynamic lesions through catheter-based intervention, lowers central venous pressure through medical therapy, minimises symptoms, and targets abnormal lymphatic perfusion when symptoms persist. This review summarises the pathophysiology of these disorders and the current evidence base regarding management, proposes treatment algorithms, and identifies future research opportunities. Key considerations regarding the development of a lymphatic intervention program are also highlighted.

Background Congestive heart failure is a leading cause of morbidity and mortality worldwide. One of the signs of congestive heart failure is fluid overload including pulmonary edema, peripheral edema, and ascites. The cause of fluid overload remains incompletely understood, and management of these patients continues to be a challenge. The role of lymphatic circulation abnormalities in the cause and pathophysiology of fluid overload also remains unclear. Here we report on a study in a large animal model of right heart failure caused by severe tricuspid regurgitation comparing cardiovascular and lymphatic findings in a group of animals that did not develop ascites with a group of animals that developed ascites. Methods and Results Thirteen Yorkshire pigs were included in this study divided into 2 groups. Group 1 included 6 animals that did not develop ascites, and Group 2 included 7 animals that had developed ascites. The groups were compared on hemodynamic parameters as well as comparison of the animal's lymphatic anatomy and function. There was no difference between the groups in degree of tricuspid regurgitation and central venous pressure, with inferior vena cava pressure measuring 11.6±1.6 versus 13.2±3.7 ( P =0.534) and superior vena cava pressure measuring 12.0±2.3 versus 13.7±3.2 ( P =0.366). There was also no difference between the groups in all measured hemodynamic parameters, including right ventricular pressure, pulmonary artery pressure, and left ventricular function. The weighted liver size in the ascites group was significantly larger than in the nonascites group (30.3±12.4 versus 63.3±14.0 mL/kg, respectively; P =0.001). The 2 groups also differed in the number of animals with regurgitant thoracic duct flow (Group 1: 1/6,17% versus Group 2: 6/7, 86%; P =0.029) and the minimal thoracic duct diameter (Group 1: 2.3±0.3 versus Group 2: 4.2±2.2; P =0.035). Conclusions In animals with right heart failure caused by severe tricuspid regurgitation, fluid overload did not correlate with hemodynamic parameters but rather with changes in the lymphatic system, including regurgitant lymphatic flow, minimal thoracic duct diameter, and liver size. This study is consistent with lymphatic dysfunction and not cardiovascular function playing a significant role in the cause of fluid overload. Further studies are needed to confirm these findings.

Congenital heart disease can lead to notable lymphatic complications such as chylothorax, plastic bronchitis, protein-losing enteropathy, and ascites. Recent improvements in lymphatic imaging and the development of new lymphatic procedures can help alleviate symptoms and improve outcomes.

Patients with dextro-transposition of the great arteries (d-TGA) require surgical repair as neonates. These patients are at risk for post-operative chylothorax. We sought to describe the presentation, imaging, and outcomes after intervention for patients with d-TGA with post-operative chylothorax. A retrospective chart review was performed in patients with repaired d-TGA who were referred from 1/1/2013 to 4/1/2020 for evaluation of chylothorax. Patient history, lymphatic imaging, and interventional data were collected. Impact of intervention on lymphatic drainage was evaluated with a student’s t-test. Eight patients met inclusion criteria for this study. Five patients had a history of central venous thrombus leading to thoracic duct outlet occlusion. Five patients underwent intervention, two were managed conservatively, and one was not a candidate for intervention. Chylothorax resolved in six patients. There was a significant difference in output from 7 days prior to first intervention (114 mL/kg/day) compared to 28 days following final intervention (27 mL/kg/day, p = 0.034). There were no procedural complications. Chylothorax in patients with repaired transposition of the great arteries is often amenable to intervention. Early surveillance and management of central venous thrombosis may reduce the burden of lymphatic disease in these patients.

Background: Lymphatic embolization therapy has proven effective for Fontan failure from plastic bronchitis or protein-losing enteropathy but not when multiple lymphatic compartments are involved; furthermore, embolization does not alter the underlying pathophysiology of lymphatic dysfunction. A technique for transcatheter thoracic duct decompression (TDD), rerouting the thoracic duct to the pulmonary venous atrium to treat multicompartment lymphatic failure is described and early outcomes presented. Methods: Initially covered stents were used to channel the innominate vein flow inside of the cavopulmonary pathway into the pulmonary venous atrium. A modified approach was developed where covered stents redirected innominate vein directly to the left atrium via an extravascular course. Baseline and follow-up data on all patients undergoing TDD were reviewed. Results: Twelve patients underwent TDD between March 2018 and February 2021 at a median age of 12 (range: 2–22) years. Lymphatic failure occurred in median of 3 compartments per patient (protein-losing enteropathy, ascites, pleural effusions, plastic bronchitis); 10 patients had lymphatic embolizations before TDD. TDD method was intra-Fontan tunnel in 4, direct approach in 7, and other in 1. There were no major procedural complications; 6 patients underwent subsequent procedures, most commonly to treat endoleaks. Lymphatic failure resolved in 6 patients, improved in 2, and was unchanged in 4 at 6 (range: 1–20) months follow-up. One patient died after TDD from Fontan failure. Conclusions: TDD is a promising new treatment for the failing Fontan physiology from multicompartment lymphatic failure. Additional work is needed to refine the technique and define optimal candidates.

Objectives The objective of this study was to assess contemporary use of operator directed sedation (ODS) and anesthesiologist care (AC) in the pediatric/congenital cardiac catheterization laboratory (PCCL), specifically evaluating whether the use of operator-directed sedation was associated with increased risk of major adverse events. Background The safety of ODS relative to AC during PCCL procedures has been questioned. Methods A multicenter, retrospective cohort study was performed studying procedures habitually performed with ODS or AC at IMPACT (Improving Adult and Congenital Treatment) registry hospitals using ODS for ≥5% of cases. The risks for major adverse events (MAE) for ODS and AC cases were compared, adjusted for case mix. Current recommendations were evaluated by comparing the ratio of observed to expected MAE for cases in which ODS was inappropriate (inconsistent with those guidelines) with those for similar risk AC cases, as well as those in which ODS or AC was appropriate. Results Of the hospitals submitting data to IMPACT, 28 of 101 met inclusion criteria. Of the 7,042 cases performed using ODS at these centers, 88% would be inappropriate. Use of ODS was associated with lower likelihood of MAE both in observed results (p Conclusions Across a range of hospitals, ODS was used safely and with improved efficiency. Clinical judgment better identified cases in which ODS could be used than pre-procedural risk score. This should inform future guidelines for the use of ODS and AC in the catheterization laboratory

Objectives The aim of this study was to enumerate and categorize quality metrics relevant to the pediatric/congenital cardiac catheterization laboratory (PCCL). Background Diagnostic and interventional catheterization procedures are an increasingly important part of the care of young patients with cardiac disease. Measurement of the performance of PCCL programs in a stringent and consistent fashion is a crucial step toward improving outcomes. To the best of our knowledge, a systematic evaluation of current quality metrics in PCCL has not been performed previously. Methods Potential metrics were evaluated by: 1) a systematic review of peer-reviewed research; 2) a review of metrics from organizations interested in quality improvement, patient safety, and/or PCCL programs; and 3) a survey of U.S. PCCL cardiologists. Collected metrics were grouped on 2 dimensions: 1) Institute of Medicine domains; and 2) the Donabedian structure/process/outcome framework. Survey responses were dichotomized between favorable and unfavorable responses and then compared within and between categories. Results In the systematic review, 6 metrics were identified (from 9 publications), all focused on safety either as an outcome (adverse events [AEs], mortality, and failure to rescue along with radiation exposure) or as a structure (procedure volume or operator experience). Four organizations measure quality metrics of PCCL programs, of which only 1 publicly reports data. For the survey, 229 cardiologists from 118 hospital programs responded (66% of individuals and 72% of hospital programs). The highest favorable ratings were for safety metrics (p Conclusions There is a limited number of PCCL quality metrics, primarily focused on safety. Confidence in current risk adjustment methodology is low. The knowledge gaps identified should guide future research in the development of new quality metrics.

The aim of this study was to determine the feasibility of using contrast-enhanced ultrasound (CEUS) evaluation to determine thoracic duct (TD) outlet patency. Nine patients referred for lymphatic imaging and intervention underwent percutaneous intranodal ultrasound contrast injection and conventional lymphangiography (CL). Eight of 9 patients had a patent TD by CEUS and CL. One patient did not have a patent TD. There was 100% agreement between CEUS and CL. These results suggest that CEUS is an imaging modality that might be as accurate as CL in determining TD patency.

Objectives To evaluate short- and middle-term outcomes after transcatheter patent ductus arteriosus (TC-PDA) closure in small infants, specifically device-related left pulmonary artery (LPA) stenosis and aortic coarctation, risk factors, and changes over time. Background Recent studies have demonstrated successful transcatheter PDA (TC-PDA) closure in small infants. LPA stenosis and aortic coarctation have been seen after TC-PDA, but it is not clear whether device-related LPA/aortic obstruction persists. Methods A single-center retrospective study of infants ≤4 kg who underwent TC-PDA closure from February 1, 2007 to September 1, 2018 was performed, evaluating the incidence and risk factors for LPA stenosis and coarctation. Results Forty-four patients underwent successful TC-PDA with Amplatzer Vascular Plug II (AVPII; n = 30), Amplatzer Duct Occluder II-Additional Sizes (n = 10), Amplatzer Duct Occluder I (n = 3), and coil-filled AVPI (n = 1) devices, all via an antegrade approach. Median birthweight and procedural weight were 890 g (range: 490-3,250) and 2.8 kg (range: 1.2-4.0), respectively. Median follow-up was 0.7 years (range: 2 days-7 years). Thirty-eight patients had post-procedure echocardiograms assessing LPA/aortic obstruction. Of those, 17 had LPA flow acceleration/stenosis (≥1.5 m/s), which improved or resolved in all patients with available follow-up; 3 developed mild coarctation (>2 m/s), which improved in the two with more than short-term follow-up; 4 developed mild flow acceleration (1.5-2 m/s) in the descending aorta, which resolved in three and increased in one (2.4 m/s). Flow acceleration in the LPA was associated with younger procedural age, larger PDA minimal diameter, and placement of a device other than the AVPII. There was no device-related mortality or need for reintervention. Conclusion TC-PDA in small infants is effective, without significant complications. Device-related LPA/aortic obstruction can improve with time/growth.

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

Introduction: Balloon and stent angioplasty of the pulmonary arteries (PAs) are frequently performed following superior cavopulmonary connection (SCPC), not only to normalize the caliber of the affected PA but also in hopes of maximizing downstream growth over time. There are limited data on the impact on subsequent PA growth prior to total cavopulmonary connection (TCPC). Methods: A single-center, retrospective cohort study was performed on children who underwent transcatheter (TC) PA intervention following SCPC between January 1, 2010, and December 31, 2018. Growth of treated and contralateral PAs was measured at the lobar bifurcation (distal branch PA [DBPA]) and in the proximal lower lobe (lower lobe branch [LLB]) on serial angiograms. Growth rate was evaluated using a mixed-effect model clustered by individual patient with an interaction term for treated PA and time to evaluate for differential growth rates between treated and contralateral PAs. Results: Thirty-five patients underwent TC PA intervention following SCPC, at a median of 70 days (interquartile range: 19-297 days) postoperatively. Significant growth was seen at both DBPA and LLB for raw (0.8 mm/year, 95% CI: 0.6-1.0, P < .001 for both) and body surface area (BSA) adjusted measures (8.4mm/m2/year, 95% CI: 5.6-11.2, P < .001; 7.9 mm/m2/year, 95% CI: 5.5-10.2, P < .001). The growth rate of the treated vessel was not significantly different from that of the contralateral vessel at the DBPA or LLB positions for raw ( P = .71, .70) or BSA-adjusted measurements ( P = .86, .64). Conclusion: Transcatheter PA intervention was associated with normal distal PA growth rate relative to the untreated side.

Background Protein‐losing enteropathy (PLE) is a significant cause of morbidity and mortality in congenital heart disease patients with single ventricle physiology. Intrahepatic dynamic contrast‐enhanced magnetic resonance lymphangiography (IH‐DCMRL) is a novel diagnostic technique that may be useful in characterizing pathologic abdominal lymphatic flow in the congenital heart disease population and in diagnosing PLE. The objective of this study was to characterize differences in IH‐DCMRL findings in patients with single ventricle congenital heart disease with and without PLE. Methods and Results This was a single‐center retrospective study of IH‐DCMRL findings and clinical data in 41 consecutive patients, 20 with PLE and 21 without PLE, with single ventricle physiology referred for lymphatic evaluation. There were 3 distinct duodenal imaging patterns by IH‐DCMRL: (1) enhancement of the duodenal wall with leakage into the lumen, (2) enhancement of the duodenal wall without leakage into the lumen, and (3) no duodenal involvement. Patients with PLE were more likely to have duodenal involvement on IH‐DCMRL than patients without PLE ( P Conclusions IH‐DCMRL findings of lymphatic enhancement of the duodenal wall and leakage of lymph into the duodenal lumen are associated with PLE. IH‐DCMRL is a useful new modality for characterizing pathologic abdominal lymphatic flow in PLE and might be useful as a risk‐assessment tool for PLE in at‐risk patients.

Fontan-associated protein losing enteropathy is a challenge to treat and is a major contributor to morbidity and mortality in patients with palliated single ventricle. Numerous strategies for management have been proposed, with confusion as to how best to stratify and implement care among the many treatments available. Medical management can be helpful in some, while novel lymphatic interventions hold potential for remission with good results. We review our institutional approach to protein losing enteropathy after Fontan operation and provide a suggested algorithm and pathway for effective care.

Introduction: Symptomatic Heart failure (HF) is a leading cause of morbidity and mortality in the world. HF with right sided symptoms may result in fluid overload including edema and ascites. The pathophysiology of fluid overload is poorly understood with no clear association between symptoms and hemodynamic parameters. Hypothesis: In animals with RHF and elevated CVP, the presence of ascites correlates with changes in lymphatic function and is independent of myocardial function and hemodynamic parameters. Methods: RHF was induced in fifteen swine animals by creating severe tricuspid regurgitation. Hemodynamics and anatomic changes were characterized using fluoroscopy, echocardiogram, and MRI. Measurements of the thoracic duct (TD) were made in the abdomen and thorax and the cross-sectional area was calculated. TD regurgitant flow was identified with ethiodized oil injection into the TD and was noted to be present or absent. Data is reported as median (IQR). Results: All 15 animals developed RHF with a dilated right atrium (RA) and right ventricle (RV). Nine animals developed ascites. Comparing animals with and without ascites, there were no differences in IVC pressure(mmHg) 11.5 (7.7-13.4) vs. 11.9 (10.2-17.4) (p= 0.364), SVC pressure 12.8 (11.3-16.8) vs. 12 (8.3-14.9) (p=0.343). Other hemodynamics parameters including pulmonary artery (PA), RV, and RA pressure were also not significantly different. In addition, left ventricular (LV) ejection fraction was normal in both groups (68.7% (57.9-74.8) vs. 55% (53.1-69.4) (p=0.135)). However, animals in the ascites group had larger relative liver volume (mL/Kg) 59.1 (65.1-51.9) vs. 34.85 (42.3-24.1) (p = 0.003), larger dimension of the minimal TD size (mm) 3.2 (5.8-2.7) vs. 2.2 (2.6-2.0) (p=0.04), and more animals had TD regurgitant flow 89% vs. 16% (p=0.01). Conclusion: In animal model with RV failure, there is no association between hemodynamic parameters and occurrence of ascites, consistent with observations in humans. In contrast, parameters of lymphatic congestion differ significantly between the two groups supporting the notion that lymphatic dysfunction is a contributing cause of tissue congestion in patients with HF and elevated CVP.

Background Recent studies suggest that lymphatic congestion plays a role in development of late Fontan complications, such as protein‐losing enteropathy. However, the role of the lymphatic circulation in early post‐Fontan outcomes is not well defined. Methods and Results This was a retrospective, single‐center study of patients undergoing first‐time Fontan completion from 2012 to 2017. The primary outcome was early Fontan complication ≤6 months after surgery, a composite of death, Fontan takedown, extracorporeal membrane oxygenation, chest tube drainage >14 days, cardiac catheterization, readmission, or transplant. Complication causes were assigned to 1 of 4 groups: (1) Fontan circuit obstruction, (2) ventricular dysfunction or atrioventricular valve regurgitation, (3) persistent pleural effusions in the absence of Fontan obstruction or ventricular dysfunction, and (4) chylothorax or plastic bronchitis. T2‐weighted magnetic resonance imaging sequences were used to assess for lymphatic perfusion abnormality. The cohort consisted of 238 patients. Fifty‐eight (24%) developed early complications: 20 of 58 (34.5%) in group 1, 8 of 58 (14%) in group 2, 18 of 58 (31%) in group 3, and 12 of 58 (20%) in group 4. Preoperative T2 imaging was available for 126 (53%) patients. Patients with high‐grade lymphatic abnormalities had 6 times greater odds of developing early complications ( P =0.001). Conclusions There is substantial morbidity in the early post‐Fontan period. Half of those who developed early complications had lymphatic failure or persistent effusions unrelated to structural or functional abnormalities. Preoperative T2 imaging demonstrated that patients with higher‐grade lymphatic perfusion abnormalities were significantly more likely to develop early complications. This has implications for risk stratification and optimization of patients before Fontan palliation.

Background: The arterial switch operation (ASO) is the gold standard operative correction of neonates with transposition of the great arteries and intact ventricular septum, with excellent operative survival. The associations between patient and surgeon characteristics and outcomes are well understood, but the associations between variation in preoperative care and outcomes are less well studied. Methods: A multicenter retrospective cohort study of infants undergoing neonatal ASO between January 2010 and September 2015 at hospitals contributing data to the Pediatric Health Information Systems database was performed. The association between preoperative care (timing of ASO, preoperative use of balloon atrial septostomy, prostaglandin infusion, mechanical ventilation, and vasoactive agents) and operative outcomes (mortality, length of stay, and cost) was studied with multivariable mixed-effects models. Results: Over the study period, 2159 neonates at 40 hospitals were evaluated. Perioperative mortality was 2.8%. Between hospitals, the use of adjuvant therapies and timing of ASO varied broadly. At the subject level, older age at ASO was associated with higher mortality risk (age >6 days: odds ratio, 1.90; 95% CI, 1.11–3.26; P =0.02), cost, and length of stay. Receipt of a balloon atrial septostomy was associated with lower mortality risk (odds ratio, 0.32; 95% CI, 0.17–0.59; P P =0.03), longer length of stay ( P P Conclusions: There was significant variation in preoperative care between hospitals. Some potentially modifiable aspects of perioperative care (timing of ASO and septostomy) were significantly associated with mortality, length of stay, and cost. Further research on the perioperative care of neonates is necessary to determine whether modifying practice on the basis of the observed associations translates into improved outcomes.

The initial "Frontiers in Fontan Failure" conference in 2015 in Atlanta, GA, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with "Failing Fontan" physiology. Four types of "Fontan Failure" were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan procedure "imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation." The purpose of the second "Frontiers in Fontan Failure" was to further the discussion regarding new data and technologies as well as novel interventions. The 2017 "Frontiers in Fontan Failure: Innovation and Improving Outcomes" was sponsored by Children's Healthcare of Atlanta, Sibley Heart Center Cardiology, and Emory University School of Medicine. Future directions in the management of Fontan failure include further investigations into the risk of sudden cardiac death and how to properly prevent it, achievable interventions in modifying the Fontan physiology to treat or prevent late complications, and improved and refined algorithms in Fontan surveillance. Finally, further research into the interventional treatment of lymphatic-related complications hold the promise of marked improvement in the quality of life of advanced Fontan failure patients and as such should be encouraged and contributed to.

Background Protein-losing enteropathy (PLE), characterized by loss of proteins in the intestine, is a devastating complication in patients with congenital heart disease. The cause of PLE is unknown, but lymphatic involvement has been suspected. Objectives The authors evaluated the use of lymphangiographic imaging and liver lymphatic embolization as a treatment for PLE. Methods This was a single-center, retrospective review of imaging and interventions used in 8 consecutive patients with liver lymphatic embolization and congenital heart disease with elevated central venous pressure complicated by PLE. Results Liver lymphangiography was performed in 8 patients (5 males, 3 females; median age, 21 years), 7 of whom demonstrated leakage of liver lymph into the duodenum through abnormal hepatoduodenal lymphatic communications. This was confirmed by duodenoscopy with simultaneous injection of isosulfan blue dye into the liver lymphatics in 6 of 7 patients. Liver lymphatic embolization with ethiodized oil in 2 patients resulted in a temporary increase in albumin blood level and symptom improvement in 1 patient, but was complicated by duodenal bleeding in both patients. Of the remaining 6 patients, liver lymphatic embolization with n-butyl cyanoacrylate glue resulted in sustained improvement of the serum albumin level and symptoms in 3 patients, temporary improvement in 2 patients, and no change in 1 patient with median follow-up of 135 days (range, 84 to 1,005 days). Conclusions The authors demonstrated liver lymph leakage as a cause of PLE in patients with congenital heart disease and elevated central venous pressure. Lymphatic embolization led to improved albumin levels and relief of symptoms. Further experience with the technique is needed to determine long-term outcome of this procedure.

Background Risk‐adjusted adverse event ( AE ) rates have been used to measure the quality of pediatric and congenital cardiac catheterization laboratories. In other settings, failure to rescue ( FTR ) has demonstrated utility as a quality metric. Methods and Results A multicenter retrospective cohort study was performed using data from the IMPACT (Improving Adult and Congenital Treatment) Registry between January 2010 and December 2016. A modified FTR metric was developed for pediatric and congenital cardiac catheterization laboratories and then compared with pooled AEs . The associations between patient‐ and hospital‐level factors and outcomes were evaluated using hierarchical logistic regression models. Hospital risk standardized ratios were then calculated. Rankings of risk standardized ratios for each outcome were compared to determine whether AEs and FTR identified the same high‐ and low‐performing centers. During the study period, 77 580 catheterizations were performed at 91 hospitals. Higher annual hospital catheterization volume was associated with lower odds of FTR (odds ratio: 0.68 per 300 cases; P =0.0003). No association was seen between catheterization volume and odds of AEs . Odds of AEs were instead associated with patient‐ and procedure‐level factors. There was no correlation between risk standardized ratio ranks for FTR and pooled AEs ( P =0.46). Hospital ranks by catheterization volume and FTR were associated ( r =−0.28, P =0.01) with the largest volume hospitals having the lowest risk of FTR . Conclusions In contrast to AEs , FTR was not strongly associated with patient‐ and procedure‐level factors and was significantly associated with pediatric and congenital cardiac catheterization laboratory volume. Hospital rankings based on FTR and AEs were not significantly correlated. We conclude that FTR is a complementary measure of catheterization laboratory quality and should be included in future research and quality‐improvement projects.

In patients with total cavopulmonary connections, elevated central venous pressures (CVP) have detrimental effects on the lymphatic system causing an imbalance in fluid production and drainage of the interstitium. This combination may result in life-threatening lymphatic complications including plastic bronchitis (PB), protein losing enteropathy (PLE), chylothorax, and ascites. While embolization of the abnormal lymphatics has greatly improved outcomes from these complications, alternative treatment strategies have been proposed that would result in improved lymphatic drainage while leaving the lymphatic system intact. We report two novel transcatheter approaches for thoracic duct (TD) decompression in two patients who developed PLE after completion of the Fontan procedure as part of staged palliation for congenital heart disease. In addition, one patient had severe concurrent PB. In both patients, a connection was created between a left superior vena cava (LSVC) to the left atrium allowing for a nonsurgical method to decompress the TD. This procedure resulted in significant clinical and laboratory improvement of both patients' PLE and other symptoms of lymphatic dysfunction.

The risks and benefits of pharmacologic treatment and operative closure of patent ductus arteriosus (O-PDA) in premature infants remain controversial. Recent series have demonstrated the feasibility of transcatheter PDA closure (TC-PDA) in increasingly small infants. The effect of this change on practice has not been evaluated.A multicenter observational study of infants treated in neonatal intensive care units in hospitals contributing data to the Pediatric Health Information Systems Database from January 2007 to December 2017 was performed to study trends in the propensities for (1) mechanical closure of PDA and (2) TC-PDA versus O-PDA, as well as interhospital variation in practice.A total of 6,214 subjects at 44 hospitals were studied (5% TC-PDA). Subject median gestational age was 25 weeks (interquartile range: 24-27 weeks). Median age at closure was 24 days (interquartile range: 14-36 days). The proportion of all neonatal intensive care unit patients undergoing either O-PDA or TC-PDA decreased (3.1% in 2007 and 0.7% in 2017, P .001), whereas the proportion in which TC-PDA was used increased significantly (0.1% in 2007 to 29.0% in 2017). Case-mix-adjusted multivariable models similarly demonstrated increasing propensity to pursue TC-PDA (odds ratio [OR] 1.66 per year, P .001) with acceleration of the trend after 2014 (OR 2.46 per year, P .001) as well as significant practice variation (P .001, median OR 4.6) across the study period.In the face of decreasing closure of PDA, the use of TC-PDA increased dramatically with significant practice variability. This demonstrates that there is equipoise for potential clinical trials.

Background Cardiac catheterization is an important but costly component of health care for young patients with cardiac disease. Measurement of variation in their cost between hospitals and identification of the reasons for this variation may help reduce cost without compromising quality. Methods and Results Using data from Pediatric Health Information Systems Database from January 2007 to December 2015, the costs of 9 procedures were measured. Mixed‐effects multivariable models were used to generate case‐mix–adjusted estimates of each hospital's cost for each procedure and measure interhospital variation. Procedures (n=35 637) from 43 hospitals were studied. Median costs varied from $8249 (diagnostic catheterization after orthotopic heart transplantation) to $38 909 (transcatheter pulmonary valve replacement). There was marked variation in the cost of procedures between hospitals with 3.5‐ to 8.9‐fold differences in the case‐mix–adjusted cost between the most and least expensive hospitals. No significant correlation was found between hospitals’ procedure‐specific mortality rates and costs. Higher procedure volume was not associated with lower cost except for diagnostic procedures in heart transplant patients and pulmonary artery angioplasty. At the hospital level, the proportion of cases that were outliers (>95th percentile) was significantly associated with rank in terms of cost (Spearman's ρ ranging from 0.37 to 0.89, P Conclusions Large‐magnitude hospital variation in cost was not explained by case‐mix or volume. Further research is necessary to determine the degree to which variation in cost is the result of differences in the efficiency of the delivery of healthcare services and the rate of catastrophic adverse outcomes and resultant protracted and expensive hospitalizations.

The purpose of this study was to evaluate the association between the method of procedural sedation and outcomes for congenital cardiac catheterization procedures.The safety of operator-directed sedation (ODS) in the pediatric/congenital cardiac catheterization laboratory has been questioned. To our knowledge, the relative safety of ODS versus general anesthesia (GA) in these cases has not to date been critically evaluated.A single-center retrospective cohort study was performed to compare the relative safety, cost, and times of catheterization procedures performed with ODS and those performed with GA from a cardiac anesthesiologist. The risk of adverse outcomes was compared using propensity-score-adjusted models. Using the same propensity score, procedure times and relative charges were also compared.Over the study period, 4,424 procedures in 2,547 patients were studied. Of these, 27% of cases were performed with ODS. ODS procedures were 70% diagnostic procedures, 17% device closure of patent ductus arteriosus, 5% balloon pulmonary valvuloplasty, and 3% pulmonary artery angioplasty. The risk of adverse event in adjusted models for ODS cases was significantly lower than in GA cases (odds ratio: 0.66; 95% confidence interval: 0.45 to 0.95; p = 0.03). Total room time and case time were also significantly shorter (p 0.001). Professional (charge ratio: 0.88; p 0.001) and hospital (charge ratio: 0.84; p 0.001) charges for ODS cases were also lower than those for GA cases.This study demonstrates that clinical judgment can identify subjects in whom ODS is not associated with increased risk of adverse events. The use of ODS was associated with reduced case times and charges. In combination, these findings suggest that the selective use of ODS can allow for greater efficiency and higher value care without sacrificing safety.

The lymphatic system was discovered in the early days of medicine, however, the ability to image and treat lymphatic abnormalities has only developed over the last 10 to 15 years. In this time, the presence of lymphatic disorders with congenital heart disease has been described and better understood. Advances in imaging modalities have led to novel MRI techniques including dynamic contrast enhanced magnetic resonance lymphangiograms (DCMRL) as well as conventional lymphangiograms to better delineate lymphatic pathology. Using these modalities, it is now possible to diagnose and treat a wide array of lymphatic abnormalities including chylous effusions, chylous ascites, plastic bronchitis, and protein losing enteropathy. Therapies have advanced to involve percutaneous, transabdominal thoracic duct access with treatment of lymphatic problems using liquid embolic material, covered stents, and coils. In cases of thoracic duct occlusion, re-establishing flow is the first line of treatment which can be performed using a novel microsurgical lympho-venous anastomosis technique. In patients with single ventricle physiology, it is essential to image the lymphatics using intra-nodal, intra-hepatic, and intra-mesenteric DCMRL prior to intervening. In these patients, care must be taken to prevent complete occlusion of the thoracic duct to avoid creating another source of lymphatic problems. Current and future lymphatic management involves a combination of catheter-based and surgical interventions as well as the use of novel medical therapies, such as MEK inhibitors, to remodel abnormal lymphatic architecture. Synopsis This article strives to review the current status of the field of lymphatic imaging and interventions in congenital heart diseases and briefly outlines future directions as the field evolves.

The lymphatic system plays an important role in immune regulation, transport of metabolites, and fluid balance. The key circulatory role of the lymphatic system is to transport fluid from tissue back into the venous system via lymphovenous connections. Despite the centuries-old recognition of this key role, there has been poor understanding of lymphatic flow pathophysiology because of a lack of a simple reliable noninvasive clinical lymphatic imaging method. This lack of clinical imaging has limited the treatment options for patients with lymphatic flow disorders. Recent development of noncontrast magnetic resonance (MR) lymphangiogram and dynamic contrast MR lymphangiography make it possible to visualize central lymphatic anatomy and flow dynamics with high spatial and temporal resolution. Dynamic contrast MR lymphangiography has provided insight into understanding the pathophysiology of several pulmonary lymphatic flow disorders and provides guidance for interventional procedures. Another important development has been intranodal lymphangiogram, which has now replaced pedal lymphangiogram as the main lymphatic interventional modality, and which provides quick and reliable access to the central lymphatic ducts for interventional procedures. These new techniques have led to a resurgence in interest in the lymphatic system and the development of new treatments for patients with lymphatic flow disorders.

Balloon and stent angioplasty of the pulmonary arteries (PA) is often performed following superior cavopulmonary connection (SCPC), not only to normalize the caliber of the affected PA but also in hopes of maximizing growth of the downstream pulmonary vascular tree. To our knowledge, this impact has

Protein-losing enteropathy (PLE) is a common cause of morbidity and mortality in patients with single-ventricle (SV) physiology. Pathologic, retrograde lymphatic flow from the liver to the duodenum and leakage of lymph into the bowel lumen is implicated in PLE. Intrahepatic dynamic contrast-enhanced

Background Pulmonary insufficiency is a consequence of transannular patch repair in Tetralogy of Fallot (ToF) leading to late morbidity and mortality. Transcatheter native outflow tract pulmonary valve replacement has become a reality. However, predicting a secure, atraumatic implantation of a catheter-based device remains a significant challenge due to the complex and dynamic nature of the right ventricular outflow tract (RVOT). We sought to quantify the differences in compression and volume for actual implants, and those predicted by pre-implant modeling. Methods We used custom software to interactively place virtual transcatheter pulmonary valves (TPVs) into RVOT models created from pre-implant and post Harmony valve implant CT scans of 5 ovine surgical models of TOF to quantify and visualize device volume and compression. Results Virtual device placement visually mimicked actual device placement and allowed for quantification of device volume and radius. On average, simulated proximal and distal device volumes and compression did not vary statistically throughout the cardiac cycle (P = 0.11) but assessment was limited by small sample size. In comparison to actual implants, there was no significant pairwise difference in the proximal third of the device (P > 0.80), but the simulated distal device volume was significantly underestimated relative to actual device implant volume (P = 0.06). Conclusions This study demonstrates that pre-implant modeling which assumes a rigid vessel wall may not accurately predict the degree of distal RVOT expansion following actual device placement. We suggest the potential for virtual modeling of TPVR to be a useful adjunct to procedural planning, but further development is needed.

Background: No previous studies have studied the variation in costs between hospitals for catheterization procedures performed in children. Variation in cost not explained by differences in case-mix can indicate opportunities to improve efficiency and quality of care. Methods: A multicenter observational study was performed, studying total hospital costs of 9 common transcatheter procedures (diagnostic procedures in 2 populations and 7 interventional procedures) performed between 2007-2015 at hospitals contributing data to the Pediatric Health Information Systems database. Mixed effects models were used to adjust costs for case mix and to assess inter-hospital variation. Bayesian methods were used to calculate risk-standardized costs for each hospital. Results: The study included 35,637 procedures from 43 hospitals. Median costs (US 2015 dollars) increased roughly with technical complexity, from $8,249 (heart transplant diagnostic catheterization) to $38,909 (transcatheter pulmonary valve replacement). There was significant inter-hospital variation in cost for each procedure ( p Conclusion: There is significant inter-hospital variation in costs of transcatheter procedures after case-mix adjustment. Exploring the etiology of these differences is an opportunity to improve care of children with heart disease.

Stenosis of the pulmonary arteries frequently occurs during staged palliation of hypoplastic left heart syndrome and variants, often necessitating stent angioplasty. A complication of stent angioplasty is compression of the ipsilateral mainstem bronchus. Following such a case, we re-evaluated our approach to PA stent angioplasty in these patients. The incident case is described. A retrospective observational study of children and adults with superior (SCPC) and/or total cavopulmonary connection (TCPC) undergoing left pulmonary artery (LPA) stent angioplasty between January 1, 2005 and January 5, 2014 and subsequent chest CT was performed to assess the incidence of bronchial compression. The current strategy of employing bronchoscopy to assess bronchial compression during angioplasty is described with short-term results. Sixty-five children and adults underwent LPA stent angioplasty. Other than the incident case, none had symptomatic bronchial compression. Of the total study population, 12 % had subsequent CT, of which one subject had moderate bronchial compression. To date, seven subjects have undergone angioplasty of LPA stenosis and bronchoscopy. In one case, stent angioplasty was not performed because of baseline bronchial compression, exacerbated during angioplasty. In the rest of cases, mild-moderate compression was seen during angioplasty. Following stent angioplasty, the resultant compression was not worse than that seen on test angioplasty. Bronchial compression is a rare complication of stent angioplasty of the pulmonary arteries in children and adults with SCPC/TCPC. Angioplasty of the region of interest with procedural bronchoscopy can help to identify patients at risk of this complication.

Background Cardiac catheterization is the standard of care procedure for diagnosis, choice of therapy, and longitudinal follow-up of children and adults with pulmonary hypertension (PH). However, the procedure is invasive and has risks associated with both the procedure and recovery period.

Background Symptomatic infants with unrestrictive ventricular septal defect (VSD) or single ventricle with severe pulmonary stenosis (PS) are typically treated surgically. High surgical risk infants may benefit from catheter-based palliative pulmonary valvuloplasty. Methods We retrospectively reviewed all patients with severe PS associated with unrestrictive VSD or single ventricle who underwent palliative pulmonary valvuloplasty as initial management and describe outcomes. Results From 2000 to 2013, 16 patients met inclusion criteria and underwent valvuloplasty at a median age and weight of 25 (range 1–352) days and 3.65 (range 2.2–12) kg. Common diagnoses included tetralogy of Fallot (50%) and double-outlet right ventricle (25%). In all, the indication was cyanosis. Reasons to defer surgery included low weight (37.5%), preference for complex operation at older age (31.25%) and significant comorbidity (18.75%). Following valvuloplasty, significant increases in oxygen saturation (83.1 ± 8.8 vs. 74.7 ± 8.7%, P = 0.008) and QP:QS (1.6 ± 0.5 vs. 0.8 ± 0.3, P = 0.0005) occurred. All two ventricle patients (n = 10) ultimately had complete operative repair at a median of 125 (range 27–382) days after valvuloplasty, during which time there was 20.3 ± 9.2 g/day of weight gain without change in oxygen saturation. Single ventricle patients who underwent cavopulmonary connection (n = 3) had similar weight gain and palliation time, but with a decrease in oxygen saturation. Two patients required reintervention to augment QP prior to definitive repair. Conclusions In select infants with unrestrictive VSD or single ventricle associated with severe PS, palliative pulmonary valvuloplasty provides an effective and durable method of increasing oxygen saturation until definitive surgical treatment can be performed. © 2015 Wiley Periodicals, Inc.

Objective To describe our center's middle-term outcomes following trans-catheter creation of atrial communication (ASD) in patients on mechanical circulatory support. Background: Trans-catheter creation of an ASD in patients on mechanical circulatory support is an adjuvant therapy to reduce left atrial pressure and associated morbidity. Data on middle term outcomes following this procedure, specifically in regards to the fate of the ASD, are limited. Methods Retrospective observational study of consecutive children and adults undergoing trans-catheter creation of an atrial septal communication between 1/1/2006 and 5/1/2014, reviewing their baseline characteristics, procedural details, and data from follow-up. Results Over the study period, 37/227 (16%) subjects undergoing veno-arterial extra-corporeal membrane oxygenation (VA-ECMO) underwent trans-catheter creation of an atrial communication. Mortality on VA-ECMO support in this subgroup was 19%, with an additional 24% transitioning to ventricular assist device. Of the 57% who survived to separation from VA-ECMO, 16/21 (76%) had residual atrial communications. 56% of these underwent closure procedures. Conclusions Following trans-catheter creation of ASD, a residual ASD is present in the majority of assessable survivors and represents a potential volume overload and/or right to left shunt that may need to be addressed. © 2015 Wiley Periodicals, Inc.

Operative correction of tetralogy of Fallot frequently results in pulmonary insufficiency and chronic volume overload, which have been linked to increased risk for adverse outcomes. No consensus recommendations for the timing of pulmonary valve replacement (PVR) exist. The aim of this study was to examine the pattern of PVR in the United States from 2004 to 2012. The Pediatric Health Information Systems database was used to perform an observational study of children and adults ≥10 years of age with diagnoses of tetralogy of Fallot who underwent PVR at 35 centers in the United States from 2004 and 2012, to assess the rate of PVR and the age at which is performed. Mixed-effects multivariate regression was used to account for patient-level covariates and center-level covariance. Additional analyses assessed for trends in cost, hospital length of stay (LOS), intensive care unit LOS, and in-hospital mortality over the study period. In total, 799 subjects at 35 centers underwent PVR over the study period. The number of PVRs performed per year increased significantly over the study period. There was significant between-center heterogeneity in age at PVR (p0.001). Age at PVR, intensive care unit LOS, hospital LOS, and cost did not change over the study period. In conclusion, PVR in patients with tetralogy of Fallot is being performed more frequently, without an accompanying change in the age at PVR or other measurable outcomes. There is significant variability in the age at which PVR is performed among centers across the United States. This highlights the need for additional research guiding the optimal timing of PVR.

Little data are available on the accuracy of phase-contrast magnetic resonance imaging (PC-MRI) velocity mapping in the vicinity of intravascular metal stents other than nitinol stents. Therefore, we sought to determine this accuracy using in vitro experiments. An in vitro flow phantom was used with 3 stent types: (1) 316L stainless steel, (2) nitinol self-expanding, and (3) platinum-iridium. Steady and pulsatile flow was delivered with a magnetic resonance imaging-compatible pump (CardioFlow 5000, Shelley Medical, London, Ontario, Canada). Flows were measured using a transit time flow meter (ME13PXN, Transonic, Inc, Ithaca, New York). Mean flows ranged from 0.5 to 7 L/min. For each condition, 5 PC-MRI acquisitions were made: within the stent, immediately adjacent to both edges of the stent artifact, and 1 cm upstream and downstream of the artifact. Mean PC-MRI flows were calculated by segmenting the tube lumen using clinical software (ARGUS, Siemens, Inc, Erlangen, Germany). PC-MRI and flow meter flows were compared by location and stent type using linear regression, Bland-Altman, and intraclass correlation (ICC). PC-MRI flows within the stent artifact were inaccurate for all stents studied, generally underestimating flow meter-measured flow. Agreement between PC-MRI and flow meter-measured flows was excellent for all stent types, both immediately adjacent to and 1 cm away from the edge of the stent artifact. Agreement was highest for the platinum-iridium stent (R = 0.999, ICC = 0.999) and lowest for the nitinol stent (R = 0.993, ICC = 0.987). In conclusion, PC-MRI flows are highly accurate just upstream and downstream of a variety of clinically used stents, supporting its use to directly measure flows in stented vessels.

Children requiring reimplantation of a branch pulmonary artery (PA) are at risk for postoperative stenosis and impaired growth of the reimplanted PA. Outcomes and risk factors for reintervention and impaired growth are incompletely described. We reviewed data on patients who underwent reimplantation of a branch PA between 1/1/99 and 5/1/15 at a single center. The primary outcome was reintervention to treat postoperative stenosis. The secondary outcome was “catch-up” growth (faster diameter growth of the affected PA compared with the unaffected PA from the preoperative to follow-up measurements.). Twenty-six patients were identified with a total follow-up of 102.2 patient-years (median 2.5 years). Diagnoses included LPA sling (n = 12) and isolated PA of ductal origin with (n = 7) or without (n = 7) tetralogy of Fallot (ToF). All had primary repair of the anomalous PA. Seventeen (65%) had reintervention with median time to first reintervention of 69 (range 1–1005) days and median of 1.5 (range 1–6) reinterventions. 94% of reinterventions were transcatheter (53% balloon and 41% stent angioplasty). Patients with reintervention were younger (hazard ratio 0.75 per log-day, p = 0.02) and lower weight (hazard ratio 0.18 per log-kg, p = 0.02) at initial repair. Of the 18 with PA growth data, 8 (44%) had catch-up growth. There were no identified differences between those who did and did not demonstrate catch-up growth. Despite a practice of primary reimplantation and aggressive postoperative reintervention, these results suggest that changes in strategy are needed or that there are intrinsic patient factors that have more influence on longer-term reimplanted PA growth.

In neonates requiring balloon aortic valvuloplasty, both anterograde and retrograde approaches are feasible. A recent comparison of these two approaches is lacking. A retrospective cohort study of neonates at a single center undergoing BAV from 9/00 to 7/14 was performed. Records were reviewed including pre- and post-intervention echocardiograms and catheterization data. Comparisons of acute efficacy and procedural safety were made based on type of approach utilized. Forty-two neonates underwent BAV. Eleven cases utilized exclusively an anterograde approach, while 31 included a retrograde approach (including 4 with both approaches used). There were no significant differences between groups in baseline demographic and clinical characteristics. Additionally, by both pre-intervention echocardiogram and catheterization, there were no differences based on approach in aortic valve gradient, degree of aortic insufficiency (AI), or degree of mitral regurgitation (MR). Both approaches were equally efficacious in gradient reduction (45 ± 17 vs. 44 ± 21 mmHg, p = 0.97), and there was no difference in post-intervention AI as assessed by both catheterization and echocardiogram (52% vs. 64% none or trivial, p = 0.74). Additionally, there was no difference in the proportion of patients with an increased severity of MR after BAV (15% vs. 22%, p = 0.52). The retrograde approach required a larger arterial catheter and was associated with a higher rate of arterial thrombosis (61% vs. 18%, p = 0.014). Both anterograde and retrograde approaches to neonatal BAV appear to be equally efficacious in the short term. The anterograde approach avoids the need for a larger arterial catheter and may reduce the risk of arterial thrombosis.

Concern for device erosion following transcatheter treatment of atrial septal defects (TC-ASD) led in 2012 to a United States Food and Drug Administration panel review and changes in the instructions for use of the Amplatzer Septal Occluder (ASO) device. No studies have assessed the effect of these changes on real-world practice. To this end a multicenter observational study was performed to evaluate trends in the treatment of ASD.A retrospective observational study was performed using data from the Pediatric Health Information Systems database of all patients with isolated ASD undergoing either TC-ASD or operative ASD closure (O-ASD) from January 1, 2007, to September 30, 2015, hypothesizing that the propensity to pursue O-ASD increased beginning in 2013.A total of 6,392 cases from 39 centers underwent ASD closure (82% TC-ASD). Adjusting for patient factors, between 2007 and 2012, the probability of pursuing O-ASD decreased (odds ratio [OR] 0.95 per year, P = .03). This trend reversed beginning in 2013, with the probability of O-ASD increasing annually (OR 1.21, P = .006). There was significant between-hospital variation in the choice between TC-ASD and O-ASD (median OR 2.79, P.0001). The age of patients undergoing ASD closure (regardless of method) decreased over the study period (P = .04). Cost of O-ASD increased over the study period, whereas cost of TC-ASD and length of stay for both O-ASD and TC-ASD was unchanged.Although TC-ASD remains the predominant method of ASD closure, the propensity to pursue O-ASD has increased significantly following changes in instructions for use for ASO. Further research is necessary to determine what effect this has on outcomes and resource utilization.

Objective Objectives To describe the results of a clinical practice pathway (CPP) for the management of postcatheterization pulse loss in a children's hospital. Background Standardized approaches to the diagnosis and management of postcatheterization arterial thrombus are lacking. As a result, substantial practice variation exists. Methods Data collected prospectively for quality improvement purposes were retrospectively reviewed. Results Since initiation of the CPP, 93/1,672 (5.4%) catheterizations resulted in pulse loss at a median patient age and weight of 73 days (1 day–5.8 years) and 4.8 kg (2–14.1 kg). Arterial thrombus was documented by ultrasound (US) in 85. Of these, 66 resolved by 12 weeks of therapy, seven patients died, and four were lost to follow-up before completing treatment. Eight patients had persistent thrombus despite a full treatment course (89% success rate in those able to complete treatment). Of patients treated with unfractionated heparin as initial therapy, 46% (17/37) achieved a therapeutic partial thromboplastin time within 12 hr with 19% (67/343) of all levels therapeutic. As a result, the CPP was modified to use enoxaparin as first line agent, of which 57% (41/72) had a therapeutic anti-Xa level after the 2nd dose and 88% by the 4th dose. No bleeding complications were observed. A priori established process metrics were achieved. Conclusions A CPP utilizing early initiation of anticoagulation and US to aid diagnosis of postcatheterization arterial thrombus and response to therapy is feasible and effective. In those able to complete up to 12 weeks of treatment, resolution occurs in nearly 90%. © 2014 Wiley Periodicals, Inc.

Objective Deficient retro-aortic rim has been identified as a risk factor for device erosion following trans-catheter closure of atrial septal defects (ASDs). Transthoracic echocardiography (TTE) is the primary screening method for subjects for possible device closure of ASD, but its reliability in measuring retro-aortic rim size has not been assessed previously. Design A single-institution cross-sectional analysis of children and adults referred for trans-catheter device closure of single ostium secundum ASD from January 1, 2005 to April 1, 2012 with reviewable TTE and trans-esophageal echocardiogram images was performed. Inter-rater reliability of measurements was tested in a 24% sample. Accuracy of TTE measurement of retro-aortic rim was assessed using a Bland–Altman plot with trans-esophageal echocardiogram measurement as the gold standard. Test characteristics of TTE detection of deficient retro-aortic rim were calculated. Risk factors for misclassification of deficient retro-aortic rim were assessed using receiver operator characteristic curves. Risk factors for measurement error were assessed through multivariate linear regression. Results In total, 163 subjects of median age 5 years (range: 0.3–46 years) were included. Trans-thoracic echocardiography had 90% sensitivity, 84% specificity, 90% positive predictive value, and 83% negative predictive value to detect deficient retro-aortic rim. Bland–Altman plot demonstrated no fixed bias (P = .23), but errors in measurement increased on average as the aortic rim increased in size (P

Background Plastic bronchitis is a rare, potentially life-threatening complication after Fontan operation. Hemodynamic alterations (elevated central venous pressure and low cardiac output) likely contribute to the formation of tracheobronchial casts composed of inflammatory debris, mucin, and fibrin. Pathologic studies of cast composition support medical treatment with fibrinolytics such as inhaled tissue plasminogen activator (t-PA). Methods This was a retrospective case series of medical, surgical, and catheter-based treatment of patients with plastic bronchitis after cavopulmonary palliation. Results Included were 14 patients (86% male, 93% white). Median age at Fontan operation was 2.7 years (range, 1.2 to 4.1 years), with median interval to plastic bronchitis presentation of 1.5 years (range, 9 days to 15.4 years). Cast composition was available for 11 patients (79%) and included fibrin deposits in 7. All patients were treated with pulmonary vasodilators, and 13 (93%) were treated with inhaled t-PA. Hemodynamically significant lesions in the Fontan pathway were addressed by catheter-based (n = 9) and surgical (n = 3) interventions. Three patients (21%) underwent heart transplantation. Median follow-up was 2.7 years (range, 0.6 to 8.7 years). Symptoms improved, such that 6 of 13 patients (46%) were weaned off t-PA. Rare or episodic casts are successfully managed with outpatient t-PA in most of the other patients. Of the 3 patients who underwent heart transplant, 2 are asymptomatic and 1 has recurrent casts in the setting of elevated filling pressures and rejection. Conclusions A systematic step-wise algorithm that includes optimization of hemodynamics, aggressive pulmonary vasodilation, and inhaled t-PA is an effective treatment strategy for patients with plastic bronchitis after cavopulmonary connection.

Background Radiation exposure in the pediatric population may increase the risk of future malignancy. Children with congenital heart disease who often undergo repeated catheterizations are at risk. One possible strategy to reduce radiation is to use X-ray Magnetic Resonance Fusion (XMRF) to facilitate cardiac catheterization. Methods Catheterization data of patients who underwent diagnostic XMRF procedures between January 1, 2009 and February 1, 2012 were reviewed. Cases were matched 1:1 to contemporary controls who did not undergo XMRF based on weight and diagnosis and were compared in radiation exposure, contrast dose, and procedural and anesthesia times. Results Forty-four matched pairs were included. Baseline demographics were similar in both groups. Patients in the XMRF group had lower indices of radiation exposure measured by fluoroscopy time (14 vs. 16.4 vs. P = 0.047), dose-area product from fluoroscopy (513.2 vs. 589.1 µGy·m2, P = 0.042), total dose-area product (625.8 vs. 995.2 µGy·m2, P = 0.027), and total air kerma dose (94.5 vs. 153.8 mGy, P = 0.017). There was also a significant reduction in contrast dose (2 vs. 3.3 cc/kg, P