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Background In infants with ductal‐dependent pulmonary blood flow, the impact of palliation strategy on interstage growth and feeding regimen is unknown. Methods and Results This was a retrospective multicenter study of infants with ductal‐dependent pulmonary blood flow palliated with patent ductus arteriosus (PDA) stent or Blalock‐Taussig shunt (BTS) from 2008 to 2015. Subjects with a defined interstage, the time between initial palliation and subsequent palliation or repair, were included. Primary outcome was change in weight‐for‐age Z‐score. Secondary outcomes included % of patients on: all oral feeds, feeding‐related medications, higher calorie feeds, and feeding‐related readmission. Propensity score was used to account for baseline differences. Subgroup analysis was performed in 1‐ (1V) and 2‐ventricle (2V) groups. The cohort included 66 PDA stent (43.9% 1V) and 195 BTS (54.4% 1V) subjects. Prematurity was more common in the PDA stent group (P=0.051). After adjustment, change in weight‐for‐age Z‐score did not differ between groups over the entire interstage. However, change in weight‐for‐age Z‐score favored PDA stent during the inpatient interstage (P=0.005) and BTS during the outpatient interstage (P=0.032). At initial hospital discharge, PDA stent treatment was associated with all oral feeds (P

Background: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention, utilizing either a staged repair (SR) or primary repair (PR) approach. They are exposed to several sources of low-dose ionizing radiation, which may contribute to increased cancer risk., Objectives: The purpose of this study was to compare cumulative radiation exposure and associated lifetime attributable risk (LAR) of cancer between treatment strategies in sTOF., Methods: Neonates with sTOF who underwent SR or PR from 2012 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Radiation exposure from all radiologic studies prior to 18 months of age was converted to organ-equivalent doses and projected LAR of cancer incidence using the National Cancer Institute dosimetry tools., Results: There were 242 neonates from 8 centers, including patients with 146 SR and 96 PR. Cumulative total effective dose was significantly higher for SR (median 8.3 mSv, IQR: 3.0-17.4 mSv) than PR (2.1 mSv, IQR: 0.8-8.5 mSv; P  < 0.001). Cumulative organ-level doses were significantly higher in SR compared to PR. Regardless of treatment strategy, LARs were higher in females compared to males. Among organs with median exposure >1 mGy in females, the LAR was highest for breast in SR (mean 1.9/1,000 patients). The highest proportion of cancers attributable to radiation exposure was projected for thyroid cancer in females undergoing SR (7.3%)., Conclusions: Cumulative radiation exposure and LARs were higher among those undergoing SR compared to PR. This will be an important factor to consider in determining the preferred neonatal treatment strategy and should substantiate efforts to reduce radiation exposure in this vulnerable population., Competing Interests: Financial support for this research was derived, in part, from the Kennedy Hammill Pediatric Cardiac Research Fund, the Liam Sexton Foundation, and A Heart Like Ava. Dr Goldstein has reported consulting relationships with Medtronic, W.L. Gore & Associates, and Edwards Lifesciences; and is an advisory board member for PECA Labs. Dr Qureshi has reported consulting relationships with W.L. Gore & Associates, Edwards Lifesciences, and Abiomed. Dr Zampi has reported consulting relationships with Medtronic and W.L. Gore & Associates. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Background: Complete repair (CR) can be delayed in neonates with symptomatic tetralogy of Fallot (sTOF) using surgical or transcatheter palliation to relieve cyanosis. Balloon pulmonary valvuloplasty (BPV) is an established treatment for pulmonary valve stenosis; however, its effectiveness in palliating neonates with sTOF has not been well investigated., Methods: A retrospective chart review between 2005 and 2017 on neonates with sTOF who underwent initial BPV from 9 participating centers of the Congenital Cardiac Research Collaborative was performed. Primary outcome was CR at >30 days after BPV without interval reintervention (RI)., Results: In total, 47 neonates with sTOF underwent BPV, of whom 27 (57%) underwent CR at >30 days after BPV without RI. The median time to CR was 151 days (106-210). RI before CR occurred in 17 patients (36%): surgical shunt (n = 7), outflow tract stenting (n = 6), patent ductus arteriosus stenting (n = 2), and surgical outflow patch (n = 2). Valve-sparing repair at CR was performed in 6 patients (13%) after initial BPV. RI or CR ≤30 days from BPV was associated with smaller infundibular diastolic diameter ( P = .004). An infundibular diastolic diameter of <3.4 mm demonstrated 75% sensitivity and 67% specificity to predict early CR or RI., Conclusions: BPV can be an effective palliative therapy in select neonates with sTOF to delay CR. A smaller diastolic infundibulum diameter is a predictor of RI or early CR, and valve-sparing repair is uncommon, making patient selection and alternative palliative methods key when considering BPV palliation in neonates with sTOF., (© 2023 The Author(s).)

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.

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.)

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: Transcatheter aortic and pulmonary valves have been used to treat stenosis or regurgitation after prior surgical tricuspid valve (TV) replacement or repair. Little is known about intermediate-term valve-related outcomes after transcatheter tricuspid valve replacement (TTVR), including valve function, thrombus, and endocarditis., Objectives: The authors sought to evaluate mid-term outcomes in a large cohort of patients who underwent TTVR after surgical TV repair or replacement, with a focus on valve-related outcomes., Methods: Patients who underwent TTVR after prior surgical TV replacement or repair were collected through an international registry. Time-related outcomes were modeled and risk factors assessed., Results: Data were collected for 306 patients who underwent TTVR from 2008 through 2017 at 80 centers; 52 patients (17%) had a prior history of endocarditis. Patients were followed for a median of 15.9 months after implantation (0.1 to 90 months), with 64% of patients estimated to be alive without TV reintervention or a valve-related event at 3 years. The cumulative 3-year incidence of death, reintervention, and valve-related adverse outcomes (endocarditis, thrombosis, or significant dysfunction) were 17%, 12%, and 8%, respectively. Endocarditis was diagnosed in 8 patients 2 to 29 months after TTVR, for an annualized incidence rate of 1.5% per patient-year (95% confidence interval: 0.45% to 2.5%). An additional 8 patients were diagnosed with clinically relevant valve thrombosis, 3 in the short term, 2 within 2 months, and 3 beyond 6 months. Only 2 of these 8 patients received anticoagulant therapy before thrombus detection (p = 0.13 vs. patients without thrombus). Prior endocarditis was not a risk factor for reintervention, endocarditis, or valve thrombosis, and there was no difference in valve-related outcomes according to TTVR valve type., Conclusions: TV dysfunction, endocarditis, and leaflet thrombosis were uncommon after TTVR. Patients with prior endocarditis were not at higher risk for endocarditis or other adverse outcomes after TTVR, and endocarditis occurred with similar frequency in different valve types. Though rare, leaflet thrombosis is an important adverse outcome, and further study is necessary to determine the appropriate level of prophylactic therapy after TTVR., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study sought to determine the safety and feasibility of transcatheter pulmonary valve replacement (TPVR) using the Melody valve in native (nonconduit) right ventricular outflow tracts (nRVOT), and to identify factors associated with successful TPVR., Background: The Melody valve is Food and Drug Administration-approved for TPVR within right ventricle-to-pulmonary artery conduits and bioprosthetic pulmonary valves. However, most patients needing pulmonary valve replacement have nRVOT and TPVR has been adapted for this indication., Methods: In this multicenter retrospective study of all patients presenting for nRVOT TPVR, we collected pre-procedural magnetic resonance imaging, echocardiography, and catheterization data, and evaluated procedural and early outcomes., Results: Of 229 patients (age 21 ± 15 years from 11 centers), 132 (58%) had successful TPVR. In the remaining 97, TPVR was not performed, most often because of prohibitively large nRVOT (n = 67) or compression of the aortic root or coronary arteries (n = 18). There were no deaths and 5 (4%) serious complications, including pre-stent embolization requiring surgery in 4 patients, and arrhythmia in 1. Higher pre-catheterization echocardiographic RVOT gradient was associated with TPVR success (p = 0.001) and larger center volume approached significance (p = 0.08). Magnetic resonance imaging anterior-posterior and lateral RVOT diameters were smaller in implanted versus nonimplanted patients (18.0 ± 3.6 mm vs. 20.1 ± 3.5 mm; p = 0.005; 18.4 ± 4.3 mm vs. 21.5 ± 3.8 mm; p = 0.002)., Conclusions: TPVR in the nRVOT was feasible and safe. However, nearly half the patients presenting for catheterization did not undergo TPV implantation, mainly because of prohibitively large nRVOT size. Improved understanding of magnetic resonance imaging data and availability of larger devices may improve the success rate for nRVOT TPVR., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

We sought to describe the acute results and short- to medium-term durability of transcatheter tricuspid valve-in-valve (TVIV) implantation within surgical bioprostheses among patients with Ebstein anomaly (EA). Cases were identified from a voluntary, multicenter, international registry of 29 institutions that perform TVIV. Demographic, clinical, procedural, and follow-up data were analyzed. Eighty-one patients with EA underwent TVIV from 2008 to 2016. Thirty-four patients (42%) were New York Heart Association (NYHA) class 3/4 at time of TVIV. The most common indication for TVIV was the presence of moderate or severe tricuspid regurgitation (40%). Most patients received a Melody valve (64%). TVIV was ultimately successful in all patients, and there was no procedural mortality. Four patients (5%) developed acute valve thrombosis, 4 patients (5%) developed endocarditis, and 9 patients (11%) developed valve dysfunction not related to thrombosis or endocarditis. Eight patients (10%) underwent reintervention (2 transcatheter, 6 surgical) due to thrombosis (3), endocarditis (2), other valve dysfunction (2), and patient-prosthesis mismatch without valve dysfunction (1). Among 69 patients who were alive without reintervention at latest follow-up, 96% of those with NYHA status reported were class 1/2, a significant improvement from baseline (62% NYHA class 1/2, p <0.001). In conclusion, transcatheter TVIV offers a low-risk, minimally invasive alternative to surgical tricuspid valve re-replacement in patients with EA and a failing tricuspid valve bioprosthesis., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Off-label use of transcatheter aortic and pulmonary valve prostheses for tricuspid valve-in-valve implantation (TVIV) within dysfunctional surgical tricuspid valve (TV) bioprostheses has been described in small reports., Methods and Results: An international, multicenter registry was developed to collect data on TVIV cases. Patient-related factors, procedural details and outcomes, and follow-up data were analyzed. Valve-in-ring or heterotopic TV implantation procedures were not included. Data were collected on 156 patients with bioprosthetic TV dysfunction who underwent catheterization with planned TVIV. The median age was 40 years, and 71% of patients were in New York Heart Association class III or IV. Among 152 patients in whom TVIV was attempted with a Melody (n=94) or Sapien (n=58) valve, implantation was successful in 150, with few serious complications. After TVIV, both the TV inflow gradient and tricuspid regurgitation grade improved significantly. During follow-up (median, 13.3 months), 22 patients died, 5 within 30 days; all 22 patients were in New York Heart Association class III or IV, and 9 were hospitalized before TVIV. There were 10 TV reinterventions, and 3 other patients had significant recurrent TV dysfunction. At follow-up, 77% of patients were in New York Heart Association class I or II (P<0.001 versus before TVIV). Outcomes did not differ according to surgical valve size or TVIV valve type., Conclusions: TVIV with commercially available transcatheter prostheses is technically and clinically successful in patients of various ages across a wide range of valve size. Although preimplantation clinical status was associated with outcome, many patients in New York Heart Association class III or IV at baseline improved. TVIV should be considered a viable option for treatment of failing TV bioprostheses., (© 2016 American Heart Association, Inc.)

Radiation exposure remains a significant concern for ASD device closure. In an effort to reduce radiation exposure, the default fluoroscopy frame rate in our Siemens biplane pediatric catheterization laboratory was reduced to 4 fps in November 2013 from an earlier 7.5 fps fluoro rate. This study aims to evaluate the components contributing to total radiation exposure and compare the procedural success and radiation exposure during ASD device closure using 4 versus 7.5 fps fluoroscopy rates. Twenty ASD device closures performed using 4 fps fluoro rate were weight-matched to 20 ASD closure procedures using 7.5 fps fluoro rate. Baseline characteristics, procedure times and case times were similar in the two groups. Device closure was successful in all but one case in the 4 fps group. The dose area product (DAP), normalized DAP to body weight, total radiation time and fluoro time were lower in the 4 fps group but not statistically different than the 7.5 fps. The number of cine images and cine times were identical in both groups. Fluoroscopy and cineangiography contributed equally to radiation exposure. Fluoroscopy at 4 fps can be safe and effective for ASD device closure in children and adults. There was no increase in procedure time, cine time, fluoro time or complications at this slow fluoro rate. There was a trend toward decreased radiation exposure as measured by indexed DAP although not statistically significant in this small study. Further study with multiple operators using 4 fps fluoroscopy for simple interventional procedures is recommended.

Background: The Coarctation of the Aorta Stent Trial (COAST) was designed to assess the safety and efficacy of the Cheatham Platinum stent when used in children and adults with native or recurrent coarctation. Acute outcomes have been reported. We report here follow-up to 2 years., Methods and Results: A total of 105 patients underwent attempted implantation, with 104 successes. There were no procedural deaths, serious adverse events, or surgical intervention. All patients experienced immediate reduction in upper- to lower-extremity blood pressure difference with sustained improvement to 2 years. Rates of hypertension and medication use decreased from baseline to 12 months and remained largely unchanged at 2 years. Six aortic aneurysms have been identified: 5 were successfully treated with covered stent placement, and 1 resolved without intervention. Stent fractures were noted in 2 patients at 1 year and 11 patients at 2 years, with evidence of fracture progression. To date, only larger stent diameter was associated with stent fracture. Twelve additional fractures have occurred after 2 years. No fracture has resulted in loss of stent integrity, stent embolization, aortic wall injury, or reobstruction. Nine reinterventions occurred in the first 2 years for stent redilation and address of aneurysms, and 10 additional reinterventions occurred after 2 years., Conclusions: The Cheatham Platinum stent is safe and associated with persistent relief of aortic obstruction. Stent fracture and progression of fracture occur but have not resulted in clinically important sequelae. Reintervention is common and related to early and late aortic wall injury and need for re-expansion of small-diameter stents., Clinical Trial Registration: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00552812., (© 2015 American Heart Association, Inc.)

Objectives: We examine normalized air Kerma area product (PKA ) by body weight (PKA /BW) as a reference value of radiation dose and benchmark PKA /BW in pediatric laboratories using a multicenter registry database., Background: Reduction of radiation dose is an important quality improvement task in pediatric cardiac catheterization laboratories. Physicians need to agree on a standard method of reporting radiation dose that would allow comparisons to be made between operators and institutions., Methods: This was a multicenter observational study of radiation dose in pediatric laboratories. Patient demographic, procedural and radiation data including fluoroscopic time and PKA (µGy m(2) ) were analyzed. PKA /BW was obtained by indexing PKA to body weight., Results: A total of 8,267 pediatric catheterization procedures (age <18 years) were included from 16 institutions. The procedures consisted of diagnostic (n = 2,827), transplant right ventricular (RV) biopsy (n = 1,172), and interventional catheterizations (n = 4268). PKA correlated with body weight better than with age and best correlated with weight-fluoroscopic time product. PKA /BW showed consistent values across pediatric ages. Interventional catheterizations had the highest PKA /BW (50th, 75th, and 90th percentiles: 72, 151, and 281 μGy m(2) /kg), followed by diagnostic (59, 105, and 175 μGy m(2) /kg) and transplant RV biopsy (27, 79, and 114 μGy m(2) /kg)., Conclusion: PKA /BW appeared to be the most reliable standard to report radiation dose across all procedure types and patient age. We recommend PKA /BW to be used as the standard unit in documenting radiation usage in pediatric laboratories and can be used to evaluate strategies to lower radiation dosage in pediatric patients undergoing cardiac catheterizations. © 2014 Wiley Periodicals, Inc., (© 2014 Wiley Periodicals, Inc.)

Objectives: To describe the immediate and midterm results of a modified method for transcatheter closure of perimembranous ventricular septal defects (pmVSDs)., Background: Transcatheter closure of pmVSDs has been associated with development of heart block due to impingement on the ventricular conduction system. Ventricular septal aneurysms (VSAs) are common; the VSA tissue can serve as a target for the device without necessitating direct contact with the conduction system., Methods: Between 2004 and 2011, 15 patients underwent transcatheter closure of a pmVSD utilizing a device implanted into a VSA. Catheterization reports were examined in addition to pre-closure, post-closure, and current clinical, ECG, and echocardiographic data., Results: The median age was 20 years (4-61 years), and the most common indication for closure was increasing LV dilation. Four different Amplatzer devices were utilized. Following device implantation there was a decrease in Qp:Qs (1.7-1.1) and in RV:LV pressure ratio (0.36-0.31). There were no deaths, no device embolizations, and no new heart block or PR interval prolongation. Three patients developed a new right bundle branch block (RBBB). The median follow-up time was 1.5 years (4 months to 7.1 years). Two patients required further procedures for important residual shunting. Six continued to have a "trivial/small" residual leak, but only one had any degree (mild) of residual LV dilation. None of the complications were significantly associated with age or weight at the time of procedure, original size of the VSD, or size or type of the device used., Conclusion: Transcatheter closure of pmVSD with placement of the device into the VSA is safe and effective, and may result in fewer instances of atrioventricular block and valve abnormalities than have been reported with alternative methods of pmVSD device closure. Persistent VSDs and new RBBBs remain an important issue., (Copyright © 2013 Wiley Periodicals, Inc.)

The arterial switch operation for correction of transposition of the great arteries can be complicated by late stenosis or occlusion of the coronary arteries that are re-implanted to the new aorta. We report the case of a young boy who underwent this operation as a neonate and was found to have an occluded anomalous left anterior descending artery (LAD) before age 3. Subsequent bypass surgery was complicated by anastomotic stricture and kinking of the left internal mammary artery graft to the LAD. At age 7, the LAD territory showed reversible ischemia on nuclear perfusion testing and he was referred for percutaneous coronary intervention. A combined approach with pediatric and adult interventional cardiologists resulted in successful retrograde PCI to recanalize the chronic total occlusion of the LAD. Important features of this technique in pediatric patients are discussed., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Objectives: We sought to characterize the use, efficacy, and performance characteristics of premounted stents relative to nonpremounted stents when used during congenital cardiac catheterization., Background: Endovascular stent implantation is an effective means of relieving vascular obstruction in patients with congenital heart disease. However, stent implantation is technically challenging and important complications occur. Premounted stents appear to offer many advantages relative to their nonpremounted counterparts, and it has been suggested that the use of premounted stents is associated with fewer complications. However, translation of these potential benefits into procedural or clinical success has been poorly examined and the data are conflicting., Methods: All stent placements performed between January 1, 1999 and December 31, 2009 were reviewed. Analysis of technical success, hemodynamic success and complications was performed., Results: 416 stents were placed over the 10 year period. 158 (38%) were premounted. There was no apparent trend in the frequency of use of premounted stents over the study period. Implanted premounted stents were smaller in diameter than nonpremounted stents 4.9 mm +/- 1.8 versus 13.9 mm +/- 3.7, and the site of stent placement differed significantly. Unadjusted and adjusted analysis of technical success with respect to the precision of stent placement, hemodynamic success, and complications showed no difference between premounted and non-premounted stents., Conclusions: We found no difference between premounted and nonpremounted stents with respect to procedural and hemodynamic success or complications. Nevertheless, there remain practical advantages to the use of premounted stents that may justify their expanding role in congenital cardiac catheterization., (© 2012, Wiley Periodicals, Inc.)

Objective: To compare the course of hypoplastic left heart syndrome (HLHS) patients diagnosed prenatally with any degree of atrial restriction with those without evidence of atrial restriction., Design: Retrospective, cohort., Methods: Prenatally diagnosed HLHS patients from August 1999 to January 2009 were categorized as nonrestrictive versus restrictive, defined by left atrial hypertension on pulmonary venous Doppler and/or an intact interatrial septum., Results: Of 73 total fetal patients identified, 49 were live-born. Survival at 2 years was 29/35 [83% confidence interval (CI): 59.5%-88.9%] for the nonrestrictive group and 6/14 (43% CI: 17.7%-6.0%) for the restrictive group (p<0.0001). Of those who underwent stage 1 palliation (35 with nonrestrictive and 10 with restrictive atrial septa), both groups had a similar incidence of preoperative acidosis and need for ventilation and inotropic support. Postoperatively, there was no difference between groups in ventilator days, length of stay, or survival to discharge. There was decreased survival at 2 years in the restrictive group, 60% (CI: 26.2%-87.8%) versus 83% (CI: 66.4%-93.4%) in the nonrestrictive group. Furthermore, a disproportionate number of interstage deaths was evident in the restrictive group., Conclusions: Prenatal presence of any degree of atrial septal restriction in the setting of HLHS confers a significant survival disadvantage, with increases in both early and late mortality., (© 2012 John Wiley & Sons, Ltd.)

Protein-losing enteropathy is a relatively uncommon complication of Fontan procedures for palliation of complex congenital cardiac disease. However, the relative infrequency of protein-losing enteropathy belies the tremendous medical, psychosocial and financial burdens it places upon afflicted patients, their families and the healthcare system that supports them. Unfortunately, because of the complexity and rarity of this disease process, the pathogenesis and pathophysiology of protein-losing enteropathy remain poorly understood, and attempts at treatment seldom yield long-term success. The most comprehensive analyses of protein-losing enteropathy in this patient population are now over a decade old, and re-evaluation of the prevalence and progress in treatment of this disease is needed. This report describes a single institution experience with the evaluation, management, and treatment of protein-losing enteropathy in patients with congenital cardiac disease in the current era, follows with a comprehensive review of protein-losing enteropathy, focused upon what is known and not known about the pathophysiology of protein-losing enteropathy in this patient population, and concludes with suggestions for prevention and treatment.

Cardiac catheterization in the adult patient with congenital heart disease clearly is now its own field. The history of the field is closely tied to pediatric cardiac catheterization, in large part because of the individual histories of its patients, and as such, at least for the foreseeable future, these two fields appear inexorably linked. For both, the pace of progress has been swift. This chapter provides a broad review of the important advances in cardiac catheterization that have occurred over the past decade as they pertain to the adult with congenital heart disease, with an emphasis on recent interventional tools and techniques that have revolutionized this exciting field., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objectives: We evaluated the anatomic and technical factors predicting stent malposition and embolization in patients undergoing endovascular stent implantation for relief of noncoronary vascular obstruction., Background: Endovascular stent implantation provides a highly effective, minimally invasive solution to vascular obstruction in patients with structural heart disease. However, stent implantation is technically challenging and stent embolization occurs in up to 5.5% of cases., Methods: We reviewed patient and procedural characteristics of all endovascular stent implantations performed for relieving noncoronary vascular obstruction from January 1, 1999, through December 31, 2009. Univariate and multivariate predictors of stent malposition or embolization were explored through logistic regression methods., Results: During the 10-year study period, 429 stents were implanted. Of these, 399 were placed for relief of vascular obstruction in 267 patients during 322 procedures. Initial implantation failure occurred in 33 patients (8.3%), including stent malposition in 18 (4.5%) and stent embolization in 15 (3.8%). Patient size and vascular obstruction caused by external compression or a vascular fold were independent predictors of stent malposition or embolization. All malpositioned and embolized stents were successfully managed without surgery, and none resulted in death, sustained hemodynamic instability, or important vascular injury., Conclusions: Endovascular stent implantation is a highly effective and safe means of relieving noncoronary vascular obstruction in patients with congenital and acquired structural heart disease. Stent embolization occurs in approximately 3.8% of implantation procedures but can be managed successfully without surgical intervention. Anatomic and technical factors predict stent malposition, and consideration of these factors may improve procedural results., (Copyright © 2010 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: The aim of this study was to prospectively examine the effects of elective Fontan fenestration closure upon exercise capacity and ventilatory abnormalities., Background: For patients undergoing Fontan procedures as palliation of single ventricle physiology, the addition of a fenestration to the procedure mitigates perioperative morbidity. Although some fenestrations may close spontaneously, many remain patent and subject patients to arterial hypoxemia and risk for paradoxical emboli. For these reasons fenestration closure is routinely performed post-operatively in the cardiac catheterization laboratory. Although closure of Fontan fenestrations typically results in an immediate improvement in systemic arterial oxygen saturation, it is also associated with an acute decrease in cardiac index and systemic O(2) delivery. The sum result of these physiologic changes upon exercise capacity has not been examined., Methods: Twenty consecutive patients, age 5 to 46 (median 10) years, underwent pre- and post-fenestration closure exercise testing with expiratory gas analysis., Results: Before fenestration closure, peak oxygen consumption (VO(2)) was depressed and there was systemic desaturation at rest that worsened with exercise. The ventilatory response to exercise was also abnormal, characterized by elevation of the minute ventilation (VE)/CO(2) elimination slope (VE/VCO(2)), a low end-tidal CO(2), and high end-tidal O(2) at the ventilatory anaerobic threshold. Although arterial saturation improved significantly after fenestration closure, there was no change in peak VO(2) (70.9 +/- 18.6% to 74.0 +/- 18.6%, p = NS), heart rate, or O(2) pulse at peak exercise. In contrast, ventilatory abnormalities (VE/VCO(2)) improved considerably (44.4 +/- 10.9 to 33.3 +/- 5.5, p

Objective: Protein-losing enteropathy (PLE) is a known complication of surgical procedures for congenital heart disease. The pathogenesis and pathophysiology of PLE remain poorly understood. However, lymphatic insufficiency appears central to the disease process. We sought to investigate the role of lymphatic obstruction and central venous catheter-related central venous thrombosis in patients with congenital heart disease and PLE., Design: A case-control study design was constructed consisting of patients with congenital heart disease and PLE and 2:1 matched controls having undergone the same definitive surgical procedure. Obstruction to lymphatic return was considered present if the thoracic duct was ligated, or if there was complete central venous obstruction at the usual site of thoracic duct drainage., Results: Obstruction to lymphatic return was identified in 4 of 16 cases (25%) and 1 of 32 controls (4%), P = .06. There was no association between PLE and central venous catheter use or duration, and no discriminating characteristics between cases and controls with respect to anatomy, pre-Fontan hemodynamic variables, operative or perioperative factors, or hemodynamic variables at the time of PLE diagnosis. Mortality for patients with PLE was 25% compared with 9% in controls (P = not significant). Long-term resolution of PLE was obtained in six patients (38%)., Conclusion: There is a high prevalence of apparent lymphatic obstruction in patients with congenital heart disease and PLE, suggesting that physical lymphatic obstruction may play an important, and previously unrecognized role in the development of PLE in patients with complex congenital heart disease.

Objective exercise testing in patients with repaired tetralogy of Fallot frequently identifies gross deficiencies in exercise capacity. These findings are typically attributed to right ventricular dysfunction, pulmonary valve regurgitation, and pulmonary artery stenosis and are used to justify referrals for surgical or transcatheter interventions. However, the relation between right-sided cardiac abnormalities and exercise capacity in this patient group is poorly understood. Cardiac magnetic resonance imaging correlates of exercise capacity in 37 patients with repaired TOF were retrospectively examined. In conclusion, on multivariate analysis, right ventricular ejection fraction was the only cardiac magnetic resonance imaging predictor of percentage of predicted peak oxygen consumption, oxygen consumption at ventilatory anaerobic threshold, and oxygen pulse.

Objective: Creation or enlargement of a ventricular septal defect is indicated in rare clinical situations. In the setting of double-outlet right ventricle requiring single-ventricle palliation, left ventricular outflow tract obstruction caused by progressive restriction at the ventricular septal defect poses an uncommon but recognized dilemma. In this situation surgical ventricular septal defect enlargement may be desirable but risks damage to the atrioventricular valve or conduction system. We report the results of a novel technique for transcatheter creation or enlargement of ventricular septal defects as an alternative to reoperation when decompression of an isolated ventricle is indicated., Methods: Eight patients had undergone transcatheter ventricular septal defect enlargement or creation, and 3 of these patients had undergone 4 prior surgical attempts at left ventricular decompression. Ventricular aneurysms had developed in 3 patients before intervention., Results: Five patients underwent ventricular septal defect creation, and 3 patients underwent enlargement of existing ventricular septal defects. Initial intervention resulted in a decreased ventricular septal defect pressure gradient from 76.9 mm Hg to 20.3 mm Hg (P = .004). There was no procedural mortality or sustained heart block. Two patients had moderate-to-severe atrioventricular valve regurgitation, and 1 required surgical repair. At last follow-up, all ventricular septal defects remained patent, with recurrent obstruction in the majority of cases caused by muscular hypertrophy beyond the stent margins. In 1 patient a ventricular aneurysm has regressed in size. Repeat intervention reduced recurrent obstruction, but recurrence was the rule., Conclusions: When reoperation is considered high risk, transcatheter creation and enlargement of ventricular septal defects is possible with excellent short-term results. Recurrent obstruction is common but responds to repeated intervention. Further studies are required to establish clinical benefit.