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Purpose of Review: To review current controversies in the transcatheter device closure of ostium secundum atrial septal defects (ASD)., Recent Findings: Transcatheter device closure of ASD (TC-ASD) has well established efficacy and safety. For most individual patients with suitable anatomy, TC-ASD is the preferred method for treating ASD. The availability of large multicenter data sets has made it possible to study practice patterns at a range of hospitals across the United States. These studies have revealed differences in practice that were not previously appreciated. Interpretation of the indications for TC-ASD, specifically the definition of right ventricular volume overload varies between hospitals. In response to concern about device erosion, an increasing proportion of patients are being referred for operative ASD closure. Over the last decade, the average age at which ASD closure occurs has decreased. These trends demonstrate previously underappreciated differences in opinion between cardiologists across the country and suggest that further research is necessary to address knowledge gaps limiting consistency of practice., Summary: As TC-ASD and congenital interventional cardiology mature as a field, studies of real-world practice provide increasingly valuable information about aspects of care in which there are disagreements about best practices and in which further research is necessary.

Increasing habitual exercise has been associated with improved cardiopulmonary exercise testing (CPET) performance, specifically maximal oxygen consumption in children with operatively corrected congenital heart disease. This has not been studied in children following Fontan palliation, a population in whom CPET performance is dramatically diminished. A single-center cross-sectional study with prospective and retrospective data collection was performed that assessed habitual exercise preceding a clinically indicated CPET in children and adolescents with Fontan palliation, transposition of the great arteries following arterial switch operation (TGA), and normal cardiac anatomy without prior operation. Data from contemporaneous clinical reports and imaging studies were collected. The association between percent predicted VO 2max and habitual exercise duration adjusted for known covariates was tested. A total of 175 subjects (75 post-Fontan, 20 with TGA, and 80 with normal cardiac anatomy) were enrolled. VO 2max was lower in the Fontan group than patients with normal cardiac anatomy (p < 0.0001) or TGA (p < 0.0001). In Fontan subjects, both univariate and multivariate analysis failed to demonstrate a significant association between habitual exercise and VO 2max (p = 0.6), in sharp contrast to cardiac normal subjects. In multivariate analysis, increasing age was the only independent risk factor associated with decreasing VO 2max in the Fontan group (p = 0.003). Habitual exercise was not associated with VO 2max in subjects with a Fontan as compared to biventricular circulation. Further research is necessary to understand why their habitual exercise is ineffective and/or what aspects of the Fontan circulation disrupt this association.

The Fontan operation is a widely used palliative procedure in patients with single-ventricle anatomy that results in liver injury. As timely identification of liver fibrosis may result in management changes to Fontan patients, the aim of our study was to identify clinically meaningful semi quantitative/quantitative pathologic parameters for biopsy assessment. We performed a retrospective review of 74 liver needle biopsies from Fontan patients. Fibrosis was assessed using quantitative % collagen deposition by Sirius red image analysis, METAVIR, congestive hepatic fibrosis score, sinusoidal fibrosis score, and sinusoidal dilation score. Contemporaneous laboratory, hemodynamic, and ultrasound data were collected. Centrilobular and peri sinusoidal fibrosis was observed in all cases, with 39.2% high grade. Portal fibrosis was observed in 93.2%, with 36.2% high-grade (METAVIR F3-F4). Cirrhosis was observed in 5.4%. % Collagen deposition was increased over control tissue (P < .001) and correlated with time from Fontan (r = 0.3, P = .009) and prothrombin time (r = 0.25, P = .034). Mildly elevated prothrombin time/international normalized ratio was the only measure of liver function consistently associated with multiple high-grade fibrosis scores (METAVIR P = .046, sinusoidal fibrosis P = .018). Abnormal liver echotexture on ultrasound was associated with high-grade congestive hepatic fibrosis score (P = .03). Pathologic gradings and %CD correlated with each other (r = 0.48-0.8, P < .001). Hepatic fibrosis in Fontan patients in our study is universally present, appears to be time dependent, and correlates with few laboratory measurements of liver function. Careful assessment of needle liver biopsies lends a more meaningful measure of liver fibrosis in the Fontan patient than clinical and laboratory data, allowing for appropriate changes to patient management., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Treatment of pulmonary atresia with major aortopulmonary collaterals (PA MAPCAs) remains a challenge. Despite variations in surgical technique, contemporary strategies all include initial intervention in the first year of life. However, a subset of patients presents later in life, and contemporary outcomes of this group have not been reported previously. We performed a retrospective case series of consecutive cases of PA MAPCAs who were seen at our center between January 2001 and February 2016, who had not undergone surgery before the age of 1 year. We describe their presenting characteristics, operative and transcatheter interventions, and outcomes. A total of eight cases were identified from 76 children with PA MAPCAs treated over the study period. Median age at presentation was 5.9 years. Seventy-five percent had confluent pulmonary arteries with a median Nakata index of 113 mm(2)/m(2). Operative intervention was performed in 5/6 cases. Two are awaiting intervention. The combination of operative and transcatheter interventions allowed for ventricular septal defect closure in 60 % of cases, all of whom had subsystemic right ventricular pressures. Operative intervention is possible in some older cases with PA and MAPCAs. Though multiple operations and transcatheter therapies are necessary, some can achieve operative correction of serial circulation with tolerable physiology. Subjects with ventricular hypoplasia and those without confluent pulmonary arteries are more challenging.

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.

Outcomes for transcatheter pulmonary valve replacement (TC-PVR) and operative pulmonary valve replacement (S-PVR) are excellent. Thus, their respective cost is a relevant clinical outcome. We performed a retrospective cohort study of children and adults who underwent PVR at age ≥ 8 years from January 1, 2011, to December 31, 2013, at 35 centers contributing data to the Pediatric Health Information Systems database to address this question. A propensity score-adjusted multivariable analysis was performed to adjust for known confounders. Secondary analyses of department-level charges, risk of re-admission, and associated costs were performed. A total of 2,108 PVR procedures were performed in 2,096 subjects (14% transcatheter and 86% operative). The observed cost of S-PVR and TC-PVR was not significantly different (2013US $50,030 vs 2013US $51,297; p = 0.85). In multivariate analysis, total costs of S-PVR and TC-PVR were not significantly different (p = 0.52). Length of stay was shorter after TC-PVR (p <0.0001). Clinical and supply charges were greater for TC-PVR (p <0.0001), whereas laboratory, pharmacy, and other charges (all p <0.0001) were greater for S-PVR. Risks of both 7- and 30-day readmission were not significantly different. In conclusion, short-term costs of TC-PVR and S-PVR are not significantly different after adjustment., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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., Objectives: The purpose of this study was to identify risk factors for catastrophic adverse outcomes in children with PH undergoing cardiac catheterization., Methods: We studied children and young adults up to 21 years of age with PH undergoing 1 or more cardiac catheterization at centers participating in the Pediatric Health Information Systems database between 2007 and 2012. Using mixed-effects multivariable regression, we assessed the association between pre-specified subject- and procedure-level covariates and the risk of the composite outcome of death or initiation of mechanical circulatory support within 1 day of cardiac catheterization after adjustment for patient- and procedure-level factors., Results: A total of 6,339 procedures performed on 4,401 patients with a diagnosis of PH from 38 of 43 centers contributing data to the Pediatric Health Information Systems database were included. The observed risk of composite outcome was 3.5%. In multivariate modeling, the adjusted risk of the composite outcome was 3.3%. Younger age at catheterization, cardiac operation in the same admission as the catheterization, pre-procedural systemic vasodilator infusion, and hemodialysis were independently associated with an increased risk of adverse outcomes. Pre-procedural use of pulmonary vasodilators was associated with reduced risk of composite outcome., Conclusions: The risk of cardiac catheterization in children and young adults with PH is high relative to previously reported risk in other pediatric populations. The risk is influenced by patient-level factors. Further research is necessary to determine whether knowledge of these factors can be translated into practices that improve outcomes for children with PH., (Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Clinical outcomes for transcatheter and operative closures of atrial septal defects (ASDs) are similar. Economic cost for each method has not been well described., Methods: A single-center retrospective cohort study of children and adults<30 years of age undergoing closure for single secundum ASD from January 1, 2007, to April 1, 2012, was performed to measure differences in inflation-adjusted cost of operative and transcatheter closures of ASD. A propensity score weight-adjusted multivariate regression model was used in an intention-to-treat analysis. Costs for reintervention and crossover admissions were included in primary analysis., Results: A total of 244 subjects were included in the study (64% transcatheter and 36% operative), of which 2% (n = 5) were ≥18 years. Crossover rate from transcatheter to operative group was 3%. Risk of reintervention (P = .66) and 30-day mortality (P = .37) were not significantly different. In a multivariate model, adjusted cost of operative closure was 2012 US $60,992 versus 2012 US $55,841 for transcatheter closure (P < .001). Components of total cost favoring transcatheter closure were length of stay, medications, and follow-up radiologic and laboratory testing, overcoming higher costs of procedure and echocardiography. Professional costs did not differ. The rate of 30-day readmission was greater in the operative cohort, further increasing the cost advantage of transcatheter closure. Sensitivity analyses demonstrated that costs of follow-up visits influenced relative cost but that device closure remained favorable over a broad range of crossover and reintervention rates., Conclusion: For single secundum ASD, cost comparison analysis favors transcatheter closure over the short term. The cost of follow-up regimens influences the cost advantage of transcatheter closure., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: The effect of genotype on the outcomes of infant cardiac operations has not been well established. The purpose of the present study was to investigate the effect of 22q11.2 deletion (22q11del) on infants with truncus arteriosus communis (TA) and interrupted aortic arch (IAA) undergoing operative correction during infancy., Methods: We conducted a retrospective cohort study of all infants who had undergone operative correction of TA or IAA at the Children's Hospital of Philadelphia from 1995 to 2007, comparing the perioperative outcomes (hospital length of stay, intensive care, mechanical ventilation, risk of cardiac and noncardiac events, number of consultations, and number of discharge medications) by 22q11del status., Results: A total of 104 patients were studied (55 with TA and 49 with IAA), of whom 40 (38%) were 22q11del positive. The 22q11del status was unknown in 9 (7 with TA and 2 with IAA). In patients with known deletion status, those with 22q11del had a longer hospital and intensive care length of stay. Subjects with 22q11del also required more frequent operative reintervention and more consultations and were prescribed more medications at discharge. No significant difference was found in method of feeding between those with and without 22q11del at discharge., Conclusions: In this study, 22q11del is associated with perioperative outcomes in infants undergoing operative correction of TA and IAA, with longer hospital stays and greater resource utilization in the perioperative period. These findings should inform counseling and risk stratification and warrant additional study to identify genotype-specific management strategies to improve outcomes., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Deficient retro-aortic rim is of concern as a risk factor for aortic erosion after device closure of atrial septal defects (ASD). However, its prevalence and contribution to technical failure and adverse outcomes have not been delineated. A single-center retrospective cohort study of children and adults undergoing cardiac catheterization for device occlusion of ASD from 1 January 1999 to 1 April 2012 was performed. Risk factors for technical failure and early adverse outcome were assessed using multivariate logistic regression. During the study period, 445 consecutive subjects with a median age of 5.9 years (range, 0.8-80 years) underwent catheterization. Of the subjects with reviewable echocardiograms, 60 % had deficient retro-aortic rim. No attempt at device closure was made for 3.6 % of the subjects. Of the remaining 429 subjects, 96 % underwent successful device occlusion. Major early adverse events occurred in 1.2 % (95 % confidence interval 0.4-2.7 %) of the cases, all of them either device embolization or malposition. Deficient retro-aortic rim was not a risk factor for composite outcome of technical failure or early major adverse event. No deaths, late reinterventions, or erosion events occurred during 2,395 total person-years (median, 5.8 years) of follow-up evaluation. Deficient retro-aortic rim was associated with increased risk of device impingement on the aorta, but no association was seen between device impingement or deficient retro-aortic rim and the development of new/progressive aortic insufficiency. Deficient retro-aortic rim is highly prevalent but did not increase the risk of adverse outcomes. Its contribution to the risk of aortic erosion could not be addressed by this study.

Previous studies of outcome after operative correction of interrupted aortic arch (IAA) have focused on mortality and rates of reintervention. We sought to investigate the clinical status of children and adolescents after surgery for IAA. A cross-sectional study of subjects with IAA between the ages of 8 and 18 years was performed with the subjects undergoing simultaneous genetic testing, electrocardiogram, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and assessment of health status and health-related quality of life as well as concurrent retrospective cohort study reviewing their postoperative use of medical care, including operative and transcatheter reinterventions, noncardiac surgeries, and hospitalizations. Twenty-one subjects with IAA with median age of 9 years were studied. Reintervention rates were 38% for left-ventricular outflow tract, 33% for AA, and 24% for both. Rates of reintervention were highest in the first year of life and decreased in subsequent years. Left-ventricular ejection fraction was preserved (72 ± 6%). Maximal oxygen consumption, maximal work, and forced vital capacity were both significantly decreased from age and sex norms (p < 0.0001). Health status and quality of life were both severely decreased. Subjects with IAA demonstrate a significant burden of operative and transcatheter intervention and large magnitude deficits in exercise performance, health status, and health-related quality of life.

Background: Studies of outcome after operative correction of truncus arteriosus communis (TA) have focused on mortality and rates of reintervention. We sought to investigate the clinical status of children and adolescents with surgically corrected TA., Methods and Results: A cross-sectional study of subjects with TA was performed. Subjects underwent concurrent genetic testing, electrocardiogram, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and completed questionnaires assessing health status and health-related quality of life. Review of their medical history provided retrospective information on cardiac reintervention and use of medical care. Twenty-five subjects with a median age of 11.8 (8.1-18.99) years were enrolled. The prevalence of 22q11.2 deletion was 32%. Incidence of hospitalization, cardiac reintervention, and noncardiac operations was highest in the first year of life. Combined catheter-based and operative reintervention rates were 52% on the conduit and 56% on the pulmonary arteries. Right ventricular ejection fraction and end-diastolic volume were normal. Moderate or greater truncal valve insufficiency was seen in 11% of subjects, and truncal valve replacement occurred in 8% of subjects. Maximal oxygen consumption (P = .0002), maximal work (P < .0001), and forced vital capacity (P < .0001) were all lower than normal for age and sex. Physical health status and health-related quality of life were both severely diminished., Conclusion: Patients with TA demonstrate significant comorbid disease throughout childhood, significant burden of operative and catheter-based reintervention, and deficits in exercise performance, functional status, and health-related quality of life., (© 2013.)

Cardiopulmonary exercise testing (CPET), particularly maximal oxygen consumption (VO2max), has been used to assess the outcome for patients with palliated congenital heart disease (CHD). Small studies correlating VO2max with noninvasive imaging measures of ventricular function have led to the hypothesis that VO2max reflects cardiac performance. In other settings, physical training is associated with increased VO2max. The authors hypothesized that habitual exercise correlates with VO2max to a greater degree than ventricular function in a relatively healthy cohort of patients with palliated CHD. The habitual exercise behavior of 208 subjects with conotruncal abnormalities (tetralogy of Fallot, truncus arteriosus, and interrupted aortic arch) at the time of previously performed, study-based CPET and cardiac magnetic resonance imaging (CMR) were retrospectively assessed via questionnaire. The association of VO2max with habitual exercise duration and CMR measures of ventricular function was tested. Of 208 subjects, 89 (43 %) completed questionnaires, and 78 % of the 89 patients had a concurrent CMR. The mean VO2max was 76 ± 21 % of that predicted. The CMR-assessed left ventricular ejection fraction was 67.6 ± 7.4 %. The hours of habitual exercise per week correlated with VO2max (p < 0.001; r (2) = 0.14). No association was found between right and left ventricular ejection fraction, cardiac index, or right ventricular end-diastolic volume and VO2max. In this study, VO2max correlated with habitual exercise to a greater degree than CMR measurements of ventricular function. These findings highlight the importance of considering the contribution of noncardiac factors when exercise data in both clinical and research settings are interpreted. The contribution of these factors to clinical outcomes deserves further study.