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Background/objective Stenting the ductus arteriosus (DAS) has become an alternative to surgical systemic to pulmonary artery shunts in neonates with ductal-dependent pulmonary blood flow (PBF). Femoral approach for a vertical ductus can be difficult secondary to the acute angle and tortuous course, thus alternative access sites have been explored. Carotid access complications have been reported in 5%-10%. The extensive use of an axillary arterial approach in the United States has not been reported. The aim of this study is to describe our experience with DAS using the axillary approach. Methods We reviewed all patients with DAS with an axillary approach in neonates with ductal-dependent PBF (May 2017-May 2018) in our institution. Procedural reports, angiograms, and clinical records of all consecutive patients were reviewed. Procedural technique, procedural outcomes, adverse events, and post-hospital courses are reported. Results Seven consecutive patients who received DAS utilizing axillary approach. All patients had ductal-dependent PBF through a vertical, tortuous ductus. Five had pulmonary atresia or near atresia, one had compromised PBF due to dynamic subvalvar obstruction, and one had Tetralogy of Fallot with isolated left pulmonary artery. Axillary access with 3.3 or 4 French sheath was obtained using ultrasound guidance. Bare metal coronary stents were deployed successfully in all. Intra-procedure, one developed in stent thrombus requiring re-stenting. There were no procedural mortalities or major adverse events from axillary access. There is a steep learning curve. Hemostasis was achieved with manual compression. Two patients had reintervention at 6-8 weeks. All patients underwent successful planned surgeries. Conclusions This series suggests DAS in neonates utilizing an axillary approach is a feasible and effective alternative for establishing PBF. Axillary arterial approach may be preferred as there is no risk to neurological sequelae and very low risk of limb complications. Larger series are needed to validate this approach.

Introduction Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics. Methods Between August 2014 and July 2017, 49 Fontan patients underwent TJLB. All the patients had hemodynamic evaluation, 28 patients had MRE (magnetic resonance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modified Ishak congestive hepatic fibrosis score. Results Median age 17.8 years, median time since Fontan 15.2 years. Primary diagnosis and Fontan type were variables, but predominantly LV morphology (30/49), lateral tunnel Fontan (29/49), originally fenestrated (37/49), and 11/49 had a pacemaker. Histologic fibrosis correlated with MRE (R = 0.62, P ≤ .001). Histologic fibrosis and MRE correlated with Fontan pressure (R = 0.38, P = .008 & R = 0.59, P ≤ .001). Morphology of the single ventricle did not correlate with liver fibrosis. The presence of a fenestration resulted in a higher cardiac index (P = .026) but did not resulted in lower liver fibrosis (P = .64). Conclusion Noninvasive tests, such as MRE, may be suitable for longitudinal follow-up in patients with single ventricle physiology. Our data suggest that there is reasonable correlation of MRE liver stiffness with biopsy scoring systems and Fontan pressures. We demonstrated the feasibility of TJLB in the setting of Fontan palliation and demonstrated its correlation with noninvasive measures particularly MRE. We recommend selective use of TJLB when MRE score is >5 KPa or when there are other clinical signs of cirrhosis.

Background In the modern era, results of the arterial switch operation (ASO) for transposition of the great arteries are excellent. However, because of the LeCompte maneuver, there may be a propensity for development of pulmonary artery stenosis. We encountered atypical complications of pulmonary artery stenting in patients after the ASO, including aorto-pulmonary fistula and coronary compression. Methods We performed a 10-year retrospective review of catheterizations performed in patients after ASO in our institution with a focus on adverse events. Results Diagnostic and interventional catheterizations were performed in 47 patients. In 29 patients, 37 interventional procedures performed, which included pulmonary artery angioplasty and/or stenting. In this group, there were five major adverse events (14%), including three aorto-pulmonary fistulae and one coronary artery compression among patients having stent implantation or stent redilation. In addition, there were 6/37 (16%) intended stent procedures, which were aborted because there appeared to be high-risk of significant adverse events. Conclusions This review suggests that percutaneous intervention on pulmonary artery stenosis after ASO has high-risk and should be undertaken advisedly. Prior thorough evaluation of coronary arteries is mandatory as coronary reimplantation sites may be adjacent to sites of pulmonary artery stenosis. Furthermore, if pulmonary artery stent implantation or stent redilation is contemplated, the risk of stent fracture and possible AP fistula should be recognized. Primary use of reinforced covered stents should be considered.

Background Right ventricular outflow tract (RVOT) stents have been used as palliation in patients with severe tetralogy of Fallot (TOF). Radiofrequency perforation of the RVOT has also been described in patients with pulmonary atresia (PA)/ventricular septal defect (VSD). However, RVOT stenting in conjunction with radiofrequency perforation as a means for establishing reliable pulmonary blood flow in patients with PA/VSD has not previously been reported. Objectives Our aim is to report our experience with using perforation of plate-like pulmonary valve atresia combined with stenting of RVOT as an alternative and equally efficacious intervention for infants with PA/VSD, as compared to a surgical pulmonary artery shunt (SPS). Methods A retrospective review of patients with PA/VSD at our institution from Jan 2006 to October 2015 was performed. Results Twenty-seven patients received palliation for PA/VSD. Five (median 2.5 kg, 2.1-4.1 kg) underwent RVOT stenting and 22 (3.5 kg, 2.1-4.6 kg) underwent surgical SPS. Of the patients who received an RVOT stent, 3 underwent guidewire perforation and balloon dilation of the plate-like pulmonary valve, and 2 underwent radiofrequency perforation of the pulmonary plate, followed by balloon dilation. Postprocedure saturations averaged 93% in the stent group and 89% in the shunt group. One of the patients who underwent RVOT stenting required additional stenting of the PDA for isolation of the LPA, but none required re-intervention. Four patients in the SPS cohort required re-intervention (18%). Two required stenting of the shunt, and 2 required surgical revision of the shunt. There was one death in the stent group, however this was secondary to withdrawal of support given an underlying genetic diagnosis (trisomy 13). There was one fatality in the SPS cohort prior to hospital discharge. Conclusions Perforation followed by RVOT stenting may be a safe and effective alternative to surgical SPS in infants with PA/VSD.

Objective To evaluate whether preprocedural transthoracic echocardiography (TTE) can be used to predict Amplatzer septal occluder (ASO) size for device closure of atrial septal defect (ASD). Design Retrospective review of patients who underwent ASD device closure at our institution between August 2006 and August 2013 was performed. Patients with complex congenital heart disease, devices other than the ASO, multiple devices, or inadequate TTE images were excluded. Those who had transesophageal echocardiography (TEE)-guided device placement were evaluated. A blinded observer reviewed their preprocedural TTE images and applied a scaled formula to predict device size. Results A total of 186 patients underwent ASO placement during the study period, 87 had TEE guidance, of which 45 met inclusion criteria. The mean predicted device size by the scaled formula was 18.0 ± 5.11 mm, compared to the mean implanted device size of 18.8 ± 5.22 mm. The mean absolute difference between each predicted and final deployed device size was 1.44 mm with 95% CI [1.08, 1.81]. The Pearson correlation showed that the predicted device size had a positive correlation coefficient of 0.94. Conclusion Preprocedural TTE assessment of ASD size using a scaling formula in patients with adequate TTE windows can accurately predict ASO device size and aid in device selection.

Objective Pulmonary insufficiency following balloon valvuloplasty for pulmonary valve stenosis has been shown to result in right ventricular dilation, which may necessitate pulmonary valve replacement. This study investigates the consequences of employing a more conservative balloon:annulus ratio of ≤1.2 against the currently used ratios of >1.2. The aim of the study was to evaluate whether a more conservative balloon:annulus ratio would maintain procedural success with adequate relief of the pulmonary valve gradient, while minimizing pulmonic insufficiency. Design Procedural data were collected on cases considered for balloon valvuloplasty for congenital pulmonic stenosis at Rady Children's Hospital, San Diego between March 31, 2003 and April 9, 2014. The study includes 98 patients whose median age at the time of procedure was 3.6 months (range: 1 day to 271 months); median follow-up was 15.2 months (range 24 days to 106 months). The subjects were divided into two groups based on their balloon:annulus ratio: ≤1.2 (n = 67) and >1.2 (n = 31). Results The difference in pulmonary gradient reduction—the marker of procedural success—was not significant between the two groups (P = .33). Although both groups had an increase in pulmonary valve insufficiency from baseline, the ≤1.2 group had significantly less insufficiency compared to the >1.2 group (P = .008). Reintervention rates were not statistically significant between both groups (P = .89). Conclusion Performing pulmonary balloon valvuloplasty with a balloon:annulus ratio of ≤1.2 produces significantly less pulmonary insufficiency than a ratio of >1.2 without reducing procedure efficacy.

Objective The objective of this study was to investigate variation in practice surrounding elective cardiac catheterization in patients with single-ventricle (SV) congenital heart disease. Design Patient and procedural characteristics and outcomes during SV catheterization were collected prospectively from eight centers using a web-based registry (Congenital Cardiac Catheterization Project on Outcomes). We attempted to identify a population of elective procedures by limiting the cohort in case type and timing. Cases were then stratified by stage of SV palliation (pre-bidirectional cavopulmonary anastomosis [pre-BCPA], pre-Fontan and post-Fontan) and limited by age. Subcohort analysis was performed by mode of airway management (assisted vs. spontaneous ventilation). Institutional variation was assessed. Results Between 2/2007 and 6/2010, 1459 (10.1%) of 14 467 cases in the registry met the inclusion and exclusion criteria, including 326 pre-BCPA, 571 pre-Fontan and 562 post-Fontan procedures. Median patient age was 0.4 (interquartile range 0.3, 0.5), 2.6 (1.0, 3.4) and 9.6 (5.2, 15.4) years and weight was 5.6 (4.8, 6.4), 12.2 (10.5, 14), and 26.3 (16.6, 51.8) kg in the pre-BCPA, pre-Fontan and post-Fontan cohorts, respectively. Cases were more commonly diagnostic in the pre-BCPA cohort (57%) whereas they were more commonly interventional in the pre-Fontan (69%) and post-Fontan (77%) cohorts. At least one adverse event (AE) occurred in 210 cases (14.4%) overall, including 20% of pre-BCPA, 11% of pre-Fontan and 14% of post-Fontan catheterizations. Mode of airway management was associated with statistically significant, but clinically small differences in hemodynamic measures in the pre- and post-Fontan cohorts, but not in the pre-BCPA group. Considerable practice variation exists across centers with variability in airway management, AE rate, case type, interventions performed and fluoroscopy time, in all SV cohorts. Conclusions Elective catheterization in SV patients, frequently performed with intervention, is common among US congenital cardiac centers. However, important variation in practice exists during these procedures in SV patients, at all stages of palliation.

Objective Intravascular stent placement in patients with congenital heart disease has replaced several surgical procedures because the desired outcome is achieved with a lower risk of complications. However, the blood vessels of children undergo a two- to threefold diameter increase due to somatic growth creating a dilemma: Can stents implanted in infants and children be serially dilated to significantly larger diameters without losing integrity and important mechanical characteristics? Our aim was to assess the expansion potential and to characterize mechanical parameters of balloon expandable stents at the maximum expansion diameter. Design Sixteen commonly used stent designs were evaluated. Physical attributes (sheath size and guide wire size) and the following mechanical parameters were determined, compared, and contrasted: fracture diameter, maximum expansion diameter, stent integrity, outer diameter, diameter uniformity, diameter recoil, length change, radial stiffness, and radial strength. Results Stents were dilated to an average of 1.7 times (range from 1.3 to 2.4 times) the nominal expansion diameter without losing mechanical integrity. Stents at the maximum expansion diameter maintained diameter uniformity (with less than 3.2% difference) and demonstrated minimal diameter recoil once the balloon catheter was deflated (less than 3.6% recoil). At the maximum expansion diameter, stent length varied depending on stent type. When dilated to the maximum expansion diameter, radial stiffness and radial strength of all stents increased compared with the stents’ nominal expansion diameter. Conclusions Balloon expandable stents maintain excellent mechanical characteristics when dilated beyond their manufacturers’ recommended nominal expansion diameter. These results support the use of balloon expandable stents in smaller vessels that require future serial redilation.

The patient was a male infant with L-transposition of great arteries (L-TGA), Ebstein's anomaly of the tricuspid valve, subvalvar aortic stenosis, ventricular septal defect (VSD), hypoplastic right ventricle, arch hypoplasia, and congenital complete heart block. He underwent a Norwood procedure, aortic arch repair, permanent pacemaker implantation, and a 3.5-mm aortopulmonary shunt at 4 days of age. At the time of his surgery, left ventricular function was in the normal range (ejection fraction [EF] = 67%). However by 3 months of age, he was noted to have developed moderate-severe biventricular dysfunction (left ventricular ejection fraction [LVEF] = 34%). Atresia of the coronary sinus with a small left superior venacava (LSVC) and a bridging vein was discovered during cardiac catheterization at this time. The coronary sinus mean pressure was 17 mm Hg, and the common atrial mean pressure was 6 mmHg. We opened the atretic coronary sinus ostium using radiofrequency ablation and stent placement. There was dramatic improvement in ventricular function observed over a 2-month period. Follow-up cardiac catheterization 5 months later revealed the stent in the coronary sinus to be widely patent with no intimal buildup, and the ventricular function was normal (LVEF = 58%). The patient had a bidirectional Glenn procedure with an uncomplicated postoperative course and is currently awaiting Fontan completion.

Objective Neonatal coronary thrombosis is a rarely reported disorder, with variable outcomes described. This study assessed the feasibility and safety of an institutional protocol using tissue plasminogen activator (tPA) administration for the treatment of neonatal coronary artery thrombi. Methods They reviewed the outcome of three neonates with clinical evidence of myocardial infarction secondary to coronary thrombosis. All three underwent the tPA treatment protocol. Results The three described cases presented at 5 hours, 15 hours, and 10 days of life. The patients identified underwent the tPA protocol at least once. There was clinical evidence of improvement in coronary flow, as well as demonstration of increased left ventricular function and decreased mitral regurgitation. No major adverse events occurred. Conclusion Thrombolytic therapy with this tPA protocol may be safe and effective in treating neonates with coronary thrombosis.

To evaluate whether preprocedural transthoracic echocardiography (TTE) can be used to predict Amplatzer septal occluder (ASO) size for device closure of atrial septal defect (ASD).Retrospective review of patients who underwent ASD device closure at our institution between August 2006 and August 2013 was performed. Patients with complex congenital heart disease, devices other than the ASO, multiple devices, or inadequate TTE images were excluded. Those who had transesophageal echocardiography (TEE)-guided device placement were evaluated. A blinded observer reviewed their preprocedural TTE images and applied a scaled formula to predict device size.A total of 186 patients underwent ASO placement during the study period, 87 had TEE guidance, of which 45 met inclusion criteria. The mean predicted device size by the scaled formula was 18.0 ± 5.11 mm, compared to the mean implanted device size of 18.8 ± 5.22 mm. The mean absolute difference between each predicted and final deployed device size was 1.44 mm with 95% CI [1.08, 1.81]. The Pearson correlation showed that the predicted device size had a positive correlation coefficient of 0.94.Preprocedural TTE assessment of ASD size using a scaling formula in patients with adequate TTE windows can accurately predict ASO device size and aid in device selection.

Objective. The purpose of this study was to establish an objective criterion to help assess adequacy of the transverse arch in newborns with coarctation and thereby optimize preoperative surgical decision making. Methods. Echocardiograms of 47 patients

Objective. Classically pericardiocentesis has been described and performed through the subxiphiod approach, which is feasible if the pericardial fluid is circumferential or collected inferior or anterior to the heart. However, not uncommonly, the collection of fluid is at the base of the heart or in the posterior or apical portion of the pericardium, necessitating a different approach. The purpose of this study is to describe echo and fluoroscopic guided pericardiocentesis to evacuate noncircumferential effusions, which are not accessible from the standard subcostal approach. Methods. Retrospective review of all patients with localized/noncircumferential effusions at Rady Children's Hospital San Diego between May 2007 and February 2010 was performed. During these procedures, effusions were identified at the point closest to the skin. The pericardial drains were introduced, directed, and advanced under echocardiographic and fluoroscopic guidance while avoiding major organs. Results. Ten patients were identified (age ranged from 20 days to 22 years, weight ranged from 1.6 kg to 94 kg). All procedures were successful in draining the pericardial effusions with minimal residual fluid and no complications. Conclusion. Echocardiographic and fluoroscopic guided pericardiocentesis is a feasible, relatively safe, and reliable technique to drain loculated pericardial effusions using nonstandard entry sites.

Scimitar syndrome is a rare variant of partial anomalous pulmonary venous return. The syndrome consists of a right-sided abnormal lung segment that is directly supplied by arteries from the aorta and has an anomalous venous drainage to the inferior vena cava. Three cases of left-sided scimitar syndrome have been reported in the literature, but it has not yet been associated with venous drainage to the azygos vein. Herein we report a left-sided scimitar syndrome with anomalous venous drainage to the azygos vein.

The patient was a male infant with L-transposition of great arteries (L-TGA), Ebstein's anomaly of the tricuspid valve, subvalvar aortic stenosis, ventricular septal defect (VSD), hypoplastic right ventricle, arch hypoplasia, and congenital complete heart block. He underwent a Norwood procedure, aortic arch repair, permanent pacemaker implantation, and a 3.5-mm aortopulmonary shunt at 4 days of age. At the time of his surgery, left ventricular function was in the normal range (ejection fraction [EF] = 67%). However by 3 months of age, he was noted to have developed moderate-severe biventricular dysfunction (left ventricular ejection fraction [LVEF] = 34%). Atresia of the coronary sinus with a small left superior venacava (LSVC) and a bridging vein was discovered during cardiac catheterization at this time. The coronary sinus mean pressure was 17 mm Hg, and the common atrial mean pressure was 6 mmHg. We opened the atretic coronary sinus ostium using radiofrequency ablation and stent placement. There was dramatic improvement in ventricular function observed over a 2-month period. Follow-up cardiac catheterization 5 months later revealed the stent in the coronary sinus to be widely patent with no intimal buildup, and the ventricular function was normal (LVEF = 58%). The patient had a bidirectional Glenn procedure with an uncomplicated postoperative course and is currently awaiting Fontan completion.

The purpose of this study was to establish an objective criterion to help assess adequacy of the transverse arch in newborns with coarctation and thereby optimize preoperative surgical decision making.Echocardiograms of 47 patients6 months of age who underwent coarctation repair from September 2005 to November 2008 and 47 age-matched healthy infants were reviewed. The proximal and distal transverse aortic arch to descending aorta ratios (TAA:DAO ratio) were calculated from the end-systolic proximal and distal transverse aortic arch diameters and diameter of the descending aorta at the diaphragm.Both the proximal and distal TAA:DAO ratios were significantly lower in the study vs. control group (P = .001) and in patients who underwent patch reconstruction of the aorta vs. extended end-to-end anastomosis (P = .014; P = .015). All patients who underwent patch reconstruction had a proximal and/or distal TAA:DAO0.65. A cutoff of 0.65 was derived based on our analyses and an algorithm was developed to guide decision making. Forty-six out of 47 patients were free from reintervention.The TAA:DAO ratio appears to be an accurate parameter by which the adequacy of the transverse arch can be assessed preoperatively using the proposed algorithm, and thus help determine the type of surgical intervention and approach.

Classically pericardiocentesis has been described and performed through the subxiphiod approach, which is feasible if the pericardial fluid is circumferential or collected inferior or anterior to the heart. However, not uncommonly, the collection of fluid is at the base of the heart or in the posterior or apical portion of the pericardium, necessitating a different approach. The purpose of this study is to describe echo and fluoroscopic guided pericardiocentesis to evacuate noncircumferential effusions, which are not accessible from the standard subcostal approach.Retrospective review of all patients with localized/noncircumferential effusions at Rady Children's Hospital San Diego between May 2007 and February 2010 was performed. During these procedures, effusions were identified at the point closest to the skin. The pericardial drains were introduced, directed, and advanced under echocardiographic and fluoroscopic guidance while avoiding major organs.Ten patients were identified (age ranged from 20 days to 22 years, weight ranged from 1.6 kg to 94 kg). All procedures were successful in draining the pericardial effusions with minimal residual fluid and no complications.Echocardiographic and fluoroscopic guided pericardiocentesis is a feasible, relatively safe, and reliable technique to drain loculated pericardial effusions using nonstandard entry sites.