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BACKGROUND Carotid artery (CA) and axillary artery (AA) access are increasingly used for transcatheter stenting of the patent ductus arteriosus (PDA), although reports are limited. METHODS The Congenital Catheterization Research Collaborative (CCRC) reviewed multicenter data from infants who underwent PDA stenting via the CA or AA approach from 2008 to 2017, and compared outcomes to those of infants undergoing PDA stenting via the femoral artery (FA) approach. Post-procedure ultrasound (US) imaging was reviewed. RESULTS Forty-nine infants underwent PDA stenting from the CA (n = 43) or AA (n = 6) approach, compared with 55 infants who underwent PDA stenting from the FA approach. The PDA was the sole pulmonary blood flow (PBF) source in 61% of infants in the CA/AA cohort, compared with 33% of the FA cohort (p

We present the case of a child with congenital heart disease repaired in infancy with diffuse central venous occlusions resulting in central venous insufficiency, superior vena cava (SVC) syndrome, and intracranial bleeds. He presented to the catheterization laboratory for multiple transcatheter interventions to recanalize central venous channels which were unsuccessful by conventional methods. Therefore, extravascular venous channels were created to decompress his upper body by creation of a neo-SVC and his lower body with a neo-azygos vein to the neo-SVC. The latter procedure required direct percutaneous access from a paraspinal approach in order to obtain continuity with the azygos vein. At latest follow-up the patient is clinically well and asymptomatic.

We describe a patient with chronic thromboembolic pulmonary hypertension who presented to the pediatric cardiac catheterization laboratory for peripheral pulmonary artery recanalization and angioplasty. This case report outlines serial pulmonary arterial rehabilitation via a retrograde approach through intrapulmonary collateral arterial connections.

OBJECTIVE Define outcomes of premounted stent implantation (PMS) for branch pulmonary artery stenosis (BPAS). BACKGROUND PMS for BPAS in children raises concern of long term viability, with limited maximal expansion. METHODS We reviewed our cardiac database over an 11-year period ending in 2013. Primary endpoint was need for surgical stent intervention (SSI). Other endpoints included acute results and repeat interventions (RI). RESULTS 82 PMS were implanted in 60 children for BPAS. Median weight was 6.3 (25th -75th 4.6-9.8) kg. Median stent diameter was 6 (range 4-9) mm. Acutely, vessel diameter improved from 2.0 (25th -75th 1.6-3.4) to 5.0 (25th -75th 4.2-5.9) mm (p < 0.001), pressure gradient decreased from 41 (25th -75th 29-50) to 11 (25th -75th 7-18) mmHg (n = 47, p < 0.001), RV:Ao pressure ratio decreased from 100% (25th -75th 85-110%) to 59% (25th -75th 49-74%) (n = 40, p < 0.001). Freedom from SSI was 81% at 1 year and 35% at 5 years. Freedom from RI was 50% at 1 year and 14% at 5 years. 86% of PMS underwent SSI during a concomitant planned cardiac surgery. 45% patients had stent redilation, improving stent diameter from 4.6 (25th -75th 4.1-5.4) to 5.7 (25th -75th 4.9-7) mm (p < 0.001). 1 stent (3%) was able to be fractured longitudinally. CONCLUSIONS PMS is an effective short term solution for BPAS in children. PMS is associated with expected early need for transcatheter reintervention to accommodate for growth, but also has high rates of SSI.

Background Unique and small anatomical features often preclude the use of available vascular stents in pediatric patients with congenital heart disease (CHD). Objectives To report our experience and outcomes tailoring stents to fit unique anatomy, particularly in small children and infants with CHD. Methods Stent tailoring techniques included trimming, folding, and flaring. Patients receiving a tailored stent November 2002 to February 2015 were included in a retrospective analysis. Results Forty-one tailored stents were implanted in 30 patients with median age and weight of 0.8 years (6 days to 17 years) and 8.1 kg (2.9–47.9 kg). Thirty stents were placed intraoperatively and 11 percutaneously. Sites included branch pulmonary arteries (BPA; n = 32), pulmonary veins (n = 6), SVC (n = 1), and the ventricular septum (n = 2). Twenty-three (56%) stents were trimmed with or without folding to avoid jailing of side branches, 16 (39%) stents were folded or flared with or without trimming to avoid excessive proximal protrusion, and two (5%) stents were folded back at both ends for implantation in ventricular septal defects. Final stent lengths were 6–15 mm. Minimal vessel diameters increased from 2.8 ± 1.4 mm to 6.7 ± 2.6 mm (P

Objectives This study sought to update benchmark values to use a quality measure prospectively. Background Congenital Cardiac Catheterization Outcomes Project – Quality Improvement (C3PO-QI), a multi-center registry, defined initial radiation dose benchmarks retrospectively across common interventional procedures. These data facilitated a dose metric endorsed by the American College of Cardiology in 2014. Methods Data was collected prospectively by 9 C3PO-QI institutions with complete case capture between 1/1/2014 and 6/30/2015. Radiation was measured in total air kerma (mGy), dose area product (DAP) (µGy*M2), DAP per body weight, and fluoroscopy time (min), and reported by age group as median, 75th and 95th%ile for the following six interventional procedures: (1) atrial septal defect closure; (2) aortic valvuloplasty; (3) treatment of coarctation of the aorta; (4) patent ductus arteriosus closure; (5) pulmonary valvuloplasty; and (6) transcatheter pulmonary valve implantation. Results The study was comprised of 1,680 unique cases meeting inclusion criteria. Radiation doses were lowest for pulmonary valvuloplasty (age 15 yrs, median mGy: 1835, DAP: 17990). DAP/kg standardized outcome measures across weights within an age group and procedure type significantly more than DAP alone. Radiation doses decreased for all procedures compared to those reported previously by both median and median weight-based percentile curves. These differences in radiation exposure were observed without changes in median fluoroscopy time. Conclusions This study updates previously established benchmarks to reflect QI efforts over time. These thresholds can be applied for quality measurement and comparison. © 2017 Wiley Periodicals, Inc.

Objectives The study evaluated the institutional experience with cardiac catheterization on extracorporeal membrane oxygenation (ECMO) support. Background There is scant literature on the outcomes of catheterization on ECMO. Methods A retrospective review was performed of all children who underwent catheterization on ECMO from 2003 to 2013. Patients were categorized as cardiomyopathy (CM) or congenital heart disease (CHD). Results During the study period, 215 children were placed on cardiac ECMO. Of these, 29.8% underwent 75 catheterization procedures while on ECMO support. The median age of the cohort was 1.5 months (range 0 days −16.7 years) and the median weight was 3.9 kg (2.2–63.1 kg). CM patients constituted 18.8% of the cohort and all of them underwent atrial septoplasty (an atrial septal stent in 7/12 and balloon atrial septoplasty or septostomy in 5). The survival to hospital discharge rate was 83% and the transplant-free survival rate was 58.3%. CHD patients constituted 81.2% of the cohort. In this group, transcatheter interventions were performed in 40.4% and subsequent surgical interventions in 40.4%. Survival to hospital discharge rate was 34.6% and transplant free survival rate was 32.7%. Overall, 76.7% underwent transcatheter or surgical interventions. The major catheterization complication rate was 6.7%. The mean ECMO-to-catheterization time was 1.6 days for survivors and 3.5 days for non-survivors (P = 0.034). Survival to discharge was better for the CM group compared to the CHD group (P = 0.01). Among CHD, survival was better with transcatheter interventions compared to no interventions or surgical interventions (P

Background Vascular thromboses are a significant cause of morbidity and mortality in children. Data in children regarding catheter-based rheolytic and aspiration thrombectomy systems are limited. We sought to review the safety and efficacy of catheter-based rheolytic and aspiration thrombectomy systems in children. Methods: Data of all children having undergone thrombectomy using specialized rheolytic or aspiration systems were reviewed. Results: Thrombectomy was performed in 50 vessels in 21 patients, median age 1.9 months (8 days–18 yrs), median weight 4.3 (1.1–67.9) kg. Thrombectomy was performed using AngioJet in 16, Helix Clot Buster in 5, Fetch catheter in 8, Pronto catheter in 1, and a combination of other systems in 20 vessels (with AngioJet in 16). Thrombectomy was successful in 47/50 (94%) vessels in 18/21 (86%) patients with additional balloon/stent therapy or tPA administration performed in 16/18 (89%) of these patients. There were 2 (9.5%) major complications (both with AngioJet) consisting of asystole when thrombectomy was performed using activation times of >5 sec. At a median follow-up of 10 months (2 weeks–7 years), all 47 successfully treated vessels are patent, with 8/18 (44%) patients requiring reintervention with angioplasty/stent placement or repeat thrombectomy. Conclusions: Catheter-based thrombectomy systems are an important adjunctive tool in the treatment of children with thrombotic vessel occlusions. Significant hemodynamic compromise seen when using AngioJet may be minimized by using activation times of ≤5 sec. © 2016 Wiley Periodicals, Inc.

Keywords: percutaneous coronary intervention; structural heart disease intervention; congential heart disease; adults; congenital heart disease; pediatrics

Objective The objective is to quantify radiation dose in children undergoing cardiac catheterization and determine the impact of increased reporting transparency on total radiation exposure. Background: Cardiac catheterization (cath) can result in significant radiation exposure in children. There has been growing interest in quantifying and reducing radiation exposure in pediatric cath procedures. Our center underwent a slight change in practice recently that resulted in direct physician reporting of radiation dose following every case. Methods We reviewed cath procedures across three different eras in four cath categories: post-heart transplant annual cath, unilateral pulmonary artery (PA) stent placement, pre-Fontan cath, and pre-Glenn cath. The eras were defined as: Era 1, 1/2009 – 1/2011; Era 2, 1/2011 – 9/2013; and Era 3, 9/2013 – 5/2014. In Era 3, the physician performing the cath was responsible for reporting the radiation data. Results Across the three eras, there were significant decreases in cumulative air KERMA (mGy) among all four cath categories. From Era 2 to Era 3, the greatest decreases in radiation were noted, particularly in dose area product (cGy·cm2) in the transplant annual evaluation and pre-Glenn cases. In Era 1, 2 cases (1.2%) had a frame rate reduction, while in Era 2, 22 cases (12.0%), and in Era 3, 83 cases (21.6%) had frame rate changes (P

Objectives The purpose of this study was to examine the outcomes of catheter dilation interventions in general upon surgical anastomotic sites in the immediate postoperative period. Background Early postoperative cardiac catheter intervention is regarded as high-risk, particularly when a dilation intervention across a fresh suture line is performed. Methods All catheter dilation interventions performed upon a fresh suture line within 30 days of congenital heart surgery between August 2005 and December 2013 were reviewed. Values are reported as median and interquartile range (IQR). Primary endpoint was procedural success, which was defined as an increase in vessel diameter of >75% of the adjacent normal vessel or 50% increase over pre-dilation diameter. Secondary endpoints included procedural complications and survival to hospital discharge. Results Forty-five patients, median age 0.15 years (0.03–0.51 years), weight 4.1 kg (3.1–6.4 kg), underwent 62 interventional procedures on median postoperative day 7 (3–13 days). Among the 62 dilation interventions at surgical anastomotic areas, were 30 stent and 32 balloon angioplasty procedures. There were two major complications including: arterial dissection during stent placement and a procedural mortality due to pulmonary artery avulsion during angioplasty. There were 12 deaths (27%) prior to hospital discharge. The median ratio of maximum stent diameter to stenosis diameter was 2.62 (2.27–3.73). The median ratio of maximum balloon diameter to stenosis diameter was 2.27 (1.84–2.94). Stent placement was more likely to result in procedural success (OR 2.1; 95% confidence interval 1.6–2.8). Conclusion Though caution is paramount, early postoperative catheter dilation intervention across fresh suture lines can be performed safely in small, critically ill children. © 2015 Wiley Periodicals, Inc.

We report a case of a child with congenital heart disease who presented with critical right ventricular outflow tract obstruction and a history of bilateral femoral vein occlusion. He underwent successful right ventricular outflow tract intervention, culminating in transcatheter pulmonary valve implantation, after recanalization of a completely obstructed femoral vein. © 2016 Wiley Periodicals, Inc.

Infants with a large patent arterial duct (PDA) may develop signs of congestive heart failure secondary to pulmonary overcirculation. As the PVR decreases, the relative pulmonary blood flow (Qp :Qs ) increases and this may cause tachypnea, respiratory distress, failure to thrive, ventricular dysfunction and even ventilator dependency. The diastolic runoff from the systemic circulation may result in intestinal hypoperfusion resulting in necrotizing enterocolitis, and in severe cases, myocardial ischemia. Cardiac ischemia due to coronary steal is a recognized clinical entity. We present two cases of infants who developed coronary artery steal with the use of supplemental oxygen therapy during anesthesia induction for PDA occlusion. These cases highlight the importance of prudent use of supplemental oxygen therapy in infants with large PDAs.

Objectives To analyze the outcomes and risk factors associated with endomyocardial biopsy (EMB) in children less than one year of age. Background EMB has proven to be an integral diagnostic tool to evaluate suspected myocarditis, identify tumor histology, and provide tissue-graft surveillance after cardiac transplantation. The morbidity and mortality of EMB has been well established in the adult literature and reviewed in the general pediatric population, but there remains limited data for children in the first year of life. Methods We retrospectively reviewed the cardiology database at our institution to identify patients less than one year of age who underwent EMB between 1984 and 2008. Cardiac catheterization reports were reviewed for patient demographics, biopsy indication, procedural details, and complications. Results A total of 99 EMBs were performed, 49 for evaluation of suspected myocarditis, 43 for transplant rejection surveillance, 3 to identify tumor histology, and 4 for suspected endocardial fibroelastosis. Forty procedures were performed in children age

Objectives We studied outcomes of neonatal aortic valvuloplasty to determine the risk factors for poor outcomes. Background Balloon aortic valvuloplasty (BAV) is the primary therapy for neonates with severe aortic stenosis (AS). Limited data are available on the mid-term and long-term outcomes in this population, and reported risk factors for poor outcomes vary among studies. Methods We reviewed all cases of BAV in neonates in our institution between 1998 and 2009. We reviewed patient characteristics, preintervention echocardiographic, and procedural details. We tracked repeat BAV, aortic valve replacement (AVR), and death/transplant. Kaplan–Meier and Cox regression analyses were performed. Changes in dimensions of left heart structures post-BAV were also studied. Results Forty-eight neonates were included—of these, 30 (62%) had critical AS. There was one procedural death. The remaining 47 neonates were followed for 4.8 ± 4.4 years. Repeat BAV was performed in 19 (40%) neonates; AVR was performed in 9 (19%), and death/transplant occurred in 4 (8%) neonates. Multivariate analysis revealed that lower LV shortening fraction pre-BAV, higher pre-BAV and final valve gradient, and need for inotropes were associated with poor long-term outcomes. LV diastolic and systolic dimensions normalized over time, and left heart structures improved in size early and late after BAV. Conclusions Neonatal BAV is associated with low mortality. Lower LV shortening fraction pre-BAV is associated with risk of future interventions, while repeat valvuloplasty is safe and effective for recurrent AS. There is significant improvement in dimensions of the LV and aortic valve annulus following BAV. © 2012 Wiley Periodicals, Inc.

Rarely is closure of the atrial septal defect indicated in infants. Yet, an atrial septal defect in the presence of congenital mitral stenosis may cause significant congestive heart failure in infancy. We present the case of an infant with Shone's syndrome, left ventricular hypoplasia, and a large ostium secundum atrial septal defect who developed pulmonary overcirculation following repair of coarctation of the aorta. The infant underwent cardiac catheterization, hemodynamic assessment, and successful percutaneous closure of the atrial septal defect. The patient improved dramatically following the intervention. Follow-up echocardiograms have demonstrated growth of mitral valve annulus diameter and left ventricular dimensions. Atrial septal defect closure in this small infant was associated with immediate improved clinical status, and improved growth of the left heart structures. © 2012 Wiley Periodicals, Inc.

Hypoxemia is a significant cause of early and interstage death in patients with single ventricle (SV). Obstruction of Blalock-Taussig shunts (BTS) in patients with SV has traditionally been managed with surgical revision. Purpose: We report on the experience at our institution of deploying endovascular stents within BTS as well as obstructed right-ventricle (RV) to pulmonary artery (PA) conduits in patients with modified Norwood (ie Sano modification). Methods: Medical records were reviewed for the time period between January 1, 2002 and November 30, 2005. All patients with SV who presented for intervention for BTS or RV-PA conduit stenosis were reviewed. Specific endpoints reviewed included pre- and post-intervention arterial oxygen saturation, type of intervention (stent vs. ballon dilation), need for subsequent surgical shunt/conduit revision, and interval to second stage palliation. Results: Fifteen patients with SV underwent intervention for acute cyanosis. Eight patients had BTS, and the other seven patients had RV-PA conduit stenosis. Coronary stents were deployed in 14 of the 15 patients. Four patients also underwent balloon angioplasty of branch PAs. Oxygen saturations improved in all patients, with a mean increase of 13.9% (p = 0.0001). Four patients died before second stage palliation — one due to complications of the catheterization. Of the eleven remaining patients, nine have undergone second stage palliation; interval from intervention to Glenn ranged from 28–205 days (mean 163d). Two patients are awaiting cavo-pulmonary anastamosis. Conclusions: Endovascular stenting in this high-risk population is effective at improving oxygen saturation as well as obviating need to surgical shunt/conduit revision. © 2006 Wiley-Liss, Inc.

Percutaneous implantation of valved stents is now routinely performed to treat pulmonary valve regurgitation and stenosis. In addition, there are isolated reports of implantation of valved stents in the tricuspid position to treat prosthetic tricuspid stenosis or regurgitation when a prosthetic valve ring exists. We present a case in which a patient with combined severe tricuspid valve and pulmonary valve disease was successfully treated with sequential implantation of percutaneous valved stents in a single procedure. The procedure was straightforward, of short duration, and dramatically improved the patient's functional status. © 2012 Wiley Periodicals, Inc.

Objective Balloon aortic valvuloplasty (BAV) is the primary therapy for congenital aortic stenosis (AS). Recurrent AS following initial BAV or initial surgical valvotomy (SV) may require a second BAV (BAV2). We sought to determine the longterm outcomes of BAV2. Design We reviewed all cases of BAV2, defined as BAV following primary BAV or SV between 1988 and 2009. Cases were reviewed for pre- and post-BAV2 echocardiographic and procedural details. Setting Tertiary care dedicated children's hospital. Patients Between 1985 and 2009, 43 patients underwent BAV2 (23 primary SV, 20 primary BAV) at median age 1.9 years (1 month–21 years) and median weight 15 (3.3–55) kg. Interventions BAV2 performed following primary SV or primary BAV. Main Outcome Measures We evaluated the following endpoints: ≥ moderate AI post-BAV2, aortic valve replacement (AVR), additional BAV or SV post-BAV2, death and heart transplantation. Results The gradient decreased from 61.4 ± 16.0 mm Hg to 26.0 ± 13.6 post-BAV2 (P mild, compared to 21 (51%) patients after BAV2. Cox regression revealed that primary BAV was associated with development of AI > mild after BAV2 (P < 0.01). Conclusion BAV2 is associated with decreased valve gradient, though with an increase in AI. However, residual AS, not AI, is associated with poor outcomes following BAV2. BAV2 effectively treats recurrent AS and postpones need for surgical intervention. © 2013 Wiley Periodicals, Inc.