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Background Patent ductus arteriosus (PDA) stenting is evolving as an alternative to surgical aorto-pulmonary shunts for infants with ductal-dependent pulmonary blood flow. Given anatomical proximity, the PDA can compress the ipsilateral bronchus. We report a case series of four patients with bronchial compression by a tortuous PDA who underwent PDA stenting. Methods Our four patients received PDA stents for ductal-dependent pulmonary blood flow despite preprocedure imaging evidence of bronchial compression. We reviewed the cross-sectional chest imaging to assess the degree of bronchial compression and the variables that affect it, namely PDA size, PDA tortuosity, and the anatomical relationship between the compressed bronchus and the PDA. Results Three out of the four patients had postprocedure imaging, and all showed relief of the previously seen bronchial compression. Post-PDA stenting patients had a smaller and straight PDA with significant lateralization away from the compressed bronchus. None of the four patients developed symptoms of bronchial compression poststenting. Conclusions Our study suggests that pre-existing bronchial compression does not preclude PDA stenting. Stent placement in an engorged and tortuous PDA led to significant improvement in pre-existing bronchial compression. Improvement may be attributed to PDA shrinkage, straightening, and lateralization. Further studies are needed to confirm our findings.

The phenomenon of coronary steal is well known in the setting of HLHS (Hypoplastic Left Heart Syndrome) early after the Classical Norwood Operation. We report a rare case of an infant with HLHS [Severe Aortic Stenosis (AS), Mitral Stenosis (MS) and small Left Ventricle (LV)], who developed aortic regurgitation and presumed coronary steal late after the Sano Modification of the Norwood Procedure. Coronary steal developed secondary to progressive aortic and mitral regurgitation and resulted in poor right ventricular function and severe tricuspid regurgitation. We describe a novel interventional approach for obliteration of the LV cavity by using hydrogel coils. LV obliteration eliminated the presumed steal and resulted in improvement in ventricular function, tricuspid regurgitation and clinical course.

Despite few institutions stenting the ascending aorta, it has been discouraged because of the proximity of the aortic valve, the coronary artery orifices, and the aortic arch branches. We describe a small case series of patients having acquired stenosis of the ascending that was relieved successfully by stenting. © 2017 Wiley Periodicals, Inc.

Objectives To examine the factors contributing to arterial thrombosis after catheterization, particularly the use of long vs. short introducer sheaths, and propose a new protocol to address risks. Background Post-procedure arterial compromise is an important concern in pediatric cardiac catheterizations. For certain interventional procedures, the use of a long sheath is necessary, however, the incidence of complications when using such sheaths has not yet been studied. Methods This retrospective review includes patients

Background Systemic-to-pulmonary artery shunt (SPS) dysfunction can be deleterious in shunt dependent patients and traditionally have undergone surgical revision. Data on transcatheter stenting of SPS is limited. We sought to evaluate feasibility, safety and outcomes of stenting SPS. Methods Retrospective review of all patients who underwent transcatheter SPS stenting from 1/2006 to 12/2013. Results Of 229 surgically implanted SPS, 25 transcatheter stent interventions were performed in 22 patients. The majority had pulmonary atresia (n = 9) or HLHS (n = 10). Their median age was 4 ms (range 10 days to 4 years) and median weight 4.9 kg (range 3–14). Nine had a central and 15 had a BT shunt with a median shunt size of 3.75 mm (range 3–6). The interval from shunt placement to intervention was 1.9 ms (range 4 days–3.8 years). The indication for intervention was increasing cyanosis in10 patients and delaying final repair in 9. Two patients were on ECMO at the time of intervention. The median shunt diameter increased from 2.3 to 4.1 mm and oxygen saturation from 72 to 85% (P

Objectives To investigate the spectrum, etiology, and management of traumatic aortopulmonary (AP) communications after transcatheter interventions on the pulmonary circulation. Background An iatrogenic AP communication is an unusual complication after balloon pulmonary artery (PA) angioplasty or stenting, or transcatheter pulmonary valve replacement (TPVR). However, with the increasing application of transcatheter therapies for postoperative PA stenosis and right ventricular outflow tract (RVOT) dysfunction, including percutaneous pulmonary valve replacement, consideration of the etiology, diagnosis, and management of this problem is important for interventional cardiologists performing such procedures. Methods and Results We present three new cases, as well as gross anatomy and histopathology data, related to AP communications after PA interventions. We also review the literature relevant to this topic. Including these new cases, there have been 18 reported cases of iatrogenic AP communication after transcatheter interventions on the PAs or RVOT, primarily patients with transposition of the great arteries who underwent PA angioplasty after an arterial switch operation, or after TPVR in patients who had undergone a Ross procedure. The likely cause of such defects is PA trauma plus distortion of the neo-aortic anastomosis resulting from angioplasty or stenting of the RVOT or central PAs, with subsequent dissection through the extravascular connective tissue and into the closely adjacent vessel through the devitalized tissue at the anastomosis. Conclusions Cardiologists performing PA or RVOT interventions should be aware of the possibility of a traumatic AP communication and consider this diagnosis when confronted with suggestive signs and symptoms. © 2015 Wiley Periodicals, Inc.

Objectives We sought to compare ductal stenting (DS) to systemic-pulmonary artery shunt (SPS) in a contemporary cohort of patients having pulmonary atresia with intact ventricular septum (PAIVS). Background Alternative palliative strategies (interventional versus surgical) are currently available for initial palliation of ductal dependent patients with various forms of congenital heart disease. There is little data available to guide strategy selection. Methods A retrospective review of patients with PAIVS at our institution from Jan 2006 to Dec 2013 was performed. Demographic, echocardiographic, procedural, and follow-up data were reviewed and analyzed. Results Twenty-nine patients were identified. Thirteen underwent DS and 16 underwent SPS. In the DS group, 6 had radiofrequency perforation of the pulmonary valve, and 12 underwent balloon pulmonary valvuloplasty (BPV) prior to DS. SPS was performed after perforation of the pulmonary valve in 1 patient and after BPV in 4 patients. Procedural success was 92.8% in the DS group versus 93.3% in the SPS group. In the DS group, one patient required emergency shunting due to intra-procedural ductal spasm. In the SPS group there was one procedure related death, and there were six significant procedure-related complications. Five patients required re-interventions prior to hospital discharge. The median length of hospital stay for the DS versus SPS group was 10 versus 23 days, respectively (P

Objective Bronchial compression as a result of pulmonary artery and aortic arch stenting may cause significant respiratory distress. We set out to limit airway narrowing by endovascular stenting, by using simultaneous flexible bronchoscopy and graduated balloon stent dilatation, or balloon angioplasty to determine maximum safe stent diameter. Methods Between August 2010 and August 2013, patients with suspected airway compression by adjacent vascular structures, underwent CT or a 3D rotational angiogram to evaluate the relationship between the airway and the blood vessels. If these studies showed close proximity of the stenosed vessel and the airway, simultaneous bronchoscopy and graduated stent re-dilation or graduated balloon angioplasty were performed. Results Five simultaneous bronchoscopy and interventional catheterization procedures were performed in four patients. Median age/weight was 33 (range 9–49) months and14 (range 7.6–24) kg, respectively. Three had hypoplastic left heart syndrome, and one had coarctation of the aorta (CoA). All had confirmed or suspected left main stem bronchial compression. In three procedures, serial balloon dilatation of a previously placed stent in the CoA was performed and bronchoscopy was used to determine the safest largest diameter. In the other two procedures, balloon testing with simultaneous bronchoscopy was performed to determine the stent size that would limit compression of the adjacent airway. In all cases, simultaneous bronchoscopy allowed selection of an ideal caliber of the stent that optimized vessel diameter while minimizing compression of the adjacent airway. Conclusion In cases at risk for airway compromise, flexible bronchoscopy is a useful tool to guide endovascular stenting. Maximum safe stent diameter can be determined without risking catastrophic airway compression. © 2014 Wiley Periodicals, Inc.

Background: Secundum atrial septal defect (ASD) closure devices were granted approval based on industry-sponsored, prospective, nonrandomized, single device studies, demonstrating acceptable efficacy and safety in selected patients. We sought to report community practice and outcomes. Methods and Results: Procedure specific data was collected on cases considered for ASD closure in the congenital cardiac catheterization project on outcomes (C3PO) between February 1, 2007 and June 31, 2010. Eight centers contributed data during this time period. All adverse events (AE) were independently reviewed and classified by a five level severity scale. In 40 months (2/07–6/10), 653 of 688 ASDs were occluded with a single device using an AMPLATZER® Septal Occluder (ASO) in 566 (87%), GORE® HELEX® Septal Occluder (HSO) in 33 (5%), and a CardioSEAL® or STARFlex™ device (CSD) in 54 (8%). Most patients had an isolated ASD (93%). 85% were >2 years of age. The ASD median diameter was 12 mm [8,16] for ASO, with smaller diameters in HSO 8 mm [7,10] and CSD 8 mm [5,10] (P

Objectives To assess the efficacy and safety of low dose tissue plasminogen activator (tPA) therapy in children with vascular thrombosis following cardiac catheterization. Background Currently, heparin is the standard of care for vascular thrombosis, however, it may not be efficacious in dissolving existing thrombi. Although tPA is an accepted thrombolytic therapy for life-threatening thrombosis in children, it is not a well-established treatment for the thrombosis of femoral vessels following cardiac catheterization. Methods A retrospective analysis was performed from 1/1/2009 until 6/30/2011 at Rady Children's Hospital—San Diego (RCHSD) reviewing all cases with venous or arterial thrombosis at the vascular access site following cardiac catheterization, and a survey was conducted among the Congenital Cardiovascular Interventional Study Consortium members regarding their experience in tPA therapy. Results At RCHSD, out of 1,155 catheterizations, 12 children developed femoral thrombosis. In three children, where 12-h heparin therapy was unsuccessful in resolving thrombosis, we used low-dose tPA according to the following regime: 0.05 mg/kg/h for 30 min, followed by 0.1 mg/kg/h for 4 h. We achieved thrombolysis in all cases evidenced by repeat ultrasound and return of palpable pulse with normal limb circulation. No complications were encountered. The survey confirmed that adult tPA dose (0.1–0.5 mg/kg/h) has 100% efficacy in resolving thrombi in children with vascular thrombosis, however, the rate of serious complications was not negligible (15%). Conclusion tPA is an efficacious therapy to dissolve thrombi that developed as a complication of cardiac catheterization in children. The rate of complications due to tPA may be reduced using a lower dose: 0.05–0.1 mg/kg/h. © 2012 Wiley Periodicals, Inc.

Background Studies have documented the importance of procedure type and hemodynamic variables on the incidence of procedure related adverse events (AE) after cardiac catheterization. However, little is known about the impact of low weight on the incidence and severity of AE. Methods: Data were prospectively collected using a multicenter registry (C3PO). Infants

Objectives: The aim of this report is to describe our experience with closure of perimembranous ventricular septal defects (PMVSD) with aneurysm using the Amplatzer Duct Occluder I (ADO I). Background: Atrio-ventricular block (AVB) remains the most troublesome complication of device closure of PMVSDs. Many PMVSDs are associated with an aneurysm that protrudes into the right ventricle. We believe that devices implanted entirely within the VSD aneurysm avoid the risk of AVB. Methods: A retrospective analysis of patients with PMVSD and aneurysm who underwent attempted ADO I device closure between 3/2004 and 8/2010 [Rady Children's Hospital-San Diego (n = 16), Mattel Children's Hospital at UCLA (n = 4), and Straub Hospital in Honolulu (n = 1)]. Results: Twenty-one patients (16 male) with PMVSD and aneurysm underwent cardiac catheterization for the purpose of VSD closure using an ADO I. Nineteen patients had successful ADO I implantation with the following devices: 10/8 (n = 8), 8/6 (n = 7), 6/4 (n = 3) and 12/10 (n = 2). The procedure was aborted in 2 cases due to temporary AVB and ventricular ectopy during catheterization. One patient developed hemolysis due device malposition and had surgical device retrieval and VSD closure. One patient had new trivial tricuspid regurgitation and in 5/19 the tricuspid regurgitation improved post device closure. Transient accelerated junctional rhythm occurred within 24 hr in 4 patients and nonsustained slow ventricular tachycardia occurred in one patient. At latest follow-up (up to 6 years, median 1.9 years) 11/18 had no residual shunt, 4/18 had trivial and 3/18 small residual shunt. No AVB has been observed. Conclusions: Percutaneous closure of PMVSD with aneurysm using the ADO I appears to have acceptable efficacy and risk. Atrio-ventricular block in up to mid-term follow up was not encountered. © 2012 Wiley Periodicals, Inc.

Objectives: To investigate the feasibility, procedural techniques, safety, and overall potential of percutaneous coronary angioplasty and stent implantation in infants with coronary artery disease and acute coronary syndrome. Background: Despite extensive experience in adult patients, percutaneous coronary intervention remains uncommon in children and extremely rare in infants. Methods: Retrospective review including all children who underwent percutaneous coronary angioplasty in Rady Children's Hospital—San Diego during a period of 4 years. Results: Seven children including four children less than 15 months of age underwent percutaneous coronary intervention with coronary stent implantation in the proximal portion of the left or right coronary arteries. Successful stent placement with excellent revascularization was achieved in all cases. Mean coronary artery diameter was 0.65 mm prior to stent placement. Balloon angioplasty resulted in suboptimal resolution of coronary stenosis, thus bare metal stents were implanted in all cases, dilated to a mean of 2.5-mm internal diameter. Average intervention-free period was 434 days after stent implantation. In-stent re-stenosis and stent thrombosis did not occur if the implanted stent diameter was greater than 2.5 mm and the patient received dual anti-platelet therapy. Conclusions: Our experience suggests that coronary artery stent implantation is a feasible and relatively safe palliative option in infants and toddlers with coronary stenosis. It is a viable strategy for bridging patients with acute ischemia or poor ventricular function to elective surgical revascularization or transplantation. © 2011 Wiley Periodicals, Inc.

Patients with unrepaired pulmonary artery atresia and ventricular septal defect (PA/VSD) depend on aortoplumonary collaterals and surgically created shunts for pulmonary blood flow. These vessels frequently develop stenoses with time, leading to hypoperfusion of lung segments and systemic hypoxemia. The purpose of this article is to describe catheter palliation of hypoxemic patients with PA/VSD who were not candidates for surgical repair. We present our experience with stent implantation for stenosis of aortopulmonary collaterals and shunts in these patients. Three patients with hypoplastic pulmonary arteries underwent stent placement in aortopulmonary collateral arteries (APCAs) or their shunts. Technical aspects of the interventional catheterization procedure are discussed in detail. Case 1 underwent placement of five stents in collateral vessels and one stent in the Blalock-Taussig shunt (BT) with dramatic increase in vessel size and improvement in saturations from 70% to 89%. Case 2 underwent placement of two overlapping stents in a collateral vessel with an increase in diameter of the collateral vessel from 2.3 to 6 mm and an improvement in saturation from 68% to 88%. Case 3 underwent placement of three overlapping stents in a BT shunt with an increase in diameter of the shunt from 2.2 to 6.6 mm and an improvement in saturation from 71% to 89%. All three patients had excellent clinical improvement and stable saturation at follow-up. Stent placement for maintaining patency of APCAs and aortopulmonary shunts is feasible and safe.

The presence of an intra-atrial patch (IAP) has been considered a relative contraindication to transseptal puncture (TSP). The purpose of this study is to determine the efficacy and safety of the TSP through baffles, conduits, pericardial patches and other prosthetic materials in the intra-atrial septum. We reviewed the records of all pediatric patients with IAP who underwent TSP at Texas Children's Hospital from November 1979 through February 1998. The review included the cardiac diagnoses, indications for TSP, technical difficulties and follow up echocardiograms specifically addressing residual atrial shunts A total of 1958 TSP were performed. Thirty-nine patients had IAP. Cardiac diagnoses in those 39 patients included D-transposition of the great arteries after Mustard (10) or Senning procedure (6), single ventricle variant post-Fontan operation (4), total anomalous venous return repair (4), atrioventricular canal repair (9) and atrial septal defect with patch repair (6). Patients' age ranged from 1-31 years (median 7 years). The duration from the time of surgical repair to TSP ranged from 0.1-21 years (median 5 years). Indications for TSP included diagnostic and therapeutic intervention for pulmonary venous obstruction (12), creation of a baffle fenestration (2), prosthetic mitral valve evaluation (1), left ventricular outflow tract evaluation (1), access the left heart for hemodynamic evaluation (23). The IAP was traversed in 38/39 patients (97.5%), followed by diagnostic or therapeutic prograde left-heart catheterization. No complications were encountered. Follow up echocardiography in 30/38 PTS demonstrated no residual shunting across the atrial septum except for two cases in which the atrial baffle had been intentionally fenestrated. Transseptal puncture through an intra-atrial patch is a safe procedure. This technique is effective in permitting diagnostic and therapeutic left heart catheterization and does not result in residual shunting through the patch.