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Objectives To determine whether X-ray fused with MRI (XFM) is beneficial for select transcatheter congenital heart disease interventions. Background Complex transcatheter interventions often require three-dimensional (3D) soft tissue imaging guidance. Fusion imaging with live X-ray fluoroscopy can potentially improve and simplify procedures. Methods Patients referred for select congenital heart disease interventions were prospectively enrolled. Cardiac MRI data was overlaid on live fluoroscopy for procedural guidance. Likert scale operator assessments of value were recorded. Fluoroscopy time, radiation exposure, contrast dose, and procedure time were compared to matched cases from our institutional experience. Results Forty-six patients were enrolled. Pre-catheterization, same day cardiac MRI findings indicated intervention should be deferred in nine patients. XFM-guided cardiac catheterization was performed in 37 (median age 8.7 years [0.5-63 years]; median weight 28 kg [5.6-110 kg]) with the following prespecified indications: pulmonary artery (PA) stenosis (n = 13), aortic coarctation (n = 12), conduit stenosis/insufficiency (n = 9), and ventricular septal defect (n = 3). Diagnostic catheterization showed intervention was not indicated in 12 additional cases. XFM-guided intervention was performed in the remaining 25. Fluoroscopy time was shorter for XFM-guided intervention cases compared to matched controls. There was no significant difference in radiation dose area product, contrast volume, or procedure time. Operator Likert scores indicated XFM provided useful soft tissue guidance in all cases and was never misleading. Conclusions XFM provides operators with meaningful three-dimensional soft tissue data and reduces fluoroscopy time in select congenital heart disease interventions.

Coronary sinus atrial septal defects are the rarest defects of the atrial septum comprising

Renal artery stenosis (RAS) accounts for 10 % of cases of systemic hypertension in children. Initial management involves anti-hypertensive therapy. Percutaneous interventions are documented for the treatment of RAS in the adult population. In children, case reports suggest benefit.This is a retrospective analysis of consecutive patients referred for catheterization for RAS between 2002 and 2010 at a single institution. Recorded variables included: age, weight, systemic blood pressure, minimal luminal diameter, interventional devices, antihypertensive medications, contrast volume, and complications.Twelve patients (median age 8.2, IQR 6-12.4 years); median weight 42.8 kg, IQR: 25-47.4 kg) were referred for renal artery catheterization and underwent percutaneous intervention. Overall, minimal luminal diameter (MLD) increased by 1.2 ± 0.9 mm for all patients (p 0.05) and by 1.3 ± 0.9 mm for post-renal transplant patients (p 0.05). Only stent angioplasty patients demonstrated significant improved blood pressure (p 0.05). One patient had stent thrombosis requiring re-intervention with repeat balloon angioplasty.This retrospective analysis suggests that percutaneous intervention might play a role in the management of RAS, with an improvement in MLD in children with RAS. Transcatheter intervention is technically feasible with low morbidity. A prospective, longitudinal study is warranted to compare standard medical therapy with percutaneous interventions.

A 7-month-old patient in congestive heart failure due to a moderate sized patent ductus arteriosus (PDA) underwent uncomplicated implantation of an Amplatzer Ductal Occluder (ADO1). Two months after percutaneous device PDA closure, left pulmonary artery (LPA) stenosis was discovered. Rather than spontaneous improvement as reported in previous cases, our patient's LPA stenosis progressed in severity 7 months after ADO1 placement. A catheterization demonstrated a 32 mm Hg peak gradient from her main pulmonary artery to her LPA. She underwent successful stent angioplasty of her LPA with an excellent result and preserved PDA closure. This case demonstrates that stent angioplasty is a feasible an effective method of relieving LPA obstruction caused by a PDA occluder device. Additionally, despite slight deflection by the stent, the ADO1 device continued to provide complete ductal closure. Stent angioplasty should be considered in patients who have LPA stenosis caused by ADO1 occluder device that does not improve over time. © 2013 Wiley Periodicals, Inc.

Transcaval access enables delivery of large-caliber devices to the aorta in patients with small or diseased iliofemoral arteries [(1,2)][1]. The technique has been described in detail elsewhere [(3)][2] and requires pre-procedural computed tomography planning to identify a calcium-free 7.6 mm × 9.

Purpose: To develop an active delivery system that enhances visualization of nitinol cardiac occluder devices during deployment under real-time magnetic resonance imaging (MRI). Materials and Methods: We constructed an active delivery cable incorporating a loopless antenna and a custom titanium microscrew to secure the occluder devices. The delivery cable was tuned and matched to 50Ω at 64 MHz with the occluder device attached. We used real-time balanced steady state free precession in a wide-bore 1.5T scanner. Device-related images were reconstructed separately and combined with surface-coil images. The delivery cable was tested in vitro in a phantom and in vivo in swine using a variety of nitinol cardiac occluder devices. Results: In vitro, the active delivery cable provided little signal when the occluder device was detached and maximal signal with the device attached. In vivo, signal from the active delivery cable enabled clear visualization of occluder device during positioning and deployment. Device release resulted in decreased signal from the active cable. Postmortem examination confirmed proper device placement. Conclusion: The active delivery cable enhanced the MRI depiction of nitinol cardiac occluder devices during positioning and deployment, both in conventional and novel applications. We expect enhanced visibility to contribute to the effectiveness and safety of new and emerging MRI-guided treatments. J. Magn. Reson. Imaging 2012;36:972–978. © 2012 Wiley Periodicals, Inc.

A 20-month-old boy was referred to the cardiac catheterization laboratory for closure of a large prosthetic mitral valve paravalvular leak. At birth, the boy had an unbalanced atrioventricular septal defect with borderline hypoplasia of the mitral component of the common atrioventricular valve and the left ventricle. At the age of 3 months, the boy underwent complete (two-ventricle) repair. The intraoperative findings included deficient left-sided atrioventricular valve tissue. As a result of the surgical septation, the coronary sinus ostium was located in the left atrium. The boy initially did well, but 6 weeks after his initial surgery, progressive and severe mitral regurgitation developed, necessitating mitral valve replacement with a 19-mm St. Jude prosthetic valve in the supraannular position. A small (posteromedial) paravalvular prosthetic mitral leak was noted approximately 6 months after surgery. This leak became progressively worse, resulting in moderate to severe functional mitral regurgitation and worsening left ventricular dilation. A dilated structure posterior to the left atrioventricular groove also was noted to be expanding. This structure was interpreted by echocardiography as an aneurysm of the coronary sinus. Three-dimensional (3D) echocardiography helped to define the anatomy of the leak (Fig. 1a/Movie 1a in supplementry material). At the age of 20 months, the boy was referred for cardiac catheterization with planned closure of the leak.

BACKGROUND: Carbon monoxide (CO) can be produced in the anesthesia circuit when inhaled anesthetics are degraded by dried carbon dioxide absorbent and exhaled CO can potentially be rebreathed during low-flow anesthesia. Exposure to low concentrations of CO (12.5 ppm) can cause neurotoxicity in the developing brain and may lead to neurodevelopmental impairment. In this study, we aimed to quantify the amount of CO present within a circle system breathing circuit during general endotracheal anesthesia in infants and children with fresh strong metal alkali carbon dioxide absorbent and define the variables associated with the levels detected. METHODS: Fifteen infants and children (aged 4 months to 8 years) undergoing mask induction followed by general endotracheal anesthesia were evaluated in this observational study. CO was measured in real time from the inspiratory limb of the anesthesia circuit every 5 minutes for 1 hour during general anesthesia. Carboxyhemoglobin (COHb) levels were measured at the 1-hour time point and compared with baseline. RESULTS: CO was detected in all patients older than 2 years (0-18 ppm, mean 3.7 +/- 4.8 ppm) and rarely detected in patients younger than 2 years (0-2 ppm, mean 0.2 +/- 0.6 ppm). Only the relationship between CO concentration and fresh gas flow to minute ventilation ratio (FGF:(.)VE) remained significant after adjustment in longitudinal regression analysis (P < 0.001). Although not powered to determine such a relationship, CO levels were weakly associated with the use of desflurane and female sex. There was no significant association between CO concentration and anesthetic concentration. Baseline COHb levels were higher in children younger than 2 years and decreased significantly at the 1-hour time point compared with baseline and children older than 2 years. However, COHb levels increased significantly from baseline in a predictable manner consistent with CO exposure in children older than 2 years. FGF:(.)VE correlated significantly with change in COHb using simple linear regression (r = 0.62; P < 0.02). CONCLUSIONS: CO was detected routinely during general anesthesia in infants and children when FGF:(.)VE was

We report a case of interrupted aortic arch and origin of the left pulmonary artery from the aorta with bilateral ductus arteriosus and discuss its possible embryological basis. To the best of our knowledge, this combination of anomalies has not been reported in the English medical literature.

A 16-year-old boy with a double-outlet right ventricle, D-malposed great vessels, and a subpulmonary ventricular septal defect status-post surgical ventricular septal defect patch closure and arterial switch procedure at 2 months of age, reported progressive exercise intolerance. He was found to have moderate right atrial enlargement, mild dilation of right and left ventricles, and a persistent residual left ventricle to right atrium (LV-RA) intracardiac shunt on echocardiographic assessment (similar physiology to a Gerbode-type defect; Figure A and Movie I in the online-only Data Supplement). Cardiac MRI delineated the LV-RA shunt (steady-state free precession cine; Figure B and Movie I in the online-only Data Supplement), with an estimated Qp:Qs of 1.4:1 (velocity-encoded MRI). Cardiac MRI–derived left-ventricular end-diastolic volume was 132 mL/m2 ( z score …

Coronary artery fistula (CAF) is a rare clinical abnormality characterized by a connection between one or more coronary arteries and an adjacent cardiac chamber or vascular structure. Although CAF complications are more common in older children over time, there is still no consensus in terms of treatment indications in children with asymptomatic fistula. We describe an asymptomatic infant diagnosed with a severely dilated right coronary artery and a coronary-cameral fistula treated successfully by transcatheter device closure. The Amplatzer Vascular Plug II appears to be safe, effective and relatively easy to use for CAF closure in infants. Resumo: A fístula da artéria coronária (FAC) é uma alteração clínica rara, caracterizada por uma ligação entre uma ou mais artérias coronárias e a câmara cardíaca adjacente ou a estrutura vascular. Embora as complicações da FAC sejam mais comuns em crianças mais velhas, não existe consenso relativamente às indicações da terapêutica em crianças com fístula assintomática. Apresentamos o caso assintomático de uma criança que foi diagnosticada com dilatação grave da artéria coronária direita e fístula coronária-câmara, cujo tratamento foi bem-sucedido através de encerramento por dispositivo percutâneo. Consideramos que o dispositivo Amplatzer Vascular Plig II é seguro, eficaz e de fácil utilização no encerramento da FAC em crianças. Keywords: Therapeutic occlusion, Fistula, Infant, Palavras-chave: Encerramento terapêutico, Fístula, Criança

An arterial switch is the corrective procedure of choice for d-transposition of the great arteries but may be associated with increased morbidity and mortality when performed in low-birth-weight infants. Conversely, delaying surgery often leads to left ventricular "deconditioning" as pulmonary arteriolar resistance decreases. We present an infant with a birth weight of 940 g with d-transposition of the great arteries with an intact ventricular septum whose bilateral pulmonary artery branch stenosis allowed for maintenance of near systemic left ventricular pressure, thereby protecting against deconditioning. This case also represents the smallest reported patient to undergo a successful balloon atrial septostomy.

Transcatheter closure of secundum-type atrial septal defects (ASDs) using the AMPLATZER™ Septal Occluder (ASO) has been in use for more than a decade since its US Food and Drug Administration approval in 2001. Device embolization remains an uncommon complication, which can sometimes occur after the initial deployment. Previous reports of ASO devices embolized to the left ventricle have primarily been managed by open-heart surgical retrieval. We present a case of an ASO device embolized to the left ventricular outflow tract (LVOT) 18 hr after initial implantation, which was successfully retrieved percutaneously, followed by successful closure of the ASD using a larger device.

Paravalvular leak following prosthetic valve surgery has the potential to cause serious complications such as hemolysis and congestive heart failure. Successful percutaneous closures of prosthetic mitral and aortic paravalvular leaks have been performed as an alternative to reoperation. This case represents the first known report of successful percutaneous closure of a prosthetic pulmonary paravalvular leak in an adult patient with a history of congenital heart disease using two muscular ventricular septal defect occluder devices.

Background We aim to deliver large appliances into the left ventricle through the right ventricle and across the interventricular septum. This transthoracic access route exploits immediate recoil of the septum, and lower transmyocardial pressure gradient across the right versus left ventricular free wall. The route may enhance safety and allow subxiphoid rather than intercostal traversal. Methods The entire procedure was performed under real-time CMR guidance. An “active” CMR needle crossed the chest, right ventricular free wall, and then the interventricular septum to deliver a guidewire then used to deliver an 18Fr introducer. Afterwards, the right ventricular free wall was closed with a nitinol occluder. Immediate closure and late healing of the unrepaired septum and free wall were assessed by oximetry, angiography, CMR, and necropsy up to four weeks afterwards. Results The procedure was successful in 9 of 11 pigs. One failed because of refractory ventricular fibrillation upon needle entry, and the other because of inadequate guidewire support. In all ten attempts, the right ventricular free wall was closed without hemopericardium. There was neither immediate nor late shunt on oximetry, X-ray angiography, or CMR. The interventricular septal tract fibrosed completely. Transventricular trajectories planned on human CT scans suggest comparable intracavitary working space and less acute entry angles than a conventional atrial transseptal approach. Conclusion Large closed-chest access ports can be introduced across the right ventricular free wall and interventricular septum into the left ventricle. The septum recoils immediately and heals completely without repair. A nitinol occluder immediately seals the right ventricular wall. The entry angle is more favorable to introduce, for example, prosthetic mitral valves than a conventional atrial transseptal approach.

When anesthetizing children with congenital heart disease for diagnostic cardiac catheterization, anesthesiologists and cardiologists seek to use anesthetic regimens that yield minimal hemodynamic changes and allow for spontaneous ventilations. Recently, dexmedetomidine has been used as an anesthesia adjunct because of its sedative and analgesic properties and minimal ventilatory depressive effects. We tested the hypothesis that the combination of sevoflurane and dexmedetomidine is non-inferior to sevoflurane alone as it refers to hemodynamic measurements during diagnostic cardiac catheterization in children with a transplanted heart, one ventricle (Fontan procedure), or normal cardiac physiology. Patients were anesthetized with inhalation of sevoflurane in nitrous oxide/oxygen and, after baseline hemodynamic measurements, successive boluses of dexmedetomidine followed by continuous infusion were administered. In this study, non-inferiority was shown when differences at steady-state (dexmedetomidine + sevoflurane) compared to baseline (sevoflurane alone) and its associated 95% confidence interval fell completely within the range of plus or minus 20%. Forty-one (26 normal physiology, 9 cardiac transplantation, and 6 Fontan) patients were enrolled. Non-inferiority of sevoflurane + dexmedetomidine compared with sevoflurane alone was shown for heart rate, but not for arterial blood pressure in patients with normal and cardiac transplant physiology. In patients with normal cardiac physiology, non-inferiority was demonstrated for bispectral index. Therefore, while the lack of depressive respiratory effects and non-inferiority for heart rate are desirable, the lack of non-inferiority of dexmedetomidine + sevoflurane combination for arterial blood pressure do not justify the routine use of this combination compared with sevoflurane alone for children with congenital heart disease undergoing cardiac catheterization.

Tricuspid stenosis secondary to ventricular pacemaker leads is uncommon. We present a unique case of iatrogenic tricuspid stenosis secondary to fusion of the valve leaflets to transvenous implanted pacing leads. This occurred in an adult with childhood repaired Tetralogy of Fallot and high grade surgical heart block following multiple pacemaker procedures. The case was complicated by superior vena cava (SVC) and innominate vein stenosis secondary to implanted pacing leads, severe tricuspid valve (TV) stenosis, perforation of the heart by one of the implanted transvenous ventricular pacing leads, prolapse of the transvenous atrial pacing lead into the right ventricle, and unusual coronary sinus anatomy. We describe a multidisciplinary approach to management.

A 16-year-old male presenting with upper extremity hypertension was found to have a severe form of discrete coarctation with complete luminal obliteration, causing a functional interruption of the thoracic aorta. Fluoroscopically guided perforation of the obstruction and creation of a neo-aortic lumen was performed. This was followed by balloon angioplasty and stent placement, successfully relieving the coarctation. The procedural method, acute and late follow-up results, and a discussion of the potential risks and benefits are presented. © 2008 Wiley-Liss, Inc.

Patent foramen ovale (PFO) is a common congenital abnormality that has been implicated in a number of disease processes, including cryptogenic stroke and migraine headaches. Medical treatment for these processes is often considered inadequate and mechanical closure of the PFO is an attractive, albeit controversial, alternative. PFO closure has become common practice in many centers, although recent guidelines limit its indication to certain subsets of patients. This review first focuses on the anatomy, physiology and pathophysiology of PFO, and then reviews the currently available and experimental devices for PFO closure, as well as the present clinical data pertaining to them. Finally, we present our perspective of the PFO closure, with regard to its current use and future directions.

The tracheoinnominate fistula (TIF) is a potentially lethal complication traditionally associated with open tracheostomy. Currently, standard treatment involves surgical diversion or occlusion of the innominate artery. Herein, we report the case of a 10-yearold girl with a TIF treated acutely with endovascular stenting; no surgical therapy was undertaken. The patient has been doing well since the procedure, with no associated complications and no further hemorrhaging. Based on the high mortality rate associated with surgical intervention, we believe that endovascular stenting should be considered as the first-line treatment for TIF in the acute setting.

An anomalous left brachiocephalic vein is an uncommon systemic venous anomaly, which usually has no clinical significance. We describe a case of tricuspid atresia with such an anomalous left brachiocephalic vein. The presence of this unusual venous anomaly had a number of implications in the surgical management of the tricuspid atresia.

We performed orotracheal intubation in 153 consecutive pediatric patients undergoing cardiac catheterization. Auscultation of bilateral breath sounds was confirmed. By fluoroscopy, the tip of the endotracheal tube (ETT) was seen in the right mainstem bronchus in 18 patients (11.8%) and in a low position, defined as within 1 cm above the carina, in 29 patients (19.0%). All of the 18 patients with right mainstem intubation were children

A new method for prograde balloon dilation of severe congenital aortic valve stenosis is described. Two TEGwire balloons were used prograde through bilateral femoral venous sheaths. Excellent hemodynamic results were achieved, without the need for retrograde left heart catheterization and femoral arterial sheath placement.

In this report, we describe an infant with HLHS, who underwent PDA stenting which allowed the infant to thrive as an outpatient without intervening surgical palliation. The patient eventually underwent successful cardiac transplantation at 3 months of age. The infant is well at 1 year after transplant follow-up.

Objectives . This report summarizes our experience with the use of occluding spring coils to close the small patent ductus arteriosus. Background . Several patent ductus arteriosus occluders (most notably the Rashkind device) have been developed and studied. Occluding spring coils have been used to close abnormal vessels and vascular connections. We previously reported the use of occluding spring coils to close the small patent ductus arteriosus in a small group of patients. This report describes our series of patients having patent ductus arteriosus closure with occluding spring coils. Methods . Between June 1990 and June 1993, 30 patients underwent cardiac catheterization to have patent ductus arteriosus closure by occluding spring coils. Selection criteria were age >6 months and narrowest patent ductus arteriosus internal dimension ⪯3.0 mm by color flow imaging. Definitive selection was based on review of aortograms performed at catheterization. A 5.2F coronary catheter was used to deliver one or two standard occluding spring coils. A loop was delivered in the main pulmonary artery, and the remainder of the coil was delivered across the patent ductus arteriosus and into the aortic diverticulum. Patent ductus arteriosus closure was confirmed by aortography or color flow imaging, or both. Follow-up after coil placement occurred at 6 weeks and 6 months and included two-view chest radiography, echocardiography and color flow imaging. Results . Of the 30 patients, 29 had successful implantation by one (27 patients) or two (2 patients) occluding spring coils. Of these 29 patients, 19 had a clinically apparent and 10 had a silent patent ductus arteriosus. Average ductus minimal internal dimension was 1.7 mm (range 1.0 to 3.0). Complete closure of the ductus was confirmed in 27 patients by aortography or color flow imaging or both (in 24 within 4 h, in 2 after 6 weeks and in 1 after 6 months). Six weeks after implantation, two patients had a tiny residual patent ductus arteriosus noted on color flow imaging. One patient did not have successful implantation. This patient had a 3.2-mm ductus, and two coils migrated to the distal left pulmonary artery and could not be retrieved. There were no deaths or any significant complications noted during early or late follow-up in these patients. Conclusions . Occluding spring coils may have additional application in closing the small patent ductus arteriosus.

Extracorporeal membrane oxygenation (ECMO) is a well-established tool of cardiopulmonary circulatory support for cardiopulmonary failure in children and adults. It has been used as a supportive strategy during interventional procedures in neonates with congenital heart disease. Herein, we describe a neonate with hypoplastic left heart syndrome who underwent stenting of the Sano shunt and left pulmonary artery after Norwood Sano operation using intra-procedural ECMO support. The use of ECMO as a bridge to recovery might be a feasible and reasonably safe adjunctive approach in the treatment of complications in selective case of neonates having undergone the Norwood Sano procedure.