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Although congenital coronary artery anomalies are relatively rare, they are the second most common cause of sudden cardiac death among young athletes. When encountered in the cardiac catherization laboratory, they are often challenging to selectively engage, requiring multiple catheters, plus increased contrast volume and radiation exposure. In the setting of acute coronary syndromes, it is not infrequent that percutaneous intervention is delayed because of the inability to engage an anomalous coronary artery. The aim of this review is to provide a comprehensive and concise overview of coronary artery anomalies, with particular attention to diagnostic and guide catheter selection for each type of anomaly and recommendations on how to recognize the vessel course angiographically.

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.

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.

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.

Objectives This study sought to evaluate feasibility of nonsurgical transthoracic catheter-based left ventricular (LV) access and closure. Background Implanting large devices, such as mitral or aortic valve prostheses, into the heart requires surgical exposure and repair. Reliable percutaneous direct transthoracic LV access and closure would allow new nonsurgical therapeutic procedures. Methods Percutaneous direct LV access was performed in 19 swine using real-time magnetic resonance imaging (MRI) and an “active” MRI needle antenna to deliver an 18-F introducer sheath. The LV access ports were closed percutaneously using a commercial ventricular septal defect occluder and an “active” MRI delivery cable for enhanced visibility. We used “permissive pericardial tamponade” (temporary fluid instillation to separate the 2 pericardial layers) to avoid pericardial entrapment by the epicardial disk. Techniques were developed in 8 animals, and 11 more were followed up to 3 months by MRI and histopathology. Results Imaging guidance allowed 18-F sheath access and closure with appropriate positioning of the occluder inside the transmyocardial tunnel. Of the survival cohort, immediate hemostasis was achieved in 8 of 11 patients. Failure modes included pericardial entrapment by the epicardial occluder disk (n = 2) and a true-apex entry site that prevented hemostatic apposition of the endocardial disk (n = 1). Reactive pericardial effusion (192 ± 118 ml) accumulated 5 ± 1 days after the procedure, requiring 1-time drainage. At 3 months, LV function was preserved, and the device was endothelialized. Conclusions Direct percutaneous LV access and closure is feasible using real-time MRI. A commercial occluder achieved hemostasis without evident deleterious effects on the LV. Having established the concept, further clinical development of this approach appears realistic.

Objectives The aim of this study was to close ventricular septal defects (VSDs) directly through the chest wall using magnetic resonance imaging (MRI) guidance, without cardiopulmonary bypass, sternotomy, or radiation exposure. Background Surgical, percutaneous, and hybrid management of VSD each have limitations and known morbidity. Methods Percutaneous muscular VSDs were created in 10 naive Yorkshire swine using a transjugular laser catheter. Under real-time MRI guidance, a direct transthoracic vascular access sheath was introduced through the chest into the heart along a trajectory suitable for VSD access and closure. Through this transthoracic sheath, muscular VSDs were occluded using a commercial nitinol device. Finally, the right ventricular free wall was closed using a commercial collagen plug intended for arterial closure. Results Anterior, posterior, and mid-muscular VSDs (6.8 ± 1.8 mm) were created. VSDs were closed successfully in all animals. The transthoracic access sheath was displaced in 2, both fatal. Thereafter, we tested an intracameral retention sheath to prevent this complication. Right ventricular access ports were closed successfully in all, and after as many as 30 days, healed successfully. Conclusions Real-time MRI guidance allowed closed-chest transthoracic perventricular muscular VSD closure in a clinically meaningful animal model. Once applied to patients, this approach may avoid traditional surgical, percutaneous, or open-chest transcatheter (“hybrid”) risks.

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.

Objectives We hypothesized that X-ray fused with magnetic resonance imaging (XFM) roadmaps might permit direct antegrade crossing and delivery of a ventricular septal defect (VSD) closure device and thereby reduce procedure time and radiation exposure. Background Percutaneous device closure of membranous VSD is cumbersome and time-consuming. The procedure requires crossing the defect retrograde, snaring and exteriorizing a guidewire to form an arteriovenous loop, then delivering antegrade a sheath and closure device. Methods Magnetic resonance imaging roadmaps of cardiac structures were obtained from miniature swine with spontaneous VSD and registered with live X-ray using external fiducial markers. We compared antegrade XFM-guided VSD crossing with conventional retrograde X-ray–guided crossing for repair. Results Antegrade XFM crossing was successful in all animals. Compared with retrograde X-ray, antegrade XFM was associated with shorter time to crossing (167 ± 103 s vs. 284 ± 61 s; p = 0.025), shorter time to sheath delivery (71 ± 32 s vs. 366 ± 145 s; p = 0.001), shorter fluoroscopy time (158 ± 95 s vs. 390 ± 137 s; p = 0.003), and reduced radiation dose–area product (2,394 ± 1,522 mG·m2 vs. 4,865 ± 1,759 mG·m2; p = 0.016). Conclusions XFM facilitates antegrade access to membranous VSD from the right ventricle in swine. The simplified procedure is faster and reduces radiation exposure compared with the conventional retrograde approach.

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.

The application of endovascular stents in new locations is continuing to expand. Recently, experience with treating both recurrent post-surgical and native aortic coarctation has been reported. The use of stents to treat aortic coarctation is increasing, mandating a comprehensive discussion of this important evolving therapeutic option. In this paper, both experimental data and published clinical results are reviewed with respect to appropriate indications and current use. Recent clinical results from Children's National Medical Center in 12 patients (nine recurrent and three native aortic coarctations) with 18-month follow-up are also presented. Technical considerations with respect to stents, balloons and delivery sheaths and their appropriate use as well as limitations are discussed. Potential complications and procedural pitfalls are described as well as strategies for their avoidance. The continued successful development of aortic coarctation stenting as a viable treatment option will depend on continued follow-up and reporting of favorable long-term patient outcomes. Finally, speculation regarding the further development and future clinical use of bio-degradable stent technologies is made.

Although rare, a congenital direct fistula connection between the proximal right pulmonary artery and the left atrium can present as cyanosis in the newborn. We report the first case in which catheter-based coil closure of such a fistula in a neonate resulted in rapid clinical improvement, obviating the need for surgical repair.

A 44-year-old man underwent aortic valve replacement with a porcine bioprosthesis 21 years ago for infective endocarditis complicated by a cerebral mycotic aneurysm and intracranial bleeding. Nine years ago, he had a second aortic valve replacement with a mechanical bileaflet tilting-disk prosthesis because of porcine prosthesis degeneration. No pseudoaneurysm was noted on the operative report. Six months ago, he developed angina and had a positive stress test for ischemia. Angiography showed severe left main coronary artery (LM) stenosis, which was treated with intravascular ultrasound–guided percutaneous coronary intervention with a zotarolimus-eluting stent. Two months ago, he again developed angina. Follow-up angiography and intravascular ultrasound revealed severe in-stent restenosis in the proximal third of the LM and systolic narrowing of the distal third, suggestive of extrinsic compression (Figure 1 and online-only Data Supplement Movie 1). In-stent restenosis was treated at that time with a sirolimus-eluting stent. A transesophageal echocardiogram revealed a large pseudoaneurysm lateral to the aortic root; color …

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.

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.

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.

Background Transcatheter closure of cardiovascular defects remains a challenge. Several occlusion devices are available, but each device has limitations. The purpose of this study was to evaluate the new Gianturco-Grifka vascular occlusion device (GGVOD) in a canine model. Methods and Results A total of 26 GGVODs were implanted as part of short- and long-term studies. In the short-term study, 1 GGVOD was implanted in each of 11 systemic arteries from 3.2 to 9.0 mm in diameter. All 11 arteries were occluded immediately. In the long-term study, an aortopulmonary shunt was placed in 10 dogs (9, Gore-tex graft; 1, subclavian artery) followed by GGVOD implantation; additionally, a GGVOD was implanted in 5 subclavian arteries. The dogs were boarded for 3 to 6 months, then recatheterized and euthanatized. Immediately after implantation, the 5 subclavian arteries and 9 Gore-tex shunts were occluded completely; the 1 subclavian artery shunt had a small residual leak. At recatheterization, all 10 shunts and 5 subclavian arteries were occluded completely. Necropsy revealed all shunts to be occluded, with the aortic and pulmonic orifices covered with a neointimal layer. The mean fluoroscopic time needed for GGVOD implantation was 9 minutes (range, 3 to 22 minutes). Conclusions (1) In a canine model, the GGVOD is effective for transcatheter occlusion of arteries and aortopulmonary shunts from 3 to 9 mm in diameter. Possible indications in children include aortopulmonary collateral vessels, long patent ductus arteriosus, systemic-pulmonary shunts, AV malformations, and arteries supplying tumors. (2) GGVOD implantation requires a short fluoroscopic time.

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.

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.