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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.

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.

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.

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.

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.

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

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.

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 …

Introduction Muscular ventricular septal defect (VSD) is common, and can be congenital, post-infarction, or post-traumatic. Real-time MRI can provide surgical-grade exposure for non-surgical procedures. One innovative pediatric surgical procedure is device closure of ventricular septal defect using catheter techniques through an open chest. We attempt to conduct this same procedure in a closed chest model using real-time MRI.

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.

Background— Real-time MR imaging (rtMRI) is now technically capable of guiding catheter-based cardiovascular interventions. Compared with x-ray, rtMRI offers superior tissue imaging in any orientation without ionizing radiation. Translation to clinical trials has awaited the availability of clinical-grade catheter devices that are both MRI visible and safe. We report a preclinical safety and feasibility study of rtMRI-guided stenting in a porcine model of aortic coarctation using only commercially available catheter devices. Method and Results— Coarctation stenting was performed wholly under rtMRI guidance in 13 swine. rtMRI permitted procedure planning, device tracking, and accurate stent deployment. “Active” guidewires, incorporating MRI antennas, improved device visualization compared with unmodified “passive” nitinol guidewires and shortened procedure time (26±11 versus 106±42 minutes; P =0.008). Follow-up catheterization and necropsy showed accurate stent deployment, durable gradient reduction, and appropriate neointimal formation. MRI immediately identified aortic rupture when oversized devices were tested. Conclusions— This experience demonstrates preclinical safety and feasibility of rtMRI-guided aortic coarctation stenting using commercially available catheter devices. Patients may benefit from rtMRI in the future because of combined device and tissue imaging, freedom from ionizing radiation, and the ability to identify serious complications promptly.

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

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.

Introduction and objective Percutaneous transthoracic left ventricular (LV) access and closure would be an enabling technology for a wide range of structural heart, electrophysiologic and proximal aorta procedures. We propose a real-time MRI guided approach to access and close the LV using novel active devices. Methods Sixteen Yorkshire swine underwent percutaneous transthoracic LV access and followed up to three months (n=8). All procedures were guided by real-time bSSFP MRI (Espree, Siemens) using two slices along the needle trajectory and a short-axis slice for cardiac monitoring. Transthoracic ventricular puncture was performed using a customized 18G active needle with integrated loop coil. Puncture trajectory was planned using realtime MRI to identify optimal LV puncture target and trajectory with respect to intraventricular and valvular structures (Figure 1A). After needle entry, an 18-French sheath was inserted and anticoagulation begun.

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.