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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.

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.

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