Left-sided scimitar vein causing cyanosis after Fontan operation: successful transcatheter device occlusion using magnetic resonance imaging X-ray fusion.
We present the case of a 3-year-old boy with asplenia-type heterotaxy syndrome and functionally single ventricle congenital heart disease who developed cyanosis early after the Fontan operation. Combined cardiac magnetic resonance imaging (MRI) and catheterization identified a large hepatic vein to pulmonary vein connection as the source of right to left shunt. The anatomy was quite unusual, suggesting an underlying diagnosis of mixed total anomalous pulmonary venous connection with left-sided scimitar vein. This pattern of pulmonary venous return has not been previously reported in a patient with asplenia. MRI x-ray fusion was used to guide transcatheter device occlusion of the scimitar vein, resulting in marked clinical improvement.
Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pa, USA; Department of Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pa, USA.
Tacy E
TE
Downing
Yoav
Y
Dori
Matthew A
MA
Harris
Andrew C
AC
Glatz
D000015:Abnormalities, Multiple; D006328:Cardiac Catheterization / Q000295:instrumentation*; D002675:Child, Preschool; D003326:Coronary Circulation; D003490:Cyanosis / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D018729:Fontan Procedure / Q000009:adverse effects*; D006352:Heart Ventricles / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D006439:Hemodynamics; D059446:Heterotaxy Syndrome / Q000150:complications; D006801:Humans; D018810:Magnetic Resonance Angiography; D053783:Magnetic Resonance Imaging, Interventional; D008297:Male; D064847:Multimodal Imaging; D011237:Predictive Value of Tests; D011652:Pulmonary Circulation; D015641:Radiography, Interventional; D012587:Scimitar Syndrome / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D013997:Time Factors; D016896:Treatment Outcome
Fontan; Heart Catheterization; Heterotaxy Syndrome; Interventional; Magnetic Resonance Imaging; Scimitar Syndrome