Transcatheter embolization of anomalous systemic arterial supply with Amplatzer Vascular Plugs II in Scimitar syndrome.
A 57-year-old woman suffering from recurrent pneumonia and considered to be high risk for surgical correction of an atrial septal defect (ASD) associated with a right lung sequestration vascularized by arteries coming from the abdominal aorta and an abnormal venous drainage into the inferior vena cava (Scimitar syndrome). Therefore stepwise transcatheter approach was offered as an alternative treatment. We performed ASD closure and embolization of the anomalous systemic arterial supply using Amplatzer Vascular Plugs II. The abnormal venous drainage was conservatively treated (Q(p) /Q(s) : 1.1/1 after ASD closure and anomalous arterial supply embolization). At 24-month follow-up, no recurrent pneumonia was reported, functional class improved and right cardiac cavities normalized.
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Available from: Ibrahim Cansaran Tanidir
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ABSTRACT: Scimitar syndrome is a rare disease associated with a right lung sequestration vascularised by arteries arising from the abdominal aorta and abnormal venous drainage into the inferior vena cava. The infantile form is generally presented with severe heart failure, pulmonary hypertension and respiratory distress. It may be associated with various intracardiac defects, including atrial septal defects, ventricular septal defects, patent ductus arteriosus or more complicated structural congenital heart defects. Here, we present a 2-month-old girl with Scimitar syndrome whose pulmonary arterial pressure decreased after transcatheter patent ductus arteriosus closure and embolization of the anomalous systemic arterial supply.
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