Interventional rerouting of scimitar vein to left atrium using an Amplatzer vascular plug

Congenit Heart Dis. 2007 Jul-Aug;2(4):265-9. doi: 10.1111/j.1747-0803.2007.00113.x.

Abstract

Scimitar syndrome is a rare congenital anomaly accounting for about 0.5% of all congenital heart diseases. In its most common form, it is characterized by anomalous drainage of one or all right side pulmonary veins into inferior vena cava or rarely into hepatic, portal, or azygous veins. Other associated anomalies can be hypoplasia or agenesis of right lung, hypoplasia of right pulmonary artery, bronchial anomalies, aberrant blood supply and (or) sequestration of right lung, and variety of intracardiac defects which commonly includes atrial or ventricular septal defects and tetrology of Fallot. The usual treatment of these patients is surgical rerouting of Scimitar vein into left atrium (LA) by baffle formation, lobectomy, embolization or ligation of aberrant blood supply and various combinations of these. Nonsurgical and interventional treatments are rarely possible. We report a rare case where complete rerouting of anomalous venous drainage to LA could be achieved by interventional treatment by a simple deployment of a vascular plug in combination with embolization of aberrant vascular supply to the same area, thus avoiding surgical intervention.

Publication types

  • Case Reports

MeSH terms

  • Blood Vessel Prosthesis*
  • Child, Preschool
  • Collateral Circulation
  • Echocardiography
  • Embolization, Therapeutic* / instrumentation
  • Embolization, Therapeutic* / methods
  • Heart Atria
  • Humans
  • Male
  • Pulmonary Veins* / abnormalities
  • Pulmonary Veins* / physiopathology
  • Regional Blood Flow
  • Scimitar Syndrome / diagnostic imaging
  • Scimitar Syndrome / physiopathology
  • Scimitar Syndrome / therapy*
  • Vena Cava, Inferior