The windsock syndrome: subpulmonic obstruction by membranous ventricular septal aneurysm in congenitally corrected transposition of great arteries

Echocardiography. 2013 Sep;30(8):E243-8. doi: 10.1111/echo.12279. Epub 2013 Jul 1.

Abstract

Anomalies of the membranous portion of the interventricular septum include perimembranous ventricular septal defect and/or membranous septal aneurysm (MSA). In congenitally corrected transposition of the great arteries (L-TGA in sinus solitus), the combination of ventricular inversion and arterial transposition creates a unique anatomic substrate that fosters subpulmonic left ventricular outflow tract obstruction by an MSA. The combination of an L-TGA with subpulmonic obstruction by an MSA is referred to as the windsock syndrome. We report a case of windsock syndrome in a 25-year-old man which is to our knowledge the first three-dimensional echocardiographic description of this congenital entity.

Keywords: L-TGA; aneurysm; congenitally corrected transposition of great arteries; obstruction; windsock.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Congenitally Corrected Transposition of the Great Arteries
  • Diagnosis, Differential
  • Echocardiography, Three-Dimensional / methods*
  • Heart Septal Defects, Ventricular / diagnostic imaging*
  • Humans
  • Male
  • Syndrome
  • Transposition of Great Vessels / diagnostic imaging*
  • Ventricular Outflow Obstruction / diagnostic imaging*

Supplementary concepts

  • Aneurysm Of Interventricular Septum