Left isomerism syndrome with total anomalous systemic connection

Asian Cardiovasc Thorac Ann. 2017 Jan;25(1):58-61. doi: 10.1177/0218492315618507. Epub 2016 Jul 11.

Abstract

We present a case of left isomerism with total anomalous systemic venous connection where the inferior vena cava was absent and all other systemic veins connected abnormally to the left atrium. The right atrium was hypoplastic with an intact atrial septum. Blood flow to the lungs was through a large ventricular septal defect. The diagnosis was made with echocardiography, angiography, and computed tomography. Complete repair was performed successfully, and the 7-year-old patient had an uneventful recovery.

Keywords: Heart atria; Heart defects; Heart septal defects; Vascular malformations; Vena cava; atrial; congenital; inferior; superior.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple*
  • Cardiac Surgical Procedures
  • Child
  • Computed Tomography Angiography
  • Echocardiography, Doppler, Color
  • Female
  • Heart Defects, Congenital* / diagnostic imaging
  • Heart Defects, Congenital* / physiopathology
  • Heart Defects, Congenital* / surgery
  • Heart Septal Defects, Ventricular* / diagnostic imaging
  • Heart Septal Defects, Ventricular* / physiopathology
  • Heart Septal Defects, Ventricular* / surgery
  • Heart Ventricles / abnormalities*
  • Heart Ventricles / diagnostic imaging
  • Heart Ventricles / physiopathology
  • Heart Ventricles / surgery
  • Hemodynamics
  • Heterotaxy Syndrome* / diagnostic imaging
  • Heterotaxy Syndrome* / physiopathology
  • Heterotaxy Syndrome* / surgery
  • Humans
  • Pulmonary Circulation
  • Treatment Outcome
  • Vena Cava, Inferior / abnormalities*
  • Vena Cava, Inferior / diagnostic imaging
  • Vena Cava, Inferior / physiopathology
  • Vena Cava, Inferior / surgery

Supplementary concepts

  • Right ventricle hypoplasia