Fetal echocardiographic evaluation of atrial morphology and the prediction of laterality in cases of heterotaxy syndromes

Ultrasound Obstet Gynecol. 2005 Oct;26(5):538-45. doi: 10.1002/uog.1934.

Abstract

Objective: To evaluate whether abnormal atrial morphology, which is well recognized in autopsy series, is detectable by fetal echocardiographic examination of the four-chamber view, and can therefore be utilized to differentiate left from right isomerism in heterotaxy syndromes.

Methods: This study was a retrospective review of 30 cases with prenatally diagnosed heterotaxy syndromes. Ultrasound video recordings and still images were reviewed with respect to atrial morphology in the four-chamber view. In 25 cases the morphology of both atria was sufficiently well visualized on the recordings to be evaluated and only these were included in the study.

Results: Two types of atrial morphology were distinguished in our cohort: a sickle-shape with the tip pointing laterally and apically, and a blunt shape resembling the usual atrial appearance in the four-chamber view. Nineteen out of the 25 cases (76%) presented with isomerism of the atria in the four-chamber view. Thirteen had bilateral sickle-shaped atrial morphology, all associated with left isomerism. Six had bilateral blunt-shaped atrial morphology, all associated with right isomerism. The atria of the remaining six cases were not isomeric, the right atrium being sickle-shaped and the left blunt-shaped. Five of the latter cases were associated with left and one with right isomerism.

Conclusions: The majority of prenatally diagnosed heterotaxy syndromes seem to present with isomeric atrial morphology in the four-chamber view. In these cases a differentiation between left and right isomerism can be based on the two distinct types of atrial morphology. This may further enhance the prenatal differentiation of these syndromes.

MeSH terms

  • Chi-Square Distribution
  • Echocardiography / methods*
  • Female
  • Heart Atria / diagnostic imaging
  • Heart Atria / embryology
  • Heart Defects, Congenital / diagnostic imaging*
  • Heart Defects, Congenital / embryology
  • Humans
  • Infant, Newborn
  • Predictive Value of Tests
  • Pregnancy
  • Retrospective Studies
  • Syndrome
  • Ultrasonography, Prenatal / methods*