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Ventricular Topology in Congenital Heart Defects Associated with Heterotaxy: Can We Find Patterns Reflecting the

Takhfif Othman1,2,3, Abdulsalam Mohammad Adnan Alsaiad1,2,3, Abdulraouf M Z Jijeh1,2,3

  • 1King Abdulaziz Cardiac Center, Ministry of National Guard Health Affairs, Riyadh 11426, Saudi Arabia.

Journal of Cardiovascular Development and Disease
|November 26, 2025
PubMed

Insights

Heterotaxy syndrome, often linked to congenital heart defects, shows varied ventricular topology. This study found a tendency for D-hand topology in left atrial isomerism, but a more random distribution in right atrial isomerism.

Area of Science:

  • Cardiology
  • Developmental Biology
  • Medical Genetics

Background:

  • Heterotaxy syndrome involves bilateral symmetry of internal organs, frequently co-occurring with complex congenital heart defects (CHDs).
  • In heterotaxy, atrial arrangement tends towards isomerism, while ventricular topology remains asymmetric (D-hand or L-hand).

Purpose of the Study:

  • To statistically analyze ventricular topology distribution in patients with CHDs and heterotaxy.
  • To investigate associations between ventricular topology, cardiovascular disorders, and patient survival.

Main Methods:

  • Retrospective cross-sectional study of 192 patients treated between 2000 and 2023.
  • Analysis of ventricular topology (D-hand vs. L-hand) in relation to atrial isomerism (LAI vs. RAI), CHDs, and clinical outcomes.

Main Results:

  • The cohort (n=192) predominantly showed D-hand ventricular topology (67%), significantly in Left Atrial Isomerism (LAI) (74%).
  • Right Atrial Isomerism (RAI) exhibited a near-equal distribution of D-hand (57%) and L-hand (43%) topologies.
  • No significant links were found between ventricular topology and major CHDs or mortality.
  • Ventricular topology showed significant associations with cardiac apex position, p-wave axis direction, and aortic arch sidedness.

Conclusions:

  • In heterotaxy, particularly RAI, ventricular topology and aortic arch sidedness display a tendency towards random occurrence.
  • This observed symmetry reflects the syndrome's inherent tendency for bilateral symmetry in anatomical arrangements.

Keywords:
atrial isomerismcardiac loopingcongenital heart defectsheterotaxytranslational researchventricular isomerismventricular topology

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