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Pediatrics3 papers

Isomerism of left atrial appendage

Last edited: 4/15/2026

Overview

Left atrial isomerism, often associated with polysplenia syndrome, involves the symmetrical development of the left atrium and inferior vena cava anomalies, including interruption and azygos continuation 1.

Diagnosis

  • Key Diagnostic Criteria: Interruption of the inferior vena cava with azygos continuation is common (≥65%) 1.
  • Recommended Tests: First-pass radionuclide angiography can accurately diagnose inferior vena cava interruption and its continuation pattern 1.
  • Confirmatory Tests: Cardiac catheterization is essential for definitive diagnosis 1.
  • Management

  • Surgical Correction/Palliation: Indicated for patients requiring intervention; specific techniques not detailed 1.
  • Monitoring: Patients awaiting surgical intervention should be closely monitored 1.
  • Special Populations

  • Pediatrics: Not specifically addressed in the provided abstracts 1.
  • Comorbidities: No specific mention of comorbidities affecting management 1.
  • Key Recommendations

  • Utilize first-pass radionuclide angiography for initial diagnosis of inferior vena cava interruption in suspected left atrial isomerism (Evidence: Moderate) 1.
  • Confirm findings with cardiac catheterization before proceeding with surgical correction or palliation (Evidence: Moderate) 1.
  • Consider surgical intervention based on clinical need, with close monitoring for patients awaiting surgery (Evidence: Expert opinion) 1.
  • References

    1 Roguin N, Lam M, Frenkel A, Front D. Radionuclide angiography of azygos continuation of inferior vena cava in left atrial isomerism (polysplenia syndrome). Clinical nuclear medicine 1987. link

    Original source

    1. [1]

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