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Ivemark syndrome: bronchial compression from anomalous pulmonary venous anatomy

Pooja H Patel1, Joel Hayden1, Randy Richardson1

  • 1Department of Radiology, St. Joseph's Hospital and Medical Center and Creighton University School of Medicine, Phoenix, AZ 85004, USA.

Journal of Surgical Case Reports
|May 2, 2017
PubMed

Insights

Ivemark syndrome, a rare condition affecting multiple organs, can cause severe heart defects. This case used 3D CT angiography to visualize complex cardiopulmonary abnormalities in Ivemark syndrome, aiding understanding of this rare disorder.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Genetics

Background:

  • Ivemark syndrome, a heterotaxy disorder, affects approximately 1 in 6000 births.
  • It is characterized by congenital heart defects and malformations of other organs.
  • Total anomalous pulmonary venous return is a common cardiac manifestation.

Observation:

  • This case report details a patient with Ivemark syndrome presenting with anomalous venous return and right isomerism.
  • Advanced imaging techniques, specifically 3D CT angiography, were employed for detailed visualization.
  • The focus was on understanding the complex cardiopulmonary anatomy.

Findings:

  • 3D CT angiography provided optimal visualization of the anomalous venous return and associated cardiac structures.
  • The reconstruction facilitated a comprehensive understanding of the cardiopulmonary system's intricate abnormalities.
  • Detailed anatomical insights were gained into the multi-organ involvement characteristic of Ivemark syndrome.

Implications:

  • Enhanced visualization aids in surgical planning and patient management for Ivemark syndrome.
  • This case contributes valuable data to the understanding of heterotaxy syndromes.
  • Further research into Ivemark syndrome may lead to improved diagnostic and therapeutic strategies.

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