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Lymphatic Disorders in Patients With Single Ventricle Heart Disease

Yoav Dori1, Christopher L Smith1

  • 1Department of Cardiology, Jill and Mark Fishman Center for Lymphatic Disorders, Children's Hospital of Philadelphia, Philadelphia, PA, United States.

Frontiers in Pediatrics
|June 27, 2022
PubMed

Insights

Lymphatic abnormalities cause Fontan failure and complications like protein-losing enteropathy and plastic bronchitis. New imaging and treatments offer hope for managing lymphatic dysfunction in single ventricle patients.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Lymphatic Physiology

Background:

  • Single ventricle physiology presents unique challenges, often leading to lymphatic abnormalities.
  • These abnormalities are linked to critical Fontan circuit complications, including protein-losing enteropathy (PLE), plastic bronchitis (PB), and chylothorax.
  • Early Fontan failure is a significant concern in this patient group.

Purpose of the Study:

  • To review recent advancements in lymphatic imaging and interventions for single ventricle patients.
  • To elucidate the role of lymphatic circulation in the pathogenesis of PLE, PB, and chylothorax.
  • To discuss emerging treatment strategies for lymphatic dysfunction in this population.

Main Methods:

  • Literature review of recent developments in lymphatic imaging techniques.
  • Analysis of current interventional strategies targeting lymphatic abnormalities.
  • Synthesis of evidence on the pathophysiology of Fontan complications related to lymphatic dysfunction.

Main Results:

  • Novel imaging modalities provide better visualization of lymphatic abnormalities.
  • Interventions targeting lymphatic circulation show promise in managing complications.
  • Understanding lymphatic dysfunction is crucial for improving Fontan patient outcomes.

Conclusions:

  • Lymphatic abnormalities are a key factor in Fontan failure and associated complications.
  • Advances in lymphatic imaging and intervention offer new therapeutic avenues.
  • Further research is needed to optimize management and improve long-term outcomes for single ventricle patients.

Keywords:
DCMRLasciteschylothoraxlymphangiographylymphaticsplastic bronchitisprotein-losing enteropathy (PLE)

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