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Lymphatic Management in Single-Ventricle Patients.

Maxim Itkin1, Christian Pizarro2, Wolfgang Radtke2

  • 1Neours/Dupont Children's Hospital, Wilmington, Delaware; Penn Medicine, Perelman School of Medicine, Hospital of University of Pennsylvania, Philadelphia, Pennsylvania.

Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
|May 2, 2020
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Summary

Novel imaging and minimally invasive lymphatic interventions are effectively treating serious lymphatic complications in single ventricle patients, improving outcomes for plastic bronchitis and protein-losing enteropathy.

Keywords:
EmbolizationPlastic bronchitisProtein-losing enteropathySingle ventricle

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Area of Science:

  • Cardiology
  • Vascular Biology
  • Pediatric Surgery

Background:

  • Lymphatic complications like plastic bronchitis, protein-losing enteropathy, and chylous pleural effusion cause significant morbidity and mortality in single ventricle patients.
  • Historically, therapeutic options for these conditions have been limited, posing a challenge for patient management.

Purpose of the Study:

  • To elucidate the pathophysiological mechanisms underlying lymphatic complications in single ventricle patients.
  • To evaluate the efficacy of novel lymphatic imaging and interventional techniques in managing these complex conditions.

Main Methods:

  • Utilized advanced lymphatic imaging techniques including intranodal lymphangiography, dynamic contrast-enhanced magnetic resonance lymphangiography, and liver lymphangiography.
  • Employed minimally invasive interventional techniques such as thoracic duct embolization, interstitial embolization, and liver lymphatic embolization to target abnormal lymphatic networks.

Main Results:

  • Identified a dual mechanism involving increased lymphatic flow due to elevated central venous pressure and lymphatic anatomical variants.
  • Demonstrated successful obliteration of abnormal lymphatic networks, leading to symptom resolution in patients undergoing interventional procedures.
  • Refined imaging and interventional methods improved patient selection and long-term outcomes.

Conclusions:

  • Novel lymphatic imaging and interventional strategies offer effective treatment for lymphatic complications in single ventricle patients.
  • Understanding the specific pathophysiological mechanisms allows for targeted therapeutic approaches, improving patient prognosis and quality of life.