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Clinical development focuses on how the drug will interact with the human body and encompasses four key phases of clinical trials, each serving a specific purpose in assessing the safety and effectiveness of new drugs. These phases overlap and build upon one another. Phase I involves a small group of healthy volunteers (typically 20-80 individuals) or, in cases where significant toxicity is expected, patients with the targeted disease, such as cancer or AIDS. The volunteers are tested for...
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Hedinger Syndrome-Lessons Learnt: A Single-Center Experience.

Mohamed El Gabry1, Sven Arends2, Sharaf-Eldin Shehada1

  • 1Department of Thoracic and Cardiovascular Surgery, West-German Heart and Vascular Centre, 45147 Essen, Germany.

Journal of Cardiovascular Development and Disease
|October 27, 2023
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Summary
This summary is machine-generated.

Surgical management of Hedinger syndrome (HS), a rare heart complication of neuroendocrine tumors (NETs), can prolong survival. Careful patient selection is crucial for this high-risk cardiac surgery.

Keywords:
Hedinger syndromecarcinoid heart disease (CD)hemoperfusionneuroendocrine tumors (NET)octreotidetricuspid valve

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Area of Science:

  • Cardiology
  • Cardiac Surgery
  • Oncology

Background:

  • Hedinger syndrome (HS), also known as carcinoid heart disease (CD), is a rare but serious complication of neuroendocrine tumors (NETs).
  • HS significantly impacts cardiac function, particularly the tricuspid valve and right ventricle.
  • Surgical intervention is often necessary for patients with advanced HS.

Purpose of the Study:

  • To report surgical strategies and midterm outcomes for patients with Hedinger syndrome.
  • To evaluate the feasibility and safety of different surgical approaches for HS.
  • To identify factors influencing survival in HS patients undergoing cardiac surgery.

Main Methods:

  • Retrospective analysis of 11 patients with HS who underwent cardiac surgery between 2005 and 2023.
  • Detailed review of surgical procedures including tricuspid valve replacement, reconstruction, and novel implantation techniques (TVI).
  • Evaluation of concomitant procedures, management of right ventricular failure (e.g., ECMO, hemoperfusion), and patient outcomes.

Main Results:

  • All patients presented with severe symptoms (NYHA class III-IV) and tricuspid valve involvement.
  • Surgical interventions included tricuspid valve replacement (4), reconstruction (3), and tricuspid valve implantation (4).
  • 30-day mortality was 18%, with a median follow-up of 2 years and an overall 72.7% mortality during follow-up.

Conclusions:

  • Cardiac surgery for HS is a high-risk but potentially life-prolonging procedure.
  • The choice of surgical strategy and patient selection are critical for successful outcomes.
  • Further research with larger cohorts and extended follow-up is necessary to optimize HS management.