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Published on: December 4, 2023
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.
Insights
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.
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.
Background:
Hedinger syndrome (HS) or carcinoid heart disease (CD) is a rare and challenging manifestation of malignant neuroendocrine tumours (NETs) involving the heart. We aimed to report our experience with surgical strategies and midterm results in HS patients.
Methods:
Eleven patients (58 ± 11 (range 41 to 79 years); 5 females) with HS who underwent cardiac surgery in our department between 07/2005 and 05/2023 were analysed.
Results:
All patients showed a New York Heart Association (NYHA) class III-IV and in all the tricuspid valve (TV) was involved. Four patients received a TV replacement, and three TV reconstruction. Recently, to preserve the geometry and function of the compromised right ventricle (RV), we have applied the TV "bio-prosthesis in native-valve" implantation technique with the preservation of the valve apparatus (tricuspid valve implantation: TVI) in four cases. Concomitant procedures included pulmonary valve replacement in four, pulmonary implantation in one, and aortic valve replacement in three cases. To treat RV failure, we adapted a combined TandemHeart®-CytoSorb® haemoperfusion strategy in Patient #10 and venoarterial extracorporeal membrane oxygenation (V-A ECMO) support avoidance, after experiencing an ECMO-induced carcinoid-storm-related death in Patient #8. Mortality at 30 days was 18% (2/11). The median follow up was 2 ± 2.1 years (range 1 month to 6 years) with an overall mortality during the follow-up period of 72.7% (8/11).
Conclusions:
HS surgery, despite being a high-risk procedure, can efficiently prolong survival, and represents a safe and feasible procedure. However, patient selection seems to be crucial. Further follow up and larger cohorts are needed.
