PMID- 37887860
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20231029
IS  - 2308-3425 (Electronic)
IS  - 2308-3425 (Linking)
VI  - 10
IP  - 10
DP  - 2023 Oct 1
TI  - Hedinger Syndrome-Lessons Learnt: A Single-Center Experience.
LID - 10.3390/jcdd10100413 [doi]
LID - 413
AB  - 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((R))-CytoSorb((R)) 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.
FAU - El Gabry, Mohamed
AU  - El Gabry M
AD  - Department of Thoracic and Cardiovascular Surgery, West-German Heart and Vascular 
      Centre, 45147 Essen, Germany.
FAU - Arends, Sven
AU  - Arends S
AUID- ORCID: 0000-0003-1300-7327
AD  - Department for Anesthesiology and Intensive Care Medicine, University of 
      Duisburg-Essen, 45147 Essen, Germany.
FAU - Shehada, Sharaf-Eldin
AU  - Shehada SE
AUID- ORCID: 0000-0003-1638-0493
AD  - Department of Thoracic and Cardiovascular Surgery, West-German Heart and Vascular 
      Centre, 45147 Essen, Germany.
FAU - Lahner, Harald
AU  - Lahner H
AD  - Department of Endocrinology and Metabolism, University Hospitals Duisburg-Essen, 
      45147 Essen, Germany.
FAU - Kamler, Markus
AU  - Kamler M
AUID- ORCID: 0000-0001-6608-8496
AD  - Department of Thoracic and Cardiovascular Surgery, West-German Heart and Vascular 
      Centre, 45147 Essen, Germany.
FAU - Wendt, Daniel
AU  - Wendt D
AD  - Department of Thoracic and Cardiovascular Surgery, West-German Heart and Vascular 
      Centre, 45147 Essen, Germany.
AD  - CytoSorbents Europe GmbH, 12587 Berlin, Germany.
FAU - Spetsotaki, Konstantina
AU  - Spetsotaki K
AUID- ORCID: 0000-0001-8965-4702
AD  - Department of Thoracic and Cardiovascular Surgery, West-German Heart and Vascular 
      Centre, 45147 Essen, Germany.
LA  - eng
PT  - Journal Article
DEP - 20231001
PL  - Switzerland
TA  - J Cardiovasc Dev Dis
JT  - Journal of cardiovascular development and disease
JID - 101651414
PMC - PMC10607344
OTO - NOTNLM
OT  - Hedinger syndrome
OT  - carcinoid heart disease (CD)
OT  - hemoperfusion
OT  - neuroendocrine tumors (NET)
OT  - octreotide
OT  - tricuspid valve
COIS- The authors declare no conflict of interest.
EDAT- 2023/10/27 12:43
MHDA- 2023/10/27 12:44
PMCR- 2023/10/01
CRDT- 2023/10/27 07:06
PHST- 2023/08/29 00:00 [received]
PHST- 2023/09/26 00:00 [revised]
PHST- 2023/09/28 00:00 [accepted]
PHST- 2023/10/27 12:44 [medline]
PHST- 2023/10/27 12:43 [pubmed]
PHST- 2023/10/27 07:06 [entrez]
PHST- 2023/10/01 00:00 [pmc-release]
AID - jcdd10100413 [pii]
AID - jcdd-10-00413 [pii]
AID - 10.3390/jcdd10100413 [doi]
PST - epublish
SO  - J Cardiovasc Dev Dis. 2023 Oct 1;10(10):413. doi: 10.3390/jcdd10100413.