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Hedinger Syndrome—Lessons Learnt: A Single-Center Experience

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...

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Autores principales: El Gabry, Mohamed, Arends, Sven, Shehada, Sharaf-Eldin, Lahner, Harald, Kamler, Markus, Wendt, Daniel, Spetsotaki, Konstantina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10607344/
https://www.ncbi.nlm.nih.gov/pubmed/37887860
http://dx.doi.org/10.3390/jcdd10100413
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author El Gabry, Mohamed
Arends, Sven
Shehada, Sharaf-Eldin
Lahner, Harald
Kamler, Markus
Wendt, Daniel
Spetsotaki, Konstantina
author_facet El Gabry, Mohamed
Arends, Sven
Shehada, Sharaf-Eldin
Lahner, Harald
Kamler, Markus
Wendt, Daniel
Spetsotaki, Konstantina
author_sort El Gabry, Mohamed
collection PubMed
description 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.
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spelling pubmed-106073442023-10-28 Hedinger Syndrome—Lessons Learnt: A Single-Center Experience El Gabry, Mohamed Arends, Sven Shehada, Sharaf-Eldin Lahner, Harald Kamler, Markus Wendt, Daniel Spetsotaki, Konstantina J Cardiovasc Dev Dis Article 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. MDPI 2023-10-01 /pmc/articles/PMC10607344/ /pubmed/37887860 http://dx.doi.org/10.3390/jcdd10100413 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
El Gabry, Mohamed
Arends, Sven
Shehada, Sharaf-Eldin
Lahner, Harald
Kamler, Markus
Wendt, Daniel
Spetsotaki, Konstantina
Hedinger Syndrome—Lessons Learnt: A Single-Center Experience
title Hedinger Syndrome—Lessons Learnt: A Single-Center Experience
title_full Hedinger Syndrome—Lessons Learnt: A Single-Center Experience
title_fullStr Hedinger Syndrome—Lessons Learnt: A Single-Center Experience
title_full_unstemmed Hedinger Syndrome—Lessons Learnt: A Single-Center Experience
title_short Hedinger Syndrome—Lessons Learnt: A Single-Center Experience
title_sort hedinger syndrome—lessons learnt: a single-center experience
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10607344/
https://www.ncbi.nlm.nih.gov/pubmed/37887860
http://dx.doi.org/10.3390/jcdd10100413
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