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Management and outcomes of carcinoid heart disease with liver metastases of midgut neuroendocrine tumours
  1. Gaspard Suc1,2,3,
  2. Agnès Cachier2,
  3. Olivia Hentic4,
  4. Baptiste Bazire1,2,3,
  5. Aurélie Sannier1,3,5,
  6. Clémence Delhomme1,2,3,
  7. Patrick Nataf1,3,6,
  8. Jamila Laschet1,3,
  9. Lydia Deschamps5,
  10. Eric Garbarz2,
  11. Phalla Ou1,3,7,
  12. Giuseppina Caligiuri2,3,
  13. Bernard Iung1,2,3,
  14. Philippe Ruszniewski1,4,
  15. Louis de Mestier1,4,
  16. Dimitri Arangalage1,2,3
  1. 1Université Paris Cité, Paris, France
  2. 2Cardiology, Bichat and Beaujon Hospitals, APHP, Paris, France
  3. 3UMRS 1148, INSERM, Paris, France
  4. 4Pancreatology, Beaujon Hospital, AP-HP, Paris, France
  5. 5Pathology, Bichat Hospital, AP-HP, Paris, France
  6. 6Cardiac Surgery, Bichat Hospital, AP-HP, Paris, France
  7. 7Radiology, Bichat Hospital, AP-HP, Paris, France
  1. Correspondence to Dr Dimitri Arangalage, Cardiology, Bichat Hospital, APHP, Paris, France; dimitri.arangalage{at}


Objective Despite recent advances in surgical and interventional techniques, knowledge on the management of carcinoid heart disease (CHD) remains limited. In a cohort of patients with liver metastases of midgut neuroendocrine tumours (NETs), we aimed to describe the perioperative management and short-term outcomes of CHD.

Methods From January 2003 to June 2022, consecutive patients with liver metastases of midgut NETs and severe CHD (severe valve disease with symptoms and/or right ventricular enlargement) were included at Beaujon and Bichat hospitals. All patients underwent clinical evaluation and echocardiography.

Results Out of 43 (16%) consecutive patients with severe CHD and liver metastases of midgut NETs, 79% presented with right-sided heart failure. Tricuspid valve replacement was performed in 26 (53%) patients including 19 (73%) cases of combined pulmonary valve replacement. The 30-day postoperative mortality rate was high (19%), and preoperative heart failure was associated with worse survival (p=0.02). Epicardial pacemakers were systematically implanted in operated patients and 25% were permanently paced. A postoperative positive right ventricular remodelling was observed (p<0.001). A greater myofibroblastic infiltration was observed in pulmonary versus tricuspid valves (p<0.001), suggesting that they may have been explanted at an earlier stage of the disease than the tricuspid valve, with therefore potential for evolution.

Conclusions We observed a high postoperative mortality rate and baseline right-sided heart failure was associated with worse outcome. In surviving patients, a positive right ventricular remodelling was observed. Prospective, multicentre studies are warranted to better define the management strategy and to identify biomarkers associated with outcome in CHD.

  • Cardiac surgery
  • Heart Valve Diseases
  • Pulmonary Valve Insufficiency
  • Tricuspid Valve Insufficiency

Data availability statement

Data are available upon reasonable request.

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  • Contributors Substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data for the work: All authors. Drafting the work or revising it critically for important intellectual content: All authors. Final approval of the version to be published: All authors. Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved: All authors. DA is responsible for the overall content as guarantor.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting or dissemination plans of this research.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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