Inferior Vena Cava Stenosis Mimicking Right Heart Failure in Carcinoid Heart Disease
Carcinoid heart disease is a well-recognized complication of serotonin-secreting neuroendocrine tumors. Chronic exposure to vasoactive substances leads to endocardial fibrosis, most commonly affecting right-sided valves. Patients typically present with progressive right heart failure due to severe tricuspid and pulmonary regurgitation. While valvular lesions dominate the clinical picture, serotonin-mediated fibrosis can extend beyond the endocardium to the vascular endothelium. Reports of venous outflow obstruction caused by carcinoid-related fibrosis are exceedingly rare but may produce a phenotype indistinguishable from cardiac decompensation. We describe 2 patients with advanced carcinoid heart disease who developed profound systemic congestion despite normal intracardiac pressures, ultimately attributed to inferior vena cava and renal vein stenosis. This series highlights a diagnostic challenge with important therapeutic implications.
Authors
- Mercedes Pérez Lafuente (ORCID: https://orcid.org/0000-0002-3051-5692)
- Núria Vallejo (ORCID: https://orcid.org/0000-0001-6644-6767)
- Gerard Oristrell (ORCID: https://orcid.org/0000-0003-3217-987X)
- Alejandro Garcia-Alvarez (ORCID: https://orcid.org/0000-0001-5647-043X)
- Gerard Martí-Aguasca
- Sofía Vila-Sanjuán
- Jorge Hernando
- Jaume Capdevila
- Ignacio Ferreira-González
- Hug Cuéllar
Institutions
- Hebron University (PS)
- Universitat Autònoma de Barcelona (ES)
- Instituto de Salud Carlos III (ES)
- Centro de Investigación en Red en Enfermedades Cardiovasculares (ES)
- Vall d'Hebron Institut de Recerca (ES)
- Vall d'Hebron Hospital Universitari (ES)
Publication Details
- Journal
- JACC Case Reports
- Published
- 2026-04-01
- DOI
- https://doi.org/10.1016/j.jaccas.2026.107765
- Primary Topic
- Neuroendocrine Tumor Research Advances
- Type
- article
- Field-Weighted Citation Impact
- 0.00