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

Institutions

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
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Inferior Vena Cava Stenosis Mimicking Right Heart Failure in Carcinoid Heart Disease

Mercedes Pérez Lafuente, Núria Vallejo, Gerard Oristrell, Alejandro Garcia-Alvarez et al.
JACC Case Reports
Neuroendocrine Tumor Research Advances
article

Inferior Vena Cava Stenosis Mimicking Right Heart Failure in Carcinoid Heart Disease

Mercedes Pérez Lafuente, Núria Vallejo, Gerard Oristrell, Alejandro Garcia-Alvarez, Gerard Martí-Aguasca, Sofía Vila-Sanjuán, Jorge Hernando, Jaume Capdevila, Ignacio Ferreira-González, Hug Cuéllar
article en

Abstract

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.

JACC Case Reports
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)
Good health and well-being
Openalex Percentile: Top 20%
Neuroendocrine Tumor Research Advances
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.