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Updated: Apr 11, 2026

Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
Inferior Vena Cava Stenosis Mimicking Right Heart Failure in Carcinoid Heart Disease
Sofía Vila-Sanjuán1, Nuria Vallejo1, Gerard Martí-Aguasca1
1Department of Cardiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
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.
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