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.
Vila-Sanjuán et al. (Wed,) studied this question.