Carcinoid Heart Disease: A Case-based Contemporary Review of Diagnosis, Imaging and Management

Carcinoid heart disease (CHD) is a major cause of morbidity and mortality in patients with carcinoid syndrome. It is characterized by serotonin-mediated fibrotic thickening and retraction of the valvular endocardium, most commonly involving the tricuspid valve and resulting in severe tricuspid regurgitation, often with associated stenosis. Pulmonary valve disease is also common. We performed a focused PubMed search of English-language studies published between January 2000 and January 2026, focusing on clinical studies, society guidance documents, expert consensus papers, and major reviews relevant to CHD. Given the heterogeneity of the available literature, the evidence was synthesized narratively. This review, illustrated by two clinical cases, summarizes the current diagnostic and therapeutic approach to CHD, with particular emphasis on the role of multimodality imaging and multidisciplinary management. Because CHD may progress over time, early detection and regular follow-up are crucial. Screening should integrate biomarkers, particularly N-terminal pro–B-type natriuretic peptide, with focused right-heart transthoracic echocardiography. Echocardiography remains the main imaging technique for diagnosis and follow-up, allowing assessment of valve morphology and dysfunction, right ventricular size and function, and disease severity. In selected patients, transesophageal echocardiography, cardiovascular magnetic resonance, and cardiac computed tomography provide additional information for anatomical definition and procedural planning. Management requires control of hormonal activity, treatment of right-sided heart failure, and timely referral for valve intervention. Transcatheter therapies may be considered in selected anatomies, although evidence on long-term outcomes is still limited.