Carcinoid Right Heart Disease

Carcinoid heart disease is an unusual cause of right heart failure. We present the case of a 53-year-old man with a history of metastatic carcinoid tumors originating from the appendix, status post-chemotherapy, and liver embolizations. The patient presented with a right-sided valvular disease with severe pulmonic valve regurgitation, right ventricle dilation and pulmonary hypertension. He had carcinoid syndrome well controlled with long-acting lanreotide. He underwent tricuspid valve and pulmonary valve replacement with a stented bioprosthetic valve, maze and cardio-septal right ventricular outflow tract patch with an improvement of ventricular dysfunction. These findings supported the diagnosis of carcinoid heart disease presenting with pure right heart failure. The patient was symptom-free due to lanreotide, but ultimately, valve surgery is the preferred definite treatment in suitable patients. Carcinoid heart disease requires a high index of suspicion, and valve surgery is the only definitive treatment.