Anaplastic Metastasis of Left-Sided Cardiac Carcinoid with Rapid Tricuspid Valve Involvement after Valve Replacement

Carcinoid tumors are rare and slow-growing neuroendocrine tumors. In the United States, the prevalence is reported at 2 cases per 100,000 persons. The classic carcinoid syndrome occurs in up to 5% of cases and its vasoactive effects can induce flushing, secretory diarrhea, bronchospasm and hypotension1. Carcinoid induced cardiac dysfunction is rare and involves development of plaques on the tricuspid and pulmonic valves, often manifesting as tricuspid insufficiency and pulmonic stenosis (TIPS). More specifically, these plaques cause hemodynamic dysfunction as a result of thickening and restricted motion of the valve leaflets. Of all cardiac carcinoid cases, the left-heart valves are affected in less than 10% of cases, due to atrial right-to-left shunt or primary bronchial carcinoid. Despite appropriate valve replacement therapy, rarely does the subsequent congestive heart failure resolve; necessitating an ongoing balance between cardiac treatment options and chemotherapy. Herein, we present a case of aggressive carcinoid heart disease manifesting as left-sided valvular plaque with subsequent development of severe tricuspid valve regurgitation after triple-valve replacement surgery. We will also review the treatment strategy in this patient with aggressive carcinoid syndrome.