Carcinoid Heart Disease and Review of Literature

Robin Fernandes, Srinivasan Sattiraju, Sanjay Mehta* and Woubeshet Ayenew

Carcinoid Heart Disease and Review of Literature.

Carcinoid Heart Disease (CHD) is one of the rare causes of acquired valvular heart disease. CHD develops as a systemic manifestation of metastatic neuroendocrine tumors (NETs). When these tumors metastasize to the liver, they can release vasoactive amines into the systemic circulation, which can result in carcinoid syndrome and CHD. The key for diagnosing CHD is a thorough history and physical examination supplemented with characteristic echocardiographic findings. We report a case with classic echocardiographic features of CHD, which unfolded the diagnosis of metastatic carcinoid tumor.

Carcinoid tumors are rare neuroendocrine tumors with an incidence of 1.2-2.1% per 100,000
population per year. The term “Carcinoid” generally implies well-differentiated NETs that arise from enterochromaffin cells within the gastrointestinal tract and, less commonly, in the bronchopulmonary system. The tumors often metastasize to the liver resulting in the release
of vasoactive amines (serotonin, bradykinin and histamine) into the systemic circulation. The
triad of cutaneous flushing, gastrointestinal hypermotility, and bronchospasm characterizes carcinoid syndrome. At diagnosis, 20-30% of cases may present with carcinoid syndrome. Up to
50% of patients with carcinoid syndrome can develop CHD, which is a major cause of morbidity and mortality. About 20% of cases may present as CHD at the time of diagnosis. Herein, we report classic findings of carcinoid heart disease.

In patients with carcinoid syndrome, Echocardiography is the gold standard for diagnosing CHD, given the pathognomonic appearance of the valve lesions. Valve surgery is the only effective treatment option for patients with symptomatic CHD and has been shown to improve survival. CHD warrants aggressive treatment as the disease carries high morbidity and mortality.

Heart Res Open J. 2016; 4(1): 1-5. doi: 10.17140/HROJ-4-132