Carcinoid heart disease (CHD) is a rare cardiac manifestation occurring in patients with advanced NENs and the carcinoid syndrome, usually involving the right-sided heart valves and eventually leading to right heart failure. The pathophysiology of CHD is still obscure and believed to be multifactorial. Patients with the rare diagnosis of CHD should be treated in specialized centres by a multidisciplinary team with experience in the treatment of this complex condition. Without a timely intervention, NET patients with CHD will eventually develop progressive right heart failure in parallel with a significant decrease in their life expectancy compared with those NET patients without CHD. Medical treatment includes firstly somatostatin analogues (SSA), based on NET cell ability to express specific somatostatin receptors on their surface membrane. Treatment with SSA improves the symptoms of CS as well as the negative haemodynamic impact of tumour vasoactive agents on CHD and on the development of heart failure. In the perioperative setting, continuous SSA infusion is of outmost importance, with a slow tapering down before treatment discontinuation. The SSA infusion is aimed at reducing serotonin release, optimizing surgical outcome by reducing perioperative complications such as hypotension, carcinoid crisis and death. Recently, telotristat etiprate, a novel serotonin synthesis inhibitor, has been reported as being highly effective for alleviating diarrhoea in patients with CS inadequately controlled by SSAs alone. This new drug may be promising for patients with CHD with intractably elevated levels of serotonin. Antihistamines to prevent flushing and bronchospasm, and corticosteroids to reduce bradykinin production may be used. The use of loop diuretics, together with fluid and salt, restriction and compression stockings, may initially relieve the symptoms of right heart failure. A better understanding of the molecular mechanisms underlying the progression of fibrosis in CHD may lead to the development of appropriate targets for targeted molecular therapy.