Unusual cyanosis in a boy with congenital pulmonary stenosis and tricuspid insufficiency. Fatal outcome after angiocardiography

Invasive carcinoid tumor of the heart

“…Cardiac manifestations of the carcinoid syndrome were first recognized in 1952 when a patient with metastatic carcinoid was found to have severe pulmonic valvular stenosis and tricuspid insufficiency [4]. The valvular heart disease is due to irreversible endocardial fibrosis, similar to the fibrosis noted in the gut wall, in the retroperitoneum, and around the mesenteric blood vessels in patients with the malignant syndrome.…”

Siegfried Oberndorfer and the Evolution of Carcinoid Disease

“…In 1943, Samuel Millman 36 described a 44-year-old female patient with flushing who was found on autopsy to have multiple ileal carcinoid tumors with metastatic spread to the liver and lymph nodes. In 1952, Biö rck et al 13 described carcinoid heart disease in a 19-year-old man who had pulmonary stenosis with tricuspid insufficiency and cyanosis. The patient was dyspneic at rest and died during cardiac angiography.…”

“…Thereafter, the protean clinical manifestations of serotonin-including flushing-became recognized as the biochemical basis for many of the symptoms. In 1952, Gunnar Biö rck et al 13 established the relationship of carcinoid heart disease with carcinoid tumors; in 1961, Charles G. Moertel et al 14 identified the association of fibrosis with carcinoid tumors.…”

The optimal use of cardiac imaging in the quantification of carcinoid heart disease

“…Carcinoid heart disease, first described by Biorck et al (1952), is a major cause of morbidity and mortality in patients with carcinoid syndrome, and in up to 20% of patients, it may be the presenting feature of the disease (Lundin et al 1988). Cardiac involvement is thought to occur in 20-50% of patients with carcinoid syndrome (Connolly & Pellikka 2006) and is characterised by a fibrous reaction causing retraction and fixation of the right-sided valve leaflets, leading to a combination of regurgitation and stenosis, which ultimately can progress to right heart failure.…”