Anesthesia Toolbox Question:

55-year-old patient has carcinoid heart disease. Which finding on echocardiography is MOST characteristic of carcinoid heart disease?

A. Left ventricular dysfunction  X

B. Tricuspid regurgitation  ✔

C. Mitral stenosis  X

Read the discussion and key information below.

Discussion:

Carcinoid Heart Disease is characterized by tricuspid/pulmonary valve disease, right ventricular dysfunction, and pulmonary hypertension. In the presence of hepatic metastases from a primary carcinoid tumor, hormonally active tumor cells secrete serotonin, tachykinins, bradykinins, and prostaglandins that bypass degradation in the liver and reach the right side of the heart. These substances can lead to carcinoid syndrome and pathophysiology of the heart. Cardiac disease is produced by endothelial injury with subsequent deposition of plaque and fibrosis on the right-sided valves, papillary muscles, chordae tendineae, and endocardium. The end result is tricuspid and pulmonary regurgitation from the fibrotic valves; although, concomitant valvular stenosis can also be present. These cardiac lesions lead to right ventricular failure in full blown cases. Additional features of carcinoid heart disease including coronary vasospasm, arrhythmias, and direct myocardial tumors. Carcinoid heart disease occurs in 50% of patient with carcinoid symptoms and is a major cause of morbidity and mortality. The left side of the heart usually remains relatively unaffected, as the lungs are thought to inactivate the bioactive substances responsible for endothelial changes. The presence of left sided Carcinoid heart disease implies an endobronchial tumor, a persistent cardiac shunt, or very severe tumor burden. Presenting symptoms include fatigue, dyspnea, peripheral edema and ascites.

Key Information:

Carcinoid Heart Disease occurs in the presence of hepatic metastases from primary carcinoid tumor that secreting vasoactive substances that bypass hepatic metabolism and travel via inferior vena cava to the right side of the heart. These affect the tricuspid and pulmonic valves. Right ventricular dysfunction and pulmonary hypertension can be seen in more severe cases of carcinoid heart disease.

References: 

Castillo J, Silvay G, Weiner M. Anesthetic management of patients with carcinoid syndrome and carcinoid heart disease: the Mount Sinai Algorithm. Journal of Cardiothoracic and Vascular Anesthesia. 2018;32(2):1023-31. PMID 29273478

Ram P, Penalver JL, et al. Carcinoid heart disease: review of current knowledge. Tex Heart Inst J. 2019;4691):21-7.

CA 1 Mini PBLD 17.2 Carcinoid