U3 L4 — Carcinoid

What is carcinoid, and what do the tumors release?

Malignant, metastatic neuroendocrine tumors that release:

1. Tryptophan

2. Serotonin

These create a toxic effect on the endothelial lining

List the 5 areas where carcinoid tumors typically originate.

• GI tract — intestines

• Ovaries

• Bronchi

• Pancreas

• Biliary tract

How is carcinoid diagnosed?

• Tissue biopsy

• Laboratory testing

List the 3 laboratory tests used for carcinoid.

• Urinary 5-HIAA

• Kinin

• Serotonin

What respiratory symptom may occur with carcinoid, and what syndrome may develop?

• Dyspnea

• Carcinoid syndrome

List the 6 findings associated with carcinoid syndrome.

• Flushing

• Hypotension

• Diarrhea

• Wheezing

• Bronchospasm

• Endocardial plaque formation

How common is carcinoid heart disease in patients with neuroendocrine carcinoid tumors?

It is present in about 60% of patients.

What may develop in the late stages of carcinoid heart disease, and list the 4 findings associated with it.

Heart failure, with:

1. JVD

2. Hepatomegaly

3. Peripheral edema

4. Ascites

What may be heard on auscultation in carcinoid heart disease?

Variable murmurs.

What happens when carcinoid tumor secretions enter the right heart?

Fibrous white plaque deposits form and affect the valves and endocardium

Which 2 valves are primarily affected by carcinoid heart disease?

• Tricuspid valve

• Pulmonic valve

List the 3 additional locations where carcinoid plaque may build up.

• Endocardium

• Pulmonary arteries

• IVC

Why is carcinoid heart disease predominantly right-sided?

Because the tumor secretions are deactivated by the lungs

List the 2 exceptions in which carcinoid disease may affect the left side of the heart.

• Bronchial/lung metastases

• Right-to-left shunting

How do the right-sided valve leaflets appear in carcinoid heart disease?

They are:

• Thickened

• Retracted

• Semi-open

The leaflets may become totally fixed in a partially open position.

What tricuspid valve disease is typically seen with carcinoid, and what Doppler findings may occur?

• Severe TR

• Mild TS

• Rapid equalization of pressures on Doppler

• To-and-fro flow

What pulmonic valve abnormalities may occur with carcinoid, and what Doppler finding occurs as PI becomes more severe?

• Varying degrees of PI and PS

• Increasing PI severity produces a steep deceleration slope

List the 5 additional echo findings of carcinoid heart disease.

• RA dilation

• RV dilation

• Right-sided volume overload

• Abnormal septal motion

• Endocardial plaque

Where may a carcinoid tumor itself be seen on echo?

It may be embedded in the myocardium.

List the 3 carcinoid mimickers.

• Fen-phen

• Pergolide

• Ergot alkaloids

What 2 treatments are listed for carcinoid valve disease?

• Valve replacement

• Balloon valvuloplasty

List the 4 treatments used to treat the carcinoid tumor.

• Hepatic artery embolization

• Chemotherapy

• Counter-regulatory hormones

• Surgical excision

What is the purpose of hepatic artery embolization?

To intentionally decrease blood flow feeding hepatic metastases.

What medication is used for right-sided heart failure in carcinoid?

Diuretics