Friday, April 28, 2017

Review of carcinoid heart disease

The full text of this review is available only by subscription, but the audio summary is open access.

Here are a few key points:

Carcinoid tumors (CT) tend to grow slowly and remain asymptomatic for long periods. Carcinoid syndrome (CS), the clinical state resulting from release of mediators by the tumor, generally does not occur until it metastasizes to the liver, though there are exceptions to this rule. CS consists of vasomotor disturbances, flushing, bronchospasm and diarrhea and is primarily mediated by serotonin although other mediators including kinins, histamine and prostaglandins are involved.

Carcinoid heart disease (CHD) is a valvulopathy (most frequently the tricuspid, followed in order of frequency by the pulmonic and then the left sided valves) caused by an inflammatory and fibroproliferative response of valvular endocardium to the circulating mediators, most notably serotonin. Regurgitation is the primary lesion affecting the valves although stenosis can occur to a lesser degree.

It is estimated that 50% of patients with CS go on to develop CHD though that number may be decreasing with improvements in recognition and treatment of CS. Once CHD develops it tends to progress rapidly and worsens the prognosis of patients with CS.

Transcatheter embolization and surgical debulking of liver metastases are indicated in some patients but become less viable options if hepatic congestion has developed due to the risk of acute liver failure and bleeding.

Valve replacement may improve the outlook for selected patients.

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