Clinical scenario

This protocol addresses patients with carcinoid syndrome who have developed carcinoid heart disease (CHD) complicated by right-sided heart valve regurgitation or stenosis and resulting right heart failure, in the context of elevated urinary 5-HIAA and elevated NT-proBNP.

Why this protocol is reached

First-line management — combining aggressive reduction of tumoural hormonal secretion with right heart failure treatment (diuretic and aldosterone antagonist therapy, fluid and salt restriction) — did not achieve adequate improvement of right heart failure symptoms. This protocol defines the step taken after that failure.

Approach

Once conservative management has proven insufficient in advanced carcinoid heart disease, a surgical intervention directed at the affected cardiac valves represents the established next step and is considered the most effective treatment for this stage of CHD. The specific operative strategy and valve selection are detailed in the full protocol.

Treatment goal

The primary clinical target is marked symptomatic improvement, with post-operative re-classification to an improved NYHA functional class.

References

Carcinoid heart disease (CHD) is a rare and complex cardiac complication occurring in patients with advanced NETs and CS, usually manifesting mainly as right-sided heart valves regurgitation/stenosis and eventually leading to right heart failure (RHF).
Surgical valve replacement is the established CHD treatment.
Cardiac surgery, which primarily consists of valve replacement, is the most effective treatment option for advanced CHD.
There is marked symptomatic improvement after valve replacement surgery in CHD patients, with majority of patients being re-classified as NYHA class I post-operatively.

DOI: 10.1111/jne.13146

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Carcinoid Heart Disease with Right-Sided Valve Regurgitation: When Initial Heart Failure Management Has Not Been Sufficient

References