Carcinoid heart disease
ICD-10 E34.0 · ICD-11 5B10.1

Treatment of Carcinoid Heart Disease in Carcinoid Syndrome with Severe Tricuspid or Pulmonary Valvular Involvement

Clinical Scenario

This protocol addresses patients with carcinoid syndrome who have developed severe carcinoid valvular heart disease of the tricuspid or pulmonary valve, with an expected survival of 12 months or longer.

Carcinoid syndrome is the primary underlying condition shaping the surgical and peri-operative strategy in this setting. Both symptomatic patients and those with asymptomatic but progressive right ventricular dysfunction or dilatation are considered — provided the survival threshold is met.
Treatment Approach (partial overview)

The strategy combines peri-operative intravenous somatostatin analogue coverage — initiated before the procedure to prevent a carcinoid crisis — with valve replacement surgery targeting the affected tricuspid and/or pulmonary valve. The full protocol specifies exact timing, continuation criteria, and the complete post-operative management…

Instant Access to Structured Evidence-Based Regimens

References

  1. Valve replacement surgery is recommended in symptomatic patients with severe carcinoid tricuspid or pulmonary VHD and an expected survival ≥12 months.
  2. Valve replacement surgery should be considered in patients with asymptomatic severe carcinoid tricuspid or pulmonary VHD, progressive RV dysfunction/dilatation, and an expected survival ≥12 months.
  3. Administration of i.v. somatostatin analogues (e.g. octreotide) is recommended to avoid a peri-operative carcinoid crisis.
  4. The infusion should be started on the morning of the procedure (up to 12 h pre-operatively), continued throughout the procedure (surgery, pre-operative coronary angiography, pacemaker implantation), and post-operatively for at least 48 h following valve surgery or until stable if a carcinoid crisis is triggered post-operatively.

DOI: 10.1093/eurheartj/ehac244

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