From the Guidelines
Carcinoid heart disease, specifically tricuspid regurgitation, is the common cause of heart failure in carcinoid syndrome.
Key Points
- Carcinoid syndrome is associated with the secretion of serotonin, histamine, or tachykinins into the systemic circulation, causing episodic flushing and diarrhea 1.
- Approximately 10% to 30% of patients with carcinoid syndrome develop valvular cardiac complications, consisting of tricuspid regurgitation and/or pulmonary stenosis 1.
- A study of 250 patients with carcinoid syndrome showed that those with 5-HIAA levels of 300 mcmol or greater and with 3 or more flushing episodes per day were more likely to have carcinoid heart disease 1.
- Tricuspid regurgitation is the most common valvular lesion in carcinoid heart disease, occurring in up to 59% of patients with carcinoid syndrome 1.
- The fibroproliferative response in carcinoid heart disease is mediated via the 5-HT2B receptor, similar to anorexiant drug-related valvulopathy 1.
- A cardiology consultation and echocardiogram should be considered in patients with carcinoid syndrome and signs and symptoms of heart disease or with planned major surgery 1.
From the Research
Carcinoid Heart Disease
The common cause of heart failure in carcinoid syndrome is carcinoid heart disease, which occurs in over 65% of patients with the syndrome 2. This condition is characterized by fibrous thickening of cardiac valves, leading to heart failure.
Pathophysiology
The pathophysiology of carcinoid heart disease is attributed to the paraneoplastic effects of vasoactive substances released by the malignant cells 3. These substances can cause desmoplastic reactions to nearby tissues, leading to valvular destruction and right-sided heart failure 4.
Clinical Manifestations
The clinical manifestations of carcinoid heart disease include:
- Valvular destruction leading to valvular regurgitation and stenosis
- Right-sided heart failure
- Metastatic carcinoid disease 3
- Tricuspid insufficiency and pulmonary stenosis (TIPS) 4
Management
The management of carcinoid heart disease involves medical and surgical treatment and requires a multidisciplinary approach for optimized care 3, 5. Early diagnosis and timely surgical intervention in selected patients are of utmost importance and offer a survival benefit 5. Valve replacement surgery is the most effective option to alleviate cardiac symptoms and contribute to survival outcomes in patients with advanced carcinoid heart disease 5.