From the Guidelines
A patient with Carcinoid Heart Disease typically presents with symptoms such as progressive dyspnea, peripheral edema, and facial flushing episodes, and management should include octreotide LAR and consideration of cardiac surgery to improve morbidity, mortality, and quality of life. The patient's medical history often reveals a small bowel neuroendocrine tumor diagnosed prior with hepatic metastases, and initial evaluation shows elevated 5-HIAA urinary levels and serum chromogranin A, with echocardiography demonstrating severe tricuspid regurgitation and moderate pulmonary valve stenosis, confirming carcinoid heart disease 1. Some key points to consider in the management of Carcinoid Heart Disease include:
- The development of Carcinoid Heart Disease leads to dramatic worsening of prognosis in patients with neuroendocrine tumors, with a 3-year survival of 31% compared to 68% in patients without Carcinoid Heart Disease 1.
- Approximately 50% to 66% of patients with carcinoid syndrome develop valvular cardiac complications consisting of tricuspid regurgitation and/or pulmonary stenosis 1.
- Management should include octreotide LAR to control carcinoid syndrome symptoms, with additional octreotide as needed for breakthrough flushing, and consideration of cardiac surgery to replace affected valves 1.
- Perioperative management should include continuous octreotide infusion to prevent carcinoid crisis, and postoperative care should focus on improving exercise tolerance and reducing peripheral edema 1. The most recent and highest quality study on this topic is from 2015, which provides guidance on the management of neuroendocrine tumors, including Carcinoid Heart Disease 1. In terms of specific treatment, octreotide LAR 30 mg intramuscularly every 4 weeks is recommended to control carcinoid syndrome symptoms, with additional octreotide 100 mcg subcutaneously as needed for breakthrough flushing. Diuretics, such as furosemide 40 mg daily, may also be prescribed to manage volume overload, and cardiac surgery should be considered to replace affected valves and improve cardiac function 1. Overall, the management of Carcinoid Heart Disease requires a multidisciplinary approach, including medical therapy with octreotide and consideration of cardiac surgery, to improve morbidity, mortality, and quality of life for patients with this condition.
From the Research
Case Study: Carcinoid Heart Disease
A 60-year-old patient with a history of carcinoid syndrome (CS) presents with symptoms of dyspnea and fatigue. The patient's urinary 5-hydroxyindoleacetic acid (5-HIAA) levels are ≥300 µmol/24 h and serum N-terminal pro B-type natriuretic peptide (NT-proBNP) levels are >260 pg/mL, indicating a high risk of developing Carcinoid Heart Disease (CaHD) 2.
Clinical Manifestations
The patient's symptoms are consistent with right-sided heart failure, which is a common manifestation of CaHD 3. The disease is characterized by valvular destruction leading to valvular regurgitation and stenosis, primarily affecting the tricuspid and pulmonic valves 4. The patient's echocardiogram shows thickened right heart valves with limited mobility and regurgitation, confirming the diagnosis of CaHD 3.
Treatment Options
The patient's treatment plan involves a multidisciplinary approach, including medical and surgical options 5, 4. The patient is started on long-acting somatostatin analogs to control symptoms and tumor growth 6. Additionally, the patient is considered for peptide receptor radionuclide therapy (PRRT) and telotristat ethyl to control diarrhea and tumor growth 2, 6. Cardiac surgery, including valve replacement, is also considered as a treatment option to relieve symptoms and improve prognosis 6.
Key Considerations
- Early screening and diagnosis are crucial in managing CaHD, as no existing therapies can reverse the fibrotic damage to the heart once it occurs 2.
- A combination of biomarkers, including NT-proBNP and 5-HIAA, and cardiac imaging are used in screening and diagnosis of CaHD 6.
- Treatment of CaHD requires a multidisciplinary approach, involving cardiologists, oncologists, and surgeons, to optimize patient care 5, 4.