Related Subjects:
|Pulmonary Stenosis
|Pulmonary Regurgitation
|Pulmonary Hypertension
|Tricuspid Stenosis
|Pulmonary Embolism
|Carcinoid Heart Disease
📖 About
- Carcinoid Heart Disease (CHD) is due to prolonged exposure to circulating serotonin (5-HT) and other vasoactive substances from carcinoid tumours. 🧪
- Primarily affects the right heart (tricuspid & pulmonary valves) → leaflet thickening, retraction, fibrosis → regurgitation or stenosis.
- Occurs in up to 50% of patients with carcinoid syndrome, especially with liver metastases (since normally the liver metabolises serotonin). ⚠️
🧬 Aetiology
- Tricuspid Valve → Regurgitation most common (leaflet thickening + retraction). 🔁
- Pulmonary Valve → Often stenosed due to fibrotic narrowing. 🚪
- Left-sided valves → Rare, unless right-to-left shunt (e.g. PFO) allows serotonin to bypass the lungs. 🌬️
🩺 Clinical Features
- Right-sided heart failure:
- Dyspnoea, fatigue 🏃
- Peripheral edema 🦶
- Ascites, abdominal distension 💧
- Carcinoid syndrome symptoms:
- Flushing of face/upper chest 🔴
- Profuse diarrhoea, cramping 💩
- Triggered by food, alcohol, stress 🍷
🔍 Investigations
- Echocardiogram (gold standard):
- Thickened, retracted tricuspid/pulmonary leaflets
- Severe TR + pulmonary stenosis
- Right heart enlargement 🫀
- Biochemical markers:
- ↑ 24-hour urinary 5-HIAA (serotonin metabolite)
- ↑ Chromogranin A
- CT/MRI → for primary tumour & liver metastases. 🩻
🧪 Pathology
- Endocardial plaque-like fibrous thickening → restricted leaflet motion.
- Valve dysfunction = regurgitation (TS/TR) or stenosis (PS).
- Right-sided predominance (tricuspid + pulmonary). Left-sided only if shunt present.
💊 Management
- Medical:
- Somatostatin analogues (octreotide, lanreotide) → ↓ serotonin release → less flushing/diarrhoea 🌙
- Diuretics → control fluid overload 💧
- Interventional:
- Valve replacement (bioprosthetic preferred to avoid thrombosis) 🛠️
- Debulking liver mets (resection or embolisation) → ↓ serotonin production 🏥
📚 References
Cases - Carcinoid Heart Disease (CHD)
- Case 1 - Classic Carcinoid Syndrome with Right-Sided Lesions 🦐:
A 58-year-old man with flushing, diarrhoea, and bronchospasm develops progressive ankle swelling and fatigue. Exam: elevated JVP with prominent ‘v’ waves, pansystolic murmur at the left lower sternal edge, hepatomegaly, and ascites. Echo: severe tricuspid regurgitation and pulmonary stenosis due to thickened, retracted leaflets.
Diagnosis: Carcinoid syndrome with right-sided valvular lesions.
Management: Octreotide for carcinoid syndrome, diuretics for right heart failure, surgical valve replacement if severe symptomatic disease.
- Case 2 - Isolated Pulmonary Valve Disease 🌬️:
A 62-year-old woman with known midgut neuroendocrine tumour presents with exertional dyspnoea and peripheral oedema. Exam: early diastolic murmur at the pulmonary area, parasternal heave. Echo: pulmonary regurgitation and stenosis due to plaque-like deposits on pulmonary valve.
Diagnosis: Pulmonary valve involvement in carcinoid heart disease.
Management: Control of tumour with somatostatin analogues; valve replacement if right ventricular dysfunction develops.
- Case 3 - Left-Sided Involvement (Uncommon) ❤️:
A 65-year-old man with bronchial carcinoid tumour (not filtered by liver) develops progressive dyspnoea and orthopnoea. Exam: mid-diastolic murmur at the apex, early diastolic murmur at the aortic area. Echo: carcinoid-related thickening of mitral and aortic valves causing regurgitation.
Diagnosis: Left-sided carcinoid valvular disease due to bronchial primary (bypassing hepatic metabolism).
Management: Somatostatin analogues for tumour control, surgical replacement of affected valves, multidisciplinary oncology + cardiology input.
Teaching Commentary 🧠
Carcinoid heart disease = endocardial fibrosis from serotonin and vasoactive substances secreted by carcinoid tumours.
- Typically affects right-sided valves (tricuspid regurgitation, pulmonary stenosis/regurgitation).
- Left-sided lesions occur if serotonin bypasses hepatic metabolism (bronchial carcinoid or PFO).
- Suspect in patients with carcinoid syndrome (flushing, diarrhoea, bronchospasm) who develop right heart failure signs.
Dx: Echocardiography + raised urinary 5-HIAA.
Mx: Tumour control (somatostatin analogues, surgery, PRRT), diuretics, valve replacement when symptomatic.
