What is the management of carcinoid heart disease?

Management of Carcinoid Heart Disease

Patients with carcinoid syndrome should be screened for carcinoid heart disease (CHD) with NT-proBNP and echocardiography, and those with confirmed CHD should be referred to specialized cardiology centers for evaluation and possible cardiac surgery. 1

Epidemiology and Clinical Presentation

Carcinoid heart disease is a serious complication affecting patients with neuroendocrine tumors (NETs) and carcinoid syndrome. Key epidemiological and clinical features include:

• Prevalence has decreased from 50-70% to about 20% of carcinoid syndrome patients due to improved treatments 1
• Up to 20% of patients with carcinoid syndrome present with CHD at diagnosis 1
• Most commonly affects right-sided heart valves (tricuspid more than pulmonary) 1
• Left-sided lesions occur in up to 15% of carcinoid syndrome patients but in 47% of CHD patients 1
• Significantly worsens prognosis: 3-year survival of 31% with CHD vs 68% without CHD 1
• Clinical presentation includes:
  • Peripheral edema
  • Ascites
  • Pulsatile hepatomegaly
  • Heart murmurs (though these may be difficult to detect due to low right heart velocities) 1

Diagnostic Approach

1. Biomarker Screening:

   • NT-proBNP is highly recommended (cut-off level of 260 pg/ml) to rule out CHD morbidity 1
   • Urinary 5-HIAA has high sensitivity (100%) but low specificity for CHD 1
   • Patients with 5-HIAA levels ≥300 μmol/24h and ≥3 flushing episodes daily have higher risk of CHD 1
   • Chromogranin A measurement may be considered 2

2. Cardiac Imaging:

   • Echocardiography is the gold standard for diagnosis 1
   • Should be performed at diagnosis and during follow-up 1
   • In pulmonary carcinoid, both left and right-side valves should be screened 1
   • Accurate echocardiographic evaluation should always be performed before surgical procedures 1

3. Cardiology Consultation:

   • Should be considered in patients with carcinoid syndrome with signs/symptoms of heart disease 1
   • Especially important before planned major surgery 1

Management Algorithm

1. Medical Management

• Somatostatin Analogs (First-Line):

  • Octreotide LAR 20-30 mg IM every 4 weeks or lanreotide 120 mg deep SC every 4 weeks 2, 3
  • Short-acting octreotide (150-250 μg SC three times daily) for breakthrough symptoms 2
  • These medications help control carcinoid syndrome symptoms and may indirectly slow progression of CHD by reducing serotonin levels 2

• Heart Failure Management:

  • Diuretics are the mainstay for symptom management in patients who develop CHD 4
  • Careful monitoring of fluid status and electrolytes

2. Surgical Management

• Valve Replacement Surgery:
  • Should be considered in appropriate cases 1
  • Only definitive treatment for CHD once it has developed 4
  • Should be performed by skilled operators in specialized centers with experience in NETs 1
  • Mortality rates have improved significantly over time (from 29% in 1985-1994 to 5% from 2005 onward) 5
  • Earlier intervention may improve overall survival 5

3. Perioperative Management

• Prophylaxis Against Carcinoid Crisis:
  • IV octreotide (preoperative bolus of 100-200 μg followed by continuous infusion of 50 μg/h) before surgical or locoregional interventions 2
  • Carcinoid crisis can be precipitated by anesthesia, surgery, or adrenergic drugs 1

4. Multidisciplinary Approach

• Referral to Specialized Centers:
  • Patients with confirmed CHD should be referred to cardiology departments with expertise in CHD 1
  • Optimal management requires collaboration among cardiology, oncology, hepatobiliary and cardiovascular surgeons, endocrinologists, anesthesiologists, and gastroenterologists 6

5. Monitoring and Follow-up

• Regular Assessment:
  • Echocardiography every 6-12 months 2
  • Regular monitoring of 5-HIAA levels and chromogranin A 2
  • Ongoing assessment of tumor burden with appropriate imaging

Common Pitfalls and Caveats

1. Delayed Diagnosis:

   • CHD is often diagnosed late when cardiac damage is irreversible
   • Early screening with NT-proBNP and echocardiography is crucial in all carcinoid syndrome patients

2. Inadequate Perioperative Management:

   • Failure to provide prophylaxis against carcinoid crisis during procedures can lead to life-threatening complications
   • Always administer IV octreotide before invasive procedures

3. Left-sided Heart Disease:

   • While less common, left-sided CHD can occur in patients with:
     • Patent foramen ovale (most common cause)
     • Bronchopulmonary NETs
     • Severe, poorly controlled carcinoid syndrome 1
   • Both left and right-sided valves should be evaluated

4. Underestimation of Disease Impact:

   • CHD significantly worsens prognosis and quality of life
   • Early surgical intervention in appropriate patients may improve survival

5. Inadequate Symptom Control:

   • Failure to optimize somatostatin analog therapy can lead to continued exposure to vasoactive substances
   • Dose adjustments or addition of telotristat for persistent diarrhea may be necessary 2

By implementing this comprehensive approach to CHD management, clinicians can improve outcomes and quality of life for patients with this challenging complication of carcinoid syndrome.