What is the approach for symptomatic treatment of infiltrative heart diseases?

Symptomatic Treatment for Infiltrative Heart Diseases

The symptomatic treatment of infiltrative heart diseases should follow heart failure management principles with specific modifications based on the type of infiltration, focusing on diuretics for fluid overload, ACE inhibitors for systolic dysfunction, and beta-blockers or calcium channel blockers for rate control, while simultaneously treating the underlying infiltrative condition. 1

General Approach to Symptomatic Management

Initial Assessment and Classification

1. Establish the specific type of infiltrative disease (sarcoidosis, amyloidosis, hemochromatosis, etc.)
2. Determine the predominant cardiac manifestation:
   • Systolic dysfunction (reduced ejection fraction)
   • Diastolic dysfunction (preserved ejection fraction)
   • Conduction abnormalities/arrhythmias
   • Restrictive physiology
   • Valvular involvement

Core Pharmacological Therapy

For Fluid Overload and Congestion

• Diuretics: Essential first-line therapy for symptomatic treatment when pulmonary congestion or peripheral edema is present 1
  • Loop diuretics (furosemide) for rapid improvement of dyspnea and increased exercise tolerance
  • Cautious use of low-dose oral diuretics in restrictive physiology
  • Monitor for hypotension, especially in infiltrative diseases with autonomic dysfunction

For Systolic Dysfunction (HFrEF)

• ACE inhibitors: First-line therapy for reduced LV systolic function 1

  • Start with low doses and gradually titrate
  • Monitor renal function and potassium levels closely
  • Follow recommended procedure for ACE inhibitor initiation (review diuretic doses, avoid excessive diuresis before treatment)

• Beta-blockers: Add after stabilization on ACE inhibitors

  • Use with caution in infiltrative diseases with conduction abnormalities
  • Monitor for bradycardia and heart block

For Arrhythmias

• For atrial fibrillation: Rate control with beta-blockers or digoxin 1
• For ventricular arrhythmias: Amiodarone is the preferred agent for symptomatic, sustained ventricular tachycardias 1

Disease-Specific Considerations

Cardiac Sarcoidosis

• Immunosuppressive therapy (usually corticosteroids) may help reduce arrhythmia burden when given in early stages of disease 1
• Combined treatment approach with steroids and antiarrhythmic medications, followed by catheter ablation if needed 1
• Consider ICD placement for prevention of sudden cardiac death in patients with significant ventricular dysfunction or ventricular arrhythmias

Cardiac Amyloidosis

• Avoid digoxin (increased binding and potential toxicity)
• Use caution with calcium channel blockers (may cause profound hypotension)
• Treatment of the underlying amyloidosis type is critical (light chain vs. transthyretin)
• ICD benefit is uncertain as many deaths are not preventable by defibrillation 1

Hemochromatosis

• Iron chelation therapy and phlebotomy are essential alongside heart failure management 2
• Standard heart failure therapy can be used as the disease is highly treatable when diagnosed early 1

Management Pitfalls and Special Considerations

Medications to Use with Caution or Avoid

• Vasodilators: May worsen symptoms in obstructive forms (e.g., HCM) 1

  • Consider discontinuation of ACE inhibitors, ARBs, and dihydropyridine calcium channel blockers if they worsen symptoms

• Verapamil: Potentially harmful in patients with severe dyspnea at rest, hypotension, or very high resting gradients 1

• Digoxin: Consider discontinuation in obstructive forms; use with caution in amyloidosis due to increased binding 1

Monitoring and Follow-up

• Regular assessment of:

  • Fluid status (daily weight monitoring)
  • Renal function and electrolytes
  • Arrhythmia burden
  • Disease progression through appropriate imaging

• Consider advanced imaging (cardiac MRI, PET) for monitoring disease activity, particularly in sarcoidosis and amyloidosis 3

Invasive Treatment Options

• For refractory symptoms despite optimal medical therapy, consider:
  • Septal reduction therapy in obstructive forms 1
  • Cardiac transplantation in eligible patients
  • Mechanical circulatory support devices in selected cases

Remember that while treating symptoms is important, addressing the underlying infiltrative process is crucial for improving outcomes. Treatment should be coordinated between cardiology and specialists in the specific infiltrative disease to optimize both cardiac symptoms and the underlying condition.