How are cardiac manifestations of Amyotrophic Lateral Sclerosis (ALS) managed?

Management of Cardiac Manifestations in ALS

Cardiac manifestations in ALS should be managed with a structured approach including baseline assessment, regular monitoring, and targeted pharmacological and non-pharmacological interventions, with careful avoidance of potentially harmful medications. 1

Baseline Assessment and Monitoring

• Initial cardiac evaluation should include:

  • Cardiac MRI
  • Echocardiography with speckle tracking
  • ECG
  • Holter monitoring
  • Cardiac biomarkers (troponin, BNP) 1

• Monitoring frequency:

  • Normal baseline findings: Annual echocardiography
  • Abnormal baseline findings: More frequent monitoring (every 3-6 months) 1

Research supports this approach, as cardiac MRI studies have shown that ALS patients may have reduced myocardial mass, decreased ejection volumes, and increased T1 enhancement (77% of patients) even without clinical cardiac symptoms 2. These structural changes may be due to sympathetic dysfunction that could account for cardiac deaths in late-stage ALS.

Pharmacological Management

• For arrhythmias:

  • Amiodarone for atrial fibrillation with reduced ejection fraction 1

• For fluid overload:

  • Diuretics with careful monitoring to avoid intravascular volume depletion 1

• For heart failure:

  • ACE inhibitors/ARBs at lowest effective dose (use with caution due to hypotension risk)
  • Beta-blockers with caution (cardiac output may be heart rate dependent) 1

• For orthostatic hypotension:

  • Midodrine 1

Non-Pharmacological Management

• Fluid balance management:

  • Salt restriction
  • Daily weight monitoring 1

• For hypotension:

  • Elastic compression garments 1

• For recurrent syncope due to arrhythmias:

  • Consider pacemaker placement 1

Medications to Avoid

• Calcium channel blockers - may worsen hypotension 1
• Digoxin - should be avoided in patients with amyloid-like cardiac infiltration due to risk of toxicity 1
• Celecoxib - associated with:
  • Increased risk of heart failure hospitalizations
  • Blunted therapeutic effects of ACE inhibitors/ARBs
  • Reduced effectiveness of diuretics
  • Increased bleeding risk when combined with anticoagulants 1

Special Considerations

• Cardiac involvement in ALS may be subclinical but present in many patients. Cardiac sympathetic hyperactivity has been detected using cardiac [123I] MIBG scintigraphy, with increased washout ratio (WR) correlating with faster disease progression and decreased survival 3.

• Global longitudinal strain measurements are abnormal in up to 94% of ALS patients, even those with normal ejection fraction, indicating subclinical cardiac dysfunction 4.

• Patients with a history of resolved infantile cardiomyopathy may continue to have residual impairment in cardiac function and require ongoing monitoring 4.

Pitfalls to Avoid

1. Overlooking subclinical cardiac involvement - Cardiac abnormalities may be present despite normal routine cardiac assessment 2.

2. Neglecting cardiac risk factors - Aggressive modification of obesity, inflammation, diabetes, and dyslipidemia is essential in ALS patients 4.

3. Delaying diagnosis and treatment - Early diagnosis and treatment of ALS and its complications, including cardiac manifestations, can improve outcomes 5.

4. Inappropriate medication use - Careful consideration of medication side effects and interactions is crucial in ALS patients with cardiac involvement 1.