What is the treatment approach for non-compaction cardiomyopathy?

Treatment Approach for Non-Compaction Cardiomyopathy

The treatment of left ventricular non-compaction cardiomyopathy (LVNC) should follow a structured approach based on the presence of symptoms, ventricular dysfunction, arrhythmias, and thromboembolic risk, with specific therapies directed at each of these manifestations. 1

Medical Therapy

Heart Failure Management

• For patients with symptoms of exertional angina or dyspnea:
  • First-line: Beta-blockers or non-dihydropyridine calcium channel blockers (verapamil, diltiazem) 1
  • Add oral diuretics when exertional dyspnea persists despite initial therapy 1
  • Standard heart failure therapy (including ACE inhibitors/ARBs) should be implemented for patients with reduced ejection fraction, though their specific efficacy in LVNC is not well established 1

Arrhythmia Management

• For symptomatic ventricular arrhythmias or recurrent ICD shocks despite beta-blocker use:
  • Antiarrhythmic drug therapy is recommended 1
• For atrial fibrillation:
  • Rate control with beta-blockers, verapamil, or diltiazem based on patient preferences and comorbidities 1

Device Therapy

ICD Implantation

• ICD implantation should follow the same criteria as for dilated cardiomyopathy, guided by:
  • Severity of LV systolic dysfunction
  • Presence of sustained ventricular arrhythmias 1

Cardiac Resynchronization Therapy

• Consider CRT for patients with significant dyssynchrony, following standard heart failure guidelines 2

Anticoagulation Therapy

• Anticoagulation is recommended for:

  • All patients with LVNC and clinical atrial fibrillation, regardless of CHA₂DS₂-VASc score 1
  • Patients with evidence of LV thrombus 1
  • Direct-acting oral anticoagulants are the first-line option, with vitamin K antagonists as second-line 1, 3

• The role of prophylactic anticoagulation in patients without atrial fibrillation or thrombus but with severe LV dysfunction remains controversial 3

Advanced Heart Failure Therapies

Heart Transplantation

• Consider heart transplantation evaluation for:
  • End-stage heart failure refractory to medical therapy
  • Recurrent poorly tolerated life-threatening ventricular tachyarrhythmias refractory to maximal therapy and ablation 1
  • In children: progressive heart failure despite maximal medical therapy, growth failure, or significant irreversible hepatic or renal dysfunction 1

Follow-up and Monitoring

• Regular cardiac follow-up is essential to monitor:

  • Ventricular function
  • Development of arrhythmias
  • Thromboembolic risk 1

• Comprehensive cardiac evaluation should include:

  • Echocardiography to assess ventricular function
  • ECG to detect arrhythmias
  • Monitoring for heart failure symptoms 1

Special Considerations

Sports Participation

• Athletes with incidental LV hypertrabeculation without symptoms, normal ECG patterns, and normal LV function may participate in competitive sports without restriction 1
• Athletes with confirmed LVNC but near-normal LV systolic function may participate in competitive sports with certain exceptions 1
• Athletes with confirmed LVNC and either impaired LV systolic function or frequent/complex ventricular arrhythmias should abstain from competitive sports 1

Genetic Testing and Family Screening

• Genetic testing and family screening should be considered for all patients with LVNC 1
• First-degree relatives should undergo screening with echocardiography and ECG 1

Common Pitfalls and Caveats

1. Diagnostic challenges: LVNC diagnosis can be challenging due to non-uniform diagnostic criteria and overlap with other cardiomyopathies 4, 5. Consider multimodality imaging (echocardiography and cardiac MRI) for accurate diagnosis 2.

2. Late diagnosis: LVNC is often diagnosed in advanced stages with significant dilation and ventricular dysfunction 6. Early recognition is crucial for timely intervention.

3. Thromboembolic risk assessment: The risk of thromboembolism in LVNC appears higher than in age-matched populations without LVNC 3. Consider anticoagulation carefully even in borderline cases.

4. Heterogeneity of presentation: Clinical presentation is highly variable, ranging from no symptoms to end-stage heart failure, lethal arrhythmias, sudden cardiac death, or thromboembolic events 3, 7. Individualized risk assessment is essential.

5. Pediatric considerations: LVNC in childhood is more frequently familial and associated with other congenital heart diseases and neuromuscular diseases 5. A comprehensive evaluation is particularly important in pediatric patients.