What is the management of noncompaction cardiomyopathy?

Management of Noncompaction Cardiomyopathy

Manage noncompaction cardiomyopathy by treating heart failure symptoms with standard guideline-directed medical therapy, using ICD criteria identical to dilated cardiomyopathy based on LV systolic dysfunction severity and sustained ventricular arrhythmias, and implementing anticoagulation for atrial fibrillation or LV thrombus. 1

Heart Failure Management

Symptomatic Patients with Preserved Ejection Fraction

• Initiate beta-blockers or nondihydropyridine calcium channel blockers as first-line therapy for exertional dyspnea or angina 1
• Add oral diuretics when symptoms persist despite beta-blocker or calcium channel blocker therapy 1
• Target heart rate control to optimize LV filling, as diastolic dysfunction is a primary driver of symptoms 1

Patients with Reduced Ejection Fraction (LVEF <50%)

• Implement full guideline-directed medical therapy for heart failure with reduced ejection fraction, including ACE inhibitors/ARBs, beta-blockers, aldosterone antagonists, and SGLT2 inhibitors 1
• Discontinue negative inotropic agents (verapamil, diltiazem, disopyramide) if systolic dysfunction develops 1
• Evaluate for concomitant causes of systolic dysfunction, particularly coronary artery disease 1

Sudden Cardiac Death Risk Stratification and ICD Therapy

The presence of noncompaction morphology alone does not justify ICD implantation 1. ICD decisions must be guided by:

Primary Prevention Criteria (Apply DCM Guidelines)

• LVEF ≤35% with NYHA class II-III symptoms despite optimal medical therapy for ≥3 months 1
• Sustained ventricular arrhythmias documented on monitoring 1
• Unexplained syncope in the setting of LV dysfunction 1

Risk Factors for Mortality

The following predict increased mortality but do not independently mandate ICD placement 1:

• Increased age at presentation
• Larger LV end-diastolic diameter
• Symptomatic heart failure (NYHA class III-IV)
• Permanent or persistent atrial fibrillation
• Bundle branch block on ECG
• Associated neuromuscular disease

Secondary Prevention

• ICD is indicated for survivors of cardiac arrest or sustained ventricular tachycardia causing hemodynamic instability 1

Anticoagulation Strategy

Clear Indications for Anticoagulation

• All patients with atrial fibrillation or atrial flutter should receive oral anticoagulation regardless of CHA₂DS₂-VASc score 1
• Documented LV thrombus requires anticoagulation 1
• History of systemic thromboembolism warrants long-term anticoagulation 1, 2

Controversial: Anticoagulation Without AF or Thrombus

The evidence is mixed regarding prophylactic anticoagulation in sinus rhythm without documented thrombus 1, 2:

• Blood stasis within deep intertrabecular recesses theoretically increases thrombotic risk 1, 2, 3
• Thromboembolic event rates range from 5-24% in observational series 1, 2
• Consider anticoagulation in patients with severely reduced LVEF (<35%), significantly dilated LV, or spontaneous echo contrast on imaging 1, 2

Anticoagulant Selection

• Direct oral anticoagulants (DOACs) are preferred over warfarin for atrial fibrillation 1, 2
• Warfarin plus aspirin is the established regimen for patients with LV assist devices, though this population differs from typical NCCM 1
• Left atrial appendage closure is reasonable for patients with contraindications to anticoagulation 1

Arrhythmia Management

Atrial Fibrillation

• Prevalence approaches 25-30% in adult NCCM patients 1, 2
• Rate control is critical given fixed stroke volume and dependence on diastolic filling 1
• Beta-blockers are first-line for rate control unless contraindicated by hypotension or severe bradycardia 1
• Amiodarone or sotalol for rhythm control if symptoms persist despite rate control 1
• Direct current cardioversion for hemodynamically unstable patients 1

Ventricular Arrhythmias

• Approximately 40% of patients demonstrate complex ventricular arrhythmias 1, 3
• Amiodarone is the preferred antiarrhythmic for ventricular arrhythmias in the setting of structural heart disease 1

Advanced Heart Failure Management

Indications for Advanced Therapies

• NYHA class III-IV symptoms despite optimal medical therapy warrant evaluation for heart transplantation 1
• Perform cardiopulmonary exercise testing to quantify functional limitation and aid in transplant selection 1
• Life-threatening ventricular arrhythmias refractory to medical therapy are an indication for transplant evaluation 1

Transplant-Free Survival Data

Children with NCCM have particularly poor outcomes 1:

• 1-year transplant-free survival: 48%
• 2-year transplant-free survival: 34%
• 5-year transplant-free survival: 22%

Family Screening and Genetic Testing

• Screen first-degree relatives with echocardiography and ECG, as 18-50% of cases are familial with autosomal dominant inheritance 1, 3
• Genetic testing identifies mutations in sarcomeric proteins, calcium-handling proteins, LMNA, LDB3, and Taffazin genes 1
• Positive genetic testing enables cascade screening of at-risk family members 3, 4

Common Pitfalls to Avoid

• Do not withhold standard heart failure therapy based solely on the noncompaction diagnosis—treat the functional phenotype 1
• Do not implant ICDs for primary prevention based on noncompaction morphology alone without meeting standard criteria for reduced LVEF or documented arrhythmias 1
• Do not assume all patients require anticoagulation—individualize based on AF, thrombus, prior embolism, or severe LV dysfunction 1, 2
• Avoid excessive beta-blockade that causes symptomatic bradycardia or chronotropic incompetence, as cardiac output depends on heart rate 1