What is the management of Left Ventricular Noncompaction (LVNC)?

Management of Left Ventricular Noncompaction (LVNC)

The management of Left Ventricular Noncompaction should be guided by the presence of symptoms, LV systolic function, arrhythmias, and thromboembolic risk, with treatment focused on preventing heart failure, arrhythmias, and embolic events rather than treating the anatomical abnormality itself. 1

Definition and Diagnosis

Left ventricular noncompaction (LVNC) is characterized by:

• Prominent trabeculations within the LV myocardium
• Deep intertrabecular recesses
• Two-layered structure of the myocardial LV wall
• Often associated with LV dysfunction

Diagnostic criteria include:

• Echocardiography: ratio of non-compacted to compacted layer >2
• Cardiac MRI: ratio >2.3
• Common ECG abnormalities: T-wave inversion, ST-segment depression, ventricular conduction delay (particularly LBBB) 1

Risk Stratification

The clinical outcome of LVNC is variable and primarily determined by:

1. LV systolic function - most important prognostic factor
2. Presence of arrhythmias - particularly ventricular tachyarrhythmias
3. Thromboembolic risk - related to blood stasis in trabeculations

Adverse outcomes are strongly associated with:

• LV systolic dysfunction
• Major ventricular tachyarrhythmias
• Increased age
• LV end-diastolic diameter at presentation
• Symptomatic heart failure
• Permanent or persistent atrial fibrillation
• Bundle branch block
• Associated neuromuscular disease 1

Management Approach

Heart Failure Management

For patients with reduced LV systolic function:

• Standard heart failure therapy following current guidelines
• Diuretics for symptom management
• Consider ICD implantation based on severity of LV dysfunction using the same criteria as for dilated cardiomyopathy 1

Arrhythmia Management

1. Monitoring:

   • Regular ambulatory ECG monitoring to detect arrhythmias
   • Exercise ECG testing to assess for exercise-induced arrhythmias

2. ICD Implantation:

   • The need for ICD should be guided by:
     • Severity of LV systolic dysfunction
     • Presence of sustained ventricular arrhythmias
     • Not indicated based on LVNC diagnosis alone 1

Anticoagulation

• For patients with LVNC and atrial fibrillation: anticoagulation is recommended 1
• For patients with LVNC and evidence of LV thrombus: anticoagulation is indicated
• For patients with LVNC in sinus rhythm with reduced EF: the effectiveness of anticoagulation compared with antiplatelet therapy is uncertain, and the choice should be individualized 1

Exercise and Sports Participation

For athletes with LVNC:

1. Athletes with incidental LV hypertrabeculation without symptoms, positive family history, abnormal ECG patterns, and normal LV function:

   • No restriction for competitive sports (Class IIa/Level B) 1

2. Athletes with confirmed LVNC but near-normal LV systolic function may participate in competitive sports (except those where syncope could cause serious harm) if:

   • Asymptomatic
   • No frequent/complex ventricular arrhythmias
   • No history of unexplained syncope (Class IIb/Level C) 1

3. Athletes with confirmed LVNC and either:

   • Impaired LV systolic function, or
   • Frequent/complex ventricular arrhythmias or non-sustained VT

   Should abstain from competitive sports and limit to leisure-time physical activities with regular clinical surveillance (Class III/Level C) 1

Follow-up and Monitoring

• Regular clinical evaluation
• Periodic assessment of LV function
• Ambulatory ECG monitoring for arrhythmia detection
• Family screening (20-40% of cases may be familial) 2

Important Caveats

1. Diagnostic challenges: Current criteria based solely on morphologic features have limitations and may lead to overdiagnosis.

2. Risk of thromboembolic events: Blood stasis within trabeculations can lead to up to 24% risk of cerebral embolism, requiring careful consideration of anticoagulation 1.

3. Genetic considerations: LVNC can be inherited as autosomal dominant or X-linked recessive disorder with mutations in multiple genes. Genetic testing and family screening should be considered 2.

4. Phenotypic variation: Clinical manifestations are highly variable, even within the same family, requiring individualized risk assessment 2.

5. Distinction from physiological trabeculation: Particularly important in athletes where increased trabeculation may represent a normal variant rather than pathology.