From the Guidelines
Apical noncompaction is a rare cardiac condition characterized by excessive trabeculations in the apex of the heart, most commonly affecting the left ventricle, and is a specific form of left ventricular noncompaction (LVNC) that predominantly affects the apex of the heart. This condition occurs when the heart muscle fails to develop properly during embryonic development, resulting in a two-layered structure with a compacted outer layer and a noncompacted inner layer with prominent trabeculations and deep recesses, as described in the 2015 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death 1.
Key Characteristics
- Excessive trabeculations in the apex of the heart
- Two-layered structure with a compacted outer layer and a noncompacted inner layer
- Prominent trabeculations and deep recesses
- Can be associated with ventricular dilatation and systolic dysfunction
- Familial disease occurs in 18-50% of adults with isolated LV noncompaction, mostly with an autosomal dominant pattern of inheritance 1
Diagnosis and Management
Diagnosis typically involves cardiac imaging techniques like echocardiography, cardiac MRI, or CT scans that can visualize the characteristic trabeculations and recesses, with MRI being superior in evaluating the extent of noncompacted myocardium and providing supplemental morphological information beyond that obtained from 2-D echocardiography 1.
- Cardiac imaging techniques: echocardiography, cardiac MRI, or CT scans
- Management focuses on treating complications and may include:
- Heart failure medications
- Anticoagulation therapy to prevent blood clots
- Antiarrhythmic drugs
- Implantable devices like pacemakers or defibrillators in severe cases Regular cardiac monitoring is essential for patients with this condition to detect and address complications early, as increased age, LV end diastolic diameter at presentation, symptomatic HF, permanent or persistent AF, bundle branch block, and associated neuromuscular disease are reported predictors for increased mortality 1.
From the Research
Definition of Apical Noncompaction
- Apical noncompaction is not directly defined in the provided studies, however, left ventricular noncompaction (LVNC) is described as a cardiomyopathy characterized by excessive trabeculation and deep intertrabecular recesses in direct communication with the left ventricular cavity 2.
- The studies provided do not specifically mention apical noncompaction, but they discuss left ventricular noncompaction and its characteristics, which may be related to apical noncompaction.
Relationship to Left Ventricular Noncompaction
- Left ventricular noncompaction is associated with heart failure, ventricular arrhythmia, and systemic thromboembolism 2.
- Hypertrabeculation, a characteristic of LVNC, is not sufficient to define a subject as at risk for complications and should not be sufficient to diagnose LVNC 2.
- The studies do not provide a direct link between apical noncompaction and left ventricular noncompaction, but they suggest that noncompaction cardiomyopathy is a complex condition that requires careful diagnosis and management.
Diagnostic Criteria and Management
- The studies do not provide specific diagnostic criteria or management strategies for apical noncompaction, but they discuss the importance of distinguishing between hypertrabeculation phenotype, non-compaction phenotype, and LVNC cardiomyopathy 2.
- The management of cardiovascular diseases, including heart failure and hypertension, is discussed in the studies, but it is not directly related to apical noncompaction 3, 4, 5.