Noncompaction
Noncompaction is a form of cardiomyopathy characterized by prominent trabeculations and deep intertrabecular recesses in the ventricular myocardium, most commonly affecting the left ventricle. It is believed to result from arrest of normal endomyocardial maturation during early fetal development, leading to a two-layered macrostructure with a thin compacted outer layer and a thick noncompacted inner layer.
Clinical presentation varies widely. Some individuals are asymptomatic, while others experience heart failure symptoms such as
Diagnosis relies primarily on cardiac imaging. Echocardiography can reveal a bilayered myocardial wall with prominent trabeculations,
Genetics are heterogeneous; many cases are sporadic, but familial forms exist with autosomal dominant inheritance and
Management focuses on symptom relief and prevention of complications. Standard heart failure therapies apply when indicated;