Key result
A 76-year-old woman was diagnosed with isolated noncompacted myocardium, representing an unusually late presentation of a rare cardiomyopathy typically found in younger populations.
Case Report (n=1)
Noncompaction of the myocardium is a rare disorder characterized by prominent myocardial trabecularizations, thought to result from an arrest of cardiac development during the compaction of myocardial fibers.
Supports transplant in refractory infantile IVNC; leaves open optimal timing and outcomes in larger series.
Noncompaction of the myocardium (NCM) is a rare disorder that is characterized by prominent myocardial trabecularizations and deep intertrabecular recesses, leading to a spongy appearance of the myocardium. In the first month of normal intrauterine development, the myocardium consists of a mesh of muscle fibers. In this mesh, trabeculae alternate with recesses, which provide the blood supply to the cardiac muscle through communication with the ventricular cavity. During the fifth to the eighth week of development, these recesses are supposed to turn into capillaries as the ventricular myocardium gradually compacts.3,4 Although the exact mechanisms that generate NCM remain unclear, it has been suggested that an arrest of cardiac development during the compaction of myocardial fibers may lead to the persistence of multiple trabeculations within the ventricular myocardium.1,2
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Miranda et al. (2008) conducted a case report in Isolated noncompacted myocardium (n=1). Medical management for heart failure and anticoagulation was evaluated. A 76-year-old woman was diagnosed with isolated noncompacted myocardium, representing an unusually late presentation of a rare cardiomyopathy typically found in younger populations.
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