The clinical case of non-compact left ventricular myocardium in combination with restrictive cardiomyopathy

Abstract

In this paper the information about a comparatively rare form of cardiomyopathy – noncompaction of the left ventricular myocardium is showed. As a result of genetical changes on the early stages of embryogenesis a disability of myocardial fibres develops, and two-layer myocardial structure is formed: thin compact layer and remaining more voluminous non-compact layer with significant trabeculation and deep intratrabecular cavities communicated with ventricular cavity. Mutations in  genes which encode sarcomeric, structural and regulatory proteins and proteins, which are responsible for ion channels functioning, are considered to be one of the main reasons of non-compact myocardium. There is a theory that considers a non-compact myocardium as a result of an exposure of various factors during lifetime – so-called non-embryonal (acquired) noncompaction myocardium. “Non-embryonal” hypothesis views non-compact myocardium as a sign of functional maladaptation, possible stage in cardiomyopathy development. By way of illustration the clinical case of 32-year-old female patient is presented in the article. The noncompaction of  the left ventricular myocardium in  conjunction with restrictive cardiomyopathy was first diagnosed in  her. The  diagnosis was confirmed by main diagnostic methods for  this pathology such as echocardiography and MRI of  a  heart. This clinical observation is interesting due to formation of disease patterns by two rare combined pathological conditions: noncompaction of the myocardium syndrome and restrictive cardiomyopathy. Changes of heart hemodynamics occurs in interaction between these pathologies. Diastolic filling of left ventricular decreases as a result of restrictive cardiomyopathy, which leads to decreasing of its myocardium load. Therefore, systolic disfunction, which is specific to non-compact myocardium, doesn’t occur. Generally, prognosis for this patient is poor due to presence of two serious pathologies.