[Isolated left ventricular non-compaction]. / La forma isolata della non compattazione del miocardio ventricolare sinistro.

Isolated left ventricular noncompaction is a genetically heterogeneous congenital disorder characterized by an altered structure of the myocardial wall. This cardiomyopathy is thought to be due to an arrest of intrauterine compaction of the myocardial fibers in the absence of any other structural heart disease. Noncompaction of the left ventricular myocardium is an uncommon finding and remains frequently overlooked even by experienced echocardiographers. However, a correct diagnosis of noncompaction has important implications due to the possible association with other cardiac abnormalities and/or muscle disorders, progressive left ventricular dysfunction, risk of thromboembolism, and life-threatening arrhythmias. Furthermore, because of the familial association described with ventricular noncompaction, screening with echocardiography of first relatives is recommended. Since echocardiography is the diagnostic technique of choice, missed diagnoses may be due to nonoptimal imaging of the lateral and apical myocardium, and/or insufficient disease awareness by echocardiographers. To increase awareness of left ventricular noncompaction, the present paper reviews embryology, genetics, clinical features and pathophysiology, diagnosis, treatment and prognosis of patients affected by isolated left ventricular noncompaction.

[Stress echocardiography in valvular heart disease]. / L'ecocardiografia da stress nelle valvulopatie: una metodica da rivalutare.

The assessment of the severity of a valvular lesion traditionally relies on the evaluation of valvular anatomy and on the demonstration of resting hemodynamic disturbances. In patients with stenotic lesions, this is usually achieved by measuring the trans-stenotic pressure difference and by calculating the surface of the stenotic orifice. In patients with regurgitant lesions, assessment of lesion severity involves the demonstration of a significant volume or pressure overload of the various chambers participating in the process. In many instances, however, discrepancies are found between the severity of valvular lesions and either the symptoms or the hemodynamic impairment. As echocardiography is also the method of choice for assessing the severity of valvular disease, stress echocardiography may offer the unique opportunity to assess the hemodynamic consequences of valvular lesions both at rest and during stress.