P322 LEFT VENTRICULAR NON COMPACTION: WHATEVER WORKS(?)

Abstract

Abstract A 36–year–old boy arrived in the emergency room for asthenia, dyspnoea and heart palpitations for several months. In PS he found atrial flutter with high ventricular penetrance complicated by severe left ventricular dysfunction (EF25%). He was therefore hospitalized in the UTIC. The blood tests also documented hepatic suffering and alteration of the coagulation indices. A new echocardium showed marked myocardial trabeculation of the left ventricle, with an appearance referable to non–compact myocardium. Clinical stability was achieved with diuretic therapy, CVE, inotropic support with a course of Levosimendan, and progressive titration of HFrEF–optimized medical therapy. Based on the clinical suspicion, the patient underwent cardiac magnetic resonance, which confirmed the echocardiographic findings, thus documenting a severe impairment of biventricular function, with LVEF of 26% and RVEF of 25%, a moderate increase in LV volumes, with an increase in the thickness of the myocardial trabeculae at the level of the lateral and inferior mid–cavitary segments and at the level of the apical segments, with a maximum trabecular thickness/ myocardial wall thickness of about 2.5, possibly related to non–compact myocardium. Following discharge, the patient underwent periodic check–up and after three months of optimized medical therapy an indication was given for PM–ICD implantation in primary prevention for persistence of EF <35%. Non–compact myocardium is a rare cardiomyopathy characterized by intrauterine arrest of the myocardial compaction process with consequent residual myocardial hypertrabeculae. Clinical manifestations range from heart failure, systemic embolisms, ventricular arrhythmias, and sudden death. The diagnosis is based on two instrumental pillars, echocardiography and magnetic resonance. The absence of well–defined diagnostic criteria is linked to a fundamental aspect of the pathology: although it is recognized as a nosological entity in its own right, the marked ventricular hypertrabeculature can be found both in the paraphysiological field and in other cardiomyopathies, such as hypertrophic and dilated cardiomyopathy. In a practical context, it is important to manage the patient clinically to increase his quality of life, hoping that the genetically compromised myocardium works enough while we try not to get lost in the diagnostic and confusing maze of this pathology.