Right ventricular noncompaction

Left ventricular noncompaction is a rare genetic cardiomyopathy characterized by non-compacted endocardial and compacted epicardial layers with a ratio >2. This condition presents an extremely high morphological variability and it is not yet defined as either a distinct cardiomyopathy or a morphologic trait shared by different cardiomyopathies. The left ventricle is the most commonly involved structure, but in rare cases, it may include only the right ventricle. Clinical presentation of this disease is variable and may be manifested at any age but in symptomatic patients has unfavorable prognosis. Electrocardiography findings are non-specific and include left ventricular hypertrophy, repolarization changes, inverted T waves, ST-segment changes, intraventricular conduction abnormalities, and atrioventricular blocks. Arrhythmias are common, including ventricular tachyarrhythmias, atrial fibrillation, and paroxysmal supraventricular tachycardia. Cardiac magnetic resonance imaging plays an essential role in the assessment of myocardial fibrosis and final diagnosis. Asymptomatic patients can be followed by clinical monitoring, whereas for symptomatic patients therapy is aimed to treat previously mentioned triads of symptoms. Heart failure symptoms treatment is carried out by the general guidelines on heart failure and includes the use of mechanical devices such as cardioverter defibrillator implantation and cardiac resynchronization therapy in primary prevention. When heart failure is refractory to medical and device therapy, heart transplantation should be considered. In patients with non compaction cardiomyopathy with implanted defibrillator either in primary or secondary prevention for sudden cardiac death, there was an appropriate shock in 25% and 50% of cases respectively, in a follow-up time of 3 years.