Noncompaction of the left ventricle: a new cardiomyopathy is presented to the clinician.

Noncompaction of the left ventricular myocardium is a morphogenetic abnormality involving loss of compaction of the myocardial fiber meshwork during intrauterine life. It is an extremely rare condition, accounting for only 0.05% of the cases evaluated in databanks. It has been described in both genders, in many ethnic groups and at different ages. Recently published studies of case series have shown a high mortality rate among these patients during follow-up of up to 48 months. Many cases have so far been misdiagnosed due to poor knowledge of the findings relating to this syndrome. There needs to be an attempt at early and accurate diagnosis, because of the need to investigate the patients family upon such diagnosis, and today this can be achieved using echocardiographic criteria.

Left atrial appendage assessment by second harmonic transthoracic echocardiography after an acute ischemic neurologic event.

Although second harmonic (sh) imaging is widely available in most contemporary ultrasound systems, its accuracy to evaluate left atrial appendage (LAA) morphology and function remains poorly characterized. We conducted a cross-sectional survey of patients with acute ischemic neurologic conditions (n = 51) who underwent both transesophageal and transthoracic echocardiography (TTE) to explore the performance of sh in LAA assessment. Doppler and LAA area evaluation by sh TTE were feasible in most patients (98%). We observed positive and significant associations between sh TTE and transesophageal echocardiography assessment of LAA peak emptying velocities (r = 0.63, P < .001) and LAA maximum area (r = 0.73, P < .001). In addition, all patients (n = 7) with LAA thrombus or spontaneous contrast had peak emptying velocities less than 50 cm/s on sh TTE (negative predictive value of 100%). In multivariate analysis, LAA peak emptying velocity remained independently associated with LAA thrombus or contrast. In conclusion, sh TTE can provide valuable and clinically relevant information of LAA morphology and dynamics.

Noncompaction of the left ventricle: a new cardiomyopathy is presented to the clinician

O miocárdio não compactado do ventrículo esquerdo é uma anormalidade morfogenética que envolve a perda da compactação da trama de fibras do miocárdio durante a vida intra-uterina. É responsável por somente 0,05% dos casos avaliados em bancos de dados. Tem sido descrita em ambos os sexos, em muitos grupos étnicos e em diversas faixas etárias. Estudos de séries de casos publicados recentemente evidenciam a alta mortalidade presente nesses pacientes quando acompanhados por até 48 meses. Talvez, muitos casos até o momento tenham sido mal diagnosticados devido ao pouco conhecimento dos achados da síndrome. O diagnóstico precoce e correto deve ser tentado, e atualmente pode ser feito com critérios ecocardiográficos. A partir do diagnóstico, a investigação familiar se impõe devido aos vários casos de recorrência entre parentes.