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Characterization of Left Ventricular Non-Compaction Cardiomyopathy

2020; Multidisciplinary Digital Publishing Institute; Volume: 9; Issue: 8 Linguagem: Inglês

10.3390/jcm9082524

2077-0383

Rebeca Lorca, Marı́a Martı́n, Isaac Pascual, Aurora Astudillo, Beatriz Díaz‐Molina, Helena Cigarrán, Elías Cuesta‐Llavona, Pablo Avanzas, J.J.R. Reguero, Eliécer Coto, César Morı́s, Juan Gómez,

Viral Infections and Immunology Research

Left ventricle non-compaction cardiomyopathy (LVNC) has gained great interest in recent years, being one of the most controversial cardiomyopathies. There are several open debates, not only about its genetic heterogeneity, or about the possibility to be an acquired cardiomyopathy, but also about its possible overdiagnosis based on imaging techniques. In order to better understand this entity, we identified 38 LVNC patients diagnosed by cardiac MRI (CMRI) or anatomopathological study that could underwent NGS-sequencing and clinical study. Anatomopathological exam was performed in eight available LVNC hearts. The genetic yield was 34.2%. Patients with negative genetic testing had better left ventricular ejection fraction (LVEF) or it showed a tendency to improve in follow-up, and a possible trigger factor for LVNC was identified in 1/3 of them. Nonetheless, cerebrovascular accidents occurred in similar proportions in both groups. We conclude that in LVNC there seem to be different ways to achieve the same final phenotype. Genetic testing has a good genetic yield and provides valuable information. LVNC without an underlying genetic cause may have a better prognosis in terms of LVEF evolution. However, anticoagulation to prevent cerebrovascular accident (CVA) should be carefully evaluated in all patients. Larger series with pathologic examination are needed to help better understand this entity.