Research progresses of left ventricular noncompaction
10.3760/cma.j.issn.1673-4408.2021.02.004
- VernacularTitle:左室心肌致密化不全研究进展
- Author:
Lin ZHU
;
Gengsheng YU
- From:
International Journal of Pediatrics
2021;48(2):87-90
- CountryChina
- Language:Chinese
-
Abstract:
Left ventricular noncompaction(LVNC)describes a rare cardiomyopathy of unknown etiology characterized by the presence of prominent trabeculations and deep intertrabecular recesses.It is one of the three major cardiomyopathies among children.LVNC can have different inheritance patterns, including X-linked recessive, autosomal dominant, and mitochondrial inheritance.Mutations in genes encoding sarcomere or cytoskeletal proteins have been identified as causes of LVNC.As a comprehensive cardiac event with serious complications such as life threatening arrhythmias, heart failure and thromboembolism, LVNC is mainly diagnosed through echocardiography, cardiac magnetic resonance and other examinations.Early improvement of genetic evaluation, screening of family members for the mutation, close follow-up of high-risk patients, and early management of complications will help to improve patients′ quality of life and prognosis.
