Advances in noncompation of the ventricular myocardium in children / 国际儿科学杂志

ABSTRACT

Noncompation of the ventricular myocardium is one of three major types of cardiomyopathy in children.More patients are diagnosed,with the understanding of this cardiomyopathy.Their clinical manifestation may vary a lot from without symptoms to severe cardiac dysfunction.This article describes the clinical characteristics of left ventricular noncompaction(LVNC) children and long-term prognosis of children at different ages.With the development of next-generation sequencing technology,more and more gene mutations have been found to cause LVNC.The article introduces the relationship between genotypes and clinical phenotypes in children.LVNC has poor long-term prognosis.At present,studies have showed the importance of family screening.For children carrying pathogenic genes mutations,they should be followed-up regularly even without clinical symptoms.