Disorders of left ventricular trabeculation/compaction or right ventricular wall formation.
Disorders of left ventricular trabeculation/compaction or right ventricular wall formation.
复制标题
左心室小梁/致密化或右心室壁形成障碍。
DOI：
10.1002/ajmg.c.31370
复制
发表时间：
2013
期刊：
影响因子：
--
通讯作者：
Chin,AlvinJ
中科院分区：
文献类型：
--
作者：
Shieh,JosephTC;Jefferies,JohnL;Chin,AlvinJ
心肌病的形式变化很大。虽然心脏可能扩张或肥厚，但心肌病的频谱包括左心室不压实/小梁亢进和右心室壁疾病。由于临床诊断的进步，这些情况在患者中得到越来越多的认识。在这里，我们介绍了心脏病理生理学的信息，从模式生物的心室壁形成和小梁到儿科和成人疾病。许多基因影响心室表型，这对破译发育和疾病途径以及应用于临床护理的测试具有重要意义。©2013 Wiley期刊公司
Cardiomyopathies are remarkably variable in form. Although hearts may be dilated or hypertrophic, the spectrum of cardiomyopathies includes left ventricular noncompaction/hypertrabeculation and right ventricular wall disorders. These conditions have been increasingly recognized in patients given advances in clinical diagnostics. Here we present information on cardiac pathophysiology, from ventricular wall formation and trabeculae in model organisms to pediatric and adult disease. Many genes to affect the ventricular phenotype, and this has implications for deciphering developmental and disease pathways and for applying testing for clinical care. © 2013 Wiley Periodicals, Inc.