title: Disorders of left ventricular trabeculation/compaction or right ventricular wall formation.
abstract: 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.
authors.affiliation: Division of Medical Genetics, Department of Pediatrics, Institute for Human Genetics, University of California San Francisco, San Francisco, CA, USA. shiehj2@humgen.ucsf.edu
authors.forename: Joseph T C
authors.initials: JT
authors.lastname: Shieh
authors.forename: John L
authors.initials: JL
authors.lastname: Jefferies
authors.forename: Alvin J
authors.initials: AJ
authors.lastname: Chin
mesh_terms: D000818:Animals; D009202:Cardiomyopathies / Q000473:pathology / Q000503:physiopathology*; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans