title: Disappearance of left ventricular hypertrabeculation/noncompaction and sudden death in a patient with Turner mosaic syndrome.
abstract: Left ventricular hypertrabeculation/noncompaction is associated with various neuromuscular and other rare genetic disorders. In a 53-year-old man with a Turner mosaic karyotype, regression of left ventricular hypertrabeculation/noncompaction was documented by cardiac magnetic resonance imaging and echocardiography after 7 years. During that time, coronary 3-vessel disease and severe left ventricular dys function developed, necessitating coronary bypass surgery. Postoperatively, left ventricular systolic function recovered to an ejection fraction of 40%. The patient died suddenly 6 months postoperatively. In conclusion, the disappearance of left ventricular hypertrabeculation/noncompaction was most likely due to scar formation.
authors.affiliation: Paracelsus Medical Private University Salzburg, Salzburg, Austria.
authors.forename: Johann
authors.initials: J
authors.lastname: Altenberger
authors.forename: Georg
authors.initials: G
authors.lastname: Hasenauer
authors.forename: Marcel
authors.initials: M
authors.lastname: Granitz
authors.forename: Claudia
authors.initials: C
authors.lastname: Stöllberger
authors.forename: Josef
authors.initials: J
authors.lastname: Finsterer
mesh_terms: D001026:Coronary Artery Bypass; D003324:Coronary Artery Disease / Q000150:complications; D003645:Death, Sudden / Q000209:etiology*; D006333:Heart Failure / Q000209:etiology; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D008944:Mitral Valve Insufficiency / Q000150:complications / Q000601:surgery; D009030:Mosaicism; D009206:Myocardium / Q000473:pathology; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D013318:Stroke Volume; D014424:Turner Syndrome / Q000150:complications*; D018487:Ventricular Dysfunction, Left / Q000150:complications
chemical_list: D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain