title: Noncompaction of ventricular myocardium in a patient with congenitally corrected transposition of the great arteries treated surgically: case report.
abstract: Noncompaction of the ventricular myocardium is a rare disorder that represents numerous prominent trabeculations and intratrabecular recesses in the ventricles. It is believed to represent not only an arrest in endomyocardial morphogenesis but also an unclassified cardiomyopathy. The pathology has been almost invariably associated with other congenital cardiac malformations. A female patient with noncompaction of the myocardium of both ventricles and congenitally corrected transposition of the great arteries (cTGA), situs inversus totalis, and atrial and ventricular septal defects is described. When she was 7 days old a permanent pacemaker was implanted because of complete heart block. Prazosin (Minipress), an alpha-receptor blocker, was administered, and the cardiac ejection fraction showed a striking increase from 20% to 42%. Despite careful and regular follow-up evaluations, the general condition of the patient slowly worsened. Five months after surgery she died of hepatorenal failure and low cardiac output. This case report is thought to be the first description of congenital complete heart block, cTGA, and situs inversus totalis with noncompaction of the myocardium of both ventricles.
authors.affiliation: Department of Cardiovascular Surgery, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Riza
authors.initials: R
authors.lastname: Dogan
authors.forename: Omer Faruk
authors.initials: OF
authors.lastname: Dogan
authors.forename: Mehmet
authors.initials: M
authors.lastname: Oc
authors.forename: Umit
authors.initials: U
authors.lastname: Duman
authors.forename: Suheyla
authors.initials: S
authors.lastname: Ozkutlu
authors.forename: Alpay
authors.initials: A
authors.lastname: Celiker
mesh_terms: D000317:Adrenergic alpha-Antagonists / Q000627:therapeutic use; D004452:Echocardiography; D015150:Echocardiography, Doppler; D017809:Fatal Outcome; D005260:Female; D006327:Heart Block / Q000150:complications / Q000151:congenital / Q000601:surgery; D006330:Heart Defects, Congenital / Q000150:complications* / Q000000981:diagnostic imaging; D006352:Heart Ventricles; D006801:Humans; D007231:Infant, Newborn; D010138:Pacemaker, Artificial; D011224:Prazosin / Q000627:therapeutic use; D012857:Situs Inversus / Q000150:complications; D013318:Stroke Volume / Q000187:drug effects; D014188:Transposition of Great Vessels / Q000150:complications* / Q000601:surgery*; D014656:Vascular Surgical Procedures
chemical_list: D000317:Adrenergic alpha-Antagonists; D011224:Prazosin