Noncompaction of ventricular myocardium in a patient with congenitally corrected transposition of the great arteries treated surgically: case report

• Published in: The Heart surgery forum Vol. 8; no. 2; p. E110
• Main Authors: Dogan, Riza, Dogan, Omer Faruk, Oc, Mehmet, Duman, Umit, Ozkutlu, Suheyla, Celiker, Alpay
• Format: Journal Article
• Language: English
• Published: United States 2005
• Subjects: Adrenergic alpha-Antagonists - therapeutic use; Echocardiography; Echocardiography, Doppler; Fatal Outcome; Female; Heart Block - complications; Heart Block - congenital; Heart Block - surgery; Heart Defects, Congenital - complications; Heart Defects, Congenital - diagnostic imaging; Heart Ventricles; Humans; Infant, Newborn; Pacemaker, Artificial; Prazosin - therapeutic use; Situs Inversus - complications; Stroke Volume - drug effects; Transposition of Great Vessels - complications; Transposition of Great Vessels - surgery; Vascular Surgical Procedures
• ISSN: 1522-6662
• DOI: 10.1532/HSF98.20041142

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.