title: Left ventricular noncompaction syndrome masquerading or misdiagnosed as congenital long QT syndrome: remember QT prolongation does not equal long QT syndrome.
abstract: Distinguishing congenital long QT syndrome from QT prolongation caused by drugs or a different underlying disease process is essential for selecting the proper treatment. Herein, we present a case of a patient referred for left cardiac sympathetic denervation as a last resort treatment option for her 19-year standing diagnosis of long QT syndrome with malignant ventricular fibrillation. However, based on her atypical clinical course and additional imaging studies, a diagnosis of left ventricular noncompaction, rather than long QT syndrome, was made. She left the clinic with a drastically different treatment plan and an improved quality of life. Because many cardiac and noncardiac diseases can demonstrate QT prolongation on electrocardiogram, all possible diagnoses should be considered before diagnosing a patient with congenital long QT syndrome especially with regard to the profound treatment implications and genetic follow-up in family members.
authors.affiliation: Mayo Medical School, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors.forename: Mira A
authors.initials: MA
authors.lastname: Coleman
authors.forename: J Martijn
authors.initials: JM
authors.lastname: Bos
authors.forename: Sabrina D
authors.initials: SD
authors.lastname: Phillips
authors.forename: Joseph J
authors.initials: JJ
authors.lastname: Souza
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Ackerman
mesh_terms: D000328:Adult; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D002304:Cardiac Pacing, Artificial; D017147:Defibrillators, Implantable; D003951:Diagnostic Errors; D004554:Electric Countershock / Q000295:instrumentation; D004562:Electrocardiography; D005260:Female; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D008133:Long QT Syndrome / Q000151:congenital / Q000175:diagnosis*; D011237:Predictive Value of Tests; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014693:Ventricular Fibrillation / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy
chemical_list: D000889:Anti-Arrhythmia Agents