Left ventricular noncompaction syndrome masquerading or misdiagnosed as congenital long QT syndrome: Remember QT prolongation does not equal long QT syndrome

Mira A. Coleman, J. Martijn Bos, Sabrina D. Phillips, Joseph J. Souza, Michael J. Ackerman

Research output: Contribution to journalArticle

2 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)492-498
Number of pages7
JournalCongenital Heart Disease
Volume6
Issue number5
DOIs
StatePublished - Sep 1 2011

Keywords

  • Arrhythmias
  • LQTS
  • LVNC
  • Left ventricular noncompaction
  • Long QT syndrome
  • QT prolongation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery
  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

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