Isolated Left Ventricular Noncompaction Syndrome

Christopher Stanton, Charles Bruce, Heidi Connolly, Peter Brady, Imran Syed, David Hodge, Samuel Asirvatham, Paul Friedman

Research output: Contribution to journalArticle

119 Scopus citations

Abstract

Isolated left ventricular noncompaction (ILVNC) is a rare congenital cardiomyopathy characterized by prominent trabeculae, deep intertrabecular recesses, and thickened myocardium with 2 distinct layers (compacted and noncompacted). Clinical characteristics, outcomes, and appropriate therapies remain poorly defined. Data were collected on patients diagnosed with ILVNC by echocardiographic criteria at the Mayo Clinic from 2001 through 2006. These data were entered prospectively into a clinical database and retrospectively analyzed. All-cause mortality, stroke, and development of atrial fibrillation (AF) were compared to community and nonischemic dilated cardiomyopathic (DC) controls. Implantable cardioverter-defibrillator (ICD) therapies were examined. Thirty patients with confirmed ILVNC were included in analyses (mean age at diagnosis 39 ± 19.5 years, 60% men). Three patients with ILVNC died during follow-up (mean 2.5 ± 1.2 years) compared to 5 DC and 1 community controls. No mortality difference was observed among these groups (p = 0.42 and 0.054, respectively). No ILVNC deaths were observed in patients with normal LV ejection fraction. New-onset AF was diagnosed in 2 patients with ILVNC, and none was observed in DC controls. Stroke occurred in 2 DC controls and none was observed in patients with ILVNC. ICDs were implanted in 11 patients with ILVNC. No appropriate therapies were identified during follow-up, but 2 patients underwent inappropriate therapies related to AF. In conclusion, mortality in patients with ILVNC is similar to that in DC patients. Deaths were observed only in patients with decreased LV ejection fraction, suggesting that ICD therapy may be reserved for this subgroup. New-onset AF may lead to inappropriate ICD discharges.

Original languageEnglish (US)
Pages (from-to)1135-1138
Number of pages4
JournalAmerican Journal of Cardiology
Volume104
Issue number8
DOIs
StatePublished - Oct 15 2009

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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