Isolated noncompaction of the left ventricular myocardium in adults: A systematic overview

Nisha L. Bhatia, A. Jamil Tajik, Susan Wilansky, D. Eric Steidley, Farouk Mookadam

Research output: Contribution to journalReview article

90 Scopus citations

Abstract

Background: Owing to inconsistent diagnostic criteria and small heterogeneous cohorts, little is known about the long-term outcomes of adult left ventricular noncompaction (LVNC), a rare cardiomyopathy with potentially serious outcomes. This systematic overview aimed to better delineate the natural history of adult LVNC. Method and Results: A comprehensive computerized search using "noncompaction" and its synonyms initially identified 206 articles, with reference lists subsequently hand scanned. These searches yielded 5 studies that were eligible for this systematic overview, identifying adult cohorts with isolated LVNC diagnosed by similar echocardiographic criteria. This combined cohort (n = 241) was followed for a mean duration of 39 months. The annualized event rate was 4% for cardiovascular deaths, 6.2% for cardiovascular death and its surrogates (heart transplantation and appropriate implantable cardioverter-defibrillator shocks), and 8.6% for all cardiovascular events (death, stroke, implantable cardioverter-defibrillator shocks, and heart transplantation.) Familial occurrence of LVNC in first-degree relatives was identified by echocardiography in 30% of index cases who were screened. Conclusion: LVNC is an increasingly recognized cardiomyopathy diagnosed by echocardiography and is associated with familial tendencies, arrhythmias, thromboembolism, advanced heart failure, and death.

Original languageEnglish (US)
Pages (from-to)771-778
Number of pages8
JournalJournal of Cardiac Failure
Volume17
Issue number9
DOIs
StatePublished - Sep 1 2011

Keywords

  • Cardiomyopathy
  • arrhythmias
  • heart failure
  • thromboembolism

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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