title: Isolated noncompaction of the left ventricular myocardium in adults: a systematic overview.
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.
  
  
  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.
  
  
  CONCLUSIONS
  LVNC is an increasingly recognized cardiomyopathy diagnosed by echocardiography and is associated with familial tendencies, arrhythmias, thromboembolism, advanced heart failure, and death.
authors.affiliation: Cardiovascular Division, Mayo Clinic Arizona, Scottsdale, Arizona 85259-5499, USA.
authors.forename: Nisha L
authors.initials: NL
authors.lastname: Bhatia
authors.forename: A Jamil
authors.initials: AJ
authors.lastname: Tajik
authors.forename: Susan
authors.initials: S
authors.lastname: Wilansky
authors.forename: D Eric
authors.initials: DE
authors.lastname: Steidley
authors.forename: Farouk
authors.initials: F
authors.lastname: Mookadam
mesh_terms: D000328:Adult; D000367:Age Factors; D002986:Clinical Trials as Topic / Q000379:methods; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000000981:diagnostic imaging* / Q000401:mortality / Q000628:therapy*; D014463:Ultrasonography