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November 2018

Clinical and Genetic Complexities of Left Ventricular Noncompaction: Preventing Overdiagnosis in a Disease We Do Not Understand

Author Affiliations
  • 1Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, Sydney, Australia
  • 2University of Sydney, Sydney, Australia
  • 3Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia
JAMA Cardiol. 2018;3(11):1033-1034. doi:10.1001/jamacardio.2018.2465

Left ventricular noncompaction (LVNC) is a cardiomyopathy characterized by prominent and excessive trabeculation of the left ventricle. A number of different subgroups of LVNC exist, including nonisolated LVNC, in which LVNC is identified in combination with an additional cardiac or syndromic disease, and isolated LVNC, in which it is not. The proposed pathogenic mechanism that underlies the development of LVNC is an arrest in compaction; however, this does not explain the development of LVNC in adulthood. Recent studies suggest that LV trabeculation can occur as a physiological adaptation to increased cardiac preload, as in athletes or during pregnancy (Box).1 Many asymptomatic individuals who fulfill diagnostic criteria for LVNC remain unaffected, and follow-up data suggest that life expectancy is consistent with the general population.2 In contrast, symptomatic individuals can develop major adverse events, including heart failure, thromboembolism, life-threatening arrhythmias, and sudden death.