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Cardiovascular Images
October 11, 2021

A Patient in Their 30s With Dyspnea and a Spongy Heart

Author Affiliations
  • 1Department of Cardiology, North Shore University Hospital, Northwell Health, Zucker School of Medicine at Hofstra/Northwell, Manhasset, New York
  • 2Department of Radiology, North Shore University Hospital, Northwell Health, Zucker School of Medicine at Hofstra/Northwell, Manhasset, New York
  • 3Multimodality Cardiovascular Imaging Laboratory, North Shore University Hospital, Northwell Health, Manhasset, New York
  • 4National Heart, Lung, and Blood Institute, National Institutes of Health, Department of Health and Human Services, Bethesda, Maryland
JAMA Cardiol. 2021;6(10):e210001. doi:10.1001/jamacardio.2021.0001

What is the most likely diagnosis in a patient in their 30s with no remarkable medical history who reported dyspnea on exertion? Heart rhythm monitoring showed episodes of nonsustained ventricular tachycardia, and echocardiography revealed reduced left ventricular (LV) systolic function. Cardiovascular magnetic resonance imaging demonstrated thinning of the LV myocardium and significantly increased trabeculations with deep intertrabecular recesses (Figure). Cine imaging confirmed severely reduced LV systolic function (Video). Late gadolinium enhancement imaging showed no definitive evidence of myocardial scarring or LV thrombus. The overall cardiovascular magnetic resonance findings strongly supported the diagnosis of noncompaction cardiomyopathy.

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