A Rare Case of Coincidental LVNC and ALVC According to CMR

Document Type : Case Report

Authors

1 Omid Hospital, Iran University of Medical Sciences, Tehran, IR Iran.

2 Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, IR Iran.

3 Mashhad University of Medical Sciences, Mashhad, IR Iran.

4 Institute for Intelligent Systems Research and Innovation, Deakin University, Geelong, VIC, Australia.

5 Institute for Physical Activity and Nutrition, Deakin University, Melbourne, Australia

Abstract

Arrhythmogenic left ventricular cardiomyopathy (ALVC) is a rare inherited cardiomyopathy characterized by the progressive fibrofatty replacement of the left ventricular (LV) myocardium in combination with arrhythmias of LV origin. Herein, we present a case of the coincidence of ALVC and left ventricular non-compaction (LVNC) in a 34-year-old woman presenting with the chief complaint of palpitation. The patient had a history of an episode of severe common cold the previous year. Her mother was diagnosed with typical hypertrophic cardiomyopathy. The patient’s preliminary echocardiography demonstrated evidence of LVNC. Subsequent evaluation using cardiac magnetic resonance (CMR) imaging showed a mildly enlarged LV without hypertrophy, along with noticeable myocardial trabeculation (Fig. 1) and a mildly decreased systolic function. Late gadolinium enhancement illustrated a fibrotic layer in the mid-anterior LV segment (Fig. 2). There was an elongated, thick stripe of subepicardial fat deposition at the base-to-mid–anteroseptal, anterior, and anterolateral LV segments (Fig. 3 & Fig. 4). Right ventricular apical trabeculation was prominent, with a regular ventricular function. The CMR tissue characterization criteria indicated the coincidence of ALVC and LVNC. Advances in noninvasive imaging modalities have significantly improved the delineation of the morphologic appearance of ventricles in different cardiomyopathies, facilitating identification and diagnosis. (Iranian Heart Journal 2024; 25(2): 92-95)

Keywords

References

  1. Corrado D, Link MS, Calkins H. Arrhythmogenic right ventricular cardiomyopathy. New England journal of medicine. 2017; 376(1):61-72.
  2. Basso C, Corrado D, Marcus FI, Nava A, Thiene G. Arrhythmogenic right ventricular cardiomyopathy. The Lancet. 2009; 373(9671):1289-300.
  3. Norman M, Simpson M, Mogensen J, Shaw A, Hughes S, Syrris P, et al. Novel mutation in desmoplakin causes arrhythmogenic left ventricular cardiomyopathy. Circulation. 2005; 112(5):636-42.
  4. Corrado D, Basso C, Judge DP. Arrhythmogenic cardiomyopathy. Circulation research. 2017; 121(7):784-802.
  5. Shemisa K, Li J, Tam M, Barcena J. Left ventricular noncompaction cardiomyopathy. Cardiovascular Diagnosis and Therapy. 2013; 3(3):170.
Iranian Heart Journal
Volume 25, Issue 2
March 2024
Pages 92-95

Files

Share

How to cite

Statistics