Journal of the Korean Society of Radiology (대한영상의학회지)

The Korean Society of Radiology (대한영상의학회)
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Left Ventricular Noncompaction in Adults: Imaging and Clinical Findings in 63 Patients

성인 좌심실 비치밀화증: 성인 63명의 영상의학적 소견과 임상양상

  • Cho, Se Jin (Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Yang, Dong Hyun (Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kang, Joon-Won (Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Lim, Tae-Hwan (Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine)
  • 조세진 (울산대학교 의과대학 서울아산병원 영상의학과, 영상의학과 연구소) ;
  • 양동현 (울산대학교 의과대학 서울아산병원 영상의학과, 영상의학과 연구소) ;
  • 강준원 (울산대학교 의과대학 서울아산병원 영상의학과, 영상의학과 연구소) ;
  • 임태환 (울산대학교 의과대학 서울아산병원 영상의학과, 영상의학과 연구소)
  • Received : 2017.04.11
  • Accepted : 2017.10.01
  • Published : 2018.03.01
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Abstract

Purpose: To describe imaging and clinical findings for a left ventricular noncompaction (LVNC) in the adult. Materials and Methods: From 2000 to 2014, 63 patients were diagnosed with LVNC by echocardiography, computed tomography, and magnetic resonance imaging at our hospital. Baseline characteristics, clinical manifestations, combined cardiac or systemic anomalies, and imaging findings were reviewed. We made a comparison between the isolated and combined disease groups. Results: Among 63 patients with LVNC, 32 (51%) patients did not have combined cardiac anomalies (isolated disease group). The mean age at the initial diagnosis was higher in the isolated than in the combined disease group (54.2 years vs. 40.2 years, p < 0.001). The combined disease group presented symptoms more frequently at initial diagnosis than the isolated disease group (94% vs. 75%, p = 0.082). Heart failure symptoms were the most common adverse events (60.3% in all patients). Thromboembolic events developed in 20 patients, and were more frequent in the combined disease group than in the isolated disease group (39% vs. 26%, p = 0.279). The most common cardiac abnormality was dilated cardiomyopathy (n = 15, 24%). There was no significant difference in the mean noncompacted/compacted ratios between both of the disease groups. Conclusion: Isolated and combined LVNC disease groups showed differences in age at diagnosis and clinical manifestations. The clinical and imaging findings may be helpful to better understand LVNC.

목적: 성인 심실 비치밀화증 환자의 영상의학적 소견과 임상양상을 기술하고자 하였다. 대상과 방법: 2000~2014년까지 본원에서 심 초음파, CT, 그리고 MRI 검사를 통해 63명의 환자가 심실 비치밀화증으로 진단되었다. 해당 심실 비치밀화증 환자를 대상으로 기본 질환 특징, 임상 양상, 동반 심 질환, 영상의학적 소견을 분석하였다. 그리고 동반 심 질환의 유무에 따라 단독 질환군과 복합 질환군을 구분하여 비교하였다. 결과: 63명의 환자 중 동반 심 질환이 없는 단독 질환군 환자는 32명(51%)이었다. 단독 질환군에서 복합 질환군에 비해 처음 진단될 당시의 나이가 많았다(54.2세 vs. 40.2세, p < 0.001). 복합 질환군에서 단독 질환군에 비해 진단 당시 증상이 더 많았다(94% vs. 75%, p = 0.082). 두 그룹 모두에서 심부전증과 연관된 증상이 가장 많았다(60.3%). 전체 환자에서 혈전색전증이 발생한 경우는 20명이었는데, 복합 질환군에서 단독 질환군보다 많았다(39% vs. 26%, p = 0.279). 동반 심 질환 중 가장 흔한 것은 확장성 심근병증이었다(n = 15, 24%). 영상의학적 소견상 두 질환군의 비치밀화/치밀화 비는 유의한 차이가 없었다. 결론: 성인 심실 비치밀화증은 단독 질환군과 복합 질환군, 두 그룹 간에 진단 당시의 나이와 임상양상에 차이가 있다. 임상양상과 영상의학적 소견은 심실 비치밀화증에 대한 이해에 도움이 될 것이다.

Keywords

Acknowledgement

Supported by : National Research Foundation of Korea (NRF), Asan Institute for Life Sciences, Asan Medical Centre

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