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Magnetic resonance imaging findings of isolated right ventricular hypoplasia

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Abstract

Isolated right ventricular hypoplasia, unassociated with severe pulmonary or tricuspid valvular malformation, is a rare congenital heart disease. Herein the authors report a case of a 6-year-old girl having isolated right ventricular hypoplasia treated with one and a half ventricular repair, and describe the cardiac magnetic resonance (CMR) imaging findings. CMR imaging revealed diffuse subendocardial or transmural late gadolinium enhancement of the right ventricle with a thin, smooth wall due to the absence of trabeculations. Noncompaction of the left ventricle was also demonstrated, although the relevance to isolated right ventricular hypoplasia is not obviously explicable.

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Authors and Affiliations

  1. Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 110-744, Korea

    Hyungjin Kim, Eun-Ah Park, Whal Lee, Jin Wook Chung & Jae Hyung Park

  2. Department of Pediatrics, Seoul National University Hospital, Seoul, Korea

    Gi Beom Kim

  3. Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul, Korea

    Woong-Han Kim

Authors
  1. Hyungjin Kim
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  2. Eun-Ah Park
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  3. Whal Lee
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  4. Jin Wook Chung
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  5. Jae Hyung Park
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  6. Gi Beom Kim
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  7. Woong-Han Kim
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Corresponding author

Correspondence to Eun-Ah Park.

Electronic supplementary material

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Online resource 1 Initial cardiac magnetic resonance (CMR): 4-chamber cine image. Right ventricular wall was akinetic except for the basal lateral wall. Bowing of interventricular septum was noted during early diastole. (MPG 216 kb)

Online resource 2 Four-year follow-up cardiac magnetic resonance (CMR): 4-chamber cine image. Right ventricle was severely distended and deviated to the left ventricular side. Paradoxical septal motion due to bowing of interventricular septum during early diastole was also aggravated. (MPG 251 kb)

Online resource 3 Four-year follow-up cardiac magnetic resonance (CMR): short-axis cine image of mid–ventricular level. The right ventricle was severely distended and deviated to the left ventricular side. Paradoxical septal motion due to bowing of interventricular septum during early diastole was also aggravated. (MPG 251 kb)

Online resource 4 Four-year follow-up cardiac magnetic resonance (CMR): through-plane phase contrast image in the tricuspid valve plane. Tricuspid regurgitation due to poor coaptation was observed. Aliasing artifact was noticed. (MPG 212 kb)

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Kim, H., Park, EA., Lee, W. et al. Magnetic resonance imaging findings of isolated right ventricular hypoplasia. Int J Cardiovasc Imaging 28 (Suppl 2), 149–152 (2012). https://doi.org/10.1007/s10554-012-0162-x

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  • DOI: https://doi.org/10.1007/s10554-012-0162-x

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