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Magnetic resonance imaging findings of isolated right ventricular hypoplasia

  • Hyungjin Kim
  • Eun-Ah ParkEmail author
  • Whal Lee
  • Jin Wook Chung
  • Jae Hyung Park
  • Gi Beom Kim
  • Woong-Han Kim
Original Paper

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.

Keywords

Isolated right ventricular hypoplasia Cyanotic congenital heart disease Cardiac magnetic resonance imaging CMR Noncompaction 

Notes

Conflict of interest

None.

Supplementary material

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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Copyright information

© Springer Science+Business Media Dordrecht 2012

Authors and Affiliations

  • Hyungjin Kim
    • 1
  • Eun-Ah Park
    • 1
    Email author
  • Whal Lee
    • 1
  • Jin Wook Chung
    • 1
  • Jae Hyung Park
    • 1
  • Gi Beom Kim
    • 2
  • Woong-Han Kim
    • 3
  1. 1.Department of RadiologySeoul National University HospitalJongno-gu, SeoulKorea
  2. 2.Department of PediatricsSeoul National University HospitalSeoulKorea
  3. 3.Department of Thoracic and Cardiovascular SurgerySeoul National University HospitalSeoulKorea

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