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Cardiovascular magnetic resonance findings in a case of Danon disease.

Piotrowska-Kownacka D, Kownacki L, Kuch M, Walczak E, Kosieradzka A, Fidzianska A, Krolicki L - J Cardiovasc Magn Reson (2009)

Bottom Line: Danon disease is a rare X-linked dominant lysosomal glycogen storage disease that can lead to severe ventricular hypertrophy and heart failure.We report a case of Danon disease with cardiac involvement evaluated with cardiovascular magnetic resonance, including late gadolinium enhancement and perfusion studies.

View Article: PubMed Central - HTML - PubMed

Affiliation: II Department of Radiology, Medical University of Warsaw, Warsaw, Poland. dpiotrowska@tlen.pl

ABSTRACT
Danon disease is a rare X-linked dominant lysosomal glycogen storage disease that can lead to severe ventricular hypertrophy and heart failure. We report a case of Danon disease with cardiac involvement evaluated with cardiovascular magnetic resonance, including late gadolinium enhancement and perfusion studies.

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Related in: MedlinePlus

On the left site SSFP image in 4 chamber orientation – signal lost due to tricuspid and mitral valves insufficiency. On the right site velocity encoded phase contrast image in short axis orientation at the level of mitral and tricuspid valves – black spots indicate valves insufficiency.
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Figure 1: On the left site SSFP image in 4 chamber orientation – signal lost due to tricuspid and mitral valves insufficiency. On the right site velocity encoded phase contrast image in short axis orientation at the level of mitral and tricuspid valves – black spots indicate valves insufficiency.

Mentions: The CMR study showed significantly reduced global function with left ventricular EF of 14%. (Additional file 1) Left ventricular end diastolic volume (EDV) and end systolic volumes (ESV) were increased (EDV 497 ml, ESV 426 ml). LV dimension was 81 × 94 mm in the short axis orientation. Significant dilatation of the right ventricle and both atria were confirmed. Tricuspid and mitral valve insufficiency were clearly visible on 4 chamber cine images and velocity encoded images in short axis orientation at valve level. (Figure 1)


Cardiovascular magnetic resonance findings in a case of Danon disease.

Piotrowska-Kownacka D, Kownacki L, Kuch M, Walczak E, Kosieradzka A, Fidzianska A, Krolicki L - J Cardiovasc Magn Reson (2009)

On the left site SSFP image in 4 chamber orientation – signal lost due to tricuspid and mitral valves insufficiency. On the right site velocity encoded phase contrast image in short axis orientation at the level of mitral and tricuspid valves – black spots indicate valves insufficiency.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2683831&req=5

Figure 1: On the left site SSFP image in 4 chamber orientation – signal lost due to tricuspid and mitral valves insufficiency. On the right site velocity encoded phase contrast image in short axis orientation at the level of mitral and tricuspid valves – black spots indicate valves insufficiency.
Mentions: The CMR study showed significantly reduced global function with left ventricular EF of 14%. (Additional file 1) Left ventricular end diastolic volume (EDV) and end systolic volumes (ESV) were increased (EDV 497 ml, ESV 426 ml). LV dimension was 81 × 94 mm in the short axis orientation. Significant dilatation of the right ventricle and both atria were confirmed. Tricuspid and mitral valve insufficiency were clearly visible on 4 chamber cine images and velocity encoded images in short axis orientation at valve level. (Figure 1)

Bottom Line: Danon disease is a rare X-linked dominant lysosomal glycogen storage disease that can lead to severe ventricular hypertrophy and heart failure.We report a case of Danon disease with cardiac involvement evaluated with cardiovascular magnetic resonance, including late gadolinium enhancement and perfusion studies.

View Article: PubMed Central - HTML - PubMed

Affiliation: II Department of Radiology, Medical University of Warsaw, Warsaw, Poland. dpiotrowska@tlen.pl

ABSTRACT
Danon disease is a rare X-linked dominant lysosomal glycogen storage disease that can lead to severe ventricular hypertrophy and heart failure. We report a case of Danon disease with cardiac involvement evaluated with cardiovascular magnetic resonance, including late gadolinium enhancement and perfusion studies.

Show MeSH
Related in: MedlinePlus