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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

Late gadolinium enhancement images in short axis orientation. LGE was present in the subendocardium and the anterior and lateral wall, apical segments and RV inferior junction point.
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Figure 3: Late gadolinium enhancement images in short axis orientation. LGE was present in the subendocardium and the anterior and lateral wall, apical segments and RV inferior junction point.

Mentions: Perfusion defects, mainly subendocardial, were visible in almost all segments on first pass images acquired at rest. They were obvious in the infero-septal segments and partly transmural in the lateral and anterior walls (Figure 2, Additional file 2). LGE was present in the subendocardium and in places transmurally in the anterior and lateral walls. A small LGE region was present in RV inferior junctional region. (Fig. 3)


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)

Late gadolinium enhancement images in short axis orientation. LGE was present in the subendocardium and the anterior and lateral wall, apical segments and RV inferior junction point.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: Late gadolinium enhancement images in short axis orientation. LGE was present in the subendocardium and the anterior and lateral wall, apical segments and RV inferior junction point.
Mentions: Perfusion defects, mainly subendocardial, were visible in almost all segments on first pass images acquired at rest. They were obvious in the infero-septal segments and partly transmural in the lateral and anterior walls (Figure 2, Additional file 2). LGE was present in the subendocardium and in places transmurally in the anterior and lateral walls. A small LGE region was present in RV inferior junctional region. (Fig. 3)

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