These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Left ventricular aneurysm in a patient with mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome): clinical and pathological correlation.
    Author: Oudit GY, Butany J, Williams WG, Siu SC, Clarke JT, Iwanochko RM.
    Journal: Cardiovasc Pathol; 2007; 16(4):237-40. PubMed ID: 17637432.
    Abstract:
    A 22-year-old man was diagnosed with mucopolysaccharidosis type VI, also known as Maroteaux-Lamy syndrome, which is a known cause of cardiac valvular disease. He presented with exercise intolerance and was diagnosed with a large, apical, left ventricular aneurysm (LV aneurysm) and subsequently underwent left ventricular aneurysmectomy with improvement in clinical status. Previous echocardiograms revealed that the LV aneurysm was new and is, therefore, likely acquired rather than congenital. Pathology confirmed a true aneurysm, replacement fibrosis, and PAS-positive material in cardiomyocytes. Subsequent echocardiography revealed progression of valvular heart disease with moderate stenosis of the aortic and mitral valves. We propose that altered metabolism of glycosaminoglycans in the extracellular matrix may have contributed to the development of the LV aneurysm in this patient.
    [Abstract] [Full Text] [Related] [New Search]