PMID: 11934600Apr 6, 2002Paper

Left ventricular aneurysm, aortic valve disease and coronary narrowing in a patient with Hunter's syndrome

Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology
David I KettlesChristopher Davidson

Abstract

Hunter's syndrome (mucopolysaccharidosis type 2, MPS 2) is an inherited disorder of glycosaminoglycan degradation commonly associated with cardiac disease. We present the case of a young man with unusual cardiac manifestations of this syndrome. When mixed aortic valve disease was noted in childhood, other classical features of the milder form of Hunter's syndrome were present. There was no symptomatic or echocardiographic cardiovascular deterioration until age 27 when the patient presented in severe biventricular failure. Investigations demonstrated cardiomegaly and a large apical left ventricular aneurysm. The patient died suddenly soon after this diagnosis. Post mortem examination demonstrated a hypertrophied left ventricle with a 6-cm apical aneurysm. Coronary arterial walls were diffusely thickened but with only mild lumenal stenosis. Mitral and aortic valve disease was also present. There is one previous report of ventricular aneurysm in Hunter's syndrome. Pathophysiological contributions to aneurysm formation may include abnormal coronary flow, the presence of aortic stenosis and abnormal myocardium. This patient's sudden deterioration after a long period of clinical stability reinforces the need for careful follow-up of ...Continue Reading

References

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Citations

Jul 12, 2011·Journal of Inherited Metabolic Disease·Elizabeth A BraunlinRoberto Giugliani
Apr 27, 2010·Cardiology in the Young·Gabriela N LealChong A Kim
Feb 11, 2014·Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology·Elizabeth BraunlinJ Carlos Manivel
Jul 17, 2010·Veterinary Pathology·P HerráezA Espinosa de Los Monteros
Jun 15, 2004·Radiologic Clinics of North America·André J Duerinckx
Nov 11, 2009·Pediatrics·Joseph MuenzerE Wraith

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