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Cardiac manifestations in mucopolysaccharoidosis

Ankur Phatarpekar1, Milind Phadke1, Charan Lanjewar1

  • 1Department of Cardiology, Seth G.S. Medical College & King Edward Memorial Hospital, Mumbai, India.

Indian Heart Journal
|May 3, 2016
PubMed

Insights

Patients with mucopolysaccharoidosis (MPS) and left ventricular (LV) dysfunction require coronary artery screening. Advanced imaging like intravascular ultrasound (IVUS) is recommended over CT scans for accurate obstruction assessment.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Imaging

Background:

  • Mucopolysaccharoidosis (MPS) is a group of rare genetic disorders.
  • Cardiac complications, including left ventricular (LV) dysfunction, are common in MPS patients.
  • Coronary artery abnormalities can occur in MPS, potentially leading to significant cardiovascular events.

Observation:

  • This case highlights a patient with MPS experiencing LV dysfunction.
  • Coronary artery screening was deemed necessary in this patient.
  • Computed tomography (CT) scan was considered suboptimal for detailed assessment of coronary obstruction.

Findings:

  • Intravascular ultrasound (IVUS) is a superior modality for delineating concentric coronary obstruction in MPS patients.
  • Early and thorough screening for hypertension is crucial in all individuals with MPS.
  • Coronary artery abnormalities necessitate careful evaluation in MPS patients with LV dysfunction.

Implications:

  • Implementing advanced imaging techniques like IVUS can improve diagnostic accuracy for coronary artery disease in MPS.
  • Systematic screening for hypertension in MPS patients may prevent or mitigate cardiovascular complications.
  • These findings underscore the importance of a multidisciplinary approach to managing cardiac health in MPS.

Keywords:
Coronary artery diseaseHypertensionMitral valveMucopolysaccharoidosis

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