Preoperative cardiac POCUS for urgent surgery in a patient with Maroteaux-Lamy syndrome

The silent development of cardiac disease is not uncommon in patients with MPS VI, as pulmonary involvement, skeletal deformities and limited exercise tolerance can mask underlying heart failure. HIPAA authorization has been obtained from the patient and parent to publish this manuscript.1 Case desc...

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Bibliographic Details
Published in:Journal of clinical anesthesia 2021-09, Vol.72, p.110296-110296, Article 110296
Main Authors: Salik, Irim, Rodhouse, Heather Brosnan, Mehta, Bhupen, Villion, Andrew
Format: Article
Language:English
Subjects:
Catheters
Enzymes
Hydrocephalus
Ostomy
Patients
Surgery
Tracheotomy
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Summary:The silent development of cardiac disease is not uncommon in patients with MPS VI, as pulmonary involvement, skeletal deformities and limited exercise tolerance can mask underlying heart failure. HIPAA authorization has been obtained from the patient and parent to publish this manuscript.1 Case description The patient was a 23-year-old male with a history of MPS-VI, presenting with skeletal dysplasia with dwarfism-like features, severe muscle weakness and joint stiffness, cervical stenosis, hydrocephalus requiring a ventriculopleural shunt, and corneal clouding leading to blindness. Preoperatively, cardiac POCUS has shown utility in patients with pericardial disease, ventricular failure, regional wall motion abnormalities, significant valvular disease, and in the evaluation of volume status [10].
ISSN:0952-8180
1873-4529
DOI:10.1016/j.jclinane.2021.110296