Acute pulmonary edema, mitral regurgitation and mucopolysaccharidosis

Case contributed by Assoc Prof Craig Hacking

Presentation

Post emergency intubation. Clinically has APO.

Patient Data

Age: 30 years
Gender: Female

The ETT and NGT are well-positioned. Tubing through the right side of the neck and over the midline of the chest likely represents VP shunt tubing which is intact.

The heart is enlarged even allowing for projection, and there is abnormal cardiac contour, in keeping with history of mitral valve disease.

There is diffuse air space opacification, more so on the right side, with perihilar infiltrates. No pleural effusion or evidence of pneumothorax. Features are in keeping with asymmetric acute pulmonary edema.

Posterior ribs are abnormal, in keeping with history of MPS.

Case Discussion

Three diagnoses on the one CXR! The patient has a background history of mitral regurgitation and is known to have MPS VI (Maroteaux-Lamy syndrome).

Mitral regurgitation is a known cause of unilateral acute pulmonary edema.

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