Fulminant valvulitis from acute rheumatic fever: successful use of extracorporeal support.

Objective: To describe an unusual case of fulminant rheumatic fever presenting acutely as severe respiratory failure managed with extracorporeal membrane oxygenation and ultimately valve replacement while on extracorporeal membrane oxygenation.

Design: Case report.

Setting: Large quaternary care pediatric intensive care unit.

Patient: A 6-yr-old female with profound respiratory failure found to be due to mitral valve dysfunction stemming from acute fulminant rheumatic fever.

Interventions And Main Results: The patient was originally maintained on venovenous extracorporeal membrane oxygenation but required conversion to venoarterial extracorporeal membrane oxygenation due to the progression of her mitral valve disease. Her condition did not improve with atrial septostomy, and she required valve replacement while anticoagulated. She was decannulated in the operating room and extubated 2 days later, and she survived to discharge. The institutional review board subsequently granted a waiver of consent for a report of this case.

Conclusions: Manifestations of rheumatic fever can develop acutely even in the setting of an industrialized country. Valvulitis with severe, isolated mitral valve dysfunction may masquerade initially as respiratory failure. Multiple invasive procedures can be performed successfully while patients are fully anticoagulated and on extracorporeal membrane oxygenation support.