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Acute Systolic Heart Failure Associated with Complement-Mediated Hemolytic Uremic Syndrome.

John L Vaughn1, Jared M Moore1, Spero R Cataland2

  • 1Department of Internal Medicine, The Ohio State University, 395 W. 12th Avenue, Third Floor, Columbus, OH 43210, USA.

Case Reports in Hematology
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Complement-mediated atypical hemolytic uremic syndrome (HUS) can cause severe heart failure. This case report shows eculizumab effectively treated a patient with atypical HUS and acute systolic heart failure, improving renal and respiratory function.

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Area of Science:

  • Nephrology
  • Cardiology
  • Hematology

Background:

  • Complement-mediated atypical hemolytic uremic syndrome (HUS) is a rare thrombotic microangiopathy.
  • Uncontrolled complement activation in atypical HUS can lead to severe systemic complications, including cardiac dysfunction.
  • The association between atypical HUS and acute heart failure is not well-established.

Purpose of the Study:

  • To report a rare case of complement-mediated atypical HUS presenting with acute systolic heart failure.
  • To highlight the clinical course and management of a critically ill patient with atypical HUS and cardiac complications.
  • To underscore the therapeutic potential of eculizumab in severe complement-mediated diseases.

Main Methods:

  • Case report of a 49-year-old female with atypical HUS.
  • Diagnostic workup included ADAMSTS13 activity, complement activation markers, renal biopsy, and echocardiography.
  • Treatment involved eculizumab, mechanical ventilation, and hemodialysis.

Main Results:

  • The patient presented with microangiopathic hemolytic anemia, thrombocytopenia, acute kidney injury, and acute hypoxemic respiratory failure.
  • Echocardiography revealed Takotsubo cardiomyopathy with severely reduced left ventricular ejection fraction.
  • Treatment with eculizumab led to significant improvement in renal function, respiratory status, and cardiac function, allowing discharge.

Conclusions:

  • Complement-mediated atypical HUS can present with severe cardiac complications, including acute systolic heart failure.
  • Eculizumab demonstrated efficacy in stabilizing a critically ill patient with atypical HUS and associated cardiac dysfunction.
  • This case emphasizes the importance of recognizing and managing systemic manifestations of atypical HUS.