Author + information
- Melody Hermel,
- Erika Jones,
- Annahita Sarcon,
- Saif Azam,
- Luanda Grazette,
- Jerold Shinbane and
- Ilene Weitz
To our knowledge, atypical Hemolytic Uremic Syndrome (aHUS) causing myocarditis in adults has not been previously reported.
A 33 year-old female with a history of Kikuchi disease and immune thrombocytopenia was admitted for cholangitis. A CT Abdomen showed biliary ductal dilation as well as a small pericardial effusion. Transthoracic echocardiogram revealed global hypokinesis with an ejection fraction of 25-30%. She was referred to cardiology for further evaluation.
Her hospital course was notable for newly diagnosed deep venous thromboses treated with low molecular weight heparin followed by apixaban. Labs were significant for anti-phospholipid syndrome. Evaluation of her hypoxia and shortness of breath included a CT pulmonary angiogram which was negative for a pulmonary embolism, but showed subendocardial hypoenhancement consistent with myocarditis. The patient underwent right and left cardiac catheterization, which revealed normal coronary arteries, with an elevated pulmonary capillary wedge pressure of 36mmHg, low cardiac output of 3.15L/min and cardiac index of 1.9L/min/m2. Right ventricular biopsy was unrevealing. Cardiac MR showed moderate left ventricular enlargement, a small thrombus in the left ventricular apex, and extensive subendocardial gadolinium enhancement of the left ventricle. Treatment of her acute decompensated heart failure included milrinone 0.5mcg/kg/min and her acute renal failure required continuous renal replacement therapy. Renal biopsy showed thromboembolic microangiopathy indicating a diagnosis of aHUS. She was treated with 3 days of 500mg methylprednisolone, 4 days of 40mg dexamethasone and Eculizumab 900mg IV weekly.
aHUS is a complement-mediated disease characterized by hemolytic anemia, thrombocytopenia and acute kidney disease. Only a limited number of cases of cardiovascular involvement in HUS related to infection have been described in children. When clinically indicated, pursuing a renal biopsy can help promptly diagnose and guide appropriate therapy. This is the first known report of aHUS - confirmed by a renal biopsy - presenting with myocarditis and cardiogenic shock in an adult.
Poster Hall, Hall A/B
Sunday, March 11, 2018, 9:45 a.m.-10:30 a.m.
Session Title: FIT Clinical Decision Making: Heart Failure and Cardiomyopathies
Abstract Category: Heart Failure and Cardiomyopathies
Presentation Number: 1191-130
- 2018 American College of Cardiology Foundation