The American Journal of Medicine
Volume 123, Issue 2 , Pages e3-e4, February 2010

Acute Cardiomyopathy in a Patient with Thrombotic Thrombocytopenic Purpura

  • Huichun Zhan, MD

      Affiliations

    • Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Md
    • Corresponding Author InformationRequests for reprints should be addressed to Huichun Zhan, Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Ross Research 1032, 720 Rutland Ave, Baltimore, MD 21205
    • ,
  • Haoyi Zheng, MD, PhD

      Affiliations

    • Division of Cardiology, University of North Carolina, Chapel Hill
    • ,
  • Alison R. Moliterno, MD

      Affiliations

    • Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Md
    • ,
  • Michael B. Streiff, MD

      Affiliations

    • Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Md

Article Outline

 

To the Editor:

Acute stress cardiomyopathy, also called Takotsubo cardiomyopathy or “broken heart syndrome,” is a unique syndrome of heart failure and transient left ventricular systolic dysfunction precipitated by acute emotional or physical stress.1 To our knowledge, thrombotic thrombocytopenic purpura has never been related to acute stress cardiomyopathy in the literature. We describe the first case of acute stress cardiomyopathy associated with thrombotic thrombocytopenic purpura.

A 45-year-old African-American woman with history of hypertension, newly diagnosed discoid lupus (on topical steroid treatment), and morbid obesity (BMI=53) presented with 2 weeks symptoms of progressive severe fatigue and shortness of breath as well as intermittent chest discomfort. Clinical examination revealed dyspnea on minor exertion. Laboratory investigations showed a white blood cell count of 16.5×109/L, hemoglobin 8.7 g/dL, platelet count 15×109/L, creatinine 1.9 mg/dL, and lactate dehydrogenase 3246 U/L. The patient had an elevated troponin I at 2.01 ng/mL (normal, 0.00-0.059 ng/mL) and an elevated creatine kinase-MB at 11 ug/L (normal, 0-7 ug/L). Initial electrocardiogram did not reveal any ST-T abnormality. There were numerous schistocytes on peripheral blood smear. The diagnosis of thrombotic thrombocytopenic purpura was considered, and the patient was started on emergent plasma exchange and methylprednisolone 100 mg intravenously every 12 hours in the cardiac care unit. Coronary angiography was not performed due to patient's severe thrombocytopenia. Beta-blocker and statin therapy was initiated and aspirin was started later when her platelet count rose above 50 ×109/L. ADAMTS-13 protease activity was markedly reduced at 1% (normal, 70%-150%) with undetectable autoantibody. The patient improved rapidly on plasma exchange and was transferred out of the cardiac care unit. Her troponin peaked at 3.92 ng/mL on hospital day 2 and creatinine peaked at 3.2 mg/dL on hospital day 3. The patient received a total of 11 plasma exchanges and 2 weekly doses of Rituximab due to her high-risk features (based on unpublished data at the Johns Hopkins Hospital) and was discharged to outpatient Hematology and Cardiology follow-up.

An echocardiogram obtained at admission revealed “moderate to severely reduced left ventricular systolic function with estimated left ventricular ejection fraction of 30% with global hypokinesis of the left ventricle, severe hypokinesis of the inferoseptal and inferior walls, and moderate-severe global hypokinesis of the right ventricle.” A repeat echocardiogram 9 days later revealed “normal left ventricular ejection fraction of 65%, normal regional wall motion, and normal right ventricular global systolic function.” Transient QT prolongation (QTc=519 ms) and nonspecific T-wave changes developed during the first 24 hours after admission, but all resolved very quickly.

Acute stress cardiomyopathy is characterized by the presence of an acute trigger, some electrocardiographic abnormalities including transient QT prolongation and T-wave abnormality, mild elevation of cardiac enzymes, an absence of significant coronary disease, and rapid recovery of left ventricular systolic function.1 The report of acute stress cardiomyopathy in our case demonstrated that cardiac injury in thrombotic thrombocytopenic purpura could occur in a spectrum—from transient cardiomyopathy to cardiogenic shock.2

Thrombotic thrombocytopenic purpura is a rare disease characterized by disseminated microvascular occlusion that causes variable signs and symptoms of organ ischemia and damage. In fact, myocardial microthrombi are frequently found in thrombotic thrombocytopenic purpura patients in autopsy studies.3 In addition, massive hemolysis and the release of free hemoglobin into the plasma in thrombotic thrombocytopenic purpura would cause accumulated oxidative stress and impaired nitric oxide bioavailability, leading to coronary endothelial dysfunction and vasoconstriction.4 All these could contribute to the pathophysiology of acute stress cardiomyopathy.

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References 

  1. Wittstein IS. Acute stress cardiomyopathy. Curr Heart Fail Rep. 2008;5:61–68
  2. Sane DC, Streer NP, Owen J. Myocardial necrosis in patients with thrombotic thrombocytopenic purpura: pathophysiology and rationale for specific therapy. Eur J Haematol. 2009;82:83–92
  3. Ridolfi RL, Hutchins GM, Bell WR. The heart and cardiac conduction system in thrombotic thrombocytopenic purpura (A clinicopathologic study of 17 autopsied patients). Ann Intern Med. 1979;91:357–363
  4. Gladwin MT, Vichinsky E. Pulmonary complications of sickle cell disease. N Engl J Med. 2008;359:2254–2265

 Funding: None.

 Conflict of Interest: The authors state that they have no conflict of interest.

 Authorship: All authors had access to the data and played a role in writing this manuscript.

PII: S0002-9343(09)00869-9

doi:10.1016/j.amjmed.2009.07.021

The American Journal of Medicine
Volume 123, Issue 2 , Pages e3-e4, February 2010

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