Iranian Journal of Medical Sciences

Document Type: Case Report(s)

Authors

1 Cardiovascular Research Center, Hormozgan University of Medical Sciences, Bandar Abbas, Iran

2 Hematologist, Hormozgan University of Medical Sciences, Bandar Abbas, Iran

3 Cardiovascular Research Center, Hormozgan University of Medical Sciences, Bandar Abbas, Iran and Molecular Medicine Research Center, Hormozgan University of Medical Sciences, Bandar Abbas, Iran

4 Internist, Hormozgan University of Medical Sciences, Bandar Abbas, Iran

Abstract

In patients with sickle cell disease, thrombotic microangiopathy is a rare complication. Also in sickle cell disease, intracardiac thrombus formation without structural heart diseases or atrial arrhythmias is a rare phenomenon. We herein describe a 22-year-old woman, who was a known case of sickle cell-βthalassemia, had a history of recent missed abortion, and was admitted with a vaso-occlusive crisis. The patient had manifestations of microangiopathic hemolytic anemia, including laboratory evidence of hemolytic anemia, thrombocytopenia, respiratory distress, fever, jaundice, and abnormal liver function and coagulation tests, accompanied by clot formation on the Eustachian valve of the inferior vena cava in the right atrium and also a long and worm-like thrombus in the right ventricle. Therapeutic plasma exchange improved her clinical condition, and her intracardiac thrombus was completely resolved after 1 week. Echocardiography, as a simple and inexpensive imaging modality, had a significant role in the diagnosis and follow-up of this patient.

Keywords

  1. Shome DK, Ramadorai P, Al-Ajmi A, Ali F, Malik N. Thrombotic microangiopathy in sickle cell disease crisis. Ann Hematol. 2013;92:509-15. doi: 10.1007/s00277-012-1647-3. PubMed PMID: 23224266.
  2. The European Cooperative Study on the clinical significance of right heart thrombi. European Working Group on Echocardiography. Eur Heart J. 1989;10:1046-59. PubMed PMID: 2606115.
  3. Savage HO, Ding N, Eso O, Sachdev B, Lefroy DL. Mobile Right Atrial Thrombi in a Patient with the Hemoglobin SC Disease. Case Rep Med. 2011;2011:897167. doi: 10.1155/2011/897167. PubMed PMID: 21912556; PubMed Central PMCID: PMC3168297.
  4. Chartier L, Bera J, Delomez M, Asseman P, Beregi JP, Bauchart JJ, et al. Free-floating thrombi in the right heart: diagnosis, management, and prognostic indexes in 38 consecutive patients. Circulation. 1999;99:2779-83. doi: 10.1161/01.CIR.99.21.2779. PubMed PMID: 10351972.
  5. Mogulkoc N, Burgess MI, Bishop PW. Intracardiac thrombus in Behcet’s disease: a systematic review. Chest. 2000;118:479‑87. PubMed PMID: 10936144.
  6. Cemri M, Erkan A, Ozdemir M, Cengel A. Behcet’s disease with a large and free right atrial thrombus. Eur J Echocardiogr. 2002;3:233-5. doi: 10.1053/euje.3.3.233. PubMed PMID: 12144843.
  7. Waller BF, Rohr TM, McLaughlin T, Grider L, Taliercio CP, Fetters J. Intracardiac thrombi: frequency, location, etiology, and complications: a morphologic review--Part V. Clin Cardiol. 1995;18:731-4. PubMed PMID: 8608674.
  8. Yeghen T, Benjamin S, Boyd O, Pumphrey C, Bevan DH. Sickle cell anemia, right atrial thrombosis, and the antiphospholipid antibody. Am J Hematol. 1995;50:46-8. doi: 10.1002/ajh.2830500109. PubMed PMID: 7668223.
  9. Tavel ME, Goldhaber SZ, Moser KM. Rapidly progressing dyspnea associated with a mass in the right side of the heart. Chest. 1995;107:866-8. PubMed PMID: 7874967.