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Acute coronary syndrome in patients with hemophilia: a delicate balancing act

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Abstract

Therapeutic advances have resulted in increased life expectancy in patients with hemophilia. Consequently, the prevalence of coronary artery disease in this population is increasing. Little is known about the optimal management of acute coronary syndrome in these patients. Current guidelines for the management of this condition are based mainly on expert opinion and generally recommend administration of the clotting factor prior to the anticoagulant, antiplatelet, and interventional therapies. We report a case that illustrates the potential harm that may come from this approach: evolution of non–ST-segment elevation acute coronary syndrome into ST-elevation acute coronary syndrome during the administration of recombinant clotting factor. We review available literature and describe the refined informatics-based guidelines for managing acute coronary syndrome in patients with hemophilia we developed in response to the presented clinical case. We propose adopting this novel informatics-based approach, which aids in the identification and early treatment of these patients, operationalizes timely involvement of hematology experts, and gathers data for further study.

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References

  1. Srivastava A, Brewer AK, Mauser-Bunschoten EP et al (2013) Guidelines for the management of hemophilia. Haemophilia 19:e1-47. https://doi.org/10.1111/j.1365-2516.2012.02909.x

    Article  CAS  PubMed  Google Scholar 

  2. Sood SL, Cheng D, Ragni M et al (2018) A cross-sectional analysis of cardiovascular disease in the hemophilia population. Blood Adv 2:1325–1333. https://doi.org/10.1182/bloodadvances.2018018226

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Plug I, Van Der Bom JG, Peters M et al (2006) Mortality and causes of death in patients with hemophilia, 1992–2001: a prospective cohort study. J Thromb Haemost 4:510–516. https://doi.org/10.1111/j.1538-7836.2006.01808.x

    Article  CAS  PubMed  Google Scholar 

  4. Rosendaal FR, Varekamp I, Smit C et al (1989) Mortality and causes of death in Dutch haemophiliacs, 1973–86. Br J Haematol 71:71–76. https://doi.org/10.1111/j.1365-2141.1989.tb06277.x

    Article  CAS  PubMed  Google Scholar 

  5. Lim MY, Pruthi RK (2011) Cardiovascular disease risk factors: prevalence and management in adult hemophilia patients. Blood Coagul Fibrinolysis 22:402–406. https://doi.org/10.1097/MBC.0b013e328345f582

    Article  CAS  PubMed  Google Scholar 

  6. Goel R, Krishnamurti L (2012) Mortality, health care utilization and associated diagnoses in hospitalized patients with haemophilia in the United States: first reported nationwide estimates. Haemophilia 18:688–692. https://doi.org/10.1111/j.1365-2516.2012.02774.x

    Article  CAS  PubMed  Google Scholar 

  7. Fefer P, Gannot S, Lubetsky A et al (2013) Percutaneous coronary intervention in patients with haemophilia presenting with acute coronary syndrome: an interventional dilemma: case series, review of the literature, and tips for management. J Thromb Thrombolysis 35:271–278. https://doi.org/10.1007/s11239-012-0802-y

    Article  PubMed  Google Scholar 

  8. Boehnel C, Rickli H, Graf L, Maeder MT (2018) Coronary angiography with or without percutaneous coronary intervention in patients with hemophilia-systematic review. Catheter Cardiovasc Interv 92:1–15. https://doi.org/10.1002/ccd.27255

    Article  PubMed  Google Scholar 

  9. Ferraris VA, Boral LI, Cohen AJ et al (2015) Consensus review of the treatment of cardiovascular disease in people with hemophilia A and B. Cardiol Rev 23:53–68. https://doi.org/10.1097/CRD.0000000000000045

    Article  PubMed  PubMed Central  Google Scholar 

  10. Schutgens REG, Tuinenburg A, Roosendaal G et al (2009) Treatment of ischaemic heart disease in haemophilia patients: an institutional guideline. Haemophilia 15:952–958. https://doi.org/10.1111/j.1365-2516.2009.02020.x

    Article  CAS  PubMed  Google Scholar 

  11. Jabbar AY, Baydoun H, Janbain M, Ferdinand KC (2018) Current concepts in the management of stable ischemic heart disease and acute coronary syndrome in patients with hemophilia. Ann Transl Med 6:299. https://doi.org/10.21037/atm.2018.05.29

    Article  PubMed  PubMed Central  Google Scholar 

  12. Fogarty PF, Mancuso ME, Kasthuri R et al (2015) Presentation and management of acute coronary syndromes among adult persons with haemophilia: results of an international, retrospective, 10-year survey. Haemophilia 21:589–597. https://doi.org/10.1111/hae.12652

    Article  CAS  PubMed  Google Scholar 

  13. Fox KAA, Dabbous OH, Goldberg RJ et al (2006) Prediction of risk of death and myocardial infarction in the six months after presentation with acute coronary syndrome: prospective multinational observational study (GRACE). BMJ 333:1091. https://doi.org/10.1136/bmj.38985.646481.55

    Article  PubMed  PubMed Central  Google Scholar 

  14. Antman EM, Cohen M, Bernink PJ et al (2000) The TIMI risk score for unstable angina/non-ST elevation MI: a method for prognostication and therapeutic decision making. JAMA 284:835–842. https://doi.org/10.1001/jama.284.7.835

    Article  CAS  PubMed  Google Scholar 

  15. Amsterdam EA, Wenger NK, Brindis RG et al (2014) 2014 AHA/ACC guideline for the management of patients with non-ST-elevation acute coronary syndromes: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines. Circulation 130:e344-426. https://doi.org/10.1161/CIR.0000000000000134

    Article  PubMed  Google Scholar 

  16. Martin K, Key NS (2016) How I treat patients with inherited bleeding disorders who need anticoagulant therapy. Blood 128:178–184. https://doi.org/10.1182/blood-2015-12-635094

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Biere-Rafi S, Tuinenburg A, Haak BW et al (2012) Factor VIII deficiency does not protect against atherosclerosis. J Thromb Haemost 10:30–37. https://doi.org/10.1111/j.1538-7836.2011.04499.x

    Article  CAS  PubMed  Google Scholar 

  18. Tuinenburg A, Mauser-Bunschoten EP, Verhaar MC et al (2009) Cardiovascular disease in patients with hemophilia. J Thromb Haemost 7:247–254. https://doi.org/10.1111/j.1538-7836.2008.03201.x

    Article  CAS  PubMed  Google Scholar 

  19. Lim MY, Pruthi RK (2012) Outcomes of management of acute coronary syndrome in patients with congenital bleeding disorders: a single center experience and review of the literature. Thromb Res 130:316–322. https://doi.org/10.1016/j.thromres.2012.02.050

    Article  CAS  PubMed  Google Scholar 

  20. Tekur Krishnamurthy V (2016) Hemophilia B with acute myocardial infarction-a case report with brief review of literature. JCDR 7:35–38. https://doi.org/10.5530/jcdr.2016.1.6

    Article  Google Scholar 

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Acknowledgements

Koki Nishimura MD was a co-author of this manuscript. However, we are unable to reach him.

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Correspondence to Jacob J. Mayfield.

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Dr. Leavitt reports having served on the Medical Advisory Boards for BioMarin, CSL, and Genentech.

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11239_2022_2664_MOESM1_ESM.pdf

Supplementary Information 1—UCSF joint cardiology and hematology clinical practice guideline foracute coronary syndromes in patients with hemophilia (PDF 114 KB)

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Mayfield, J.J., Leavitt, A.D., Tanriverdi, T. et al. Acute coronary syndrome in patients with hemophilia: a delicate balancing act. J Thromb Thrombolysis 54, 323–329 (2022). https://doi.org/10.1007/s11239-022-02664-y

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