Abstract

Abstract 3495

Poster Board III-432

Background

It is well known that patients with hemophilia (both A and B) have a lower incidence of coronary artery disease (CAD). However, patients with hemophilia may develop atherosclerosis, and with increases in life expectancy it is possible that the incidence of CAD may increase in these patients. There are no studies analyzing the prevalence of CAD in patients with hemophilia and possible risk factors.

Aims

To determine the prevalence of CAD in patients with hemophilia treated in Brazil.

Methods

All patients with hemophilia in Brazil receive therapy with Factor VIII or Factor IX at government-designed hemophilia centers. We thus contacted all centers in Brazil by e-mail and information from databases concerning health information for all patients was reviewed by the responsible physician at each center. A questionnaire about risk factors for CAD and history of coronary events was sent to patients that had a positive history of CAD.

Results

There are 6,881 patients registered with a diagnosis of hemophilia A and 1,291 patients with a diagnosis of hemophilia B in Brazil. Fifty-six percent of hemophilia centers answered the query, and information on 71.7% of patients with hemophilia A (4,934 patients) and 61.4% of patients with hemophilia B (792 patients) was reviewed. There were only 3 patients (0.05%) with a positive history of CAD. Their clinical characteristics are summarized in Table 1. During surgical treatment for CAD, replacement with Factor VIII and Factor IX was done by continuous infusion to increase Factor activity to 80-100%. Antiplatelet therapy was used in all patients at some point, and one patient (#1) developed hemarthrosis after two months of ASA. Patient #3 did not start antiplatelet therapy after CABG, and developed thrombosis of the graft. ASA and clopidogrel were started after percutaneous coronary angioplasty.

Conclusion

We found a very low incidence of CAD in hemophiliac patients, even after doing a nationwide survey that included more than 5,000 patients. No information about risk factors can be gleaned due to the small number of patients. Surgical treatment after increasing factor activity appears to be safe in such patients. Despite a theoretical risk for increased bleeding, therapy with antiplatelet agents appears to be necessary in hemophiliac patients with CAD.

Table 1

Clinical features of patients with CAD and Hemophilia

UPI Age, years Type Comorbidities Acute Events Medical Therapy Surgical Therapy 
51 Diabetes Mellitus MI ASA CABG 
62 None UA ASA Percutaneous angioplasty with stent 
37 HIV+ receiving HAART UA Clopidogrel ASA CABG Percutaneous angioplasty with stent 
UPI Age, years Type Comorbidities Acute Events Medical Therapy Surgical Therapy 
51 Diabetes Mellitus MI ASA CABG 
62 None UA ASA Percutaneous angioplasty with stent 
37 HIV+ receiving HAART UA Clopidogrel ASA CABG Percutaneous angioplasty with stent 

Abbreviations: UPI, unique patient identifier; MI, myocardial infarction; ASA, acetylsalycilic acid; CABG, coronary artery bypass graft; UA, unstable angina; HAART, highly active anti-retroviral therapy

Disclosures:

No relevant conflicts of interest to declare.

Author notes

*

Asterisk with author names denotes non-ASH members.