Journal of Thrombosis and Thrombolysis 20(1), 43–46, 2005. C  2005 Springer Science + Business Media, Inc. Manufactured in The Netherlands. Myocardial Infarction, Other Arterial Thrombosis and Invasive Coronary Procedures, in Hemaophilia B: A Critical Evaluation of Reported Cases A. Girolami, M. L. Randi, E. Ruzzon, E. Zanon, and B. Girolami University of Padua Medical School, Department of Medical and Surgical Sciences & Northeastern Italy Association for Hemophilia and other coagulation disorders, Padua, Italy Abstract. Myocardial infarction and other arterial thrombosis are commonly maintained to be rare in hemophilia patients. This, in general, seems true but the occurrence of a thrombotic event in hemophilia B is not exceptional. A thorough search of the literature has yielded 13 patients with myocardial infarction and 1 patient with a cerebrovascular accident. There were three fatalities. In five cases MI occurred after infu- sion of Prothrombin Complex Concentrates. In three additional patients the event occurred after infusion of plasma, Feiba or cryoprecipitate supernatant. Four patients had an antero-lateral infarction. Two had a non-Q infarction and one each showed a multiple or a posterior-inferior form. Several therapeutic coro- nary procedures (GABG and PTCA) were carried out in hemophilia B patients without undue complication pro- viding adequate level of FIX were maintained. Heparin prophilaxis was used in all patients but one. The anal- ysis of the literature indicates that (1) MI may occur in hemophilia B patients and (2) that invasive coronary artery therapeutic procedures may be carried out with- out complications. Key Words. hemophilia B, myocardial infarction, angioplasty, cerebrovascular accident Introduction The occurrence of thrombotic phenomena in pa- tients with congenital bleeding disorders is always a surprising event [1]. It is commonly thought that hemophilia protects from both arterial and venous thrombosis [2]. Even carries of hemophilia have been reported to have a lower incidence of atherothrombosis [3]. However, it has been recently shown that my- ocardial infarction (MI) is rare but not exceptional in hemophilia A patients. A total number of 35 cases have been gathered from the literature [4]. In most instances the MI occurred in patients with inhibitors being treated with by-passing agents such as APC concentrates or rFVIIa concentrates. In some cases associated risk factors such as diabetes, hypertension and dyslipidemia were present. On the contrary, venous thrombosis seem less frequent [5]. All these studies are in good accordance with the recent interest but the hemophilia or thrombotic manifestations occur in bleeding disorders [6,7]. Because of these findings we thought it worthed to gather and discuss all cases of hemophilia B disease who presented an arterial thrombotic phe- nomena and/or who underwent invasive therapeutic procedures for coronary diseases. Patients, Material and Methods All patients reported in the literature to have had a myocardial infarction or an arterial occlusion were the object of the present research. Cases were gath- ered from personal files and by an extensive Pub Med and/or Medline search going back, when possible, 30 years. Acta Med Services, Soragno, Switzerland and the University of Padua Pinali Library were widely used to obtain the original articles. Only cases with accepted criteria for a correct di- agnosis of hemophilia B were included in the study. Hemophilia B were subdivided in severe (FIX level <1%), moderate (FIX level of 1–5%) and mild (FIX level of 5-25%). Myocardial infarction were classified whenever possible as (1) antero-lateral (2) postero- inferior (3) massive or multiple and (4) non-Q accord- ing to accepted procedures. The arterial occlusion end-point was accepted as valid only if diagnosis was firmly established by con- sistent clinical and laboratory investigations. All pa- tients with hemophilia B who underwent invasive coronary arteries therapeutic procedures, prior or post a MI, or without a MI were also gathered. Results Fourteen patients with hemophilia B and an arterial occlusion were gathered from the literature [8,23]. In thirteen cases the arterial occlusions concerned Address for correspondence: Prof. A. Girolami, Department of Medical and Surgical Science, via Opsedale 105, 35128 Padua, Italy. 43