Abstract
IntroductionCongenital fibrinogen deficiency is a rare coagulation disorder usually responsible for hemorrhagic diathesis. However, it can be associated with thrombosis and there have been limited reports of arterial thrombotic complications in these patients.Case presentationA 42-year-old Tunisian man with congenital hypofibrinogenemia and no cardiovascular risk factors presented with new onset prolonged angina pectoris. An electrocardiogram showed features of inferior acute myocardial infarction. His troponin levels had reached 17 ng/L. Laboratory findings confirmed hypofibrinogenemia and ruled out thrombophilia. Echocardiography was not useful in providing diagnostic elements but did show preserved left ventricular function. Coronary angiography was not performed and our patient did not receive any anticoagulant treatment due to the major risk of bleeding. Magnetic resonance imaging confirmed myocardial necrosis. Our patient was managed with aspirin, a beta-blocker, an angiotensin-converting enzyme inhibitor and statin medication. The treatment was well tolerated and no ischemic recurrence was detected.ConclusionAlthough coronary thrombosis is a rare event in patients with fibrinogen deficiency, this condition is of major interest in view of the difficulties observed in managing these patients.
Highlights
Congenital fibrinogen deficiency is a rare coagulation disorder usually responsible for hemorrhagic diathesis
Conclusion: coronary thrombosis is a rare event in patients with fibrinogen deficiency, this condition is of major interest in view of the difficulties observed in managing these patients
Case presentation We report the case of a 42-year-old Tunisian man, born out of a consanguineous marriage, who had no cardiovascular risk factors and was followed-up for hypofibrinogenemia diagnosed three years previously due to
Summary
Hypo- and afibrinogenemia are rare congenital coagulation disorders with an estimated incidence of one in 500,000 births [1]. It is noteworthy that an etiological investigation for acute myocardial infarction in a young adult was otherwise negative; our patient did not have any protein C or protein S or an antithrombin deficiency; there was neither a Factor V Leiden nor prothrombin G20210 mutation, no anti-phospholipid antibodies (lupus type v inhibitor, anticardiolipin or anti-2-glycoprotein-1 antibodies) were detected and his plasma homocysteine level was normal. His fasting glucose (0.9 g/L) and hemoglobin A1C (4.7%) levels, lipid profile (cholesterol: 1.8 g/L; high density cholesterol: 0.47 g/L; triglycerides: 1.6 g/L; low density cholesterol (Friedewald formula): 1.0 g/L) and liver function tests were normal. A cardiac MRI performed one month after the acute episode identified a zone of myocardial necrosis and wall thinning, confirming the initial diagnosis (Figure 3)
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