Abstract
Primary pulmonary hypertension is a disease of unknown etiology, characterised by the elevation of pulmonary arterial pressure eventually leading to right ventricular failure and death [1]. Although the pathogenetic mechanisms are heterogeneous [1, 2], evidence from histopathological studies suggests that a primary thrombotic process may be responsible for the disease in most patients [3, 4]. Among the conditions predisposing to thrombosis is congenital homocystinuria [5], which, in a case report, was shown to be associated with acute cor pulmonale due to the formation of thrombi in pulmonary vessels [6]. Since also moderate hyperhomocysteinemia is associated with increased risk for early onset venous and arterial thrombosis [7–9], we measured its prevalence in 10 patients with primary pulmonary hypertension.
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