A rare cause of Pulmonary Hypertension - Protein S Deficiency.
Abstract
Protein S deficiency is a rare form of inherited
thrombotic disorder. This is a
case report of protein S deficiency presenting
as chronic thromboembolic pulmonary
hypertension. Middle aged male
patient presented with features of pulmonary
hypertension and right heart failure
with past history of Mesenteric Venous
Thrombosis (MVT) few years back. Investigations
revealed mosaic pattern of
attenuation in CT pulmonary angiogram
and Protein S deficiency. Incidentally this
patient belongs to Bombay blood group.
Protein S is a vitamin K dependent
plasma glycoprotein. It facilitates the anticoagulant
activity of activated protein C.
Protein S deficiency can cause both venous
and arterial thrombosis. Among unselected
outpatients presenting with venous
thromboembolism (VTE) 2 to 3 may
have low levels of protein S. Management
of protein S deficiency includes lifelong
anticoagulation.
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