Congenital protein C deficiency and myocardial infarction:concomitant factor VII hyperactivity may play a role in the onset of arterial thrombosis.
A 29-year-old man with congenital protein C deficiency and acute myocardial infarction is reported. Four hours after the onset of chest pain, he was treated intravenously with tissue-type plasminogen activator. Subsequent coronary angiography revealed only slight stenosis of the left anterior descending coronary artery without any atherosclerosis. The propositus, his brother, and his mother, showed low levels of both protein C activity and antigen, while plasma thrombomodulin levels were normal. His grandfather had died from acute myocardial infarction at 38 years of age. We investigated several other risk factors for arterial thrombosis, including factor VII, fibrinogen, heparin cofactor II, lipoprotein (a), and anticardiolipin antibodies. No other haemostatic abnormalities apart from factor VII hyperactivity were detected in this family. To study the effects of protein C and factor VII on procoagulant activity, prothrombin time was measured after the addition of activated protein C and factor VII to protein C-deficient plasma. The prothrombin time ratio decreased along with an increase in the factor VII level. It also decreased with a decrease in the activated protein C level. These findings indicated that the procoagulant activity of factor VII was enhanced by low protein C levels, suggesting that concomitant factor VII hyperactivity may cause acute myocardial infarction in patients with protein C deficiency.
Kario
K
K
Department of Internal Medicine, Hyogo Prefectural Awaji Hospital, Japan.
Matsuo
T
T
Tai
S
S
Sakamoto
S
S
Yamada
T
T
Miki
T
T
Matsuo
M
M
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001241:Aspirin / Q000627:therapeutic use; D001779:Blood Coagulation Factors / Q000032:analysis; D005167:Factor VII / Q000032:analysis*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009203:Myocardial Infarction / Q000097:blood / Q000188:drug therapy / Q000209:etiology*; D010375:Pedigree; D020151:Protein C Deficiency; D011517:Prothrombin Time; D012307:Risk Factors; D013927:Thrombosis / Q000209:etiology*; D010959:Tissue Plasminogen Activator / Q000627:therapeutic use
D001779:Blood Coagulation Factors; D005167:Factor VII; D010959:Tissue Plasminogen Activator; D001241:Aspirin