Related Experiment Videos
Hereditary heparin cofactor II deficiency and coronary artery disease
T Matsuo1, K Kario, S Sakamoto
1Department of Internal Medicine, Hyogo Prefectural Awaji Hospital, Japan
Insights
Hereditary heparin cofactor II deficiency may lead to recurrent thrombosis, such as restenosis after angioplasty. Argatroban, a potent thrombin inhibitor, showed success in preventing reocclusion in a patient with this deficiency.
Area of Science:
- Cardiovascular Medicine
- Hematology
- Genetics
Background:
- Hereditary heparin cofactor II (HC II) deficiency is a rare genetic disorder.
- HC II plays a crucial role in inhibiting thrombin, a key factor in blood coagulation.
Observation:
- A Japanese family presented with type I hereditary HC II deficiency.
- The propositus experienced recurrent restenosis following percutaneous transluminal coronary angioplasty (PTCA) despite standard anticoagulant therapy.
- Two family members had a history of cerebral infarction.
Findings:
- The patient exhibited significantly reduced HC II activity and antigen levels (49% and 50%).
- Standard heparin therapy was ineffective in preventing restenosis, suggesting accelerated thrombin generation at the site of vascular injury.
- Administration of argatroban, a direct thrombin inhibitor, successfully prevented reocclusion after PTCA.
Implications:
- This case suggests a potential link between HC II deficiency and an increased risk of thrombosis.
- Standard heparin may be less effective in patients with HC II deficiency undergoing procedures like PTCA.
- Argatroban may offer a more effective therapeutic option for preventing thrombosis in individuals with HC II deficiency due to its potent thrombin inhibition.
Abstract:
We here present a Japanese family with type I hereditary heparin cofactor II (HC II) deficiency. The propositus (a 61-year-old man) suffered from angina pectoris and coronary artery disease was confirmed by coronary angiography. He underwent percutaneous transluminal coronary angioplasty (PTCA) four times in one year. Combined use of heparin, aspirin, and nitrates could not prevent the return of his symptoms and restenosis of segment 6 of the left anterior descending artery. His HC II activity and antigen levels were 49% and 50%, respectively, and his daughter also showed similar low levels. Cerebral infarction had occurred in two family members. Argatroban, a selective potent thrombin inhibitor, was administered after the fourth PTCA for the purpose of preventing reocclusion and achieved a successful outcome. A relationship between HC II deficiency and thrombosis has not yet been established. Our case suggests that standard heparin therapy is not effective in preventing restenosis in such individuals, in whom the process is accelerated by thrombin generation at the site where PTCA produces rupture of the atherosclerotic plaque. Argatroban may be more effective under low HC II conditions because of its potent inhibition of thrombin activity at sites of vascular wall damage.