Prolonged Activated Clotting Time Immediately Prior to Open Cardiac Surgery

Area of Science:

• Hematology
• Cardiovascular Surgery
• Genetics

Background:

• Prekallikrein deficiency is an extremely rare genetic disorder characterized by reduced or absent functional prekallikrein in plasma.
• This deficiency can lead to a prolonged activated clotting time, a finding not always detected by routine preoperative screening.

Observation:

• A 15-year-old male with an atrial septal defect presented with a prolonged activated clotting time (ACT) detected preoperatively.
• Routine preoperative laboratory tests, including CBC, PTT, PT, and platelet function assays, were unremarkable.

Findings:

• Specialized testing revealed Fletcher factor deficiency, also known as prekallikrein deficiency.
• The patient successfully underwent open cardiac surgical repair for his atrial septal defect.

Implications:

• This case highlights the importance of ACT monitoring in detecting rare coagulation disorders like prekallikrein deficiency.
• Anesthesiologists play a crucial role in identifying potential hemostatic abnormalities through vigilant intraoperative monitoring.
• Management with fresh frozen plasma (FFP) proved effective for perioperative hemostasis in this patient, enabling successful cardiac surgery.