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Ann Thorac Surg 1988;46:283-288
© 1988 The Society of Thoracic Surgeons

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Articles

Cardiovascular Surgery in Patients with Congenital Plasma Coagulopathies

From the Departments of Cardiovascular Surgery and Pathology, Texas Heart Institute and St. Luke's Episcopal Hospital, and the Division of Cardiovascular Surgery and Department of Pathology, University of Texas Health Science Center, Houston, TX

Accepted for publication February 23, 1988.

^* Address reprint requests to Dr. Vander Woude, Texas Heart Institute, PO Box 20345, Houston, TX 77225.

From 1978 to 1986, fifteen cardiovascular operations were performed on 13 patients with known congenital bleeding disorders. The patients (10 men and 3 women) had a mean age of 51.1 ± 3.4 years. Four were seen with cardiovascular lesions and documented hemophilia A (Factor VIII deficiency); 3 had hemophilia B (Factor IX deficiency); 3 had Factor XI deficiency; 2 had von Willebrand's disease, and 1 had dysfibrinogenemia. All patients had a history of major hemorrhage after dental extractions or general surgical procedures, and had clearly documented coagulation disorders on hematological evaluation. Elective cardiovascular procedures performed in these patients included aortocoronary bypass grafting (eight), cardiac valve replacement or repair (five), aortic graft placement (one), and carotid endarterectomy (one). The mainstay of perioperative management included appropriate replacement therapy with blood components. Coagulation factor levels were measured routinely to guide therapy. There were no deaths. Two hemorrhagic complications necessitated reexploration. We conclude that in patients known to have congenital coagulation disorders, cardiovascular operations using systemic heparinization can be performed with minimal morbidity and mortality when carried out with preoperative and perioperative support from the hematology service, adequate replacement therapy using blood components, and careful monitoring of the coagulation status.

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