ABSTRACT
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.
Subject(s)
Arteriosclerosis/surgery , Blood Coagulation Disorders/congenital , Heart Valve Diseases/surgery , von Willebrand Diseases/congenital , Afibrinogenemia/congenital , Arteriosclerosis/blood , Arteriosclerosis/complications , Blood Coagulation Factors/analysis , Factor XI Deficiency/congenital , Female , Heart Valve Diseases/blood , Heart Valve Diseases/complications , Heart Valve Diseases/congenital , Hemophilia A/congenital , Hemophilia B/congenital , Hemorrhage/prevention & control , Heparin/therapeutic use , Humans , Male , Methods , Middle Aged , Postoperative Complications/prevention & control , Retrospective Studies , Severity of Illness IndexABSTRACT
A case of subdural and intracerebral hematoma which appeared spontaneously in a neonate with severe hemophilia A is reported. This rare complication justifies some preventive measures in families at risk : antenatal ultrasonographic diagnosis of sex, vitamin K administration to the mother at the end of pregnancy and plasma assay for factors VIII and IX in the male neonate. Clinical and ultrasonographic examination of the intracranial content must be repeated during the first week of life and specific replacement therapy must be instituted if the clinical condition requires it.
