Tricuspid atresia with christmas disease (hemophilia B). Report of a case.
A case combining Christmas disease and tricuspid atresia is presented. A severe coagulation defect in a hypoxic, polycythemic patient poses an added grave risk to major surgery. In the presence of marked and worsening symptoms, however, major surgery can be performed in these difficult cases. Close liason with a skilled hematology department is essential. Minimal tissue dissection is advised, and the usefulness of a reversed saphenous vein conduit is described.
D000293:Adolescent; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000150:complications / Q000601:surgery; D002836:Hemophilia B / Q000150:complications*; D006801:Humans; D008297:Male; D009922:Oregon; D011086:Polycythemia / Q000150:complications; D014261:Tricuspid Valve / Q000002:abnormalities* / Q000601:surgery