David’s procedure in a patient with haemophilia

Abstract

Haemophilia A is a sex-linked recessive disorder characterized by deficiency of factor VIII. Cardiac surgery in haemophiliac patients is challenging in terms of bleeding, due to the requirement of heparin, cardiopulmonary bypass and hypothermia. The complex coagulation disturbances triggered by cardiopulmonary bypass are further exacerbated by the lack of adequate haemostasis in haemophiliacs. We present this case report of a young haemophiliac with a dilated ascending aorta and aortic root, who underwent a David’s operation successfully. We establish that with meticulous haemostasis, adequate factor VIII replacement protocol and perioperative management of factor VIII levels, good results can be achieved in these patients.