Acute Type-A Dissection in a Patient with Severe Hemophilia A

Abstract

EMOPHILIA A is a rare coagulation disorder characterized by low circulating levels of factor VIII of the coagulation cascade and an increased hazard of bleeding. Hemophilia A is classified as mild, moderate, or severe according to the circulating levels of factor VIII. In recent decades, considerable advances have been made in the management of hemophilia, and, as a result, the life expectancy of many hemophiliacs currently is estimated to be 60 to 70 years. 1 Thus, a broad new spectrum of pathologies is increasing in this population, such as acquired heart disease, some of which require specific and urgent management. Acute type-A aortic dissection is a life-threatening emergency condition associated with high mortality rates. Early complex surgical intervention is required, during which coagulopathy is often an important issue. The present report presents a case of a man with known severe hemophilia A who was treated urgently for acute type-A dissection. To the authors’ knowledge, this is the first report of a patient with hemophilia A and acute type-A dissection in the current literature. Challenging patient management issues are presented and therapeutic strategies are suggested for this rare combined clinical entity.