Case Report: Successful concomitant surgical interventions for dilated ascending aorta and aortic valve insufficiency in a severe haemophilia A case.

INTRODUCTION: Cardiac surgery is the most challenging major invasive procedure in haemophilia. It involves the use of coagulation factor replacement, heparin therapy, antifibrinolytics, platelet antiaggregants, and is associated with surgical trauma, extracorporeal circulation, cardioplegia, and hypothermia, making it highly complex.

METHOD: Case presentation.

RESULTS: We present our experience managing a 64-year-old patient with severe haemophilia A (SHA) without inhibitors who underwent open aortic valve replacement and reduction plasty of the dilated ascending aorta under cardiopulmonary bypass (CPB). His history included SHA complications and cardiac failure due to severe aortic regurgitation. In accordance with international guidelines, the following have been decided: surgical intervention, complex medical therapy, a rigorous multidisciplinary team approach, and accurate, proper laboratory monitoring. The biological valve was successfully implanted, and the aorta was tubularly reduced to a 35 mm diameter, while maintaining its symmetry. The patient was cared for in the intensive care unit for 2 days, with CPB weaned after 1 h and 20 min; he was discharged after 10 days, with a recommendation for continuous prophylaxis with FVIII, combined with fractionated heparin for 3 months, and thereafter switched to aspirin for another 3 months. Immediate and long-term outcomes were good, with no bleeding, thrombotic events, or other complications.

DISCUSSION/CONCLUSION: Balancing conflicting hemostatic factors in a controversial biological environment was a challenging life-saving task. It required accurate, continuous lab monitoring as a key prerequisite for personalised therapy. The immediate and long-term evolution was good, free of complications.