Successful Aortic Valve Replacement Surgery in a Patient with Severe Haemophilia a with Low Titre Inhibitor

Abstract

In spite of the modern day innovations, managing severe Haemophilia patients with inhibitors continues to be a challenge. The management of patients with severe haemophilia with inhibitors who are undergoing major surgeries like open heart surgery is technically demanding, fraught with peri-operative complications and needs a multidisciplinary approach. We describe a young man with severe haemophilia with low titre inhibitors who underwent a successful open heart surgery and aortic valve replacement, supported only with bolus doses of Factor VIII and tranexamic acid without any complications.

Modern Treatment of Valvulopathies in Patients with Congenital Hemophilia

“…Surprisingly, there was a wide etiological spectrum, although many authors reported only the implantation of the prosthesis or the valve repair and not the pre-existing valvular lesions. The youngest patients, aged between 23 and 27, underwent surgery for rheumatic valvular disease [ 4 , 12 , 13 ]. Three cases of infective endocarditis (IE) were reported, two affecting the aortic valve (AV) [ 6 , 14 ] and one the mitral valve (MV) [ 15 ].…”

“…There were a few exceptions. A 47-year-old patient with severe HA [ 18 ] and a 23-year-old patient with severe HA and inhibitors [ 12 ] received aortic bioprostheses. A Bentall operation with a biologic composite graft was performed on a 43-year-old patient with severe HA [ 22 ].…”

“…As far as the available literature shows, few patients with hemophilia and inhibitors underwent cardiac surgery for valvular disease [ 12 , 17 ]. One patient with HA had high-titer inhibitors of 12.4 BU before surgery, which declined to 6.2 BU at a 6-month follow-up [ 17 ].…”

“…One patient with HA had high-titer inhibitors of 12.4 BU before surgery, which declined to 6.2 BU at a 6-month follow-up [ 17 ]. The second patient had low-titer inhibitors, and due to the absence of bleeding during follow-up, the reevaluation of the titer was not considered necessary [ 12 ]. Therefore, careful planning of replacement therapy led to a favorable outcome.…”