A 53-year-old female with RA presented with edema in the upper extremities and neck, cough, and orthopnea. She was receiving anti-TNF therapy (over one year) and infliximab (over 1 month).
The patient’s initial clinical picture was consistent with superior vena cava compression syndrome. Imaging revealed significant mediastinal lymphadenopathy that was compressing the airway and the superior vena cava (Fig. 1). An echocardiogram showed aortic valve vegetation and severe aortic regurgitation (AR). Thus, her acute decompensated congestive heart failure symptoms were due to severe aortic insufficiency. The patient denied a history of fever and blood cultures were negative. The multidisciplinary team agreed that the most likely cause was non-bacterial thrombotic endocarditis. The patient was treated with empiric vancomycin and ceftriaxone, and steroids were initiated. A supraclavicular lymph node biopsy was negative for malignancy; however, ecchymosis developed throughout her neck after the procedure. Intravenous solumedrol was started to reduce the swelling.
Diagnosis of AHA
Coagulation testing noted a prolonged activated partial thromboplastin time (aPTT). An anti-Xa assay was performed to confirm the absence of any systemic anticoagulation. A subsequent mixing study with normal plasma failed to improve the PTT (90.5 s after one hour) indicating the presence of a factor inhibitor. Lupus testing was negative; however, the analysis of coagulation factors revealed an absence of FVIII and the presence of a FVIII inhibitor (quantified at 17.60 Bethesda units). These results, along with normal von Willebrand factor antigen and activity, resulted in the diagnosis of AHA (Fig. 2).
Perioperative management
A trial dose of porcine recombinant FVIII (rFVIII) was given and initiated a robust response (peak levels of FVIII = 239). The ecchymosis in her neck improved; however, her cardiopulmonary condition deteriorated. Due to severe AR and an aortic root abscess, an urgent aortic valve repair (AVR) was deemed necessary. The preoperative Society of Thoracic Surgeon’s risk score was 18.39%.
An additional dose (3728 units) of the rFVIII was given 1 h prior to surgery. After anesthesia, 1 g of tranexamic acid (TXA) was given intravenously; 2.5 g of TXA was given during the course of the surgery. The cardiopulmonary bypass pump (CBP) was primed with 1.048 L of fresh frozen plasma (FFP), and 12,000 IU of systemic heparin was given. The activated clotting time (ACT) was 582 s. Following cannulation, CBP was initiated. Retrograde autologous priming was performed. The procedure was performed under aortic crossclamp, cardioplegia, and mild hypothermia. The AVR was performed with a bioprosthetic valve and root reconstruction using a pericardial patch.
Upon warming, the hematocrit level was 22 mg/dl even after 1250 mL of hemoconcentration; therefore, we added 1 unit of packed red blood cells (pRBCs). After termination of CPB, 130 mg of protamine normalized the ACT (95 s). The patient received an additional 1 unit of pRBCs, 1 unit of FFP, and 1 unit of platelets. Moreover, she received an additional dose of rFVIII (1900 units). The timing and dosage of the post-CPB rFVIII was based on preoperative coagulation data. According to serial FVIII levels (Fig. 2), the patient cleared 50% of the rFVIII in approximately 4–6 h. This preoperative information was valuable as it provided guidance on how to achieve target FVIII levels (100 IU/dL) and hemostasis during the procedure. Estimated blood loss was 1300 cc, and she received 450 mL of salvage blood. The procedure was uneventful, and she was transferred to the cardiac unit.
The patient experienced no significant bleeding events after the procedure and was extubated on postoperative day (POD) 1. A bolus of FVIII was given every 6 h during the first postoperative 18 h to ensure that the FVIII level remained about 50%. The patient received an additional bolus of FVIII at postoperative hour 30. Rituximab was added to prednisolone to eliminate the FVIII inhibitor. On POD 3, FVIII activity was 85 indicating a favorable response, and all chest tubes were removed. Her postoperative course was uneventful. A continuous decline of the FVIII inhibitor and normalization of the PTT was observed. She was discharged at POD 10.