There are a few critical issues when considering valve surgery. First, APS is by definition a hypercoagulable state. It has to be remembered that factor XII activation will occur as thrombogenic surfaces are going to be used as part of the routine setup and equipment in cardiac surgery. The most important example to consider is extracorporeal tubing. Also, intraoperative management of anticoagulation is likely to be the most important issue in patients with APS. Attention was drawn to this previously by Hogan et al. concerning a young female patient [47]. Of critical importance is thrombocytopenia as well as the prolonged clotting times. Intraoperative monitoring is a substantial part of the surgical strategy.

The conducting of the operation at our department as part of the strategy across the entire process has included ­standard cardiopulmonary bypass with full heparinization [activated clotting time (ACT) >450 s] and cardioplegic arrest with intermittent cold blood cardioplegia administered through the aortic root and coronary ostia and/or the retrograde route. Postoperative oral anticoagulation with vitamin K antagonist (VKA) and anticoagulants were started after 48 h postoperatively, and low-molecular-weight heparin was continued until the international normalized ratio (INR) reached a range between 2.5 and 3.5. A key component in surgery is a more aggressive management of anticoagulation with ACT lasting twice as long as in regular operations. However, this is a relatively empirical process as the clinical experience is scanty.

Our experience has been reported earlier [24]. It now extends to ten clinical cases, with all patients being operated on for valve disease. Two of these patients died, for an in-hospital mortality of 20 %. One of them presented with stroke on the 5th postoperative day, and the second had intracranial bleeding in the form of subdural hematoma that was later followed by carotid artery thrombosis. These two cases are good illustrations of the difficulties in the overall management of patients with APS undergoing cardiac ­surgery. As stated, protean manifestations and an extreme tendency to thrombotic events in patients are always associated with long-standing steroid therapy. The excellent review of Gorki et al. identified 57 patients who had undergone valve surgery, other than the patients in our own cases, and yielded interesting observations after a meta-analysis of the accumulated experience up to 2007. The first is that the average age of cardiac surgical APS patients is younger than that of the average patient in westernized cardiac surgical units. The second is that there is a trend toward increasing aggressiveness in the intraoperative management of the anticoagulation with more liberal use of heparin and less aggressive reversal by using less protamine sulfate. Finally, the possibility of the eventual appearance of catastrophic APS with multiple vascular occlusions must always be kept in mind [8, 9, 48].

Controversy still exists about how to choose a valve replacement device. Table 38.2 shows the procedures ­performed by different authors. Our policy has usually been to choose a mechanical valve, and actually six out of ten patients had a mechanical valve implanted. Two had a tissue valve and, and they had uneventful postoperative stays. This is similar to the experience reported in different series. Mechanical valves are usually the first choice considering that these patients will be in oral anticoagulation regimens with or without antiplatelet therapy, an issue that is also currently under discussion. Isolated cases, including one case from our experience [46] (Figs. 38.1, 38.2, and 38.3), may be treated with preservation of the valve, but this is uncommon. A very recent experience by Erdozain et al. [49] that reviewed a multicenter experience with 33 patients treated over a period of 27 years also stressed on similar issues, namely high perioperative morbidity and mortality and the need of the prevention of hemorrhagic and thrombotic complications.