CASE PRESENTATION: We present a successful aortic valve replacement with cardiopulmonary bypass in a 48 years old lady with antiphospholipid syndrome, who has severe aortic regurgitation as a result of Libman-sacks endocarditis. Antiphospholipid antibodies were positive and the clinical data showed both negative cultures and infective parameters. Surgically resected vegetations revealed sterile fibrinous and verrucous vegetations on aortic valve. Valve replacement and the course of cardiopulmonary bypass was uneventful, and the patient was discharged well.
CONCLUSIONS: Classically Libman-Sacks endocarditis is often and more commonly associated with autoimmune diseases such as systemic lupus erythematosus, although it can occur in both primary and secondary antiphospholipid syndrome. It is not a common entity, and it is a frequent underestimated disease as most clinicians do not routinely screen for valvular lesion in patients with antiphospholipid syndrome unless they are symptomatic. However, due to its high prevalence of cardiac involvement, clinicians should have a high index of suspicion in the attempt to minimize cardiovascular and haemodynamic complications. Valve surgery in patients with antiphospholipid syndrome carries considerable early and late morbidity and mortality, usually caused by thromboembolic and bleeding events. The perioperative anticoagulation management and haemostatic aspect of antiphospholipid syndrome present an exceptional challenges to clinicians, surgeons, anaesthetists and laboratory personnel.
METHODS: This study is a review of 419 children (≤18 years) with RHD who underwent primary isolated MV surgery between 1992 and 2015, which comprised MV repair (336 patients; 80.2%) and MV replacement (83 patients; 19.8%). The replacement group included mechanical MV replacements (MMVRs) (n = 69 patients; 16.5%) and bioprosthetic MV replacements (n = 14 patients; 3.3%). The mean age with standard deviation at the time of operation was 12.5 ± 3.5 (2-18) years. Mitral regurgitation (MR) was predominant in 390 (93.1%) patients, and 341 (81.4%) patients showed ≥3+ MR. The modified Carpentier reconstructive techniques were used for MV repair.
RESULTS: Overall early mortality was 1.7% (7 patients). The mean follow-up was 5.6 years (range 0-22.3 years; 94.7% complete). Survival of patients who underwent repair was 93.9% both at 10 and 20 years, which was superior than that of replacement (P