Systemic lupus erythrematosus; its implication in cardiac surgery: Institutional case report

Abstract

Introduction Systemic lupus erythematosus (SLE) is an autoimmune disease that primarily affects young women. According to the literature, the prevalence of cardiovascular involvement in patients with systemic lupus erythematosus (SLE) has been estimated to be more than 50%. Valvular involvement is the most frequent cardiac manifestation in SLE. Functionally, valvular regurgitation hasbeen reported to occur in up to 74% of patients. Meanwhile valvular endocarditis is a frequent manifestation of SLE, and the mitral valve is most frequently affected. However, any valve or multivalvular affection may occur. Valvular lesions resulting from lupus can cause severe mitral regurgitation (MR). The most classic cardiac valvular abnormality in patientswith SLE is known as Libman–Sacks endocarditis, which consists of noninfective, verrucousvegetations(marantic endocarditis). They occur most frequently onthe mitral valve. Most of the valves that have vegetations are usually associated with diffuse thickeningor regurgitation. However, although cardiac involvement in patients with SLE has been recognized since the early 20th, But cardiac surgery was infrequently performed in patients with SLE, and its clinical outcome was reported only in small series. Also the impact of SLE on provision of anesthesia has never beeninvestigated, and the lack of evidence combined with theheterogeneity of disease manifestations makes it difficult toestablish definitive management protocols. That’s why, We herein describe a 22 years old female patient with SLE,with end stage renal diseaserequiring peritoneal dialysis. presented to our hospital (Al-Demerdash, Ain Shams University Hospital), with signsof congestiveheart failure such as dyspnea on exertion andorthopnea, paroxysmal nocturnal dyspnea, and fatigue, refractory to medical management.Her therapy for SLE required longterm prednisolonand hydroxychloroquine. Physical examination revealed no facial malar rash or generalized discoid rashes. The breath sounds were diminished at the lower lung zones. Cardiac auscultation showed regular heart beat with a heart rate of 102 beats/min, a grade III/VI systolic murmur at the left lower sternal border and the apex, and a pericardial friction rub. Palpation of the abdomen showed hepatomegaly. The extremities were notable for mild pitting edema. There was no clubbing, cyanosis or deformity of joints. Preoperative data Complete blood count revealed Hg 9.9 g/dl, WBC 11.100/ul, Platelet 247,000/ul. Bleeding Profile showed PT 16.4 sec, INR 1.45, PTT 44 sec. biochemistry work-up showed, Glucose 75 mg/dl, Creat 2.9 mg/dl with hemodialysis session, ALT 15U/l, AST 20U/l. Preoperative transesophageal Echocardiography showed: A large mass is attached to the atrial surface of the anterior mitral leaflet measuring 6 × 7 mm. the mass perforates the leaflet causing severe mitral regurgitation. And, another large mass measuring 6 × 9 mm attached to the non-coronary aortic valve cusp with 2 small masses attached to each of the other two leaflets causing severe aortic regurgitation.EF 60%,RVSP 50 mmHg. So the patient was scheduled for double valve replacement. Although valve repair and bioprosthetic valve replacement are not the best solution, since accelerated native valve and bioprosthetic valve calcification tend to occur because of the high calcium turnover. Also Porcine valves have become affected by valvulitiswith perforation of valve cusps. But, our cardiac surgical team decided to replace mitral and aortic valve with bioprothetic valve, as they believe that Anticoagulation may present higher risks in our young patient who require prolonged steroid use and who have end stage renal failure, the resultant dependence on dialysis. Also previous reports described patients with SLE who underwent mitral valve replacement, had a number of complications, and died secondary to anticoagulation. Also, because ofsuccessful placement of the porcine Carpentier-Edwards bioprosthesis in patients with SLE has been reported before. Finally weaning from cardiopulmonary bypass was uneventful (adrenaline 50 n/kg/min). The patient’s recovery from surgery was uncomplicated, and she was discharged on the 6th postoperative day. In conclusion, although the postoperative complication is common, cardiac operation could be performed in patients with SLE.