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Cardiac surgery in patients with systemic lupus erythematosus
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     Department of Surgery, National Taiwan University Hospital, National Taiwan University College of Medicine, No. 7, Chung-Shan S. Rd. Taipei, Taiwan 100, ROC

    Abstract

    Cardiac surgery was infrequently performed in patients with systemic lupus erythematosus (SLE), and its clinical outcome was reported only in small series. We sought to evaluate the clinical outcome of cardiac operation in patients with SLE. Between January 1996 and March 2005, 9 patients with SLE underwent cardiac surgery at the authors' hospital. Six patients underwent coronary artery bypass grafting (three conventional and three on-pump beating heart), two patients underwent valve replacement and 1 patient underwent simultaneous heart–kidney transplantation. All 6 patients with coronary artery bypass grafting had saphenous venous grafts and two of them had additional left internal mammary artery graft. The overall in-hospital mortality rate was 11% (1/9). Major postoperative complications occurred in 4 patients (44%) including profuse postoperative bleeding, ventricular tachycardia and early graft thrombosis. There were two late deaths including sudden cardiac death and sepsis. The median follow-up duration was 23 months (range, 1–110). In conclusion, although the postoperative complication was common, cardiac operation could be performed in patients with SLE.

    Key Words: Systemic lupus erythematosus; Cardiac surgical procedures; CABG

    1. Introduction

    Cardiac involvement in patients with systemic lupus erythematosus (SLE) has been recognized since the early 20th century. SLE involves all components of the heart, including the pericardium, conduction system, myocardium, heart valves, and coronary arteries [1]. Cardiac valvular abnormalities and coronary artery disease are common complications of SLE. Studies of clinical outcomes for coronary artery disease have shown a prevalence of 8.3% in patients with SLE [2]. More sensitive investigations including myocardial perfusion imaging and electron-beam computed tomography have demonstrated a prevalence of ischemic heart disease in about 30–40% of SLE patients [3,4]. Cardiac surgery was infrequently performed in patients with SLE, and its clinical outcome was reported only in small series [5–9]. Here, we report 9 cases of cardiac surgery performed in patients with SLE.

    2. Patients and methods

    2.1. Patients

    Information was collected retrospectively on all patients with SLE who underwent cardiac surgery between January 1996 and March 2005. All patients fulfilled the 1982 American Rheumatism Association revised classification criteria for SLE. Data on age, sex, medical co-morbidities, surgical procedure and clinical outcome were recorded.

    2.2. Cardiac surgery

    All the cardiac surgical procedures were performed through a median sternotomy. Cardiopulmonary bypass was instituted with an ascending aortic and separate caval venous cannulation. Before bypass was started, heparin sodium was administered at an initial dose of 300 IU/kg. Additional heparin was administered if the activated clotting time became less than 500 s. During cardiopulmonary bypass, the hematocrit was maintained between 18% and 25%, perfusion flow between 2.4 and 2.8 l/min/m2, mean arterial pressure between 50 and 70 mmHg, and systemic temperature between 28 and 32 °C. After the surgical procedure, heparin was neutralized by continuous intravenous administration of protamine sulfate during a 5-min period. All cases were treated by administration of methyl-predonisolone during cardiopulmonary bypass.

    Commencing from June 2000, patients with coronary artery disease were treated with coronary artery bypass grafting without the use of cardiopulmonary bypass or off-pump coronary artery bypass grafting. The operation was also performed through a median sternotomy. For patients with unstable conditions, we performed on-pump beating heart coronary artery bypass grafting [10]. Cardiopulmonary bypass was instituted using an ascending aortic perfusion cannula and single right atrial cannula. Coronary artery bypass grafting was performed without aortic cross-clamping and cardioplegic cardiac arrest.

    Postoperative management in the intensive care unit was the same as that in routine patients receiving cardiac surgery. Blood components were given whenever needed and no aprotinin was used in our patients.

    2.3. Clinical outcome

    Major complications were classified as neurological (consciousness disturbance, seizure or stroke), pulmonary (prolonged ventilator support for more than 48 h), infectious (wound infection, bacteremia, pneumonia, or urinary tract infection), gastrointestinal, renal (acute renal failure or need of new dialysis), bleeding (profuse chest tube drainage in need of reoperation), and hepatic decompensation (hepatic function deterioration or hepatic failure). Late follow-up information was collected from the patients' most recent clinical visits or by telephone interviews.

    3. Results

    3.1. Patient characteristics

    Nine patients were identified. There were 2 males and 7 females. The median age was 51 years (range 13–66 years). Patient demographics and co-morbid conditions are listed in Table 1. The causes of heart disease were coronary artery disease in 6 patients, valvular heart disease in 2 patients, and dilated cardiomyopathy in 1 patient. The major co-morbid medical diseases included hypertension in 2 patients and uremia in 2 patients. There were 3 patients with renal dysfunction, including two dialysis patients and one patient with serum creatinine of 2.4 mg/dl.

    The median duration of SLE was 10 years (range, 1–22). In all patients, SLE was in remission at the time of surgery. Six patients were taking prednisone, 5 patients were taking hydroxychloroquine, and 1 patient was taking azathioprine at the time of the operation. Two of 6 patients with coronary artery disease had been referred after unsuccessful percutaneous transluminal coronary angioplasty.

    3.2. Surgical procedures

    Operative data are shown in Table 2. All 9 patients underwent cardiac surgery with the use of cardiopulmonary bypass: 6 patients underwent coronary artery bypass grafting, 1 patient underwent mitral valve replacement, 1 patient underwent aortic valve replacement and mitral valve repair, and 1 patient underwent orthotopic heart transplantation. The median duration of cardiac ischemic time was 89 min (range, 0–137). The median duration of cardiopulmonary bypass was 162 min (range, 78–187).

    A total of 6 patients underwent coronary artery bypass grafting. Three patients underwent conventional coronary artery bypass grafting, 2 patients underwent on-pump beating heart coronary artery bypass grafting, and 1 patient with severe ischemic mitral regurgitation underwent on-pump beating heart coronary artery bypass grafting and mitral valve repair. All 6 patients had saphenous venous grafts and two of them had additional left internal mammary artery graft to the left anterior descending artery. The mean number of distal coronary anastomosis was 3.3±1.5.

    3.3. Clinical outcomes

    The overall in-hospital mortality rate was 11% (1/9). The only patient with hospital death was a 51-year-old woman with acute myocardial infarction and congestive heart failure. She underwent urgent coronary artery bypass grafting because of repeated ventricular tachycardia. She died of severe postcardiotomy shock and multiple organ failure 9 days after the operation.

    The median duration of intensive care unit stay and hospital stay were 3 days (range, 2–20) and 13 days (range, 8–36), respectively. Major postoperative complications occurred in 4 patients (44%) including profuse postoperative bleeding in 2 patients, ventricular tachycardia in 1 patient and early graft thrombosis in 1 patient. One patient with early graft thrombosis was a 47-year-old man with triple vessel coronary artery disease. He underwent conventional coronary artery bypass grafting with left internal mammary artery graft to the left anterior descending artery and a sequential saphenous venous graft to diagonal artery, obtuse marginal arteries, and posterior descending artery. He had recurrent chest pain 16 days after the operation and repeated coronary angiography showed occluded venous graft to posterior descending artery. He was successfully treated with percutaneous transluminal coronary angioplasty and intra-coronary thrombolysis with urokinase. He was alive and remained free of chest pain for more than 9 years.

    The median duration of follow-up was 23 months (range, 1–110). Two patients had late death. The first was a 19-year-old woman with severe degenerative aortic regurgitation and congestive heart failure. She underwent aortic valve replacement and mitral valve repair. The postoperative course was complicated with ventricular tachycardia, which was controlled with antiarrhythmic agents. However, she died of sudden cardiac death 5 months after the operation. The second was a 54-year-old woman, who underwent on-pump beating heart coronary artery bypass grafting for double vessel coronary artery disease. The postoperative course was smooth. However, she died of overwhelming sepsis caused by Klebsiella pneumoniae 4 months after the operation.

    4. Discussion

    There have been several case reports of coronary artery bypass grafting on SLE patients [5–9]. Although coronary artery bypass grafting in SLE patients can be performed with acceptable morbidity and mortality, it is still not clear as to whether coronary artery bypass grafting can provide satisfactory long-term results similar to those established in patients without SLE. A collective series of patient data obtained from several hospitals in California showed that the in-hospital mortality rate of coronary artery bypass grafting in 70 SLE patients was 5.7% and it was not significantly different from mortality in patients without SLE [11]. However, detailed patient data and long-term outcome were not described. Ura et al. [6] retrospectively reviewed 8 cases of coronary artery bypass grafting in 7 patients with SLE. In their study, early postoperative coronary angio- graphy was performed 2–3 weeks after surgery. The in-hospital mortality rate was 12.5% and early graft patency rates were 87.5%. Because of a coexisting medical disease such as diabetes mellitus, hyperlipidemia and lupus nephropathy in SLE patients, saphenous vein grafts appear to deteriorate early. In SLE patients, total autogenous arterial bypass grafting is advised by some surgeons and intraoperative biopsies of the internal mammary artery are meaningful in patients with SLE [5,9]. But there is still no definite answer as to which conduit should be used for coronary artery bypass in SLE patients. Our series adds to the literature six cases of coronary artery bypass grafting in SLE patients. The in-hospital mortality rate was 17%. Among five operative survivors, one patient died of non-cardiac cause. Although one patient had early graft thrombosis, the other 4 patients had no cardiac events in late outpatient follow-up. Although our case number was small and postoperative coronary angiography was not done in most of our cases, our results reveal no evidence to preclude the use of saphenous venous grafts in SLE patients.

    Coronary artery bypass grafting in SLE patients is a surgical challenge because these patients have unique patient characteristics of multiple organ involvement and long-term use of steroids. As an alternative, the method of performing coronary artery bypass grafting on beating heart technique can be regarded as more advantageous in patients with SLE. To our knowledge, there was only one case report in the literature undergoing off-pump coronary artery bypass grafting in SLE patients [7]. On-pump beating-heart coronary artery bypass without aortic cross-clamping is an intermediary approach in high-risk patients [10]. In this study, we performed on-pump beating-heart coronary artery bypass in 3 patients with heart failure and pulmonary hypertension. Early results were satisfactory.

    Infection is an important risk factor for morbidity and mortality in patients with SLE [12]. Among the cardiovascular abnormalities associated with SLE, Libman-Sacks endocarditis is the most common lesion and consists of multiple sterile fibrin and platelet accumulations. These lesions may cause valvular changes or serve as a nidus for microbial infection, placing patients at risk of developing infective endocarditis. Although valvular heart disease is common in patients with SLE, infective endocarditis and heart valve surgery is reported only in sporadic cases [13]. Infective endocarditis due to non-typhoid Salmonellas is infrequently reported [14]. Salmonella endocarditis can be successfully treated with antimicrobials alone. Valve replacement should be reserved for patients with cardiac failure or persisting sepsis. In this study, we add a 41-year-old woman having a history of infective endocarditis caused by non-typhoid Salmonellas that was diagnosed 5 years before the operation. She was successfully treated with mitral mechanical valve replacement. She is on anticoagulation and at the present time is still well. Although non-typhoid Salmonella infection is common in SLE patients, this is the first case of infective endocarditis caused by non-typhoid Salmonella in SLE patients.

    Patients with connective tissue disorders are generally excluded from consideration for heart transplantation because of multiple organ involvement and donor organ shortages. In this study, a 13-year-old girl with SLE and end-stage renal disease under peritoneal dialysis was referred for evaluation of severe heart failure. She underwent simultaneous heart–kidney transplants and received cyclosporine, mycophenolate mofetil, and prednisone on a long-term basis. The patient remains functionally well without clinical manifestations of SLE for 18 months. It indicates that SLE does not necessarily represent absolute contraindication to heart transplantation [15].

    In conclusion, although the postoperative complication was common, cardiac operation could be performed in patients with SLE.

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