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Emergency Aortic Valve Replacement in Systemic Lupus Erythematosus
CASE REPORT
Case Report
Emergency Aortic Valve Replacement in Systemic Lupus Erythematosus Sanjay Kumar, MCh, FRCS a,∗ , Bharati Sinha, MD, MRCP b and Edwin Ravikumar, MCh c a
Department of Cardiothoracic Surgery, 177, ‘D’ Floor, Yorkshire Heart Centre, Leeds General Infirmary, Great George Street, Leeds LS1 3EX, UK b Department of Cardiology, Bristol Childrens’ Hospital, Bristol, UK c Christian Medical Colleges and Hospital, Ida Scudder Road, Vellore 632 004, India
The valvular heart disease in systemic lupus erythematosus (SLE) is associated with substantial morbidity and mortality. Current therapy includes symptomatic measures and valve replacement. Overall mortality of valve replacement has been reported to be as high as 25%. Most cases of Libman–Sacks endocarditis in the literature reported dominant aortic regurgitation. We present this unusual case of a young female patient with SLE and glomerulonephritis warranting emergency isolated aortic valve replacement (AVR) for severe calcific aortic stenosis. The literature is reviewed with specific focus on the pathogenesis of and acute treatment options for this extremely rare occurrence. (Heart, Lung and Circulation 2006;15:397–399) © 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. Systemic lupus erythematosus; Valvular heart disease; Aortic valve replacement; Emergency aortic valve replacement
Introduction
R
ecent reports suggest an increased incidence of significant valvular dysfunction in patients with systemic lupus erythematosus (SLE) who have received longterm corticosteroid treatment.1,2 The occurrence of aortic stenosis is far more seldom than regurgitation in such patients.2,3 The renal function, pulmonary status and cerebral complications require special attention in these highsurgical-risk patients. The present report of a patient under prolonged steroid therapy and associated renal dysfunction necessitating emergency aortic valve replacement (AVR) for acute refractory left ventricular dysfunction poses further challenge.
Case report A 37-year-old woman presented with complaints of exertional dyspnoea, chest pain and palpitation that had persisted for 1-year. Her symptoms worsened 6 months ago when she developed bilateral leg swelling, abdominal distension and orthopnoea. The transthoracic echocardiographic (TTE) examination revealed severe calcific aortic stenosis and mild aortic and mitral incompetence. Since Received 25 March 2006; accepted 5 April 2006; available online 11 July 2006 ∗ Corresponding author. Tel.: +44 7980631248; fax: +44 1173738046. E-mail address: [email protected] (S. Kumar).
then she had been on digoxin and frusemide as antifailure medication. She denied having any past history suggestive of rheumatic fever. She was a known case of SLE with antiphospholipid antibodies for last 15 years. She was on complete remission with oral prednisolone 40 mg on alternate days and chloroquin 250 mg once a day. She had had cyclophosphamide to control her symptoms in past. As a complication of SLE, she had chorea and mild renal function impairment due to grade 1 glomerulonephritis. She was admitted for AVR as she was in complete remission of SLE and NYHA functional class 2. Her condition was being optimized for cardiac catheterisation. However, her functional status acutely deteriorated with the development of low cardiac output state requiring emergency endotracheal intubation and preoperative inotropic support. We decided to undertake emergency AVR as a high surgical risk. The procedure was performed through median sternotomy. Cardiopulmonary bypass (CPB) was established with aortobicaval cannulation. The myocardial protection was achieved with cold antegrade and retrograde blood cardioplegia. She had moderate cardiomegaly with left ventricular hypertrophy. The ascending aorta was dilated with small-sized aortic annulus. The aortic valve was tricuspid, heavily calcified and stenotic. The calcification was mostly on the cusps and the annulus, but there were no degenerative changes. On the basis of the intraoperative transoesophageal echocardiographic assessment and in view of small-sized left atrium, the trivial mitral regur-
© 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2006.04.002
398
Kumar et al. Aortic valve replacement in SLE
CASE REPORT
gitation was considered to be functional. The aortic valve was excised and annulus was thoroughly decalcified. Sorin bicarbon aortic valve of size 19 mm was implanted using interrupted 2 ‘0’ Ticron vertical mattress suture bolstered with teflon pledgets. After 300 cc of hotshot cardioplegia, the patient was rewarmed and weaned off bypass on high inotropic support. However, at this stage the patient developed hypotension not responding to higher doses of inotropes. Hence, secondary CPB was commenced with ascending aortic and single right atrial venous cannula. The system was supported for 40 min and the patient was successfully weaned off CPB with good haemodynamics on intraaortic balloon pump support. The total pump time was 1 h (1st time) and 40 min (secondary CPB). The total aortic cross-clamp time was 35 min. She was weaned off IABP with good haemodynamics in 36 h. The patient was electively ventilated for 30 h. She was judiciously covered with intravenous prednisolone and diuretics in perioperative period to prevent acute deterioration of SLE and renal function, respectively. She had mild impairment of renal function parameters, which improved subsequently. Her ICU stay was free of any haemodynamic or haemostatic complications. She was discharged 2 weeks after the operation. The histological examination of the excised valve revealed the picture consistent with steroid-modified Libman–Sacks endocarditis. The echocardiographic follow-up performed on 12th postoperative day and at 3 months, 1 year and 2 years revealed good haemodynamics of the prosthetic valve with good LV function. Her SLE has been in complete clinical remission 2 years after the operation.
Heart, Lung and Circulation 2006;15:397–399
included pericardial effusion and/or thickening (37%), left ventricular hypertrophy (12%), global left ventricular hypokinesis (5%), segmental abnormalities of left ventricular wall motion (4%), right ventricular enlargement (4%), focal verrucous valvar thickening (12%), gross valvar thickening and dysfunction (8%), mitral regurgitation (25%) and aortic regurgitation (8%).4 The increased incidence of mitral valve insufficiency associated with systemic lupus erythematosus seems to be related to the treatment with corticosteroids.1,3 Corticosteroids heal Libman–Sacks endocarditis, but thereby they lead to fibrotic, retracted leaflet tissue and thus to severe valvular dysfunction.2,5 Macroscopic and microscopic examinations of the valves usually do not reveal any active endocarditis. Massive haemorrhage in SLE patients usually occurs in the central nervous system or alimentary tract so great care must be taken in regulating anticoagulant therapy.2 However, a conservative operation does not alter the natural history and progression of the valvular disease. Hence, the valve reconstruction in these young patients, with the expectation of avoiding prosthetic valve dysfunction, anticoagulation and repeat operation, is not always possible.1 The prosthetic-valve-related morbidity and mortality remain high in patients with systemic lupus erythematosus.4,5 Bioprosthetic valve replacement eliminates the need for anticoagulation during steroid treatment.1 However, SLE valvulopathy affects the bioprosthetic valve.8 Hence, our decision for mechanical prosthesis for this 37-year-old woman is consonant with others and is valid.5,8
Conclusion Comments Systemic lupus erythematosus is an autoimmune inflammatory disease with the potential of affecting virtually all organ systems.1 The cardiac complications of SLE may involve the endocardium, myocardium, pericardium and coronary vessels.2,4 The valvular abnormalities occur in 36–48% of patients with systemic lupus erythematosus but patients rarely present with it.3,4 Clinically significant valvular heart disease due to systemic lupus erythematosus has been generally considered rare, and Libman–Sacks endocarditis has been thought to be predominantly an autopsy finding.2,5 The valve involvement in chronic SLE is similar to that of chronic rheumatic disease. Valvular heart disease, with a propensity for the left valves, is the most important cardiac manifestation of SLE.5 Lupus valvulopathy includes leaflet thickening, vegetations, nodularity and poor coaptation leading to regurgitation and stenosis. The pathogenesis of valvulopathy may involve interaction of antiphospholipid antibody with antigens on the valve surface, resulting in valvulitis.2,4 A literature survey shows that significant morbidity from valvular dysfunction, mostly mitral regurgitation leading to congestive heart failure, occurs in 4 and 6% of SLE patients.4 SLE is considered as one of the uncommon causes of acute aortic insufficiency.6,7 A controlled study has revealed that patients with lupus had an increased prevalence of echocardiographic abnormalities. These
We conclude that attentive cardiac examination and systematic echocardiography should be part of routine follow-up of patients with disseminated lupus erythematosus. The emergency surgical treatment for valvular diseases may be considered even in the patient with SLE. It is crucial to reduce inflammation pre- and postoperatively and to carefully select the operative procedures when treating such patients. Both the aortic and mitral valves should be explored. During valve replacement, a careful manipulation should be done because of friability of cardiovascular tissue and the suture line should be reinforced. Patients should be adequately covered with steroids in the perioperative period. Special attention must be given to the prevention of intraoperative thromboembolism and prompt and aggressive postoperative anticoagulation.
References 1. Dajee H, Hurley EJ, Szarnicki RJ. Cardiac valve replacement in systemic lupus erythematosus. A review. J Thorac Cardiovasc Surg 1983;85(5):718–26. 2. Hojnik M, George J, Ziporen L, Shoenfeld Y. Heart valve involvement (Libman–Sacks endocarditis) in the antiphospholipid syndrome. Circulation 1996;93(8):1579–87. 3. Fluture A, Chaudhari S, Frishman WH. Valvular heart disease and systemic lupus erythematosus therapeutic implications. Heart Dis 2003;5(5):349–53.
4. Galve E, Candell-Riera J, Pigrau C, Permanyer-Miralda G, Garcia-Del-Castillo H, Soler-Soler J. Prevalence, morphologic types, and evolution of cardiac valvular disease in systemic lupus erythematosus. N Engl J Med 1988;319(13): 817–23. 5. Georghiou GP, Shapira Y, Drozd T, Erez E, Raanani E, Vidne BA, et al. Double-valve Libman–Sacks endocarditis: an entity that demands special consideration. J Heart Valve Dis 2003;12(6):797–801. 6. Moynihan T, Hansen R, Troup P, Olinger G. Simultaneous aortic and mitral valve replacement for lupus endocarditis: report
Kumar et al. Aortic valve replacement in SLE
399
of a case and review of the literature. J Thorac Cardiovasc Surg 1988;95(1):142–5. 7. Thandroyen FT, Matisonn RE, Weir EK. Severe aortic incompetence caused by systemic lupus erythematosus. A case report. S Afr Med J 1978;54(4):166–8. 8. Gordon RJ, Weilbaecher D, Davy SM, Safi HJ, Quinones MA, DeFelice CA, et al. Valvulitis involving a bioprosthetic valve in a patient with systemic lupus erythematosus. J Am Soc Echocardiogr 1996;9(1):104–7.
CASE REPORT
Heart, Lung and Circulation 2006;15:397–399
Case Report
Emergency Aortic Valve Replacement in Systemic Lupus Erythematosus Sanjay Kumar, MCh, FRCS a,∗ , Bharati Sinha, MD, MRCP b and Edwin Ravikumar, MCh c a
Department of Cardiothoracic Surgery, 177, ‘D’ Floor, Yorkshire Heart Centre, Leeds General Infirmary, Great George Street, Leeds LS1 3EX, UK b Department of Cardiology, Bristol Childrens’ Hospital, Bristol, UK c Christian Medical Colleges and Hospital, Ida Scudder Road, Vellore 632 004, India
The valvular heart disease in systemic lupus erythematosus (SLE) is associated with substantial morbidity and mortality. Current therapy includes symptomatic measures and valve replacement. Overall mortality of valve replacement has been reported to be as high as 25%. Most cases of Libman–Sacks endocarditis in the literature reported dominant aortic regurgitation. We present this unusual case of a young female patient with SLE and glomerulonephritis warranting emergency isolated aortic valve replacement (AVR) for severe calcific aortic stenosis. The literature is reviewed with specific focus on the pathogenesis of and acute treatment options for this extremely rare occurrence. (Heart, Lung and Circulation 2006;15:397–399) © 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. Systemic lupus erythematosus; Valvular heart disease; Aortic valve replacement; Emergency aortic valve replacement
Introduction
R
ecent reports suggest an increased incidence of significant valvular dysfunction in patients with systemic lupus erythematosus (SLE) who have received longterm corticosteroid treatment.1,2 The occurrence of aortic stenosis is far more seldom than regurgitation in such patients.2,3 The renal function, pulmonary status and cerebral complications require special attention in these highsurgical-risk patients. The present report of a patient under prolonged steroid therapy and associated renal dysfunction necessitating emergency aortic valve replacement (AVR) for acute refractory left ventricular dysfunction poses further challenge.
Case report A 37-year-old woman presented with complaints of exertional dyspnoea, chest pain and palpitation that had persisted for 1-year. Her symptoms worsened 6 months ago when she developed bilateral leg swelling, abdominal distension and orthopnoea. The transthoracic echocardiographic (TTE) examination revealed severe calcific aortic stenosis and mild aortic and mitral incompetence. Since Received 25 March 2006; accepted 5 April 2006; available online 11 July 2006 ∗ Corresponding author. Tel.: +44 7980631248; fax: +44 1173738046. E-mail address: [email protected] (S. Kumar).
then she had been on digoxin and frusemide as antifailure medication. She denied having any past history suggestive of rheumatic fever. She was a known case of SLE with antiphospholipid antibodies for last 15 years. She was on complete remission with oral prednisolone 40 mg on alternate days and chloroquin 250 mg once a day. She had had cyclophosphamide to control her symptoms in past. As a complication of SLE, she had chorea and mild renal function impairment due to grade 1 glomerulonephritis. She was admitted for AVR as she was in complete remission of SLE and NYHA functional class 2. Her condition was being optimized for cardiac catheterisation. However, her functional status acutely deteriorated with the development of low cardiac output state requiring emergency endotracheal intubation and preoperative inotropic support. We decided to undertake emergency AVR as a high surgical risk. The procedure was performed through median sternotomy. Cardiopulmonary bypass (CPB) was established with aortobicaval cannulation. The myocardial protection was achieved with cold antegrade and retrograde blood cardioplegia. She had moderate cardiomegaly with left ventricular hypertrophy. The ascending aorta was dilated with small-sized aortic annulus. The aortic valve was tricuspid, heavily calcified and stenotic. The calcification was mostly on the cusps and the annulus, but there were no degenerative changes. On the basis of the intraoperative transoesophageal echocardiographic assessment and in view of small-sized left atrium, the trivial mitral regur-
© 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.
1443-9506/04/$30.00 doi:10.1016/j.hlc.2006.04.002
398
Kumar et al. Aortic valve replacement in SLE
CASE REPORT
gitation was considered to be functional. The aortic valve was excised and annulus was thoroughly decalcified. Sorin bicarbon aortic valve of size 19 mm was implanted using interrupted 2 ‘0’ Ticron vertical mattress suture bolstered with teflon pledgets. After 300 cc of hotshot cardioplegia, the patient was rewarmed and weaned off bypass on high inotropic support. However, at this stage the patient developed hypotension not responding to higher doses of inotropes. Hence, secondary CPB was commenced with ascending aortic and single right atrial venous cannula. The system was supported for 40 min and the patient was successfully weaned off CPB with good haemodynamics on intraaortic balloon pump support. The total pump time was 1 h (1st time) and 40 min (secondary CPB). The total aortic cross-clamp time was 35 min. She was weaned off IABP with good haemodynamics in 36 h. The patient was electively ventilated for 30 h. She was judiciously covered with intravenous prednisolone and diuretics in perioperative period to prevent acute deterioration of SLE and renal function, respectively. She had mild impairment of renal function parameters, which improved subsequently. Her ICU stay was free of any haemodynamic or haemostatic complications. She was discharged 2 weeks after the operation. The histological examination of the excised valve revealed the picture consistent with steroid-modified Libman–Sacks endocarditis. The echocardiographic follow-up performed on 12th postoperative day and at 3 months, 1 year and 2 years revealed good haemodynamics of the prosthetic valve with good LV function. Her SLE has been in complete clinical remission 2 years after the operation.
Heart, Lung and Circulation 2006;15:397–399
included pericardial effusion and/or thickening (37%), left ventricular hypertrophy (12%), global left ventricular hypokinesis (5%), segmental abnormalities of left ventricular wall motion (4%), right ventricular enlargement (4%), focal verrucous valvar thickening (12%), gross valvar thickening and dysfunction (8%), mitral regurgitation (25%) and aortic regurgitation (8%).4 The increased incidence of mitral valve insufficiency associated with systemic lupus erythematosus seems to be related to the treatment with corticosteroids.1,3 Corticosteroids heal Libman–Sacks endocarditis, but thereby they lead to fibrotic, retracted leaflet tissue and thus to severe valvular dysfunction.2,5 Macroscopic and microscopic examinations of the valves usually do not reveal any active endocarditis. Massive haemorrhage in SLE patients usually occurs in the central nervous system or alimentary tract so great care must be taken in regulating anticoagulant therapy.2 However, a conservative operation does not alter the natural history and progression of the valvular disease. Hence, the valve reconstruction in these young patients, with the expectation of avoiding prosthetic valve dysfunction, anticoagulation and repeat operation, is not always possible.1 The prosthetic-valve-related morbidity and mortality remain high in patients with systemic lupus erythematosus.4,5 Bioprosthetic valve replacement eliminates the need for anticoagulation during steroid treatment.1 However, SLE valvulopathy affects the bioprosthetic valve.8 Hence, our decision for mechanical prosthesis for this 37-year-old woman is consonant with others and is valid.5,8
Conclusion Comments Systemic lupus erythematosus is an autoimmune inflammatory disease with the potential of affecting virtually all organ systems.1 The cardiac complications of SLE may involve the endocardium, myocardium, pericardium and coronary vessels.2,4 The valvular abnormalities occur in 36–48% of patients with systemic lupus erythematosus but patients rarely present with it.3,4 Clinically significant valvular heart disease due to systemic lupus erythematosus has been generally considered rare, and Libman–Sacks endocarditis has been thought to be predominantly an autopsy finding.2,5 The valve involvement in chronic SLE is similar to that of chronic rheumatic disease. Valvular heart disease, with a propensity for the left valves, is the most important cardiac manifestation of SLE.5 Lupus valvulopathy includes leaflet thickening, vegetations, nodularity and poor coaptation leading to regurgitation and stenosis. The pathogenesis of valvulopathy may involve interaction of antiphospholipid antibody with antigens on the valve surface, resulting in valvulitis.2,4 A literature survey shows that significant morbidity from valvular dysfunction, mostly mitral regurgitation leading to congestive heart failure, occurs in 4 and 6% of SLE patients.4 SLE is considered as one of the uncommon causes of acute aortic insufficiency.6,7 A controlled study has revealed that patients with lupus had an increased prevalence of echocardiographic abnormalities. These
We conclude that attentive cardiac examination and systematic echocardiography should be part of routine follow-up of patients with disseminated lupus erythematosus. The emergency surgical treatment for valvular diseases may be considered even in the patient with SLE. It is crucial to reduce inflammation pre- and postoperatively and to carefully select the operative procedures when treating such patients. Both the aortic and mitral valves should be explored. During valve replacement, a careful manipulation should be done because of friability of cardiovascular tissue and the suture line should be reinforced. Patients should be adequately covered with steroids in the perioperative period. Special attention must be given to the prevention of intraoperative thromboembolism and prompt and aggressive postoperative anticoagulation.
References 1. Dajee H, Hurley EJ, Szarnicki RJ. Cardiac valve replacement in systemic lupus erythematosus. A review. J Thorac Cardiovasc Surg 1983;85(5):718–26. 2. Hojnik M, George J, Ziporen L, Shoenfeld Y. Heart valve involvement (Libman–Sacks endocarditis) in the antiphospholipid syndrome. Circulation 1996;93(8):1579–87. 3. Fluture A, Chaudhari S, Frishman WH. Valvular heart disease and systemic lupus erythematosus therapeutic implications. Heart Dis 2003;5(5):349–53.
4. Galve E, Candell-Riera J, Pigrau C, Permanyer-Miralda G, Garcia-Del-Castillo H, Soler-Soler J. Prevalence, morphologic types, and evolution of cardiac valvular disease in systemic lupus erythematosus. N Engl J Med 1988;319(13): 817–23. 5. Georghiou GP, Shapira Y, Drozd T, Erez E, Raanani E, Vidne BA, et al. Double-valve Libman–Sacks endocarditis: an entity that demands special consideration. J Heart Valve Dis 2003;12(6):797–801. 6. Moynihan T, Hansen R, Troup P, Olinger G. Simultaneous aortic and mitral valve replacement for lupus endocarditis: report
Kumar et al. Aortic valve replacement in SLE
399
of a case and review of the literature. J Thorac Cardiovasc Surg 1988;95(1):142–5. 7. Thandroyen FT, Matisonn RE, Weir EK. Severe aortic incompetence caused by systemic lupus erythematosus. A case report. S Afr Med J 1978;54(4):166–8. 8. Gordon RJ, Weilbaecher D, Davy SM, Safi HJ, Quinones MA, DeFelice CA, et al. Valvulitis involving a bioprosthetic valve in a patient with systemic lupus erythematosus. J Am Soc Echocardiogr 1996;9(1):104–7.
CASE REPORT
Heart, Lung and Circulation 2006;15:397–399