Mitral Valve Replacement and Repair: Report of 5 Patients with Systemic Lupus Erythematosus

Abstract

Severe mitral valve regurgitation due to systemic lupus erythematosus is a rare cause of valvular heart disease, necessitating valve surgery. Currently, there are 36 case reports in the world medical literature of mitral valve replacement or repair in patients who have lupus. The current trend in mitral valve surgery is toward anatomic valve repair. In patients who have systemic lupus erythematosus, however, valve repair often leads to repeat surgery and valve replacement. We report the cases of 5 patients with lupus and severe mitral valve regurgitation who underwent mitral valve surgery. In 3 of these patients, replacement with a mechanical prosthetic mitral valve was performed with good long-term results. In the other 2 patients, mitral valve repair was performed, but only 1 of the repairs was successful. The 2nd patient required subsequent replacement with a mechanical valve.

To our knowledge, this report of 5 patients is the largest series of mitral valve surgery in patients with lupus. These results, along with a review of the literature, suggest the superiority of mechanical prosthetic valve replacement to repair in patients who have systemic lupus erythematosus.

Valvular endocarditis is a frequent manifestation of systemic lupus erythematosus (SLE), and the mitral valve is most frequently affected. ¹ Valvular lesions resulting from lupus can cause severe mitral regurgitation (MR). Recently, transesophageal echocardiography was used during a 2-year period to study 69 patients who had SLE. ¹ At the end of this time, echocardiograms showed that 52% of the patients had valvular thickening, 28% had MR, and 6% required mitral valve surgery.

In 1974, Myerowitz and colleagues ² were the 1st to report successful surgical management of severe MR through mitral valve replacement in a patient with SLE. Recently, anatomic repair of the mitral valve has been developed to correct severe MR. Such repair has been applied in patients with SLE-induced MR. The benefits of repair include the absence of any anticoagulation requirement, improved left ventricular function, and preservation of the papillary muscle apparatus. Herein, we review the world medical literature and report our experience with 5 patients who underwent mitral valve operations for SLE valvulitis.

Case Reports

Patient 1

A 45-year-old white woman presented at our institution with severe SLE that had been diagnosed originally in May of 1985. Manifestations of her disease included vasculitic skin lesions, leg ulceration, pleurisy, nephritis, proteinuria, osteonecrosis, and central nervous system vasculitis. Laboratory findings included antinuclear antibodies, anti-double-stranded DNA antibodies, and depressed complement C3 and C4. Her therapy for SLE required intermittent cyclophosphamide and long-term prednisone. She developed symptoms of congestive heart failure: orthopnea, paroxysmal nocturnal dyspnea, and fatigue, refractory to medical management.

In 1992, echocardiography revealed that the patient had severe MR, severe mitral stenosis, and severe tricuspid and aortic insufficiency. Cardiac catheterization on 15 September 1992 showed normal coronary anatomy, severe pulmonary hypertension (90/40 mmHg), elevated pulmonary capillary wedge pressures (42 mmHg), and elevated left ventricular end-diastolic pressures (25 to 30 mmHg). There was no gradient across the aortic valve. Left ventriculography indicated normal left ventricular systolic function with severe MR.

On 20 October, due to the patient's symptoms of heart failure, we placed 2 St. Jude mechanical valves (St. Jude Medical; St. Paul, Minn): a #29 in the mitral position and a #21 in the aortic position. In addition, we performed a DeVega tricuspid annuloplasty. ³ Intraoperatively, the patient was found to have calcific pericarditis requiring a pericardiectomy. The excised mitral valve was thickened and fibrotic, with focal calcific vegetations measuring 6 × 4 × 3 mm. On microscopic exam, the valve showed myxoid and hyaline degeneration.

The patient's recovery from surgery was uncomplicated, and she was discharged on the 6th postoperative day. Since her surgery, the patient has undergone hip replacements because of bilateral avascular necrosis of the femoral head. She has also had episodes of gastrointestinal bleeding that have been intensified by anticoagulation. When last seen in June of 2000, she was free from symptoms of heart failure, and follow-up echocardiograms showed preservation of left ventricular function.

Patient 2

A 23-year-old white woman presented with hypertension; she had been diagnosed with lupus in 1990. Clinical findings at that time included arthritis, rash, fevers, and deep venous thrombosis. A renal biopsy for proteinuria revealed stage-4 lupus nephritis. Laboratory findings included the presence of antinuclear antibodies, anti-double-stranded DNA antibodies (1:1160), and low complement C3 and C4.

In April 1998, the patient began experiencing progressive exertional dyspnea, orthopnea, and paroxysmal nocturnal dyspnea. Echocardiography revealed preserved left ventricular systolic function, severe MR, left atrial enlargement, and a small pericardial effusion. Her symptoms were refractory to medical therapy. Right- and left-heart catheterization were performed in May of 1998, which showed unobstructed coronary arteries and severe MR. She had pulmonary hypertension of 55/30 mmHg, a large V wave on the pulmonary artery tracing, and left ventricular end-diastolic pressure that was elevated to 16 mmHg. Her ejection fraction was preserved at 0.55 to 0.60, and the cardiac output was 5.4 L/min/m².

A transesophageal echocardiogram performed on 31 August 1998 showed an ejection fraction of 0.60, with a dilated left atrium, a thickened mitral valve, and severe MR. Mild aortic insufficiency and tricuspid regurgitation were also noted.

The patient underwent mitral valve replacement on 6 October 1998 with a #29 St. Jude mechanical valve. Intraoperatively, the native valve was pinkishtan in appearance. Pathologic examination revealed myxomatous changes, hyalinizing fibrosis, and minimal inflammation. The patient was discharged after an uneventful postoperative course. As of May 2000, she was doing well, without recurrence of heart failure or MR.

Patient 3

A 54-year-old hypertensive white woman presented with SLE that had been diagnosed in 1985. By June of 1992, she had experienced a worsening of the SLE, which in turn led to lupus valvulitis. She also had arthralgias and rash. Echocardiographic examination documented moderate aortic insufficiency and moderate MR. The left atrium was dilated and the left ventricular function was preserved (estimated ejection fraction, 0.40 to 0.45).

In September of 1992, she presented at the cardiology clinic with dyspnea on exertion and fatigue. Chest radiography showed mild pulmonary edema. Cardiac catheterization on 14 September showed normal pulmonary artery pressures, moderate aortic insufficiency, severe MR, and normal coronary arteries. The ejection fraction was 0.40, and the cardiac index was 2.2 L/min/m².

On 21 September, the patient underwent mitral valve repair, aortic valve annuloplasty, and dèbridement of vegetations from the right and left coronary cusps. Pathology examination of the dèbrided mitral valve revealed fibrosis with focal vascularization and macrophage infiltration.

These procedures gave the patient temporary relief from symptoms. However, in 1995, she again experienced dyspnea on exertion and fatigue. Transesophageal echocardiography on 27 February indicated severe MR and moderate aortic insufficiency; these findings were confirmed by right- and left-heart catheterization.

On 23 March 1995, the patient underwent double valve replacement with St. Jude mechanical valves: a #25 in the mitral position and a #21 in the aortic position. Pathology examination of the excised mitral valve showed fibrosis, focal vascularization, and infiltration with inflammatory cells. When last seen in June of 2000, the patient was doing well.

Patient 4

A 56-year-old white woman had initially presented to her rheumatologist in 1987 complaining of a rash on the sun-exposed areas of her body and worsening of her rheumatoid arthritis. She had a history of smoking and hyperlipidemia. Her serum complement C3 and C4 were depressed, and she had anti-double-stranded DNA antibodies. Systemic lupus erythematosus was diagnosed and she underwent immunosuppression with oral steroids. Her physical exam revealed a blowing systolic murmur radiating to the left axilla. Echocardiography revealed moderate MR. She was seen by the rheumatologist for 8 years and gradually developed dyspnea and exertional angina.

In October of 1995, percutaneous transluminal angioplasty of the right coronary artery was performed. By April of 1996, her exertional angina with shortness of breath had returned. She underwent repeat cardiac catheterization on 11 April. She was found to have moderately severe (3+) MR, triple-vessel coronary artery disease, and preserved left ventricular systolic function. She chose medical therapy at that time, including angioplasty, which was attempted in the left circumflex coronary artery on 20 August 1997. There was acute dissection of the distal vessel with evidence of a lateral wall infarct. Echocardiography showed marked worsening of the MR.

The patient continued to experience severe continuous angina and shortness of breath. On 29 October 1997, she underwent right- and left-heart catheterization in anticipation of mitral valve replacement. The pulmonary artery pressure was 32/25 mmHg, the pulmonary capillary wedge pressure was 12.2 mmHg, and the cardiac index was 3.0 L/min/m². The left ventriculogram revealed minimal inferobasal hypokinesia, preserved left ventricular function, and severe (4+) MR.

On 20 November 1997, the patient underwent mitral valve replacement with a #27 St. Jude mechanical prosthesis, and a bypass with a left internal mammary artery to the 1st diagonal branch of the left anterior descending coronary artery. Intraoperatively, the mitral valve leaflets were pink and the chordae tendineae were thin and fragile. Postoperatively, the patient required amiodarone to control atrial fibrillation. She was well when we saw her in May of 2000, with no recurrence of dyspnea or MR.

Patient 5

A 64-year-old white woman presented in 1996 with dyspnea and anginal chest pain. She had originally come to our institution in 1994 with a history of deep venous thrombosis, pulmonary emboli, and hypertension. At that time, the presence of antinuclear antibodies and depressed levels of complement C3 and C4 indicated a diagnosis of SLE, and she underwent treatment for 2 years. Upon her return in 1996, the patient had a blowing systolic murmur radiating to the left axilla. Cardiac catheterization revealed 4+ MR and preserved systolic function, with an ejection fraction of 0.70. Her circumflex coronary artery was totally occluded, the left anterior descending coronary artery had a 99% lesion in the mid-portion, and the right coronary artery had a 60% stenosis. Echocardiography on 20 September 1996 confirmed the presence of severe MR and showed prolapse of the posterior mitral leaflet with thickening of the mitral valve.

On 22 September, she underwent mitral valve repair, including a 2-cm quadrangular resection of the posterior leaflet and placement of a 31-mm Duran ring (St. Jude Medical). A left internal mammary artery graft was attached to the left anterior descending coronary artery, a gastroepiploic artery to the right coronary artery, and a saphenous vein graft to the obtuse marginal branch.

Pathology examination of the excised mitral valve leaflet showed it to be pliable, with small pink raised nodules on the left atrial surface measuring 0.1 × 0.1 × 0.1 cm. The attached chordae tendineae were partially fused and myxomatous with focal fibrosis.

By March of 1997, the patient's anginal symptoms had returned. Exercise thallium stress testing demonstrated ischemia in the posterolateral and posterior basal walls of the left ventricle. Cardiac catheterization showed the gastroepiploic graft to the right coronary artery to be insufficient. Therefore, the patient underwent angioplasty and stenting of the right coronary artery stenosis with a 3.0-mm Palmaz-Schatz® stent (Johnson & Johnson; Warren, NJ).

When we saw the patient in May of 2000, she was doing well.

Discussion

Libman and Sacks ⁴ 1st described valvular heart disease caused by SLE in 1924. However, it was not until Myerowitz and coworkers ² reported their case of mitral valve replacement for MR due to SLE in 1974 that a number of others followed (Table I ^2,5–34). In March of 1995, Kalangos and associates ²⁹ reported that until then, 14 cases of mitral valve replacements had been performed in SLE patients. Those authors described the 1st mitral valve reconstruction in a patient with severe MR and SLE. One year postoperatively, the patient had good valve function. They suggested that repair might be a preferable approach due to the elimination of the need for anticoagulation. Anticoagulation may present higher risks in young patients who require prolonged steroid use and who frequently experience renal failure, and the risks are especially high in patients who are undergoing hemodialysis and require valve replacement. ²⁹

Table I. Reported Mitral Valve Repair and Replacement in Patients with SLE

Other attempts at mitral valve repair in patients with SLE have not been as successful. Chauvaud and coworkers ³⁰ described a 17-year-old patient who was 1st treated with mitral valve repair. Follow-up revealed excessive calcification of the valve, necessitating mitral valve replacement with a cryopreserved homograft. They suggested that a conservative operation does not alter the progression of the disease and that continued valve thickening and valve calcification will affect the repaired valve.

The use of bioprosthetic porcine grafts in patients with lupus has also been hindered by complications. Porcine valves have become affected by valvulitis with perforation of valve cusps, thus requiring replacement. ³² Successful placement of the porcine Carpentier-Edwards bioprosthesis in patients with SLE has, however, been reported. ³²

Hoffman and associates ³⁵ reported that survival of patients with SLE has improved with use of corticosteroid therapy, but this therapy can cause scarring and shrinking of cardiac valves. The group presented a case in which the rapid development of severe MR occurred after a few weeks of high-dose corticosteroid therapy for lupus. ³⁵ It is notable that all of the patients in our series and most of the patients in the literature who required mitral valve surgery were on steroid therapy, often long-term.

Our series adds to the literature 3 mitral valve replacements and 2 mitral valve repairs in patients with severe MR and SLE. All of our patients were women; the ratio of men to women in Table I ^2,5–34 corresponds to previous reports that a large percentage of patients affected by SLE are women. The surgical success of valve replacement and repair in our patients was high, with no death or reoperation within the first 30 days after surgery.

In our patients, valve repair produced mixed results. Patient 3 required reoperation after 3 years and underwent mechanical valve replacement. In Patient 5, the repair was successful; however, she will likely require lifelong anticoagulation for antiphospholipid syndrome and venous thrombotic episodes. In retrospect, she might have been better served by valve replacement.

When patients with SLE need mitral valve surgery, we recommend mechanical valve replacement rather than repair. The use of mechanical valves requires anticoagulation; however, such treatment is often necessary in these patients, regardless. Antiphospholipid antibodies are frequently present in patients with SLE and valvular heart disease. Current therapeutic guidelines for antiphospholipid antibody syndrome include antithrombotic prevention with long-term anticoagulation. ³⁶ Due to embolic events in patients with lupus and the procoagulant effect of the lupus anticoagulant, many patients with lupus are placed on warfarin. Moreover, atrial fibrillation is frequently a concomitant condition affecting these patients with severe MR who require mitral valve surgery, so anticoagulation is not to be avoided in such patients.

The difficulty of anticoagulation in patients with SLE is the frequent development of end-stage renal disease and the resultant dependence on dialysis. However, valve repair and bioprosthetic valve replacement are not the best solution in the setting of lupus-induced renal insufficiency, since accelerated native valve and bioprosthetic valve calcification tend to occur because of the high calcium turnover.

Kalangos and co-authors ²⁹ stated that mortality rates remain high in the previously reported cases of mitral valve replacement in patients with SLE. Araki ³⁴ described a patient with SLE who underwent mitral valve replacement, had a number of complications, and died secondary to anticoagulation. Morin's group ³³ reviewed 25 cases of SLE patients undergoing mitral valve replacement that had been published in the literature up until December of 1996 and added 3 cases. Those authors suggested that mitral valve replacement is generally uneventful and makes surgery a feasible option without posing a major risk to patients with compensated organ function.

The experience with mitral valve replacement and repair in lupus patients is limited. Our experience and review of the literature, however, suggest that mitral valve replacement is superior to valve repair in patients who have systemic lupus erythematosus.