Surgical Treatment of Mitral Valve Disease in Patients with Systemic Lupus Erythematosus

ABSTRACT IMAGE

INTRODUCTION

Systemic lupus erythematosus (SLE) is an autoimmune condition that predominantly affects young women and presents with a wide spectrum of clinical features. Among these, cardiovascular complications contribute significantly to both morbidity and mortality.^[1] Libman–Sacks endocarditis is the most distinctive cardiac manifestation of SLE, first described in 1924,^[2] and its association with antiphospholipid syndrome (APS) was later described in 1985.^[3] Mitral valve lesions are common in SLE, affecting over one-third of patients and often, leading to mitral regurgitation (MR) and occasionally mitral stenosis.^[4] The association of mitral valve prolapse (MVP) syndrome with SLE is sometimes overlooked.^[5]

While SLE and rheumatic heart disease share complex immunological mechanisms, the coexistence of rheumatic heart disease with SLE is extremely rare,^[6] with only a few case reports existing.

Transthoracic and transoesophageal echocardiography are the diagnostic modalities of choice for evaluating mitral valve disease accurately and histopathology can provide a definitive diagnosis.^[7]

There is a dearth of information on the results of mitral valve surgery in patients with SLE. We offer a range of five surgically treated cases with varying clinical manifestations in this study.

MATERIALS AND METHODS

RESULTS

Preoperative patient characteristics

Five patients with SLE underwent mitral valve surgery. All the 5 patients were females. The mean age was 27.6 years, and the range was 13–36 years. Presenting complaint in all the five patients was dyspnea on exertion New York heart association (NYHA) II-III. Major comorbidities noted in the study were lupus nephritis in one patient and APS with accelerated hypertension in one patient who also had a history of cerebrovascular accident and seizures 2 years back. Coexisting rheumatic heart disease was noted in one patient [Table 1].

Table 1: Patient characteristics.

Age
Mean	27.6 years
Range	13–36 years
Sex
Males	None
Females	5 (100%)
Chief presenting complaint	Dyspnoea on exertion
APLA (+)	1 patient (20%) case 5
Lupus Nephritis (+)	1 patient (20%) case 3
History of CVA (+)	1 patient (20%) case 5
CRHD (+)	1 patient (20%) case 4

+: Present, Apla: Antiphospholipid antibody, CVA: Cerebrovascular accident, CRHD: Chronic rheumatic heart disease

All the patients underwent transthoracic and transesophageal echocardiography [Figure 1-3]. Among 5 patients, 4 patients had verrucous lesions over the mitral valve [Figure 4] and in one patient small vegetation was noted intraoperatively over the anterior mitral leaflet [Table 2].

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Figure 1:

Echocardiography image: Dotted line indicating mitral regurgitation jet area 12 cm².

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Figure 2:

Echocardiography image showing vegetation over the mitral valve.

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Figure 3:

Postoperative echo image showing mitral regurgitation jet area 3 cm².

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Figure 4:

Intraoperative image of mitral valve.

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Table 2: Echocardiography and immunological profile.

Case profile	Case 1	Case 2	Case 3	Case 4	Case 5
SLE	+	+	+	+	+
Valve pathology	Severe MR MRJA 12 (cm2)	MVP with Severe MR and Severe TR MRJA 12 (cm2)	Severe MR MRJA 13 (cm2)	CRHD with Sever MS	Severe MR MRJA 14 (cm2)
ANA (immunofluorescence) and Anti ds DNA antibodies	+	Negative	Negative	+	Negative
ANA (immunoblot)	+	+	+	+	+
ASO titres	Negative	Negative	Negative	+	Negative
CRP	+	+	+	+	+
Anti-cardiolipin antibodies	Negative	Negative	Negative	Negative	+
Lupus anticoagulant	Negative	Negative	Negative	Negative	+
Anti-smith antibody	+	+	+	+	+

+: Present, SLE: Systemic lupus erythematosus, ANA: Antinuclear antibody, CRP: C-reactive protein, ASO: Antistreptolysin-O, MR: Mitral regurgitation, MRJA: Mitral regurgitation jet area, MVP: Mitral valve prolapse, CRHD: Chronic rheumatic heart disease

Surgical outcomes and perioperative patient course

All the patients were stabilized and taken up for surgery. Mitral valve repair was done in 1 patient (20%), whereas a valve replacement [Figure 5] was done in 4 patients (80%). A bio-prosthetic valve was used in 2 patients and a mechanical valve was used in 2 other patients [Table 3]. One patient underwent concomitant tricuspid valve repair. The intraoperative period was uneventful in all the patients. Postoperatively, one patient developed acute kidney injury while another patient had developed acute exacerbation of lupus nephritis. Both the patients were managed conservatively. All the patients were asymptomatic during the follow-up period with acceptable mitral valve gradients. Histopathology results were consistent with Libman–Sacks endocarditis [Figure 6].

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Figure 5:

Intraoperative image showing mitral valve replacement with a mechanical valve.

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Figure 6:

Histopathology of excised mitral valve leaflet showing fibrinous exudate. Mitral valve stained with hematoxylin and eosin and 10× magnification.

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Table 3: Operative data and clinical outcomes.

Case details	Case 1	Case 2	Case 3	Case 4	Case 5
Valve procedure	Mitral valve repair with Teflon felt sized to 23 mm Carpentier Edward ring	Mitral valve replacement with 27 mm Biocor tissue valve and tricuspid valve repair with 29 mm Duran ring	Mitral valve replacement was done with 25 mm Biocor tissue valve	Mitral valve replacement was done with 27 mm St. Jude medical mechanical valve	Mitral valve replacement was done with 27 mm St. Jude medical mechanical valve
Immediate postop	Acute kidney injury (+)	Uneventful	Exacerbation of lupus nephritis (+)	Uneventful	Uneventful
Postoperative stay	15 days	5 days	20 days	7 days	5 days
Postop mitral valve gradients	10/6 mmHg	12/6 mmHg	9/4 mmHg	10/6 mmHg	9/5 mmHg
Follow up	Operated in 2019	Operated in 2019	Operated in 2022	Operated in 2022	Operated in 2024
	5-year follow-up uneventful	5-year follow-up uneventful	2-year follow-up uneventful	2-year follow-up uneventful	3 months follow-up uneventful

DISCUSSION

SLE is an autoimmune disease with female preponderance, especially during their reproductive years.^[8] In our case series, all five patients were women aged between 13 and 36 years, which aligns with the epidemiological patterns.

According to the literature, cardiovascular involvement in SLE is estimated to be more than 50%.^[1,7] Libman and Sacks first described nonbacterial verrucous endocarditis in SLE in 1924,^[2] and Myerowitz et al.^[9] were the first to report mitral valve replacement for MR in SLE.

Heart valves on the left side are most typically impacted, among which the mitral valve is most frequently involved followed by the aortic valve.^[10] In our study, all the patients with SLE had disease of the mitral valve, while a single patient had a concomitant tricuspid valve regurgitation as well.

Mitral valve pathology in SLE can typically manifest with several characteristic features such as leaflet thickening, vegetations, regurgitation, and stenosis.^[1,7] Valve regurgitation represents the most predominant abnormality with stenosis being the least common.^[11,12] 80% of the patients in our study had a severe regurgitant lesion of the mitral valve.

Antiphospholipid antibodies (APLs).^[13] are frequently implicated in the development of valve lesions in SLE. However, as observed in both our study and prior reports, not all cases with SLE and Libman–Sacks endocarditis exhibit positive antiphospholipid markers.^[14] In our series, only one patient exhibited positive anticardiolipin antibodies.

Mitral valve prolapse (MVP) and SLE have a well-known association. MVP syndrome is generally a benign disorder. It can present with various clinical symptoms, and echocardiography is essential for diagnosis.^[15,16] According to two studies, MVP is more prevalent in SLE patients than in the general population and it is an early marker of connective tissue disorder. MVP and anticardiolipin antibodies have also been demonstrated to positively correlate, yet the patient in our series had a negative result for anticardiolipin antibody.^[17,18]

The coexistence of chronic rheumatic heart disease and SLE and manifesting symptoms at the same time is quite unusual^[6] and has only been described in a few reports. In our case with dual pathology, diagnosis was confirmed based on clinical findings and serologic evidence, including a positive history and positive anti-double-stranded deoxyribonucleic acid antibody titer.

The decision between mitral valve repair and replacement, and the choice between mechanical and biological valves is influenced by multiple factors, and there is no uniform consensus in the medical literature.^[7,19] However, in the current surgical practices, there is a clear understanding of the supremacy of valve repair over replacement whenever feasible, particularly for cases of MR.^[7] In our series, a 13-year-old patient underwent successful mitral valve repair. Chauvaud et al.^[20] attempted mitral valve repair in a case of the 17-year-old SLE patient; however, literature^[16,21] also highlights the risk of long-term degeneration and calcification of the repaired valves in SLE patients, necessitating reoperation.

Mitral valve replacement (MVR) is the mainstay to remove the diseased valve tissue en bloc and prevent recurrence.^[22] In our study, 2 patients received bio-prosthetic valves due to their desire for future pregnancy, thus avoiding the teratogenic risks of long-term anticoagulation associated with mechanical valves and 2 patients had undergone MVR with a mechanical valve. Despite the shortcomings associated with the bioprosthetic valves, the successful placement of porcine Carpentier–Edwards bioprosthetic valve has been reported by Gordon et al.^[23]

High mortality was noted by Kalangos et al.^[24] in the reported cases of valve replacement in SLE patients. Nonetheless, Morin et al.^[25] in their review of 25 cases with SLE who had undergone MVR reiterate the fact that mitral valve surgery is a reasonable option without posing a major risk to the patient.

A 2025 systematic review Agarwal et al.^[26] examined 14 studies involving 277 patients diagnosed with SLE and APS who underwent cardiac surgery. The overall results were comparable to those seen in high-risk cardiac cases but highlighted a significant occurrence of thromboembolic events and catastrophic APS.

In our study, there was no operative mortality. However, two patients experienced postoperative complications of which, one patient developed acute kidney injury following surgery and the other patient had developed an acute exacerbation of lupus nephritis. Both the patients were managed conservatively. All 5 cases were discharged in stable hemodynamic condition and remained asymptomatic during follow-up.

CONCLUSION

Although infrequent, mitral valve involvement is a recognized complication in patients with SLE. Surgical correction of the mitral valve lesions in SLE is both justified and effective – not only to alleviate the hemodynamic compromise but also to prevent potential thromboembolic complications. Our case series demonstrates that timely surgical intervention can lead to favorable outcomes, with symptomatic improvement and enhanced quality of life in affected individuals.