Deepa Prasad MD, Sannya Hede MD, Claire Cigarroa MD
Driscoll Children’s Hospital, Texas, USA
Clinical History
An 18-year-old, obese female presented to the hospital with acute confusion, syncope, aphasia, and right-sided hemiparesis. Her head computed tomography (CT) revealed an ill-defined hypodensity in the left frontoparietal lobe (Figure 1). Brain MRI/MRA identified occlusion of the M2 and M3 branches of the left middle cerebral artery, with acute and subacute infarcts in the left frontoparietal and temporal lobes, without evidence of hemorrhagic transformation (Figure 1). Neck MRA and lower extremity Doppler ultrasound were unremarkable.
Figure 1. CT head without contrast and brain MRI/MRA in axial projection. CT head without contrast showing hypointense lesion in the left frontoparietal lobe (A). Brain MRI/MRA shows a hyperintense lesions in the left frontoparietal and temporal lobes on axial diffusion weighted imaging (B) and T2 fluid attenuated inversion recovery (FLAIR) spectral presaturation with inversion recovery (SPIR) (C).
Laboratory evaluation revealed positive anti-Smith antibodies, lupus anticoagulant with antiphospholipid antibodies, and anti-nuclear antibodies. The patient exhibited nephrotic-range proteinuria and hematuria. Renal biopsy confirmed Class II lupus nephritis. Karius (microbial cell-free DNA testing) and serial blood cultures were negative. She was diagnosed with systemic lupus erythematosus (SLE) with secondary antiphospholipid syndrome.
Transthoracic echocardiography (TTE) revealed multiple, small, non-mobile masses on the anterior and posterior mitral valve leaflets, suggestive of Libman-Sacks endocarditis (Figure 2, Movie 1) and normal left ventricular (LV) systolic function. Electrocardiogram (ECG) demonstrated sinus bradycardia with inverted T waves in leads III and aVF (Figure 3). CMR was performed to characterize the mitral valve masses further and evaluate myocardial involvement.
Figure 2. Four chamber (A) and parasternal short axis (B) TTE images showing multiple, small, non-mobile masses on the anterior and posterior mitral valve leaflets (arrows).
Movie 1. Parasternal long axis (A) and short axis (B) TTE images showing multiple, small, non-mobile masses on the anterior and posterior mitral valve leaflets.
Figure 3. Twelve lead ECG demonstrating sinus bradycardia with inverted T waves in leads III and aVF.
CMR Findings
CMR was performed on 1.5 Tesla Ingenia scanner (Philips Healthcare, Best, The Netherlands). Multiple, small, non-mobile and isointense mitral valve masses were identified on cine balanced steady state free precession (bSSFP) imaging (Figure 4, Movie 2), which had dense hyperenhancement on late gadolinium enhancement (LGE) imaging (Figure 4).
The LV demonstrated normal systolic function with no regional wall motion abnormalities on cine bSSFP imaging. There was no evidence of myocardial edema on T2-weighted, black blood turbo-spin echo imaging.
Figure 4. Four chamber bSSFP at end diastole (A) and phase sensitive inversion recovery (PSIR) (B) images. There are small isointense masses on the anterior and posterior mitral leaflets (orange arrows, A) with corresponding hyperenhancement on LGE imaging (orange arrows, B). There is a small subendocardial hyperenhancement in the apical lateral segment (small embolic infarct versus an artifact) (blue arrow, B).
Movie 2. LV two chamber (A), three chamber (B), four chamber (C) cine bSSFP images showing small isointense masses on the anterior and posterior mitral valve leaflets.
A focal perfusion defect involving a small coronary territory was visualized on dynamic rest first-pass perfusion scan in the mid-inferior wall of the LV (American Heart Association (AHA) segment #10) (Figure 5, Movie 3). This area correlated with a focal, dense, nearly transmural area of LGE (Figure 6), consistent with an embolic infarct. There was an additional area of small sub-endocardial LGE in the apical lateral segment (blue arrow, Figure 4), which is suggestive of an embolic infarct vs an artifact as it was not visualized on the short-axis image. An additional area of small mid-myocardial area of hyperenhancement in the mid anterior wall was noted without corresponding area of a focal perfusion defect (Figure 6). This may be suggestive of a small, non-ischemic lesion related to the inflammatory process.
Figure 5. Mid first pass perfusion sequence with a subendocardial perfusion defect (orange arrow) in the mid-inferior wall of the LV.
Movie 3. Mid first pass perfusion sequence with focal, subendocardial perfusion defect in the mid-inferior LV wall.
Figure 6. Mid short axis PSIR (A,B) showing dense, nearly transmural LGE in the mid-inferior wall of LV (orange arrows), and an additional small mid-myocardial area of hyperenhancement in the mid-anterior wall (orange arrow, A).
Conclusion
Her clinical history, laboratory findings, and cardiac imaging were most consistent with non-infectious Libman-Sacks endocarditis and coronary embolic infarcts without evidence of myocarditis. Coronary angiography was discussed, but was not done because the embolic infarct was a small area. Treatment was initiated with intravenous methylprednisolone, rituximab, hydroxychloroquine, mycophenolate for SLE, aspirin for thromboembolic risk and lisinopril for hypertension. She did not receive any additional anticoagulation because of hemorrhagic risk in the brain. She received aggressive rehabilitation to improve her muscle strength. Her neurological symptoms have significantly improved, and she continues to receive physical therapy, speech and language therapy. She will undergo a follow up CMR in 6 months.
Perspective
This case highlights the role of CMR in the comprehensive assessment of antiphospholipid syndrome in patients with SLE. CMR can add incremental value beyond TTE in defining the extent and nature of cardiac involvement. CMR is particularly useful because of its ability to define embolic coronary artery events by LGE imaging, and this could affect treatment strategies. This case highlights that normal LV function does not exclude myocardial infarction.
LGE pattern and perfusion findings are central to the diagnosis. Cardiac involvement in antiphospholipid syndrome secondary to SLE is prevalent and can be immune-mediated or thromboembolic. Valvular abnormalities affect up to 30% of patients with secondary antiphospholipid syndrome, and coronary artery involvement is frequent, significantly increasing morbidity and mortality.[1,2] Embolic coronary vascular events occur in up to 22% of patients with Libman-Sacks endocarditis.[3,4] Secondary antiphospholipid syndrome causes early myocardial infarction and death in approximately 10% of patients.[2] The association between myocarditis and antiphospholipid syndrome remains poorly understood. The presence of Libman-Sacks endocarditis in patients with SLE increases the risk for embolic cerebrovascular disease, increasing morbidity and mortality.[3] Cerebrovascular disease can manifest as stroke, transient ischemic attack, seizures, and neurocognitive defects.[3]
In summary, Libman-Sacks endocarditis can lead to a broader cardiovascular and cerebrovascular burden in patients with SLE and early use of CMR during diagnostic evaluation provides valuable information for both diagnosis and clinical management [5], as TTE alone cannot fully assess the embolic infarcts and extent of myocardial involvement.
CMR in SLE frequently demonstrates non‑ischemic LGE, typically in a mid‑wall or subepicardial distribution, reflecting prior immune-mediated myocardial injury rather than coronary artery disease. One case series showed 30% of patients with SLE had non-ischemic LGE on CMR and no patient in the cohort had evidence of ischemic LGE.[6] In cases where LGE is present without accompanying T2-based evidence of myocardial edema, the findings most likely represent chronic fibrosis from prior lupus-associated inflammation that is no longer active. This pattern underscores the ability of CMR to distinguish between active inflammatory myocarditis and residual fibrosis, even in patients with otherwise clinically silent disease. This patient had evidence of both ischemic and non-ischemic LGE owing to infarct from embolic phenomenon and fibrosis from SLE.
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References
- Zuily S, Huttin O, Mohamed S, Marie PY, Selton-Suty C, Wahl D. Valvular heart disease in antiphospholipid syndrome. Curr Rheumatol Rep. 2013 Apr;15(4):320. doi: 10.1007/s11926-013-0320-8. PMID: 23456852.
- Kolitz T, Shiber S, Sharabi I, Winder A, Zandman-Goddard G. Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form. Front Immunol. 2019 May 10;10:941. doi: 10.3389/fimmu.2019.00941.31134062; PMCID: PMC6522847.
- Roldan CA, Sibbitt WL, Qualls CR, et al. Libman-Sacks Endocarditis and Embolic Cerebrovascular Disease. JACC Cardiovasc Imaging. 2013;6(9):973-983.
- Saric M, Armour AC, Arnaout MS, et al. Guidelines for the Use of Echocardiography in the Evaluation of a Cardiac Source of Embolism. J Am Soc Echocardiogr. 2016;29(1):1-42.
- Sacher AC, Ahmed R, Kung A, Kahlam J. Myocarditis: A Rare Cardiac Manifestation of Antiphospholipid Syndrome. JACC Case Rep. 2025 Apr 16;30(8):102667. doi: 10.1016/j.jaccas.2024.102667. Epub 2025 Mar 17. PMID: 40250918; PMCID: PMC12046890.
- Burkard T, Trendelenburg M, Daikeler T, Hess C, Bremerich J, Haaf P, Buser P, Zellweger MJ. The heart in systemic lupus erythematosus – A comprehensive approach by cardiovascular magnetic resonance tomography. PLoS One. 2018 Oct 1;13(10):e0202105.
Case Prepared By:
Avanti Gulhane, MD, DNB, FSCMR
Editorial Board, Cases of SCMR
University of Washington
