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Acute papillary muscle infarction and rupture in the puerperium complicating Libman-Sacks endocarditis in a patient with systemic lupus erythematosus and antiphospholipid syndrome: a case report.

ABSTRACT: Background:Acute heart failure caused by severe mitral regurgitation (MR) due to papillary muscle rupture has been described in the puerperium by case reports; however, the majority of cases of papillary muscle rupture are caused by myocardial infarction. We describe papillary muscle rupture occurring in the postpartum period in a patient with systemic lupus erythematosus (SLE), antiphospholipid syndrome (APLS), and chronic Libman-Sacks endocarditis and explore the multifactorial nature of the papillary muscle infarction and rupture in the setting of postpartum fluid shifts, chronic myocardial injury from Libman-Sacks, and high thrombotic risk. Case summary:A 29-year-old woman presented with acute heart failure 2 weeks' postpartum and was found to have acute MR due to a flail leaflet caused by papillary muscle rupture. She proceeded to emergency surgery with mitral valve (MV) replacement and the histology revealed evidence of chronic Libman-Sacks endocarditis and papillary muscle infarction with thrombi in the intramyocardial arteries. Discussion:This is the second case report of papillary muscle rupture in the puerperium in a patient with SLE in the literature, the other case was caused by catastrophic APLS. However, in this case, the cause of the rupture is likely to be multifactorial; as a consequence of thrombosis in the microvasculature causing isolated papillary muscle ischaemia, and fibrosis of the muscle due to chronic Libman-Sacks endocarditis resulting in limited pliability which caused rupture of the papillary muscle when faced with the added stress of increased volume that occurs in the puerperium.

SUBMITTER: Curtis E

PROVIDER: S-EPMC7026610 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature

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Acute papillary muscle infarction and rupture in the puerperium complicating Libman-Sacks endocarditis in a patient with systemic lupus erythematosus and antiphospholipid syndrome: a case report.

Curtis Elizabeth E Corkill Michael M Amir Nezar N Haydock David D

European heart journal. Case reports 20191113 4

<h4>Background</h4>Acute heart failure caused by severe mitral regurgitation (MR) due to papillary muscle rupture has been described in the puerperium by case reports; however, the majority of cases of papillary muscle rupture are caused by myocardial infarction. We describe papillary muscle rupture occurring in the postpartum period in a patient with systemic lupus erythematosus (SLE), antiphospholipid syndrome (APLS), and chronic Libman-Sacks endocarditis and explore the multifactorial nature ...[more]

PMID: 32099964

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Project description:ObjectivesThe aim of this study was to determine whether Libman-Sacks endocarditis is a pathogenic factor for cerebrovascular disease (CVD) in systemic lupus erythematosus (SLE).BackgroundA cardioembolic pathogenesis of SLE CVD manifested as: 1) neuropsychiatric systemic lupus erythematosus (NPSLE), including stroke and transient ischemic attacks (TIA); 2) neurocognitive dysfunction; and 3) magnetic resonance imaging of focal brain lesions has not been established.MethodsA 6-year study of 30 patients with acute NPSLE (27 women, 38 ± 12 years of age), 46 age- and sex-matched SLE controls without NPSLE (42 women, 36 ± 12 years of age), and 26 age- and sex-matched healthy controls (22 women, 34 ± 11 years of age) who underwent clinical and laboratory evaluations, transesophageal echocardiography, carotid duplex ultrasound, transcranial Doppler ultrasound, neurocognitive testing, and brain magnetic resonance imaging/magnetic resonance angiography. Patients with NPSLE were re-evaluated after 4.5 months of therapy. All patients were followed clinically for a median of 52 months.ResultsLibman-Sacks vegetations (87%), cerebromicroembolism (27% with 2.5 times more events per hour), neurocognitive dysfunction (60%), and cerebral infarcts (47%) were more common in NPSLE than in SLE (28%, 20%, 33%, and 0%) and healthy controls (8%, 0%, 4%, and 0%, respectively) (all p ≤ 0.009). Patients with vegetations had 3 times more cerebromicroemboli per hour, lower cerebral blood flow, more strokes/TIA and overall NPSLE events, neurocognitive dysfunction, cerebral infarcts, and brain lesion load than those without (all p ≤ 0.01). Libman-Sacks vegetations were independent risk factors of NPSLE (odds ratio [OR]: 13.4; p < 0.001), neurocognitive dysfunction (OR: 8.0; p = 0.01), brain lesions (OR: 5.6; p = 0.004), and all 3 outcomes combined (OR: 7.5; p < 0.001). Follow-up re-evaluations in 18 of 23 (78%) surviving patients with NPSLE demonstrated improvement of vegetations, microembolism, brain perfusion, neurocognitive dysfunction, and lesion load (all p ≤ 0.04). Finally, patients with vegetations had reduced event-free survival time to stroke/TIA, cognitive disability, or death (p = 0.007).ConclusionsThe presence of Libman-Sacks endocarditis in patients with SLE was associated with a higher risk for embolic CVD. This suggests that Libman-Sacks endocarditis may be a source of cerebral emboli.

Project description:BackgroundLibman-Sacks endocarditis in patients with systemic lupus erythematosus (SLE) is commonly complicated with embolic cerebrovascular disease (CVD) or valve dysfunction for which high-risk valve surgery is frequently performed. However, the role of medical therapy alone for Libman-Sacks endocarditis and associated acute CVD remains undefined.ObjectiveTo determine in this cross-sectional and longitudinal study if conventional anti-inflammatory and anti-thrombotic therapy may be an effective therapy in SLE patients with Libman-Sacks endocarditis and associated acute CVD.Methods and materials17 SLE patients with Libman-Sacks endocarditis detected by two-and-three-dimensional transesophageal echocardiography (TEE) and complicated with acute CVD [stroke/TIA, focal brain injury on MRI, or cognitive dysfunction] were treated with conventional anti-inflammatory and anti-thrombotic therapy for a median of 6 months and then underwent repeat TEE, transcranial Doppler, brain MRI, and neurocognitive testing for re-assessment of Libman-Sacks endocarditis and CVD.ResultsValve vegetations decreased in number, diameter, and area (all p ≤0.01); associated valve regurgitation significantly improved (p = 0.04), and valve thickening did not progress (p = 0.56). In 13 (76%) patients, valve vegetations or valve regurgitation resolved or improved in number and size or by ≥1 degree, respectively, as compared to 4 (24%) patients in whom vegetations or valve regurgitation persisted unchanged or increased in size or by ≥1 degree (p = 0.03). Also, cerebromicroembolism, lobar and global gray and white matter cerebral perfusion, ischemic brain lesion load, and neurocognitive dysfunction resolved or significantly improved (all p ≤0.04).ConclusionThese preliminary data suggest that combined conventional anti-inflammatory and antithrombotic therapy may be an effective treatment for Libman-Sacks endocarditis and its associated CVD and may obviate the need for high-risk valve surgery.

Project description:BackgroundLibman-Sacks endocarditis, characterized by Libman-Sacks vegetations, is common in patients with systemic lupus erythematosus and is commonly complicated with embolic cerebrovascular disease. Thus, accurate detection of Libman-Sacks vegetations may lead to early therapy and prevention of their associated complications. Although two-dimensional (2D) transesophageal echocardiography (TEE) has high diagnostic value for detection of Libman-Sacks vegetations, three-dimensional (3D) TEE may allow improved detection, characterization, and clinical correlations of Libman-Sacks vegetations.MethodsTwenty-nine patients with systemic lupus erythematosus (27 women; mean age, 34 ± 12 years) prospectively underwent 40 paired 3D and 2D transesophageal echocardiographic studies and assessment of cerebrovascular disease manifested as acute clinical neurologic syndromes, neurocognitive dysfunction, or focal brain injury on magnetic resonance imaging. Initial and repeat studies in patients were intermixed in a blinded manner with paired studies from healthy controls, deidentified, coded, and independently interpreted by experienced observers unaware of patients' clinical and imaging data.ResultsThe results of 3D TEE compared with 2D TEE were more often positive for mitral or aortic valve vegetations, and 3D TEE detected more vegetations per study and determined larger sizes of vegetations (P ≤ .03 for all). Also, 3D TEE detected more vegetations on the anterior mitral leaflet, anterolateral and posteromedial scallops, and ventricular side or both atrial and ventricular sides of the leaflets (P < .05 for all). In addition, 3D TEE detected more vegetations on the aortic valve left and noncoronary cusps, coronary cusps' tips and margins, and aortic side or both aortic and ventricular sides of the cusps (P ≤ .01 for all). Furthermore, 3D TEE more often detected associated mitral or aortic valve commissural fusion (P = .002). Finally, 3D TEE detected more vegetations in patients with cerebrovascular disease (P = .01).ConclusionsThree-dimensional TEE provides clinically relevant additive information that complements 2D TEE for the detection, characterization, and association with cerebrovascular disease of Libman-Sacks endocarditis.

Dataset Information

Acute papillary muscle infarction and rupture in the puerperium complicating Libman-Sacks endocarditis in a patient with systemic lupus erythematosus and antiphospholipid syndrome: a case report.

Publications

Acute papillary muscle infarction and rupture in the puerperium complicating Libman-Sacks endocarditis in a patient with systemic lupus erythematosus and antiphospholipid syndrome: a case report.

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