Cardiogenic Shock and Mitral Valve Chord Rupture: A Rare Presentation of Libman-Sacks Endocarditis.
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ABSTRACT:
SUBMITTER: Lampert J
PROVIDER: S-EPMC8299131 | biostudies-literature | 2020 Oct
REPOSITORIES: biostudies-literature
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Cardiogenic Shock and Mitral Valve Chord Rupture: A Rare Presentation of Libman-Sacks Endocarditis.
Lampert Joshua J Halista Michael M Pujadas Elisabet E Alexander Steven S Bier Benjamin B Hadley Michael M Healy Michael M Yunus Fahd F Goldman Martin M Fuster Valentin V
JACC. Case reports 20200909 12
Distinguishing Libman-Sacks endocarditis from other valvular heart disease etiologies has important implications for management. We present a case of a 23-year-old man who presented in extremis with fever and cardiogenic shock caused by Libman-Sacks endocarditis with associated mitral valve chord rupture. (<b>Level of Difficulty: Beginner.</b>). ...[more]
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Project description:Libman-Sacks (LS) endocarditis is one of the most common cardiac manifestations of systemic lupus erythematosus. Rarely, however, it can lead to serious complications, including severe valvular regurgitation or superimposed bacterial endocarditis. We describe the initial diagnostic challenges, clinical course, imaging studies and histopathological findings of a patient who presented with life-threatening lupus complicated by hemoptysis and respiratory failure secondary to a rare complication of LS endocarditis, acute mitral valve perforation. We review the current literature on valve perforation in the setting of LS endocarditis. In conclusion, although the disease is often asymptomatic and hemodynamically insignificant, it can result in serious and potentially fatal complications secondary to valve perforation, which may demand emergency surgical management.
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Project description:Libman-Sacks endocarditis is a cardiac manifestation of systemic lupus erythematosus (SLE) and is characterized by non-bacterial verrucous vegetations, causing valvular stenosis and/or regurgitation. The effectiveness of immunosuppressive therapy for valve dysfunction due to Libman-Sacks endocarditis has not been reported. A 67-year-old woman with a history of chronic atrial fibrillation was emergently admitted with acute decompensated heart failure. Transoesophageal echocardiogram revealed severe mitral regurgitation (MR) due to oedematous thickening and poor coaptation of the medial edge of A2/P2 segments and the lateral edge of A3/P3 segments. Serial blood culture results were negative, suggesting bacterial infective endocarditis to be a less likely cause of valvular damage. Because the patient developed photosensitivity, livedo reticularis, and pancytopenia, Libman-Sacks endocarditis with rapidly progressive SLE was diagnosed on the basis of positive test results of anti-double-stranded DNA-IgG and its complement titer. Two months after, immunosuppressive therapy including corticosteroids, a transoesophageal echocardiogram revealed thinning of the degenerative mitral valve leaflets and a reduction of MR from severe to mild. Corticosteroid therapy for Libman-Sacks endocarditis reportedly increases the extent of fibrosis and scarring of the valve leaflets, resulting in worse valve function. In our patient, MR decreased from severe to mild after corticosteroid therapy. Because low-echoic thickening of the mitral valve leaflets suggested acute oedematous changes without scarring and fibrosis and other clinical symptoms of SLE rapidly progressed, early initiation of immunosuppressive therapy for Libman-Sacks endocarditis lead to a benign clinical course in our patient.
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Project description:BackgroundSystemic lupus erythematosus (SLE) valvulopathy can manifest as a spectrum of pathologies and treatment of severe valvular dysfunction thus far has been surgical. However, surgery in patients with SLE is frequently associated with high morbidity and mortality due to the presence of significant co-morbidities.Case summaryWe report the case of a 41-year-old woman with SLE and anti-phospholipid syndrome with extensive co-morbidities including lupus nephritis, pancytopaenia, cerebrovascular accident, and severe airway obstruction from ipsilateral lung collapse and bronchiectasis. She had severe mitral regurgitation (MR) from Libman-Sacks endocarditis and in recent months developed heart failure with progressive exertional dyspnoea from New York Heart Association (NYHA) functional Class from New York Heart Association (NYHA) functional class II to III. In addition, there was progressive left ventricular dilatation and reduction in left ventricular ejection fraction. In view of the high surgical risk, she underwent transcatheter edge-to-edge repair (TEER) of the mitral valve with the MitraClip system. At 1-month follow-up, she was back to NYHA functional Class II with mild MR.DiscussionOur case demonstrates that in select patient with suitable anatomy, TEER is a potential treatment option for severe MR from SLE valvulopathy.
| S-EPMC8517867 | biostudies-literature
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.
| S-EPMC3941465 | biostudies-literature
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.
| S-EPMC7886205 | biostudies-literature
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.
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Project description:BackgroundAcute 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 summaryA 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.DiscussionThis 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.
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Project description: Not available
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Project description:BackgroundPapillary muscle rupture due to infective endocarditis is a rare event and proper management of this condition has not been described in the literature. Our case aims to shed light on treatment strategies for these patients using the current guidelines.Case presentationThis case presents a 58-year-old male with acute heart failure secondary to papillary muscle rupture. He underwent an en bloc resection of his mitral valve with a bioprosthetic valve replacement. Specimen pathology later showed necrotic papillary muscle due to infective endocarditis. The patient was further treated with antibiotic therapy. He recovered well post-operatively and continued to do well after discharge.ConclusionIn patients who present with papillary muscle rupture secondary to infective endocarditis, clinical symptoms should drive the treatment strategy. Despite the etiology, early mitral valve surgery remains treatment of choice for patients who have papillary muscle rupture leading to acute heart failure. Culture-guided prolonged antibiotic treatment is vital in this category of patients, especially those who have a prosthetic valve implanted.
| S-EPMC9270734 | biostudies-literature
Project description:ObjectivesMechanical circulatory support is often challenging in patients with cardiogenic shock secondary to valvular heart disease because of challenging device placement, decreased efficacy, the need for a concomitant device for left ventricular unloading, or contraindications. Left atrial venoarterial-extracorporeal membranous oxygenation (LAVA-ECMO) is an emerging technique to achieve simultaneous ventricular unloading and circulatory support unaffected by valvular disease. The use of LAVA-ECMO for high-risk transcatheter valvular replacement has not been described.Key stepsWe describe the case of a patient with cardiogenic shock resulting from dual aortic and mitral bioprosthetic degeneration who was treated with LAVA-ECMO-supported dual-transcatheter aortic and mitral valve-in-valve replacement.Potential pitfallsAmong many precautions worth mentioning, operators should be aware of the care and adjustments of the ECMO circuit required during transcatheter valvular replacement to achieve technical success without complications. The importance of a careful case planning in a multidisciplinary heart team meeting cannot be overemphasized.Take-home messageLAVA ECMO enables high-risk valvular replacement in patients in valvular cardiogenic shock.
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