An extraordinary case of recurrent stroke, disseminated thrombosis and endocarditis

Thomas, Laurent; Shaikh, Niaz Ahmed; Pradeep, Ranjana

doi:10.1136/bcr-2018-224172

Cited by 2 publications

(1 citation statement)

References 26 publications

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“…In the majority of these cases, the pathologic findings showed evidence of myocardial infarction and valvular disease. In addition, Libman‐Sacks endocarditis has been reported in 13% of CAPS patients with heart involvement and may be accompanied by stroke [ 16 , 17 ]. Cardiomyopathy is less frequently described, but may develop as a consequence of coronary artery occlusion or significant valvulopathy [ 18 ].…”

Section: Introductionmentioning

confidence: 99%

Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review

Hermel

Azam

et al. 2020

eJHaem

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Catastrophic antiphospholipid antibody syndrome (CAPS) is a rare form of antiphospholipid syndrome, an autoimmune condition characterized by vascular thromboses, pregnancy loss, and antiphospholipid (aPL) antibodies. Diagnosis of CAPS relies on thrombosis of at least three different organs systems over 1 week, histopathological evidence of small vessel occlusion, and high aPL antibody titers. In a subset of precipitating circumstances, activation or disruption of endothelial cells in the microvasculature may occur along with cardiomyopathy. We present two cases of CAPS‐associated dilated cardiomyopathy at our institution, focusing on disease management, pathophysiology, and treatment. These patients were of Southeastern Asian descent, raising the possibility of genetic polymorphisms contributing to the development of cardiomyopathy. Both met CAPS criteria and both demonstrated clinicopathologic thrombotic microangiopathy (TMA) and complement activation and developed severe dilated cardiomyopathy with shock. Complement activation plays an important role in the development of CAPS and may be important in the pathogenesis of CAPS‐associated cardiomyopathy. Clinical suspicion for TMA as a pathophysiologic mechanism of unexplained heart failure in CAPS is important and increased awareness of cardiac side effects is necessary so that early treatment can be initiated to halt further cardiac and systemic complications.

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Section: Introductionmentioning

confidence: 99%

Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review

Hermel

Azam

et al. 2020

eJHaem

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Heart Valve Involvement in Patients with Antiphospholipid Syndrome: A Long-Term Follow-Up Study of a Single Centre

Pons

Louro

Sitges

et al. 2023

JCM

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Background: Valve involvement is the most common cardiac manifestation in antiphospholipid syndrome (APS). The objective of the study was to describe the prevalence, clinical and laboratory features, and evolution of APS patients with heart valve involvement. Methods: A retrospective longitudinal and observational study of all APS patients followed by a single centre with at least one transthoracic echocardiographic study. Results: 144 APS patients, 72 (50%) of them with valvular involvement. Forty-eight (67%) had primary APS, and 22 (30%) were associated with systemic lupus erythematosus (SLE). Mitral valve thickening was the most frequent valve involvement present in 52 (72%) patients, followed by mitral regurgitation in 49 (68%), and tricuspid regurgitation in 29 (40%) patients. Female sex (83% vs. 64%; p = 0.013), arterial hypertension (47% vs. 29%; p = 0.025), arterial thrombosis at APS diagnosis (53% vs. 33%; p = 0.028), stroke (38% vs. 21%; p = 0.043), livedo reticularis (15% vs. 3%; p = 0.017), and lupus anticoagulant (83% vs. 65%; p = 0.021) were more prevalent in those with valvular involvement. Venous thrombosis was less frequent (32% vs. 50%; p = 0.042). The valve involvement group suffered from higher mortality (12% vs. 1%; p = 0.017). Most of these differences were maintained when we compared patients with moderate-to-severe valve involvement (n = 36) and those with no or mild involvement (n = 108). Conclusions: Heart valve disease is a frequent manifestation in our cohort of APS patients and is associated with demographic, clinical and laboratory features, and increased mortality. More studies are needed, but our results suggest that there may be a subgroup of APS patients with moderate-to-severe valve involvement with its own characteristics that differs from the rest of the patients with mild valve involvement or without valve involvement.

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An extraordinary case of recurrent stroke, disseminated thrombosis and endocarditis

Cited by 2 publications

References 26 publications

Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review

Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review

Heart Valve Involvement in Patients with Antiphospholipid Syndrome: A Long-Term Follow-Up Study of a Single Centre

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