Dados do Trabalho

Title

CARDIAC INVOLVEMENT AS NON-CRITERIA MANIFESTATION OF ANTIPHOSPHOLIPID SYNDROME: TWO CASE REPORT OF VALVULOPATHIES.

Background

The antiphospholipid syndrome (APS) is defined by thrombotic or obstetrical events in patients with antiphospholipid antibodies (aPLs) persistently positive. The most frequently aPLs are lupus anticoagulant (LA), anticardiolipin antibodies (aCL), and anti-β2-glycoprotein I (β2 GPI) antibodies. APS occurs as a primary disorder (PAPS) or as a secondary manifestation to another autoimmune disease (SAPS), most commonly systemic lupus erythematosus (SLE). Due to its vascular nature, various organs and tissues may be affected, including the cardiac system. The heart can be involved through immune mediated and /or thrombotic mechanisms. Mortality due to cardiovascular problems is elevated in APS. However, the cardiovascular risk in patients with PAPS compared with SLE-APS is yet to be established. Cardiac manifestations in APS include valve abnormalities, occlusive arterial disease (atherosclerosis and myocardial infarction), intracardiac emboli, ventricular dysfunction, and pulmonary hypertension. The present work aims to report two cases of APS with different valvulopathies complications.

Case report

Case1: CCQ, female, 40 years old, no pregnancies before, only hypothyroidism (Hashimoto disease) as comorbidity. This double-positive aPL (ACL and LA) patient presented recurrent ischemic strokes and persistent livedo reticularis. As a complementary imaging strategy, transthoracic echocardiography (TTE) was performed and showed aortic and tricuspid valves with thickening and vegetations, suggesting Libman-Sacks endocarditis. The treatment consisted in oral anticoagulation, statin and aspirin. There were no relevant changes in the follow-up images, despite our therapeutic.

Case2: JASJ, male, 33 years old, diagnosis of SLE in 2008 with nephritis and skin lesion predominantly. He presented posteriorly (in 2013) involuntary movements which were classified as disease activity in the CNS. In 2018 the patient had an ischemic stroke, preceded by a thickening and thrombosis in the right anterior tibial artery. In the TTE a double lesion of the aortic valve with no systemic repercussions was found. Important laboratory results were ANA 1/160 nuclear homogeneous and a triple-positive aPL antibodies. After the event, the chosen treatment was oral anticoagulation, aspirin, statin and hydroxychloroquine.

Conclusion

Clinicians should bear in mind that heart valve lesions are the most common cardiac manifestation, observed in approximately one third of PAPS patients and usually do not cause hemodynamic significance. Valve involvement include valvular thickening, valve nodules (also referred to as Libman-Sacks endocarditis) and valvular regurgitation/stenosis. To date, a classification system that can distinguish the cardiac manifestations in PAPS from SAPS has not yet been established. We recommend performing TTE in every APS patient as an initial evaluation.

Área

Antiphospholipid syndrome