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

Abstract

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.

Antiphospholipid Antibodies Are Major Risk Factors for Non-Thrombotic Cardiac Complications in Systemic Lupus Erythematosus

“…They also found that valvulopathies are more common in arterial hypertension and arterial thrombosis at the diagnosis of APS, as well as in livedo reticularis. They found no difference between the primary APS and secondary SLE-associated APS patients [ 30 ]. Djokovic and colleagues analyzed a total of 374 patients, out of whom 260 patients were diagnosed with primary APS and 114 patients had SLE-associated secondary APS.…”

Epidemiology of antiphospholipid syndrome: macro- and microvascular manifestations

“…Even though cardiac valve abnormalities can be detected by cardiac transthoracic or transoesophageal ultrasound in more than one-third of APS patients [ 33 , 95 ], overt thrombotic cardiac manifestations are relatively rare [ 51 ]. Myocardial infarction in APS is observed in ∼2–5.5% of patients [ 8 , 9 , 30 , 31 , 34–37 ], and often presents as the first manifestation of the disease (73%) [ 96 ].…”

Management of Cardiovascular Complications in Antiphospholipid Syndrome: A Narrative Review with a Focus on Older Adults

“…The mitral and aortic valves are most commonly affected [ 12 ]. Moreover, the available literature on heart valve surgery and prosthetic valve management in APS indicates that there is a heightened risk of morbidity and mortality, both in the short and long term [ 13 , 14 , 15 , 16 ]. Patients with APS also face a higher risk of coronary artery disease and left ventricular dysfunction, pulmonary thromboembolism, microvascular thrombosis, and the rupture of atherosclerotic plaques [ 17 , 18 ].…”