Background/Purpose: Cardiac involvement in antiphospholipid syndrome (APS) includes valvular, coronary, and myocardial manifestations. Valvular involvement is the most frequent, affecting approximately 30% of patients, while myocardial infarction is the second leading cause of death in APS. Although a few studies have examined the progression of valvular disease, data on coronary and myocardial involvement are lacking. Furthermore, the prognostic implications of the different types of APS-related cardiac involvement have not been directly compared. This study aimed to evaluate the long-term prognosis associated with each type of cardiac involvement in APS.

Methods: We conducted a bicentric, retrospective observational cohort study in France, including patients followed at La Pitié-Salpêtrière Hospital (Paris) and Toulouse University Hospital. Patients met the 2023 APS classification criteria and were diagnosed between January 1, 2000, and July 1, 2023. The primary endpoint was the occurrence of a major APS-related complication, defined as death, catastrophic APS (CAPS), admission to intensive care, heart failure, myocardial infarction, or intra-alveolar hemorrhage. Overall survival and incidence of major complications were compared between groups using the log-rank test.

Results: A total of 138 patients were included, of whom 93 were women (67.4%), with a median age of 41 years (IQR 33–52). Cardiac involvement was the initial APS manifestation in 69 patients (50.0%). Compared with those with coronary or myocardial involvement, patients with valvular involvement more frequently had a history of stroke, livedo reticularis, and Sneddon syndrome. The median follow-up was 6 years (IQR 3–11). During follow-up, 89 patients (64.5%) experienced at least one APS-related complication. The probability of event-free survival was 70% at 1 year and 43% at 5 years. No significant differences in overall or event-free survival were observed across the different types of cardiac involvement. However, patients with myocardial involvement had a significantly higher incidence of arrhythmias (p = 0.050), intensive care unit admissions (p = 0.031), and hemorrhagic events (p = 0.046).

Conclusion: Cardiac involvement in APS is frequent and associated with a substantial risk of adverse outcomes over time. Although overall and event-free survival did not differ significantly across valvular, coronary, and myocardial subtypes, myocardial involvement was independently associated with a higher incidence of severe complications, including arrhythmias, intensive care unit admissions, and hemorrhagic events. These findings underscore the prognostic relevance of the type of cardiac involvement and support the need for stratified risk assessment and targeted management in patients with APS.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/prognosis-of-cardiac-involvement-in-antiphospholipid-syndrome/