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Non-valvular cardiac disease in the antiphospholipid syndrome (APS) has been scanty studied. We wanted to assess the prevalence and evolution of left myocardial disease, pulmonary hypertension and intracardiac thrombi in a cohort of APS patients.A total of 53 patients with APS, either primary (n = 34, 64%) or associated to lupus (n = 19, 36%) and 20 controls were included. Initial transthoracic echocardiography assessment was performed in patients at diagnosis, with echocardiography controls performed along mean follow-up of 12 years. Prevalence of myocardial disease in APS cohort was assessed taking into account primary risk factors (hemodynamically significant valvular disease, systemic hypertension, diabetes, alcoholism, myocardial infarction or myocarditis), the same as for pulmonary hypertension (severe left ventricular dysfunction or chronic lung disease).Left myocardial disease had a prevalence of 3.8% (2/53 patients), not different from controls (P = 0.12). Both patients had diastolic dysfunction grade I that maintained stability throughout echocardiographic follow-up period. Pulmonary hypertension had a prevalence of 11.3% (6/53 patients), not different from controls (P = 0.12); all cases were related to pulmonary embolism. Patients diagnosed with pulmonary hypertension in baseline maintained stable pressures throughout follow-up in the absence of new thrombosis. Intracardiac thrombi had a prevalence of 1.8% (1/53 patients), not different from controls (P = 0.4), without changes along echocardiographic follow-up.Pulmonary hypertension is the most prevalent non-valvular cardiac manifestation in APS, with an evolution associated with thromboembolic disease, while left myocardial disease and intracardiac thrombi would be rare manifestations in APS.