The antiphospholipid syndrome (APS) is an autoimmune disorder of acquired hypercoagulability characterized by vascular thrombosis, increased pregnancy morbidity, and elevated levels of antiphospholipid antibodies. Cardiac involvement in APS may be presented as heart valve disease affecting approximately a third of patients or less frequently as intracardial thombosis, pulmonary hypertension, right or left ventricular dysfunction, micro-vascular thrombosis, coronary artery, or micro-vascular disease with overt or silent clinical presentation.
Noninvasive cardiovascular imaging plays a crucial role in the evaluation of heart involvement in APS. Transthoracic or transoesophageal echocardiography enable early, accurate diagnosis and severity assessment of HVD as well as of ventricular dysfunction and pulmonary hypertension. Studies by echocardiography and nuclear imaging have detected abnormalities in myocardial perfusion in approximately 30% of primary APS. CT scan is the technique of choice for the assessment of pulmonary embolism and can effectively detect intracardiac thrombi. Myocardial perfusion defects have been detected by 13N-ammonia PET in 40% of APS. Cardiovascular magnetic resonance (CMR) has identified an unexpectedly high prevalence of occult myocardial scarring and endomyocardial fibrosis in APS, and is the technique of choice, if quantification of heart valve disease and stress myocardial perfusion-fibrosis is needed.
Noninvasive, nonradiating imaging techniques, such as echocardiography and CMR are superior to CT or nuclear techniques and are of great value for the diagnosis and follow-up of both clinically overt and silent cardiac disease in APS.
The high incidence of cardiac involvement in APS demands early diagnosis/treatment and multimodality cardiovascular imaging is of great importance.