ABSTRACT
Introduction: In Antiphospholipid Syndrome (APS) myocardial tissue can be involved through immune-mediated or thrombotic mechanisms, giving chest pain and increase of myocardial cytolysis markers. This may occur without any signs of myocardial injury at the moment of echocardiography, coronarography and cardiac magnetic resonance (CMR). The aim is to increase the awareness about this life-threating condition.Case summary. We present the case of 26-year-old woman few days after childbirth, affected by APS in anticoagulant therapy with previous deep vein thrombosis and without any other cardiovascular risk factors. She was symptomatic for intermittent chest-epigastric pain, fever and skin livedo reticularis. Lab Tests: TnI 750 ng/L, C-reactive protein 9.6 mg/dL, D-dimer 1693 μg/L;anemia;Antithyroid Antibodies and ANA 1:160 (SSB/LA). Blood cultures and COVID test were negative.Results. Echocardiography showed normal left/right ventricular function, but minimal pericardial effusion was present. Pulmonary Angio-CT revealed small thromboembolic event, ground-glass lungs compatible with hemorrhagic alveolitis.After a few days, the patient presented increased epigastric pain, headache, vomiting up to presenting a comatose state. Thrombotic or hemorrhagic events with cerebral CT and MR were excluded. Total-body CT was negative, except for peri-splenic and recto-uterine pouches.For the increase of TnI up to 4741 ng/L, the patient underwent coronary angiography which demonstrated non-obstructive coronary arteries. The assembled Neuro-Cardio-Rheumatology team suspected a rapidly developing Catastrophic APS which was developing quickly with multi-organ and life-threatening involvement.The patient underwent 4 cycles of plasmapheresis, intravenous human IgG and corticosteroids, with rapid clinical improvement. CMR subsequently demonstrated a small transmural late enhancement area on lateral left ventricle wall.The patient was discharged from the hospital on Day 6 post- therapies.Conclusions. APS may involve more organs, including myocardial tissue with different mechanisms of damage and high mortality rate. The presented case poses a multidisciplinary challenge, because thrombotic multiorgan microangiopathy may be not always diagnosed. Imaging methods such as CMR could be optimized with adenosine stress-CMR. Clinical attention is required among women with APS, to reach early diagnosis of myocardial thrombotic microangiopathy and to establish the best effective treatment.