Title : Catastrophic antiphospholipid syndrome: A case report
Abstract:
Introduction
Antiphospholipid syndrome (APS) is a disorder of autoimmune system presented clinically by arterial or venous thrombosis. One of the rare and severe forms of APS is known as catastrophic APS (CAPS). The incidence of CAPS has been reported in 0.8% of patients with APS. Case reports on cardiac involvement of CAPS including microvascular thrombosis, coronary artery disease or valvular thickening or lesions are limited. Here, we are reporting a case of CAPS with cardiac manifestation associated as a result of the reversal of anticoagulation.
Case summary
A 27-year-old lady with known primary APS presented with 4-week history of dizziness and heavy per vaginal (PV) bleeding. She was normally on Warfarin due to her previous history of deep vein thrombosis (DVT), pulmonary embolism (PE) and subendocardial myocardial infarction (MI). Blood tests were done which showed a haemoglobin (Hb) of 67 with an international normalised ratio (INR) of 4.3. Her warfarin was withheld, and she received 10mg intravenous Vitamin K to reverse her anticoagulation. After a few hours, she developed chest pain radiating to back and left arm, without ECG changes. Her high sensitivity troponin I showed significant rise. Serial bedside transthoracic echocardiograms (TTEs) showed worsening ejection fraction and global hypokinesia, and a subsequent cardiovascular magnetic resonance imaging (cMRI) scan confirmed multiple infarcts in all 3 coronary artery territories. A diagnosis of myocardial infarction due to catastrophic Antiphospholipid Antibody Syndrome was made. The patient was treated with Intravenous Immunoglobulin (IVIg) and Methylprednisolone, and her anticoagulation with Warfarin was reinstituted along with bridging Enoxaparin, aiming for a target INR higher than 2.5. She was subsequently transferred to a regional centre for treatment with Rituximab.
Conclusion
Reversal of anticoagulation in patient with known APS should be carried out cautiously after balancing the risks and benefits. A multidisciplinary approach should be considered. For patients with myocardial infarction secondary to CAPS, treatment with IVIg, IV plasmapheresis, and corticosteroids should be included.
Audience Take Away Notes
- To provide insight into a rare event of cardiac manifestation of CAPS following anticoagulation reversal
- To improve understanding of the CAPS, reduce the risk of similar events happening in the future, and optimise patient care.
- To act as a reflective opportunity for the case
