Catastrophic antiphospholipid syndrome (CAPS) precipitated by cardiac surgery: A case report and review of the literature

Background: A devastating complication of antiphospholipid syndrome (APS) is catastrophic antiphospholipid syndrome (CAPS), which is extremely rare, difficult to diagnose, and portends a poor prognosis with high mortality. Intra-abdominal and obstetric surgeries have been regarded as precipitating factors in the development of CAPS in patients with pre-existing APS. We present the unique case of a patient who developed CAPS as an immediate complication after valvular replacement surgery. Case presentation: A 57-year-old female was admitted for acute decompensated heart failure secondary to mitral valve stenosis. Her past medical history was suspicious for APS, but she was not formally diagnosed or started on anticoagulant therapy. She ultimately underwent mitral valve replacement. One week post-operatively, she developed intractable abdominal pain, progressive renal failure and thrombocytopenia, and behavioral changes over the course of several days. Extensive investigation for infection, thrombocytopenic thrombotic purpura (TTP), heparin-induced thrombocytopenia (HIT) and disseminated intravascular coagulation (DIC) did not explain her rapid clinical deterioration. She did, however, fulfill the criteria for probable CAPS, given that she had rapid involvement of four organ systems and persistent antiphospholipid antibodies. She was started on both plasmapharesis and hemodialysis 12 days postoperatively, and showed significant improvement in her mentation, abdominal pain, thrombocytopenia, and renal function. Conclusion: This case illustrates the high degree of clinical suspicion and understanding of precipitating factors that are required to make a diagnosis of CAPS; furthermore, it delineates the life-saving impact of effective treatment. The correlation of CAPS and surgery has been observed in several reports. However, the current literature posits that CAPS is seldom a complication of cardiac surgery, and more frequently an immediate complication after abdominal or obstetric surgeries. For this reason, this case is novel in that it represents one of the few cases in which CAPS was triggered by cardiac surgery involving cardiopulmonary bypass (CPB).