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

Abstract

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 andobstetric surgeries have been regarded as precipitating factors in the development of CAPS in patients with pre-existing APS. Wepresent 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 forinfection, thrombocytopenic thrombotic purpura (TTP), heparin-induced thrombocytopenia (HIT) and disseminated intravascularcoagulation (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 bothplasmapharesis 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 requiredto make a diagnosis of CAPS; furthermore, it delineates the life-saving impact of effective treatment. The correlation of CAPSand surgery has been observed in several reports. However, the current literature posits that CAPS is seldom a complication ofcardiac surgery, and more frequently an immediate complication after abdominal or obstetric surgeries. For this reason, this caseis novel in that it represents one of the few cases in which CAPS was triggered by cardiac surgery involving cardiopulmonarybypass (CPB).