Catastrophic antiphospholipid antibody syndrome associated with ischaemic cardiomyopathy

Area of Science:

• Cardiology
• Rheumatology
• Vascular Medicine

Background:

• Antiphospholipid antibody syndrome (APS) is a rare autoimmune disorder associated with blood clots.
• Cardiac involvement in APS, particularly ischemic cardiomyopathy, is uncommon, especially in adolescents.
• Pulmonary emboli can lead to significant cardiovascular compromise.

Purpose of the Study:

• To report a rare case of catastrophic APS-induced ischemic cardiomyopathy in a young female.
• To highlight the diagnostic challenges and clinical presentation of this severe condition.
• To emphasize the association between APS, cardiac dysfunction, and connective tissue disease.

Main Methods:

• Case report of a 16-year-old female.
• Review of clinical presentation, including cardiogenic shock and bilateral pulmonary emboli.
• Cardiac MRI findings suggestive of microvascular coronary injury.
• Diagnostic workup for autoimmune and thrombotic disorders.

Main Results:

• The patient presented with cardiogenic shock and biventricular dysfunction.
• Cardiac MRI revealed findings concerning for microvascular coronary injury.
• The final diagnosis was catastrophic antiphospholipid antibody syndrome-induced ischemic cardiomyopathy.
• An underlying autoimmune connective tissue disease was suspected.

Conclusions:

• Catastrophic APS can manifest as severe ischemic cardiomyopathy, even in young patients.
• Early recognition and management of APS are crucial to prevent life-threatening cardiac events.
• This case underscores the importance of considering APS in patients with unexplained cardiac dysfunction and thrombotic events.