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Published on: August 18, 2015
Cardiac thrombus with risk of fulminant pulmonary embolism in paediatric antiphospholipid syndrome
M Albinski1, M Hufnagel2, J Schelling2
1Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Freiburg, Freiburg.
Insights
Antiphospholipid Syndrome (APS) can cause dangerous blood clots in children. A rare case highlights a 15-year-old with deep vein thrombosis and a tumor that was actually a thrombus, leading to a full recovery.
Area of Science:
- Cardiology
- Hematology
- Immunology
Background:
- Antiphospholipid Syndrome (APS) is a systemic autoimmune disorder characterized by autoantibodies targeting membrane components.
- APS involves coagulation pathways, complement factors, and immune cells, leading to thrombosis in various blood vessels.
- Clinical manifestations of APS are diverse, depending on the affected organ, with limited data available for pediatric cases.
Observation:
- A 15-year-old patient presented with deep vein thrombosis and a right ventricular tumor.
- The tumor posed a significant risk of a life-threatening pulmonary embolism.
- Surgical removal of the mass was performed.
Findings:
- Histological examination confirmed the right ventricular mass to be a thrombus.
- The patient experienced a complete recovery following surgical intervention.
- Long-term anticoagulation therapy was initiated for the patient.
Implications:
- This case underscores the importance of considering thrombotic events in pediatric patients presenting with unusual masses.
- Early diagnosis and appropriate management, including surgical removal and anticoagulation, are crucial for favorable outcomes in pediatric APS.
- Further research into pediatric APS is warranted to improve understanding and treatment strategies.
Abstract:
Antiphospholipid Syndrome (APS) describes a systemic disease caused by autoantibodies to membrane components. Involving coagulation pathways, complement factors and immune cells, it results in thrombosis in any blood vessel. Its clinical presentation varies considerably depending upon the organ affected. Paediatric data on APS remain sparse. Most case reports focus on catastrophic APS with multiple small-vessel occlusions and a life-threatening course. Here, we report on a 15-year-old patient with deep vein thrombosis and a right ventricular tumour posing the risk of a fulminant pulmonary embolism. The tumour was surgically removed. Histology revealed it to be a thrombus. The patient fully recovered and is currently treated with long term anticoagulation.
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