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Published on: February 11, 2022
Reversible cardiac valvular disease in catastrophic antiphospholipid syndrome
C C Teunisse1, A J Kalsbeek, S T de Vries
1Department of Cardiology, Isala Klinieken, Zwolle, the Netherlands. c.c.teunisse@isala.nl
Insights
Catastrophic antiphospholipid syndrome (CAPS) can cause severe heart problems. Treatment led to reversible valve issues in two patients, avoiding surgery.
Area of Science:
- Cardiology
- Rheumatology
- Internal Medicine
Background:
- Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening condition with high mortality.
- Cardiac involvement is common in CAPS, affecting approximately 50% of patients.
- Previous reports described valve thickening and vegetations in CAPS-related cardiac issues.
Observation:
- Two female patients with CAPS presented with severe cardiac manifestations.
- Echocardiography documented significant valvular regurgitation in both cases.
- These cardiac issues were associated with papillary muscle dysfunction and myocardial stunning.
Findings:
- Both patients experienced regression of valvular regurgitation with medical treatment.
- Valve replacement therapy was successfully avoided in these cases.
- This suggests a potential for reversibility in CAPS-associated cardiac valve dysfunction.
Implications:
- Microvascular disturbances may underlie reversible valvular regurgitation in CAPS.
- Understanding these mechanisms can refine treatment strategies for cardiac manifestations.
- This highlights the importance of echocardiographic monitoring and tailored medical management in CAPS patients.
Abstract:
Catastrophic antiphospholipid syndrome (CAPS) is a severe form of antiphospholipid syndrome (APS). It frequently leads to multiorgan failure with an approximate mortality rate of 50%. The heart is involved in about 50% of the patients with CAPS. We report two cases with CAPS and severe heart manifestations, documented by echocardiography. Both women show regression of the valvular regurgitation under treatment. Valve replacement therapy was no longer necessary. In earlier studies and case reports, cardiac valve involvement had been characterised by valve thickening and vegetations. We suppose that (sometimes reversible) microvascular disturbances lead to valvular regurgitation via papillary muscle dysfunction and myocardial stunning.
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