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Rheumatic Heart Valve Disease Pathophysiology and Underlying Mechanisms
Livia S A Passos1, Maria Carmo P Nunes2, Elena Aikawa1,3
1The Center for Excellence in Vascular Biology, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, United States.
Frontiers in Cardiovascular Medicine
|February 4, 2021
Summary
Rheumatic heart valve disease (RHVD) stems from an abnormal immune response to streptococcal pharyngitis, causing valve damage. Understanding RHVD
Area of Science:
- Immunology
- Cardiovascular Medicine
- Rheumatology
Background:
- Rheumatic heart valve disease (RHVD) is a significant cause of cardiovascular mortality in young individuals, particularly women in low- and middle-income countries.
- It results from an aberrant immune response to streptococcal pharyngitis, leading to chronic inflammation, fibrosis, and valve dysfunction.
- Despite decades of research, the precise mechanisms of self-tolerance failure and the interplay of immune responses in RHVD remain poorly understood.
Purpose of the Study:
- To review the current understanding of RHVD pathophysiology.
- To explore the underlying immune mechanisms contributing to valvular damage.
- To highlight knowledge gaps regarding self-tolerance breakdown and immune cell involvement.
Main Methods:
- This review synthesizes existing literature on RHVD pathogenesis.
- It analyzes proposed pathological mechanisms and their limitations.
- Focuses on the immune response following streptococcal infections.
Main Results:
- RHVD involves long-term inflammation and fibrosis, disrupting valve structure and function.
- The exact failure of self-tolerance and the connection between humoral and cellular immunity are not fully elucidated.
- Existing models do not adequately explain the mitral valve's preferential involvement.
Conclusions:
- Further research is critical to unravel the complex immune pathways in RHVD.
- Understanding these mechanisms is essential for developing targeted therapies.
- Addressing the lack of investment in RHVD research is crucial for advancing patient care.
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