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Cardiovascular disease of connective tissue origin
Insights
Inherited connective tissue disorders, like Marfan and Ehlers-Danlos syndromes, significantly impact cardiovascular health. This review details these cardiovascular features, aiding diagnosis and treatment strategies.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Rheumatology
Background:
- Inherited connective tissue disorders frequently manifest with cardiovascular complications.
- Prominent examples include Marfan syndrome and Ehlers-Danlos syndrome, known for significant cardiovascular involvement.
- Connective tissue abnormalities can also present in isolation, such as isolated valvular prolapse and anuloaortic ectasia.
Purpose of the Study:
- To review the cardiovascular manifestations of inherited connective tissue abnormalities.
- To discuss diagnostic and therapeutic approaches for these conditions.
- To provide a classification framework for inherited cardiovascular connective tissue disorders.
Main Methods:
- Literature review of inherited connective tissue disorders and their cardiovascular impact.
- Synthesis of information on diagnosis and treatment strategies.
- Analysis of classification systems for these disorders.
Main Results:
- Cardiovascular abnormalities are a common feature of inherited connective tissue disorders.
- Marfan syndrome and Ehlers-Danlos syndrome exemplify prominent heritable conditions with cardiovascular effects.
- Isolated valvular prolapse and anuloaortic ectasia represent connective tissue abnormalities without distinct syndrome features.
Conclusions:
- Understanding the spectrum of cardiovascular involvement in connective tissue disorders is crucial.
- Effective diagnosis and management require a comprehensive approach to these heritable conditions.
- A clear classification aids in the study and treatment of inherited cardiovascular connective tissue abnormalities.
Abstract:
Inherited abnormalities of connective tissue elements often cause changes in the structure and function of the cardiovascular system. Well-known heritable disorders of connective tissue in which cardiovascular abnormalities are prominent include the Marfan syndrome and the Ehlers-Danlos syndrome. Connective tissue abnormalities also occur without the associated features of a recognized syndrome. These include isolated valvular prolapse and anuloaortic ectasia. In this review, the cardiovascular features of connective tissue abnormalities--both the recognized syndromes and the isolated abnormalities--are described, important concepts in the diagnosis and treatment of these disorders are reviewed, and the classification of inherited connective tissue abnormalities of the cardiovascular system is discussed.