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Is Marfan Syndrome Associated with Primary Structural Changes in the Left Atrium?
Kun Zhang1,2, Lucas Ernst1, Isabel Schobert3
1Department of Internal Medicine and Cardiology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt Universität zu Berlin, and Berlin Institute of Health, 10117 Berlin, Germany.
Diagnostics (Basel, Switzerland)
|October 28, 2023
Summary
Marfan syndrome (MFS) primarily affects the left atrium (LA), causing increased size and activity. These changes in MFS patients are not solely due to left ventricular issues, suggesting a primary cardiac pathology.
Area of Science:
- Cardiology
- Genetics
- Connective Tissue Diseases
Background:
- Marfan syndrome (MFS) is a genetic disorder affecting connective tissues, commonly impacting the heart.
- Understanding cardiac involvement, particularly left atrial (LA) function, is crucial for managing MFS patients.
Purpose of the Study:
- To investigate cardiac function, with a specific focus on the left atrium (LA), in adult Marfan syndrome patients.
- To compare cardiac parameters between MFS patients and a healthy control group, and to assess the impact of prior aortic surgery.
Main Methods:
- A cohort of 231 adult MFS patients and 106 healthy controls underwent transthoracic echocardiography.
- Cardiac function, LA size, and NT-proBNP levels were assessed; genetic testing for FBN1 mutations was performed in most patients.
Main Results:
- Left ventricular function was normal in MFS patients.
- Increased LA size and NT-proBNP levels were observed in MFS patients, even with normal left ventricular filling pressures.
- These LA changes were more pronounced in patients with a history of aortic surgery; right heart changes were also noted post-surgery.
Conclusions:
- Marfan syndrome leads to structural alterations in the left atrium independent of left ventricular dysfunction.
- These findings suggest that left atrial changes may represent a primary pathology in Marfan syndrome.
- Further research into the mechanisms of primary atrial pathology in MFS is warranted.
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