Peri-vascular adipose tissue attenuation on chest computed tomography in patients with Marfan Syndrome: a case series
Domenico Tuttolomondo1, Massimo De Filippo2, Daniele Sartorio3
1Department of Cardiac Surgery, Parma University Hospital, Via Gramsci 14, 43125, Parma, Italy.. d.tuttolomondo@hotmail.it.
Background And Aim Of The Work:
Marfan Syndrome is a genetic disorder that determines histopathological alterations of the aortic vascular wall leading to increased inflammatory component. The peri-vascular adipose tissue attenuation is a method able to capture localized vascular inflammation by mapping spatial changes of perivascular tissue attenuation on computed tomography.
Methods:
We measured peri-vascular adipose tissue attenuation around the ascending aorta in three consecutive subjects with confirmed genetic diagnosis of Marfan Syndrome. All subjects received the genetic diagnosis of fibrillin-1 gene mutation as part of the family screening of patients with known Marfan Syndrome. Chest computed tomography was performed in such asymptomatic subjects after genetic confirmation of Marfan Syndrome. None of these subjects showed aortic aneurysms or suffered from chronic inflammatory/infectious disease.
Results:
In the three subjects identified with Marfan Syndrome the value of aortic peri-vascular adipose tissue attenuation measured at chest computed tomography was higher than normal and the volume of aortic peri-vascular adipose tissue was lower.
Conclusion:
These preliminary observations suggest that peri-vascular adipose tissue attenuation is unexpectedly high in patients with Marfan Syndrome, notwithstanding the normal aortic diameter at the time of computed tomography. Whether this observation may find a clinical use in suspected Marfan Syndrome or in predicting aortic complications in Marfan Syndrome is worth to be assessed in prospective studies.
Insights
Perivascular adipose tissue attenuation is elevated in Marfan Syndrome patients, even with normal aortic size. This imaging finding may help detect vascular inflammation in Marfan Syndrome (MFS).
Area of Science:
- Cardiovascular Imaging
- Genetics
- Vascular Biology
Background:
- Marfan Syndrome is a genetic disorder causing aortic wall changes and inflammation.
- Perivascular adipose tissue (PVAT) attenuation on CT can detect localized vascular inflammation.
Purpose of the Study:
- To assess PVAT attenuation around the aorta in asymptomatic Marfan Syndrome patients.
- To explore PVAT attenuation as a potential imaging biomarker for Marfan Syndrome.
Main Methods:
- Computed tomography (CT) was used to measure PVAT attenuation and volume in three Marfan Syndrome patients.
- Patients had confirmed fibrillin-1 gene mutations and no aortic aneurysms or chronic inflammatory diseases.
Main Results:
- Aortic PVAT attenuation was higher than normal in all three Marfan Syndrome subjects.
- The volume of aortic PVAT was reduced in these patients.
Conclusions:
- Elevated aortic PVAT attenuation in Marfan Syndrome suggests underlying vascular inflammation.
- This finding warrants further investigation for clinical utility in Marfan Syndrome diagnosis and complication prediction.
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