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Giant Aortic Root Aneurysm in Marfan's Syndrome.
Dinkar Bhasin, Gaurav Kumar Arora, Sourabh Agstam
1Department of Cardiology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi-110029, India. drhsisser@gmail.com.
The Journal of Invasive Cardiology
|March 1, 2021
Summary
Marfan syndrome can cause aortic root and ascending aorta dilation, leading to symptoms like palpitations. Early diagnosis and surgical intervention, such as the Bentall procedure, are crucial for managing this serious connective tissue disorder.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Connective Tissue Diseases
Background:
- Marfan syndrome is an autosomal-dominant inherited connective tissue disorder.
- It predisposes patients to progressive aortic root and ascending aorta dilation.
- Cardiovascular complications are a major cause of morbidity and mortality in Marfan syndrome.
Observation:
- A 25-year-old man presented with exertional palpitations and dyspnea.
- Skeletal features suggestive of Marfan syndrome were noted.
- Contrast-enhanced computed tomography revealed aneurysmal dilation of the aortic root and ascending aorta.
Findings:
- The patient's condition necessitated a Bentall procedure for aortic valve, aortic root, and ascending aorta replacement.
- This case highlights the severe cardiovascular manifestations of Marfan syndrome.
Implications:
- Early diagnosis and regular echocardiographic monitoring are essential for patients with Marfan syndrome.
- Surgical intervention, like the Bentall procedure, can effectively manage aortic dilation.
- Understanding the genetic basis and clinical progression of Marfan syndrome is vital for patient care.
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