Anaesthesia and Marfan's syndrome: case report

Area of Science:

• Cardiology
• Pulmonology
• Genetics

Background:

• Marfan syndrome is a genetic disorder affecting connective tissue, leading to cardiovascular, skeletal, and ocular complications.
• Ascending aortic dissection is a life-threatening cardiovascular complication common in Marfan syndrome.
• Patients with Marfan syndrome often require elective surgeries for various associated conditions.

Observation:

• Pulmonary issues in Marfan syndrome include lung function restriction from pectus excavatum/kyphoscoliosis and intrinsic lung diseases like emphysema.
• Spontaneous pneumothorax is a significant risk, exacerbated by positive pressure ventilation.
• Cardiovascular complications, particularly aortic dissection and rupture, are the leading causes of sudden death.

Findings:

• Preoperative echocardiography is essential to assess aortic root size.
• Anesthetic techniques should aim to reduce myocardial contractility and prevent sudden increases.
• Careful patient assessment and tailored anesthetic management are crucial for surgical safety.

Implications:

• Optimized preoperative evaluation and anesthetic strategies can mitigate surgical risks in Marfan syndrome patients.
• Early detection and management of cardiovascular and pulmonary complications are vital for improving patient outcomes.
• This case highlights the importance of a multidisciplinary approach in managing Marfan syndrome patients undergoing surgery.