Anesthesia Considerations 

Marfan's Syndrome

 

 

Considerations

 

• Airway problems:

  • Possibly difficult: high arched palate 

  • Potential cervical spine (C1/2) ligamentous instability

  • TMJ laxity & potential dislocation with laryngoscopy 

• Multisystem disease:

  • Cardiovascular dysfunction

    • Valvular disease (AI, MR, MVP)

    • Aortic arch aneurysm, aortic rupture & dissection risk 

    • MIs secondary to medial necrosis of the coronary arterioles

    • Arrhythmias & conduction defects

  • Respiratory dysfunction:

    • Scoliosis, pectus carinatum/excavatum & restrictive lung disease, pulmonary hypertension, cor pulmonale

    • Spontaneous pneumothorax (bullous lung disease), emphysema 

  • Ocular: lens dislocation, retinal detachement, glaucoma

• Potentially difficult positioning & regional anesthesia

  • Rule out dural ectasia 

 

 

Goals

 

• Minimize ↑ in aortic wall tension through avoidance of sustained ↑ in systolic BP 

• Establish airway with minimal c-spine movement

• Maintain hemodynamic goals of associated valvular lesions

• Lung protective ventilation considering restrictive lung disease & potential bullae

• Careful positioning (lax joints & potential peripheral nerve injury)

• Post-op pain (neuraxial or regional preferrably), post-op disposition

• These patients are for elective aortic repair when ≥ 5cm 

 

 

Potential conflicts

 

• Coexisting aortic root dilation (need to reduce cardiac output) vs MR/AI/LV dysfunction

 

 

Pregnancy Considerations  

 

• If ∅ symptoms & aorta diameter < 4cm → no special considerations & vaginal delivery ok 

• If aortic root dilatation/AI → multidisciplinary management with cardiology/cardiac surgery/obstetrics 

• Some authorities recommend cesarean section for aortic diameter > 4.5cm, labor if > 4 & < 4.5cm

• Issues: 

  • Airway might be even more difficult 

  • Neuraxial very good option for vaginal delivery & cesarean section  

  • Aortic dilatation with risk dissection/rupture 

  • Monthly echocardiography during pregnancy 

  • Big focus is to reduce shear forces on aorta 

  • Consider very early epidural 

  • Need invasive monitoring

  • Drug therapy to prevent tachycardia & elevated BP (keep systolic < 120mmHg) = labetalol good agent

  • Avoid ergotamine due to hypertension risk 

• Dural ectasia: 

  • NOT an absolute contraindication to epidural placement but higher risk for failed block & dural puncture & PDPH

  • Widening of the dural sac, asymptomatic or may present with low back pain, headache, or proximal leg pain, weakness, or numbness

  • Consider CT/MRI