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