Pregnant patient with pulmonary embolism

PIG Meeting: 25th February 2021

34yo patient for elective CS, complex chronic and acute health problems.

Background

  • Ehlers Danlos Syndrome
    • Hypermobility type
    • ‘Marfans features’ (but not Marfans) with lens dislocation, high arched palate, spontaneous L PTx and known small bullae on R.
    • Bowel and gastric stasis requiring caecostomy.
  • Central and obstructive OSA – adherent to CPAP
  • BMI 38
  • Uveitis HLAB27 +
  • Raynaud’s phenomenon
  • Pregnancy – G1P0, nil foetal complications.

Issues

  •  PE – clinical symptoms + V/Q mismatch. On therapeutic clexane since ~10/7
  • ? Risk of aortic root aneurysm
    • Somewhat unusual picture. Risk difficult to quantify
    • Minimal harm from TTE (n.b. normal aortic root on subsequent TTE).
  • Risk of local anaesthetic failure
    • Patient has previously had some failed peripheral nerve blocks but successful rescue blocks

Discussion

  • What is Ehlers Danlos Syndrome?
    • The most common group of disorders in the family of genetically determined heritable connective tissue disorders.
    • Ligament laxity, joint hypermobility, fragility of connective tissues, poor wound healing.
    • Multiple subtypes with cardiac, GIT, autonomic and chronic pain issues.
  • Anaesthetic technique
    • Morphine adverse drug reaction (rash).
    • Neuraxial ideal. Consider CSE as colorectal team on standby given previous bowel surgery.
    • TAP catheters useful to avoid opioid exacerbation of gut stasis postop + risks of resp depression with opioids. Some reports of ineffective or partially effective LAs however not contraindicated and may provide substantial benefit.
    • If GA required, lung protective ventilation to minimise risk of bullae rupture. 
    • Note that Ehlers Danlos patients also have increased risk of bleeding post-operatively
  • Postoperative location (? Delivery suite, ? K2)
    • High VTE risk postop however this risk continues for weeks so no specific observation needed.
    • OSA well optimised
    • Normal care on K2 reasonable
  • Anticoagulation management
    • Discussed ? Need for conversion to heparin given known PE, to minimise time off anticoagulation
    • Overall thought that 24h off clexane reasonable and the logistics in starting/stopping heparin make it of limited value for this short period of time.

Pregnant patient with mitochondrial degenerative disorder

PIG Meeting: 25th February 2021

34yo lady booked for elective CS.

Background

  • Mitochondrial degenerative disorder POLG (DNA Polymerase gamma gene – responsible for the integrity of mitochondrial DNA)
    • OSA – Central and peripheral
    • Bulbar dysfunction
    • Ophthalmoplegia
    • Ataxia, pre-pregnancy 2km with FASF, now mostly in wheelchair. Falls
    • Epileptiform EEG
  • Obesity BMI 38
  • Pregnancy:
    • G1PO
    • Pregnancy itself uneventful.
    • Nil genetic testing of partner or foetus
    • On prophylactic clexane due to immobility
  • Distance patient (Tamworth)

Issues

  • Exercise tolerance reduction
    • TTE in December normal, but known risk of cardiac decompensation
    • Fatigue and SOB increasing. Progressive orthopnoea + possible PND. ? Pregnancy v. Pathologic process
    • Risk of cardiac dysrhythmias. Nil reported palpitations
    • Hasn’t seen cardiologist as part of antenatal care
    • Spirometry in clinic showed normal volumes
  • ? Seizures
    • Recent episodes of 50-60 desaturation events overnight. ? CPAP malfunction v. CCF v. seizures
    • Known epileptiform EEG.
    • Risks of rapid progression from partial seizures to refractory, fatal seizures with POLG
    • Lamotrigine being empirically up-titrated by neurologist
    • Ambulatory EEG cancelled by patient due to logistic challenges and lack of understanding about significance
    • Sodium valproate and MgSO4 contraindicated.
  • CPAP use
    • Machine alarming ? Cause
    • Patient now non-adherent

Discussion

  • Pre-op investigations
    • Baseline lactate (in anaesthetic bay) helps team to understand intra/postoperative levels
    • Baseline CK, CMP
  • Preop multidisciplinary reviews
    • Needs input from cardiology – speak with the cardiologist (Hatton) who works with high risk obstetric patients.
    • Needs CPAP review and optimisation
    • Needs ambulatory EEG
  • Post op care location
    • Given risks of refractory seizures, and need for repeated lactate monitoring and strict fluid balance, ICU level 2 necessary
    • Patient accepting of this.
    • Father of baby will be admitted to care for baby.
  • Anaesthetic technique
    • Neuraxial
      • Nil specific contraindications.
      • Spinous processes palpable and patient’s body habitus relatively conducive to positioning.
      • May require some head-up positioning. Negotiate with surgeons.
      • IT morphine(+/-) and TAP catheters to minimise postop systemic opioids to avoid gut stasis (known issue with POLG) and hypoventilation
      • Patient prefers SAB but happy to go with safest option
      • EDB (without spinal) for slow onset (avoid sudden resp accessory muscle paralysis) ideal.
      • Intrathecal catheter also an option
    • GA?
      • No MH risk
      • Non-depolarising MRs safe
      • Attention to vent/CO2 mx given seizure propensity
      • Difficult to detect seizures
    • Dedicated obstetric anaesthetist
    • IVF with dextrose while fasting

Update on pregnant patient with mitochondrial degenerative disease

PIG Meeting: 4th March 2021

34yo lady booked for elective CS.

Background

  • Mitochondrial degenerative disorder POLG
    • OSA – Central and peripheral
    • Bulbar dysfunction
    • Ophthalmoplegia
    • Ataxia, pre-pregnancy 2km with FASF, now mostly in wheelchair. Falls
    • Epileptiform EEG
  • Obesity BMI 38
  • Pregnancy:
    • G1PO
    • Pregnancy itself uneventful.
    • Nil genetic testing of partner or foetus (for mitochondrial issues or for CF known to exist in partner’s family)
    • On prophylactic clexane due to immobility
  • Distance patient (Tamworth)

Issues

  • Multidisciplinary involvement needed
    • Cardiology – TTE and Holter monitor required due to symptoms of possible decompensated heart failure, and the risk of cardiomyopathy and dysrhythmias associated with this condition.
    • Neurology – ambulatory EEG required due to recent symptoms possibly due to nocturnal seizures and the risk of refractory, fatal seizures with this condition
    • Resp –  CPAP manlfunctioning
  • Concern that this patient required high level multidisciplinary care which is impossible to access from her distant location. Also mindful of the risk of further decompensation of her medical issues or an obstetric issue necessitating delivery in Tamworth, where the outcome may be poor for both mother and foetus.
  • Suggested to obstetric team that this patient be admitted to enable all reviews and investigations, and then remain an inpatient with ICU level 2 after the delivery.