Eisenmenger syndrome: e.g. for elective inguinal hernia repair

Assessment:

Goals	· How bad is the disease (RV function? PA pressure?) · How stable is the disease? · How urgent is the surgery? · Where to anaesthetize? · How to anaesthetize?
History	· Hernia reducible? · NYHA · Severity and frequency of exacerbations · Medications · Last review -retrieve letter
Examination	· General inspection: cyanosis, cachexia, work of breathing · Degree of R-> L shunt: ∝ hypoxaemia · Signs of RV failure: ↑JVP, pitting oedema, hepatomegaly · Hernia: size? Reducible?
Investigations	· TTE: TAPSE, RVSP · Right heart cath: PASP, mPAP
Discussion	· Everyone: transfer to tertiary care? · Cardiologist: optimisation? Delay? · Surgeon: local only? · ICU: post-op admission?

Goals	· Avoid ↑R->L shunt = hypoxaemia · Avoid RV ischaemia · Avoid RV failure
Avoid GA	· Local only o Patient willing o Surgeon willing o Pathology amenable · CSE o Pressor infusion + euvolaemia o 1mL plain 0.5% bupivacaine into spinal o 5mL q10mins 2% lignocaine with adrenaline into epidural
Minimise shunt	↓PVR: · Physical o Lung volume at FRC o Avoid positive pressure o Avoid laparoscopy · Physiological: o ↑PaO2: O2 supp o ↓PaCO2: no sedation with local/neuraxial o ↔Temp: forced air warmer o ↔pH · Pharmacological: o Peri-op: PDEi, ETRA, CCB, PGI2 o If GA: propofol, sevoflurane, iNO (avoid N2O and ketamine) ↑SVR: · Physical: flex hips if dire · Pharmacological: euvolaemia + pressor + avoid too much anaesthetic
Avoid RV ischaemia	· ↑Perfusion pressure i.e. ↑mAP o Metaraminol vs noradrenaline · ↑Perfusion time = ↓heart rate = reflex suppression o If neuraxial: adequate block height o If GA: opioid
Avoid RV failure	· ↑↔Contractility: noradrenaline · ↔Heart rate: reflex suppression · ↓Afterload = ↓PVR (see above) · ↔Preload = euvolaemia