01 / Management of a patient with Eisenmenger Syndrome requiring emergency laparotomy in a District General Hospital-A case report

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Management of a patient with Eisenmenger Syndrome requiring emergency laparotomy in a District General Hospital-A case reportA. Rivers1, N. Ijaz2, M. Raza3, S. J. Wort41Consultant Anaesthesia,Luton&Dunstable Hospital - Luton (United Kingdom), 2Locum Consultant Anaesthesia,Luton&Dunstable Hospital - Luton (United Kingdom), 3Consultant Anaesthesia&Critical Care,Luton&Dunstable Hospital - Luton (United Kingdom), 4Consultant Pulmonary Hypertension,Royal Brompton Hospital - London (United Kingdom)BackgroundEisenmenger syndrome is a cyanotic heart condition where long standing left-to-right shunt causes pulmonary hypertension,pulmonary vascular remodelling,right ventricular hypertrophy and ultimately reversal of shunt1.Case Report:Male,35years,presented with incarcerated umbilical hernia.History included Down syndrome,complete AV septal defect,established Eisenmenger syndrome,obesity, obstructive sleep apnoea requiring CPAP and home oxygen.His longstanding hernia was refused elective repair owing to high perioperative risks.His vitals were stable.CT confirmed a large anterior abdominal wall hernia containing bowel loop which appeared compromised.Echocardiogram showed severe pulmonary hypertension(90mmHg).We involved multidisciplinary teams including specialist from tertiary centre for guidance since the surgery was deemed urgent to prevent bowel infarction and transfer to a tertiary centre was not a safe option.Pre-induction noradrenaline and milrinone was started.Modified rapid sequence induction was done with fentanyl 200 mcg, ketamine 100 mg,rocuronium 70 mg and anaesthesia was maintained with sevoflurane in oxygen.Surgery took 90 minutes and the incarcerated bowel was reperfused successfully.At the end of surgery patient deteriorated with SpO2 55% and BP of 50/30.We used a combination of noradrenaline, adrenaline, milrinone, as well as selective pulmonary vasodilators sildenafil and nebulised iloprost, to rebalance his pulmonary and systemic circulations.He then developed a narrow complex tachycardia with multiple ectopics.He was transferred to ICU where he was maintained on vasopressin and milrinone infusion while we weaned off noradrenaline.Iloprost and sildenafil were continued in the postop period.He was extubated after 2 days and was discharged after a week.DISCUSSIONS:Eisenmenger physiology necessitates maintaining a balance between systemic and pulmonary circulations with respective vascular resistances determining flow across the shunt.We used pulmonary vasodilators including Sildenafil and iloprost to maitain this balance.Ketamine helped defend the SVR in face of severe pulmonary hypertension.LEARNING POINTS:Efficient management of patient with cyanotic heart conditions like Eisenmenger, presenting for emergency surgery in non-specialist hospital implicates continuing input from patient's specialist team and early involvement of pharmacist for procurement of specific drugs.REFERENCES:1)Intensive Care Medicine 2011;37:502-9.

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