SummaryWe describe the peri-operative care of a patient with Eisenmenger's syndrome presenting for laparotomy. These patients require techniques to prevent the potential increase in intracardiac shunt caused by anaesthesia, by minimising increases in pulmonary artery pressure and reductions in systemic vascular resistance. The successful use of combined epidural and general anaesthesia with elective use of inhaled nitric oxide as a pulmonary vasodilator, and intra-operative trans-oesophageal echocardiography is described. In 1897, Eisenmenger described pulmonary vascular pathology related to a ventricular septal defect with right ventricular hypertrophy [1]. In 1958, the term Eisenmenger complex was used to describe pulmonary hypertension as a consequence of raised pulmonary vascular resistance (over 800 dyne.s )1 .cm) with reversed or bidirectional shunting through a large ventricular septal defect [2]. The term Eisenmenger's syndrome is applied where communication exists between the pulmonary and systemic circulations, with resultant pulmonary vascular pathology and cyanosis as a consequence of a reversed or bidirectional shunt [2,3], the shunt position being immaterial [2].With advances in medical care, patients with Eisenmenger's syndrome are surviving well into adulthood and are more likely to require non-cardiac surgery [4]. In this group of patients, the leading cause of peri-operative death relates to cardiac morbidity associated with alteration of the ratio of pulmonary to systemic pressures secondary to surgery and anaesthesia. Mortality rates of up to 30% are frequently quoted for this group of patients undergoing non-cardiac surgery [4].
Case reportA 43-year-old woman with Crohn's disease presented with repeated progressive episodes of bowel obstruction, secondary to a terminal ileal stricture. At 27 years of age, following the birth of her fourth child, a diagnosis of pulmonary hypertension secondary to an atrial septal defect was made, which progressed to Eisenmenger's syndrome over the next 5 years.History revealed a patient with only mild symptoms of dyspnoea on reaching the top of a flight of stairs, but who continued to smoke cigarettes, with a 20 pack year history. Daily medications were warfarin, inhaled steroid and b 2 agonist, with nicardipine for treatment of pulmonary hypertension. She was taking no medications for Crohn's disease.On examination, she weighed 45 kg, had finger clubbing, a loud second heart sound and raised jugular venous pulse but no peripheral oedema or hepatomegaly. Arterial pressure measured non-invasively was 85 ⁄ 40 mmHg, and heart rate was 90 beats.min )1 . Electrocardiogram showed sinus rhythm with right axis deviation and right ventricular hypertrophy. Results of a recent cardiac catheterisation (performed for pulmonary assessment) revealed pulmonary artery pressures of 98 ⁄ 27 mmHg (mean 53 mmHg), mean right atrial pressure of 4 mmHg, mean pulmonary capillary wedge pressure 6 mmHg, with systemic arterial pressures of 95 ⁄ 42 mmHg and pulmonary-systemic flow ratio ...