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Background
Trisomy 21 is a chromosomal disorder associated with a higher risk for congenital heart disease (CHD). Septal defects are common, which initially produce a left-to-right cardiac shunt. If uncorrected, the ensuing pulmonary hypertension increases right-sided heart pressures, and ultimately the shunt may reverse to a right-to-left direction. This is known as Eisenmenger's syndrome. 1 Because of its rarity, anaesthetic experience with this condition is limited. Median life expectancy in patients with Eisenmenger's syndrome is reduced by about 20 years. 1 The perioperative death rates in such patients can be as high as 24%. 2 The American Heart Association (AHA) and the American College of Cardiology (ACC) guidelines on management of patients with congenital heart disease recommend that the anaesthetic approach to patients with Eisenmenger's syndrome should be individualised. 3 Case reports are the primary source of information on anaesthetic management of patients with Eisenmenger's syndrome, and several techniques have been described. 4-6
Case presentation
A 49-year-old man with trisomy 21 was admitted with a hip fracture. He had an uncorrected atrioventricular septal defect, which was monitored regularly by cardiologists. His medical history included hospital admissions for recurrent supraventricular tachycardias and heart failure. He also had scoliosis, spondylosis, hypothyroidism and secondary polycythaemia. He had a left hemiparesis following a suspected embolic stroke. His medications included warfarin, allopurinol, amitriptyline, bendroflumethiazide, codeine, folic acid, levothyroxine, omeprazole, paracetamol and sodium decusate.
He possessed poor exercise tolerance (New York Heart Association class III). His weight was 54 kg, and height of 1.58 m giving a body mass index of 22. On examination the patient had central cyanosis, digital clubbing and a loud pansystolic murmur. His heart rate was 86 bpm and arterial pressure was 130/70 mm Hg.
The perioperative team and the relatives discussed the risks of anaesthesia and surgery and also considered the option of non-surgical management. This would have entailed a long period of immobilisation in traction. Despite the risks, there was a consensus that surgery was the better alternative.
Investigations
A thorough preoperative assessment is essential in patients with Eisenmenger's syndrome, and should include pulse oximetry, ECG, transthoracic echocardiogram, a full blood count and coagulation screen. 3 All these tests were performed on this patient including arterial blood gases analysis. The tests revealed pH of 7.29,...