The management of Eisenmenger syndrome in the modern treatment era: a case report

Abstract

To the Editor: A 41-yr-old female with a history of Eisenmenger syndrome (ES) was referred to the Royal Brompton Hospital, London, UK in 2002 for tertiary care. The patient had undergone closure of a large ventricular septal defect (VSD) via medial sternotomy at the age of 8 yrs elsewhere. At the time, the patient had a left-to-right shunt with high pulmonary artery saturation of 87% and low aortic saturation of 93% in air and evidence of pulmonary vascular disease, with systemic pulmonary artery pressure and a calculated pulmonary vascular resistance (PVR) of 6 Wood units. Her post-operative course was somewhat complicated and the VSD patch dehisced. The patient remained relatively well until her teenage years, when her condition deteriorated and she was diagnosed with ES. At 22 yrs she was sterilised following termination of an unplanned pregnancy; her risk of maternal mortality was quoted as 30–50% at that time. She was put on a waiting list for transplantation during the 1980s, but was relisted to a “wait/see” status in the 1990s as she seemed to be doing well. Her medical history included recurrent transient ischaemic attacks and iron deficiency anaemia, and the presence of paroxysmal atrial flutter. Following assessment at the Royal Brompton Hospital, the patient was included in a pilot study of bosentan in congenital heart disease. Treatment was initiated with 62.5 mg bosentan twice daily, and her dose was uneventfully uptitrated to 125 mg twice daily after 4 weeks. There was no drop in resting oxygen saturations during initiation. Transient ankle oedema occurred during the first week of treatment, but this resolved spontaneously by 1 month. After 3 months of treatment, the patient showed marked improvement; her oxygen saturations had improved from 83% to 87% in air, blood pressure was stable (99/70 from 114/62 mmHg), her 6-min …