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When Viktor Eisenmenger first described (1897) and later Paul Wood (1958) expanded on the Eisenmenger syndrome (ES), the options of treatment of this condition were very limited. The pulmonary hypertension (PH) associated with this condition was believed to be irreversible. Palliative care management and heart/heart-lung transplant were the only available treatment available.
The study conducted by Arnott and colleagues have once again reminded us that there are now more effective options. Their study showed that, in a binational cohort of patients with ES followed up in expert congenital heart centres in Australia and New Zealand for a mean duration of 8.3 years, advanced therapy (mostly bosentan) was associated with a reduction in mortality.1 This study should spur us to do more for these patients.
Challenging the notion that ES is not as important as idiopathic pulmonary arterial hypertension (iPAH)
The prognosis of ES has been frequently compared with iPAH and often said to be superior for several years. However, recent studies have showed that this may not be the case due to the inherent biases present in the earlier studies.2 Mortality rates in these patients remain high with adjusted 10-year mortality rates of up to 40%. In addition, ES patients have very poor exercise tolerance and reduced quality of life. For the same degree of cyanosis, patients with ES had much poorer cardiopulmonary exercise test results than other cyanotic heart patients.3 In fact, …
Competing interests None declared.
Provenance and peer review Commissioned; internally peer reviewed.