Eisenmenger's Syndrome

Abstract

In general, most authors advocate nonintervention in Eisenmenger's syndrome, but an awareness of potential palliative measures to avoid destabilizing a delicately balanced physiology is needed as well. This approach has failed to alter long-term outcome, however. Survival for patients with Eisenmenger's syndrome has not improved substantially in the past several decades. Quality of life is universally altered by the presence of cyanosis, exercise intolerance, and the comorbid conditions associated with erythrocytosis. We therefore believe that the use of novel alternatives, as they become available, is warranted and that these alternatives are likely to be best evaluated in multicenter collaborative trials. The approach to the patient with pulmonary vascular obstructive disease (PVOD) should begin with maximization of palliative therapy and should, as compliance and teaching are ensured, proceed to the use of therapies designed to reverse the underlying proliferative changes in the pulmonary vasculature. Frequent checking of potential supplemental oxygen responsiveness and use of inhaled oxygen as needed to maximize systemic arterial saturation should be considered, although evidence of the value of home oxygen use is currently lacking. We favor systemic anticoagulation to a target international normalized ratio of 2.0 to 2.5. There are currently no published data supporting this practice in patients with PVOD, but we believe that as in patients with primary pulmonary hypertension, benefit is likely to outweigh risk. In the setting of a meticulous outpatient anticoagulation service, we have witnessed acceptably low bleeding event rates. A controlled clinical trial is warranted. Selective pulmonary vasodilators and antiproliferative agents hold significant promise in altering the natural history of PVOD associated with intracardiac shunting. The risk of paradoxic embolism and the theoretical worsening of right- to-left shunting compound the already high risk of systemically administered therapies; neither, to date, has been limiting in our patients. Studies of infused or newer subcutaneous and inhaled formulations are under way, and preliminary experience suggests real benefit--improved hemodynamics, improved exercise tolerance, and increased systemic arterial saturation--in this group of patients. Lung transplantation still trades a disease for another set of problems associated with long-term immunosuppression and chronic graft rejection in patients with previous sternotomy and thoracotomy and with a high acute surgical risk. Population studies of mortality and morbidity in patients with PVOD associated with congenital heart disease who receive transplants do not seem to suggest significant improvement with this therapy. In the future, the management of Eisenmenger's syndrome will probably include a multipharmacologic approach that targets several factors in the inflammatory cascade leading to vascular proliferation, perhaps offered in concert with novel surgical or transcatheter strategies designed to limit intracardiac shunting and, if desired, provide complete repair of intracardiac defects.