This Article Figures Only Full Text Full Text (PDF) View Related Cardiosource Journal Scan Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Beghetti, M. Articles by Galiè, N. Search for Related Content PubMed Articles by Beghetti, M. Articles by Galiè, N. Related Collections Related Article

STATE-OF-THE-ART PAPER

Eisenmenger Syndrome

A Clinical Perspective in a New Therapeutic Era of Pulmonary Arterial Hypertension

Maurice Beghetti, MD^_*,_* and Nazzareno Galiè, MD

^_* University Hospital of Geneva, Geneva, Switzerland
University of Bologna, Bologna, Italy

Manuscript received August 28, 2008; revised manuscript received November 11, 2008, accepted November 16, 2008.

^_* Reprint requests and correspondence: Prof. Maurice Beghetti, Unité de Cardiologie Pédiatrique, Département de l'enfant et de l'adolescent, Hôpital des Enfants, 6 rue Willy Donzé, 1211 Genève 14, Switzerland (Email: maurice.beghetti{at}hcuge.ch).

Eisenmenger syndrome (ES), the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart disease, is a devastating condition that has a considerable impact on patients' lives. Patients who develop ES typically exhibit 1 or more of a range of cardiac defects, including ventricular septal defects, atrial septal defects, and patent ductus arteriosus. The nature of the congenital defect underlying ES is important because it has prognostic implications. Although ES shares similar morphological findings with idiopathic PAH, clinical differences exist between the 2 etiologies. Adults with ES exhibit increased survival and more favorable hemodynamics than those with idiopathic PAH. Treatment options for patients with ES have historically been limited; however, recent successes have been achieved with the use of therapies targeted against the pathophysiological pathways that underlie PAH. The dual endothelin receptor antagonist bosentan was demonstrated to improve hemodynamics and exercise capacity without compromising oxygen saturation, both in the short and long term. Improvements in hemodynamics also have been observed with the single endothelin receptor antagonist sitaxsentan. The phosphodiesterase type V inhibitor sildenafil may improve functional class, oxygen saturation, and hemodynamics in patients with ES, and beneficial effects of prostacyclin and prostacyclin analogs in patients with ES have been reported. The treatment of patients with PAH with the use of combinations of targeted therapies is becoming increasingly commonplace and may offer an alternative option for treatment of patients with ES. The authors of future studies may seek to investigate whether the pulmonary vascular remodeling in ES can be targeted and reversed.

Key Words: Eisenmenger syndrome • pulmonary arterial hypertension • physiopathology • therapy

Abbreviations and Acronyms   ASD = atrial septal defect   CHD = congenital heart defects   ES = Eisenmenger syndrome   IPAH = idiopathic pulmonary arterial hypertension   PAH = pulmonary arterial hypertension   PVR = pulmonary vascular resistance   VSD = ventricular septal defect

Related Article

Inside This Issue
J. Am. Coll. Cardiol. 2009 53: A31. [Full Text] [PDF]

This article has been cited by other articles:

				Authors/Task Force Members, N. Galie, M. M. Hoeper, M. Humbert, A. Torbicki, J.-L. Vachiery, J. A. Barbera, M. Beghetti, P. Corris, S. Gaine, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT) Eur. Heart J., October 2, 2009; 30(20): 2493 - 2537. [Full Text] [PDF]

				M. A. Gatzoulis, R. Alonso-Gonzalez, and M. Beghetti Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease Eur. Respir. Rev., September 1, 2009; 18(113): 154 - 161. [Abstract] [Full Text] [PDF]