Yasser Rodriguez1, Michael Ghannam1, Alexander Katz1, Michael Shea1,2. 1. Department of Internal Medicine, University of Michigan, 2F208 UH, 1500 E. Medical Center Dr., SPC 5052, Ann Arbor, MI 48109-5052, USA. 2. Division of Cardiology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
Abstract
BACKGROUND: Eisenmenger syndrome (ES) in the adult population has become exceedingly rare in developed countries owing to better recognition and treatment of congenital malformations of the heart. In the absence of transplantation, most patients survive an average of 20-30 years before succumbing to the cardiovascular and hemostatic sequelae. We present a rare case of an elderly woman with ES secondary to an uncorrected atrial-septum defect. She continues to have a high functional capacity despite impressive hemodynamic parameters. CASE: A 69-year-old woman with ES presented to our facility with atrial fibrillation and volume overload. She has a known sinus venosus atrial septal defect and associated severe pulmonary hypertension. She was managed conservatively and discharged on bosentan given her intricate physiology. CONCLUSION: Given the similarity of the pathology with idiopathic pulmonary arterial hypertension (IPAH) and ES, drugs that are usually reserved for IPAH such as prostanoids and phosphodiesterase type 5 inhibitors may be beneficial in the management of ES. This case highlights that with our improving understanding of ES, non-operative management may have an expanding role in the care of these complex patients.<Learning objective: Advanced therapy for Eisenmenger syndrome (ES) is geared toward the endothelin-1 mediated pulmonary arterial plexus. Given the similarity of the pathology with idiopathic pulmonary arterial hypertension (IPAH) and ES, drugs that are usually reserved for IPAH such as prostanoids and phosphodiesterase type 5 inhibitors may be beneficial in the management of ES. Conservative management can achieve a favorable outcome, even in elderly patients with advanced disease.>.
BACKGROUND: Eisenmenger syndrome (ES) in the adult population has become exceedingly rare in developed countries owing to better recognition and treatment of congenital malformations of the heart. In the absence of transplantation, most patients survive an average of 20-30 years before succumbing to the cardiovascular and hemostatic sequelae. We present a rare case of an elderly woman with ES secondary to an uncorrected atrial-septum defect. She continues to have a high functional capacity despite impressive hemodynamic parameters. CASE: A 69-year-old woman with ES presented to our facility with atrial fibrillation and volume overload. She has a known sinus venosus atrial septal defect and associated severe pulmonary hypertension. She was managed conservatively and discharged on bosentan given her intricate physiology. CONCLUSION: Given the similarity of the pathology with idiopathic pulmonary arterial hypertension (IPAH) and ES, drugs that are usually reserved for IPAH such as prostanoids and phosphodiesterase type 5 inhibitors may be beneficial in the management of ES. This case highlights that with our improving understanding of ES, non-operative management may have an expanding role in the care of these complex patients.<Learning objective: Advanced therapy for Eisenmenger syndrome (ES) is geared toward the endothelin-1 mediated pulmonary arterial plexus. Given the similarity of the pathology with idiopathic pulmonary arterial hypertension (IPAH) and ES, drugs that are usually reserved for IPAH such as prostanoids and phosphodiesterase type 5 inhibitors may be beneficial in the management of ES. Conservative management can achieve a favorable outcome, even in elderly patients with advanced disease.>.
Authors: Nazzareno Galiè; Maurice Beghetti; Michael A Gatzoulis; John Granton; Rolf M F Berger; Andrea Lauer; Eleonora Chiossi; Michael Landzberg Journal: Circulation Date: 2006-06-26 Impact factor: 29.690
Authors: W J Cantor; D A Harrison; J S Moussadji; M S Connelly; G D Webb; P Liu; P R McLaughlin; S C Siu Journal: Am J Cardiol Date: 1999-09-15 Impact factor: 2.778
Authors: Harald Kaemmerer; Siegrun Mebus; Ingram Schulze-Neick; Andreas Eicken; Pedro T Trindade; Alfred Hager; Erwin Oechslin; Koichiro Niwa; Irene Lang; John Hess Journal: Curr Cardiol Rev Date: 2010-11