Conservative management of an elderly patient with Eisenmenger syndrome

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.

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.

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.>.