The Sophistication of Simplicity: Ventricular Function and Oximetry Predict Survival in Eisenmenger Syndrome.

Abstract

> Simplicity is the ultimate sophistication. > > -Leonardo da Vinci In 1897, Victor Eisenmenger described a patient with cyanotic congenital heart disease and pulmonary hypertension who died at the age of 32. Although >100 years have passed since that initial report, the prognosis of patients with Eisenmenger syndrome has changed relatively little: Eisenmenger syndrome remains incurable, and patients have a high symptom burden and markedly diminished life expectancy, reduced by between 20 and 40 years, depending on the complexity of the underlying anatomy.1,2 Despite the grim prognosis, those with Eisenmenger syndrome live much longer with their disease than patients with other forms of severe pulmonary arterial hypertension (PAH).3 Importantly, the natural history of Eisenmenger syndrome is better than that of combined heart and lung transplantation.4,5 Therefore, conservative management for years, or even decades, is often appropriate in Eisenmenger syndrome patients who have degrees of pulmonary hypertension that would trigger transplantation consideration in other forms of PAH. See Article by Jensen et al However, in Eisenmenger syndrome, deterioration is often rapid and inexorable after many years of clinical stability. Patients with Eisenmenger syndrome often cannot be supported with mechanical circulatory assist devices or palliated with atrial septostomy, techniques which can temporize deterioration in other forms of PAH. Multiorgan system dysfunction is common which makes patients with Eisenmenger syndrome ineligible for transplantation once they begin to worsen. Therefore, accurate methods of risk stratifying patients with Eisenmenger syndrome are critical. Improved prognostication would allow for appropriate timing of advanced therapies, including transplant consideration. However, most patients with Eisenmenger syndrome will …