Nomenclature of Eisenmenger Syndrome

Eisenmenger syndrome, originating from congenital heart defects, is defined by systemic-to-pulmonary blood flow communication, pulmonary arterial disease, and cyanosis. It typically arises from left-to-right shunting due to an atrial septal defect (ASD) or ventricular septal defect (VSD), which leads to increased pulmonary artery pressure, causing endothelial damage and hyperplasia in the lungs. Over time, this develops into a right-to-left shunt, resulting in cyanosis. Although the term "Eisenmenger syndrome" was coined later, it draws from the contributions of various medical experts who helped categorize congenital heart conditions. Current treatment guidelines focus on addressing the underlying anatomical issues contributing to pulmonary hypertension and shunting. Surgical options include septal defect closure, pulmonary artery banding, or systemic-to-pulmonary shunts. The decision to operate depends on factors such as pulmonary artery dilation and blood flow ratios, with heart-lung transplantation being a potential option for advanced cases.

Victor Eisenmenger's detailed descriptions laid the foundation for the syndrome, despite the term being coined later. His work reshaped the understanding of congenital heart disease by emphasizing its developmental origins. Maude Abbott, a trailblazing female physician, made key contributions to the study of congenital heart anomalies, including Eisenmenger syndrome, which she distinguished from other conditions like Tetralogy of Fallot in her Atlas of Congenital Cardiac Disease. Paul Wood, a renowned cardiologist, later coined the term "Eisenmenger Syndrome" and expanded on its pathophysiology, particularly highlighting the roles of pulmonary hypertension and shunting. He also explored treatments like acetylcholine and noted the protective effects of pulmonary artery stenosis in managing the condition.