Abstract
Eisenmenger syndrome (ES) is a rare congenital heart disease (CHD) that occurs in persons with large intracardiac anatomical defects which may go undetected for many years, consequently results in reduced life expectancies and high mortality in the third and fourth decade of life. Pregnancy in patients with CHD and pulmonary arterial hypertension (PAH) is presaged with challenges, and perioperative management of such cases remains a topic of debate. Our case had both, atypical presentation, not diagnosed until her third trimester of third pregnancy and a quick progression to a severe stage with PAH and CorPulmonale. The atypical presentation being persistent polycythemia, raising clinical suspicion and thorough clinical examination revealing differential cyanosis and clubbing of hands and feet in pre-anesthesia checkup (PAC). A multidisciplinary approach clinched the diagnosis, and the use of a novel integrated method for perioperative management incorporating all available literature and modern interventions highlighted the critical role of anesthesiologists at a tertiary care setup.