Pathophysiology, Workup, and Treatment Options

Eisenmenger syndrome is an end-stage complication of congenital heart disease that occurs when a left to right shunt causes pulmonary over-circulation, leading to vascular remodeling, increased vascular resistance, and ultimately even shunt reversal. Aside from cardiac complications, this pathology has unique complications secondary to chronic cyanosis. In this episode of CardioNerds co-founder Dr. Amit Goyal, ACHD series co-chair Dr. Josh Saef, and Dr. Khaled Tuwairqi (ACHD cardiologist at King Faisal / Elite Hospitals) join Dr. Alexander (Sasha) Optowsky (Director of the Adult Congenital Heart Disease Program at Cincinnati Childrens) to discuss diagnosis and management of Eisenmenger syndrome. Show notes were drafted by Dr. Anna Scandinaro and episode audio was edited by CardioNerds Academy Intern Dr. Akiva Rosenzveig. The CardioNerds Adult Congenital Heart Disease (ACHD) series provides a comprehensive curriculum to dive deep into the labyrinthine world of congenital heart disease with the aim of empowering every CardioNerd to help improve the lives of people living with congenital heart disease. This series is multi-institutional collaborative project made possible by contributions of stellar fellow leads and expert faculty from several programs, led by series co-chairs, Dr. Josh Saef, Dr. Agnes Koczo, and Dr. Dan Clark. The CardioNerds Adult Congenital Heart Disease Series is developed in collaboration with the Adult Congenital Heart Association, The CHiP Network, and Heart University. See more CardioNerds Adult Congenital Heart Disease PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Pearls - Eisenmenger Syndrome First described in 1897 by Victor Eisenmenger, Eisenmenger syndrome is a long-term complication of unrepaired left to right shunts, resulting from pulmonary vascular remodeling and pulmonary hypertension. This eventually leads to reversal of the shunt, with right to left flow causing cyanosis. Evaluation for Eisenmenger syndrome should include a comprehensive history, physical exam, ECG, echocardiogram, cardiac catheterization, and laboratory work to identify multi-system complications of cyanosis and secondary erythrocytosis. The most definitive means to diagnose Eisenmenger syndrome in a patient with a prior left-to-right shunt lesion is with a right heart cardiac catheterization showing right to left shunting (Qp:Qs < 1). Eisenmenger syndrome is a multi-organ disease and many manifestations occur due to secondary erythrocytosis. Prevention and treatment of these complications are the major goals of care in this population. Complications of Eisenmenger syndrome include gout, bilirubin gallstones, stroke, paraganglioma/pheochromocytoma, thrombophilia, retinal changes, hypertrophic osteoarthropathy, and kyphoscoliosis. Emergency non-cardiac complications of Eisenmenger syndrome include cerebral abscess and hemoptysis. Pregnancy is contraindicated in Eisenmenger syndrome due to high maternal and fetal mortality. Notes- Eisenmenger Syndrome 1. How does Eisenmenger syndrome develop? Does everyone with a left-to-right shunt develop it? Can it develop as an iatrogenic complication? The pulmonary vasculature is not used to seeing the increased flow it receives in the context of a left to right shunt. Over time this leads to an increase in pulmonary vascular resistance and pulmonary hypertension. When pulmonary pressures exceed systemic pressures, this causes shunt reversal with right to left shunting causing deoxygenated blood to cross from right side of the heart to the left side bypassing the lungs and causing cyanosis. The process of developing Eisenmenger syndrome is chronically progressive and so adaptive changes have time to occur. Not all persons with unrepaired shunts will develop Eisenmenger syndrome ...