Alexandra Arvanitaki, Michael A Gatzoulis, Alexander R Opotowsky, Paul Khairy, Konstantinos Dimopoulos, Gerhard-Paul Diller, George Giannakoulas, Margarita Brida, Massimo Griselli, Ekkehard Grünig, Claudia Montanaro, Peter David Alexander, Rebecca Ameduri, Barbara J M Mulder, Michele D'Alto
Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease, Eisenmenger syndrome (ES) is still encountered and remains a complex clinical entity with multisystem involvement, including secondary erythrocytosis, increased thrombotic and bleeding diathesis, high arrhythmogenic risk, progressive heart failure, and premature death. Clearly, care for ES is best delivered in multidisciplinary expert centers. In this review, we discuss the considerable recent progress in understanding the complex pathophysiology of ES, means of prognostication, and improvement in clinical outcomes achieved with pulmonary arterial hypertension-targeted therapies...
March 29, 2022: Journal of the American College of Cardiology