Eisenmenger Syndrome: A Multisystem Disorder—Do Not Destabilize the Balanced but Fragile Physiology

Publication date: December 2019Source: Canadian Journal of Cardiology, Volume 35, Issue 12Author(s): Marie-A. Chaix, Michael A. Gatzoulis, Gerhard-Paul Diller, Paul Khairy, Erwin N. OechslinAbstractEisenmenger syndrome is the most severe and extreme phenotype of pulmonary arterial hypertension associated with congenital heart disease. A large nonrestrictive systemic left-to-right shunt triggers the development of pulmonary vascular disease, progressive pulmonary arterial hypertension, and increasing pulmonary vascular resistance at the systemic level, which ultimately results in shunt reversal. Herein, we review the changing epidemiological patterns and pathophysiology of Eisenmenger syndrome. Multiorgan disease is an integral manifestation of Eisenmenger syndrome and includes involvement of the cardiac, hematological, neurological, respiratory, gastrointestinal, urinary, immunological, musculoskeletal, and endocrinological systems. Standardized practical guidelines for the assessment, management, risk stratification, and follow-up of this very fragile and vulnerable population are discussed. Multidisciplinary care is the best clinical practice. An approach to the prevention and management of a broad spectrum of complications is provided. Relevant therapeutic questions are discussed, including anticoagulation, noncardiac surgery, physical activity, transplantation, and advanced-care planning (palliative care). Advanced pulmonary arterial hypertension therapies are indicated i...
Source: Canadian Journal of Cardiology - Category: Cardiology Source Type: research

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